Académique Documents
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Culture Documents
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Cardiology
. Myocarditis:
. Is an inflammation of the myocardium, Caused by: infections, toxins, and
autoimmune diseases.
. Viral infection (enteroviruses in particular) the most common cause of myocarditis
in children.
. Classically occurring in spring and summer, myocarditis develops more often in
infants and young children than in older children and adolescents.
. Leads to tissue necrosis, thereby worsening myocardial function, dilating the
heart, and increasing end diastolic volume with resultant pulmonary edema and
CHF.
. Myocarditis typically presents as a flulike illness: fever, anorexia, lethargy, and
irritability followed by respiratory distress from acute heart failure.
. A holosystolic murmur may be identified secondary to dilated cardiomyopathy and
the resulting functional mitral regurgitation.
. Hepatomegaly can develop due to passive congestion Other features of heart
failure include fatigue, tachypnea, tachycardia, and decreased perfusion.
. Affected children should be monitored in the intensive care unit because of the
risk of acute decompensation and fatal arrhythmias.
. The workup for suspected myocarditis includes a (CBC) with differential, ESR and
C-reactive protein, cardiac enzymes, and blood and viral cultures.
. A chest radiograph demonstrates cardiomegaly and pulmonary edema.
. Electrocardiography most commonly shows low voltage QRS and sinus
tachycardia.
. Echo.is the best means of evaluating myocardial function and often shows global
hypokinesis, left vent. hypertrophy, left vent. dysfunction & pericardial effusion.
. The gold standard for diagnosis is a myocardial biopsy, which also allows for
disease stage classification.
. Ventricular septal defect (VSD):
__________________________________
. The most common congenital heart malformation.
. VSDs range from small and asymptomatic to large with significant left-to-right
shunting.
. Indications of significant VSD is: left-to-right shunt First, he is symptomatic
Second, he has the telltale murmur.
(Affected patients have pansystolic murmurs loudest at the left lower sternal
border.
. There maybe also a diastolic murmurs at the apex because of increased flow
across the mitral valve ).
. This patient should have an echocardiogram with a bubble study to evaluate his
congenital heart defect.
. Most small ventricular septal defects close spontaneously and require no
treatment, as long as there are no signs of pulmonary vascular disease.
. Reassurance and surveillance via EKG and echocardiography are needed in most
patients.
. Oxygenated blood from the lungs is returned to the lungs by the left side of the
heart through the pulmonary artery.
. During intrauterine life, the fo ramen ovale and ductus arteriosus provide mixing
of the deoxygenated and oxygenated blood,
resulting in an almost normal oxygenation of the fetal circulation.
. A patent foramen ovale or ductus arteriosus can maintain life after birth, as seen
in a patient with normal Apgar scores.
. The foramen ovale and ductus arteriosus begin to close after birth, leading to
decreased mixing and poor oxygenation.
. As the aorta is located in front of the pulmonary artery, the S2 aortic component
is better heard than the S2 pulmonic valve component and is audible as a single S2.
. There is usually no murmur because the foramen ovale and ductus arteriosus
have closed.
. The neonate then presents with cyanosis and tachypnea within the first few hours
of life.
. Chest x-ray can be initially normal but show some evidence of increased
pulmonary blood flow after 1-2 weeks.
. Echocardiography confirms the diagnosis.
. Prostaglandin E1 can be intravenously given to stabilize the neonate by
maintaining the patency of the ductus arteriosus, which is important for survival.
. Surgical treatment is definitive.
.N.B:
- Prostaglandin E1 is a vasodilator used to prevent ductus arteriosus closure in such
infants,
to increase or maintain pulmonary blood flow and improve oxygenation until
definitive surgery can be performed.
- Closure of the ductus arteriosus in such patients would cause progressive hypoxia
and metabolic acidosis.
. Coarctation of the aorta (COA):
_________________________________
. A congenital defect characterized by stenosis of the aorta, usually near the ductus
arteriosus.
. present with hypertension in the upper part of the body (high BP in the arms) and
relative hypoperfusion in the lower part of the body (low BP in the legs).
. The prevalence of COA in patients with Turner's syndrome is approximately 7%.
. The clues to the diagnosis are occasional headaches and increased BP in both
arms, leg muscle fatigability while climbing stairs (due to lower body
hypoperfusion).
. And a mild, continuous murmur heard all over the chest (due to the development
of collaterals between the hypertensive and hypoperfused vessels).
. Rib notching caused by the dilatation of the collateral chest wall vessels is specific
for coarctation.
. Mitral stenosis:
__________________
. also causes a diastolic rumble but does not typically cause a pansystolic murmur
at the left lower sternal border unless there is tricuspid regurgitation as well.
. Other features to suggest rheumatic fever such as fever, arthritis, or skin lesions.
. Tricuspid atresia:
____________________
. Suspect tricuspid atresia in a cyanotic infant with left axis deviation.
. This condition is characterized by an absent connection between the right heart
cavities, and a hypoplastic or absent right ventricle.
. Ventricular septal defect (holosystolic murmur on auscultation) occur in 90 % of
cases.
. Transposition of the great arteries occur in 30% of cases (which is not likely to be
in a case where the chest x- ray does not demonstrate pulmonary overcirculation).
. The associated heart defects, such as atrial septal defect (ASD), ventricular septal
defect (VSD), or patent ductus arteriosus (PDA), are necessary for survival.
. Venous blood passes through the ASD to reach the left cavities, and then through
the VSD back to the outlet portion of the right ventricle to reach the pulmonary
circulation.
. Sometimes, there is an associated pulmonary stenosis, and the blood passes
through the PDA to reach the pulmonary circulation.
. Patients with normal arteries (without TGA) present in the first two weeks of life
with progressive cyanosis, tachypnea, easy fatigability, and poor feeding.
. EKG reveals left ventricular hypertrophy, which occurs since the left ventricle
handles both pulmonary and systemic venous returns.
. Echocardiography shows a fibromuscular membrane in the place of the tricuspid
valve, a variably small right ventricle, ventricular septal defect, and a large left
ventricle.
. The treatment generally includes PGE1 (to keep the ductus arteriosus open) and
balloon atrial septostomy
(if the ASD is not large enough to allow an adequate flow from the right to left
atrium)
. postpericardiotomy syndrome:
______________________________
. It is reactive pericarditis with a pericardial effusion that present after surgery for
congenital heart disease.
. Symptoms usually occur 1-6 weeks after surgery.
. Although the cause is not known, it is thought to be an autoimmune response,
possibly to a viral infection.
. Most children develop mild symptoms which are self limited. In infants, pericardial
effusions can present with abdominal pain, vomiting, and decreased appetite.
. Older children may complain of chest pain Fever is often present On examination,
findings are consistent with pericardial and/or pleural inflammation,
including tachycardia, poor perfusion, pulses paradoxus, distant heart tones, and
jugular venous distention.
. If the effusion is large enough to affect the function of the heart , pericardial
tamponade can occur.
N.B:
- Most cardiac murmurs in childhood are benign (innocent) and require only
observation. Know the following features of a benign murmur:
systolic ejection quality.
varies with position;
Asymptomatic patient
Murmur intensity grade 2 or less
Normal S2
No audible clicks
Normal pulses
No other abnormalities.
- Pathological murmurs may indicate or lead to congenital heart disease, and are
very likely when the patient has any of the following:
symptomatic patient
Murmur intensity more than grade 2.
Pansystolic murmur
Murmur loudest at upper left sternal border
Absent or diminished femoral pulses
Murmur's quality is unchanged with position.
. Rheumatic fever:
__________________
. Suspect rheumatic fever in a child with sore throat, low-grade fever, pericarditis,
arthritis, chorea and subcutaneous nodules.
. Other features include erythema marginatum, elevated ESR and prolonged PR
interval on EKG.
. Rheumatic fever is caused by is group A streptococcus.
. Diagnosis is made if two major or one major and two minor criteria (Jones criteria)
are satisfied, in addition to
evidence of recent streptococcal infection (via detection of antistreptolysin
antibodies, cultures and other various antigen detection kits).
. Jones criteria:
----------------. The five major criteria are:
- Polyarthritis.
- Carditis.
- Chorea.
- Subcutaneous nodules.
- Erythema marginatum.
. The three minor criteria are:
- Fever.
- Arthralgia.
- Previous rheumatic fever.
. The treatment is benzathine penicillin G.
. Patients with chorea, pericarditis and arthritis may require additional therapy with
anti-epileptics, salicylates, and codeine, respectively.
. Jervell-Lange-Nielson syndrome:
_________________________________
. Is an autosomal recessive disease characterized by congenital deafness and QT
interval prolongation on EKG.
. QT interval prolongation predisposes to a particular type of ventricular
tachycardia called torsades de pointes.
. Torsades de pointes cause syncopal episodes and sudden death.
. The clue to the diagnosis is a syncopal episode without following disorientation
(post-episode confusion is more characteristic for a seizure), hearing impairment &
family history.
. If the patient is asymptomatic or does not have a clearly documented syncope,
beta blocker alone is sufficient.
. If the patient is symptomatic or has a documented episode of syncope, the
treatment will be beta-blocker plus a DDD pacemaker.
. Wolff-Parkinson-White (WPW) syndrome:
_______________________________________
. Characterized by an accessory pathway between the atrium and ventricle
resulting in preexcitation and an increased risk for tachyarrhythmias.
. WPW is usually seen in otherwise healthy children or adolescents.
. Affected patients can either be asymptomatic or present with chest pain,
palpitations, syncope, or cardiac arrest.
. In WPW, an accessory pathway conducts antegrade from the atria to the
ventricles faster than the conduction through the AV node, which
allows part of the ventricle to depolarize early.
. This results in the classic EKG findings of a shortened PR interval, a slurred initial
portion of the QRS (the delta wave), and a widened QRS complex.
. If there is retrograde conduction from the ventricles to the atria, supraventricular
tachycardia (SVT) can result.
. The EKG features of WPW will not be apparent during episodes of SVT, but the
delta wave should be visible once the rhythm is slowed.
. Sudden death can occur from dysrhythmias if WPW is untreated.
. A patent ductus arteriosus (PDA):
___________________________________
. Usually associated with a continuous flow murmur due to constant movement of
blood from the high-pressure aorta to the low-pressure pulmonary artery.
. Small PDAs are often asymptomatic and detected incidentally on routine cardiac
auscultation.
. Physical examination is often unremarkable in small PDA, although mildly
accentuated peripheral pulses can occur.
==================================================
============= ENT
==================================================
===========================
. Epiglottitis:
_______________
. In unimmunized children H. influenza type B is the most likely cause.
. In Hib vaccinated patients:H. influenzae type A and nontypable strains, H.
parainfluenzae, Streptococcus pneumoniae
. Sudden onset of; fever, sore throat, dysphagia and drooling.
. Airway obstruction is the most concerning complication of epiglottitis.
. This is a pediatric emergency and should be managed emergently with
endotracheal intubation; however, since intubation of such patients is difficult,
preparation for possible tracheostomy is also standardly performed.
. Allergic Rhinitis:
____________________
. Allergic rhinitis is characterized by an immunoglobulin E (lgE) mediated
hypersensitivity to fo reign allergens.
. It may be acute or chronic, and can be confused with recurrent colds.
. It may present with itchy eyes and tearing, swollen mucosa, headaches and nasal
obstruction,if the age of onset is in the second decade of life.
. Diagnosis is by allergy testing and findings of eosinophilia in the nasal discharge.
.N.B:
- The most common predisposing factor for acute bacterial sinusitis is a viral upper
respiratory infection.
. Choanal atresia: Extremely high yield question for USMLE.
__________________
. Suspect choanal atresia in an infant who presents with cyanosis that is
aggravated by feeding and relieved by crying.
. Failure to pass a catheter through the nose 3-4 cm into the oropharynx is
suggestive of the diagnosis.
. The diagnosis is confirmed by CT scan with intranasal contrast.
. First step in management consists of placing an oral airway and lavage feeding.
. Definitive treatment involves repairing the obstruction with surgery or endoscopy.
. Brain abcess after otitis media:
__________________________________
. An acute onset of headaches and focal neurologic symptoms after an episode of
acute otitis media or sinusitis most likely has a brain abscess.
. Hypothermia, hypotension.
. Ring-enhanc ing lesions are usually seen on CT or MRI scan.
. Acute bacterial sinusitis:
____________________________
. Streptococcus pneumoniae is the most common cause of acute sinusitis in
childhood, followed by Haemophilus influenzae and Moraxella catarrhalis.
. Staphylococcus aureus and anaerobes are common etiologic agents of chronic
sinusitis, and are rarely seen in acute cases.
. Diagnosis of acute bacterial sinusitis for children <6 years of age should be based
on clinical rather than radiographic criteria.
. Diagnosis can be confirmed with either plain X-ray or a CT scan of the neck.
. Lateral neck X-ray often show widening of the soft tissue space between the
trachea and the spine.
. Due to false +ve & -ve results of X-ray, so the diagnosis should be confirmed by
CT, which will delineate the abscess and help distinguish between an abscess and
cellulitis.
. N.B:
- Inflamed, fluctuant cervical lymph nodes are typically caused by streptococcal or
staphylococcal infection, The antibiotic of choice is dicloxacillin.
- dicloxacillin will treat infections caused by methicillin-sensitive Staphylococcus
aureus (MSSA) but not methicillin- resistant Staphylococcus aureus (MRSA).
. Croup:
________
. Also known as laryngotracheitis or laryngotracheobronchitis (LTB).
. Is characterized by laryngeal inflammation that results in hoarseness, a barking
cough, and varying degrees of respiratory distress over time.
. The typical patient is less than 3 years of age, and the most common causative
agent is Parainfluenza virus.
. Croup is usually a clinical diagnosis Lateral neck x- rays show subglottic
narrowing.
. Give a trial of epinephrine in cases of croup before intubation.
. A trial of racemic epinephrine should precede any invasive procedure in cases of
croup, as this decreases the need for intubation.
. Epinephrine acts by its alpha-adrenergic that is beneficial by reducing bronchial
secretions and mucosal edema and
. Beta-adrenergic effects that help croup patients who are also wheezing by
promoting smooth muscle relaxation.
. Laryngomalacia or congenital flaccid larynx :
________________________________________________
. is the most common cause of chronic inspiratory noise in infants.
. Laryngoscopy shows flaccidity of the larynx, and collapses during inspiration.
. It is a self-limiting condition in most cases, and generally subsides by 18 months
of age.
. The mother should be instructed to hold the child in an upright position for half an
hour after feeding, and to never feed the child when he is lying down.
==================================================
=============== Ophthalmology
==================================================
================
. Neonatal Conjunctivitis:
__________________________
. Gonococcal conjunctivitis: A copious amount of purulent drainage in newborns
who are 2-5 days old is most consistent with gonococcal conjunctivitis.
==================================================
======================= Neurology
==================================================
========
. Night Terrors:
________________
. Occurs in young children 2-12 years during Non-REM sleep.
. Characherized by fear, craying or screaming, decreased level of consciousness.
. and amnesia of the event next morning.
N.B.:
- Todd's palsy is characterized by transient hemiplegia that occurs after a seizure.
. Migraine headaches:
_____________________
. are the most common cause of headaches in the pediatric population, occurring in
50% of cases before the age of 20 years.
. The aura can be visual (e g , blurred vision, scotoma, photophobia, distortion of
objects) or
sensory (e g perioral paresthesia, numbness of extremities, shoulder pain,
abdominal pain, otalgia).
. Migraine headache is a clinical diagnosis, and further investigations are not
usually indicated.
. Neuroimaging is required only when there are behavioral changes, decline in
school performance, decline in growth parameters,
early morning headaches, seizures, orabnormal neurologic findings.
. In complicated migraines, neurologic signs (e g , basilar signs, ophthalmoplegia,
or hemiparesis) may be present, although the work-up reveals nothing abnormal.
. These neurologic symptoms may last only during the headache or persist for
several days or weeks.
. Brain abscesses:
_________________
. can present with fever, headache, focal neurologic changes, seizure, spasticity, or
signs of increased intracranial pressure.
. Congenital heart disease (particularly right-to-left shunts), head trauma, infections
of the jaw or mouth, infections of the face or scalp, meningitis, and
cranial instrumentation increase the risk of developing a brain abscess.
. Absence (petit mal) seizures:
_______________________________
. Characterized by a sudden cessation, without warning, of ongoing mental activity.
. Each episode rarely lasts longer than 30 seconds.
. A typical absence seizure is characterized by brief (typically lasting for a few
seconds) periods of impaired consciousness.
. Patients may also present with automatisms, but do not have a post- ictal state.
. Hyperventilation during the EEG reveals a generalized 3Hz spike-and-wave
pattern on a normal background.
. Minor motor symptoms are common, but complex automations and clonic
activities do not occur.
. Best diagnosed with EEG studies.
. An EEG with activation procedures (hyperventilation, photic stimulation, sleep)
helps in further diagnosis and classification of seizures.
. Ethosuximide is used almost exclusively for childhood absence seizures & valproic
acid is the 2nd choice.
.N.B:
- An atypical absence seizure lasts longer.
- The characteristic EEG pattern is slow spike-and-wave activity with a frequency
less than 2.5Hz
. Seizures:
___________
___________________
. Diagnostic Criteria of Febrile seizures:
- Seizure associated with a temperature >38C (1004F).
- Age <6 years.
- No CNS infection, No acute systemic metabolic cause of seizure & No history of
previous afebrile seizures.
. classified as: simple or complex.
. A simple febrile seizure is a generalized tonic-clonic seizure that lasts <15
minutes and usually occurs once in a 24-hour period.
. It does not cause permanent brain injury or significantly increase the child's future
risk of epilepsy.
. Although febrile seizures can recur, children who experience simple febrile
seizures are less likely to have a recurrence.
. A complex febrile seizure is diagnosed if the seizure is focal, lasts >15 minutes, or
occurs more than once with a total duration of seizure activity >30 minutes.
. Children with complex febrile seizures are at increased risk for future febrile or
afebrile seizures, especially if there is family history of epilepsy in a parent or
sibling.
. Febrile seizures are associated with viral or bacterial infections (e.g. otitis media)
However, serious intracranial infections (e.g. meningitis) must be ruled out.
. Once an intracranial infection has been ruled out, the patient can be treated for
the fever and underlying infection.
. The patient can then be discharged home with parental education about the use
of antipyretics and seizure precautions.
. Cerebral palsy:
_________________
. Cerebral palsy is a non-progressive disorder characterized by impaired motor
functioning.
. The most common cause is cerebral anoxia.
. Present as: hypotonia, hyperactive deep tendon reflexes, learning disabilities,
along with an intrapartum history of prolonged labor and low APGAR scores at birth.
. Hydrocephalus:
________________
. Symptoms: Poor feeding, irritability, decreased activity, Vomiting.
. Physical examination: Tense and bulging fontanelle, Prominent scalp veins, widely
spaced cranial sutures, rapidly increasing head circumference.
. The best diagnostic approach in symptomatic children with rapidly increasing
head circumference is to perform diagnostic imaging.
. A CT scan of the brain is the best initial choice in an infant who is acutely
symptomatic & will reveal ventricular dilation as well as the infant's anatomy.
. In otherwise stable and asymptomatic infant, a sedated MRI could be considered
to spare the child radiation exposure.
. Both CT and MRI provide greater detail than ultrasound, which requires a widely
open anterior fontanelle and is most useful in infants under 6 months.
. Treatment: a shunt that is placed from the ventricle to the peritoneum, pleura, or
right atrium; which allows the excess CSF to drain.
. Guillain-Barre syndrome:
___________________________
. Suspect Guillain-Barre syndrome in a child who presents with an ascending
polyneuropathy one week after an apparent viral infection (e.g. diarrhea).
. The underlying pathology involves mainly the peripheral motor nerves, although
sensory and autonomic nerves may also be affected.
. Friedreich Ataxia:
_____________________
. an autosomal recessive condition and genetic counsel ing is recommended for
prenatal diagnosis for parents with one affected child.
. FA is associated with necrosis and degeneration of cardiac muscle fibers leading
to myocarditis, myocardial fibros is and cardiomyopathy.
. Cardiac arrhythmia and congestive heart failure contribute to a significant number
of deaths.
. Klumpke's paralysis:
_____________________
. is a brachial palsy that occurs in newborns following excessive traction on the
arm.
. It consists of hand paralysis and ipsilateral Homer's syndrome (ptosis and miosis).
. It is secondary to injury to seventh and eighth cervical nerves and first thoracic
nerve.
. Confirmation MRI, which demonstrates nerve root avulsion or rupture.
. Treatment depends on the severity of the injury and, generally, it consists of
partial immobilization and appropriate positioning to prevent contractures.
. Gentle massages and range-of-motion exercises can be started by 7-10 days of
age.
. If by 3-6 months there is no improvement, neuroplasty, neurolysis, end-to-end
anastomosis, and nerve grafting may be attempted.
. Erb-Duchenne palsy:
_____________________
. is another type of brachial palsy in which the injury involves the fifth and sixth
cervical nerves.
. It presents with: absent Moro reflex and intact grasp reflex of the affected arm.
. Patients present with a characteristic position, which consist of adduction and
internal rotation of the arm with pronation of the forearm.
. Subarachnoid hemorrhage (SAH):
________________________________
. SAH can be caused by an intraventricular hemorrhage, which is common in
premature infants.
. Accumulation of the blood in the subarachnoid space may lead to destruction of
the arachnoid villi and cisterns, thereby blocking the flow or decreasing the
absorption of CSF, and leading to communicating hydrocephalus.
. SAH is the most common cause of communicating hydrocephalus.
. CT scan: dilation of the entire ventricular system with distinct enlargement of the
subarachnoid space over the cerebral cortex is very suggestive of nonobstructive
or communicating hydrocephalus secondary to a subarachnoid hemorrhage (SAH).
.N.B:
- Dandy-Walker anomaly and Chiari malformation will both reveal CT findings
consistent with obstructive or noncommunicating hydrocephalus.
. Dandy-Walker Anomaly will demonstrate a cystic expansion of the fourth ventricle,
and Chiari malformation will reveal protrusion
of the structures of the posterior fossa through the foramen magnum.
==================================================
============================= Hematology
==================================================
=========
. Acquired aplastic anemia:
___________________________
. present by: Normocytic or macrocytic anemia, Leukopenia, reticulocytopenia and
thrombocytopenia.
. B.M. biopsy (essential for diagnosis) --> profound hypocellularity in all cells with
fatty infiltration.
. Result from B.M. supression due to acquired or congenital causes:
A) Acquired Causes of Aplastic Anemia:
-
Idiopathic.
Drugs (e.g, NSAIDs, sulfonamides, etc).
Toxic chemicals (e.g. benzene, glue, etc ).
Viral infections (e.g. HIV, EBV, etc ).
Immune disorders.
Thymoma.
- Individuals with sickle cell trait have a heterozygous genotype (AS) and are
generally asymptomatic.
- Symptoms occur very rarely, and the most common of these is painless
hematuria.s
- In a patient with a chronic hemolytic anemia and compensatory reticulocytosis,
increased demand for folic acid can lead to folic acid deficiency,
- For this reason, patients with sickle cell anemia should be on folic acid
supplementation
- vitamin B12 is also required However, in contrast to folic acid, B12 stores can be
adequately maintained by most every patient via diet alone.
. Hereditary Spherocytosis:
___________________________
. Is a common cause of hemolysis and hemolytic anemia in the newborn.
. Indicators of hemolysis: reticulocytosis and hyperbilirubinemia.
. A positive Coombs' test points towards autoimmune hemolytic anemia, and a
positive osmotic fragility test indicates hemolysis confirms hereditary spherocytosis.
. The peripheral smear may reveal anisocytosis and polychromatophilic
reticulocytes and spherocytes.
. Severe anemia and hyperbilirubinemia may require phototherapy or exchange
transfusions.
. HS is an autosomal dominant trait and is the most common hereditary hemolytic
anemia in white population.
. There is congenital RBC membrane defect in HS resulting in extravascular
hemolysis occurring only in the presence of spleen.
. The osmotic fragility test is the next best test for the diagnosis of the patient.
.N.B: Other types of anemia:
---------------------------1- Iron deficiency anemia often presents with pallor, pica, and fatigue, although
these symptoms are often not present in children.
- Laboratory studies typically show a microcytic, hypochromic anemia with a low
reticulocyte count, high ROW, low serum iron, high TI BC, and low serum ferritin.
2- Anemia of chronic disease is unlikely in a child with no medical problems.
- This type of anemia can be normocytic or microcytic, and laboratory studies will
show a low serum iron and low TIBC.
3- Vitamin B12 deficiency presents as a macrocytic anemia with an elevated MCV.
4- Reticulocytosis is a physiologic response of the bone marrow to anemia provided
adequate iron stores are present.
5- In hemolytic anemia (spherocytosis), the reticulocyte counts are much higher
(sometimes as high as 9%) Spherocytosis is unlikely with just 3% reticulocyte count.
.N.B.:
- Approximately 5%- 15% of the patients with thymic tumors have pure red cell
aplasia, This finding is most common in older women.
- Sugar water test is done for the diagnosis of Paroxysmal Nocturnal
Hemoglobinuria (PNH).
___________
. Leukemic patients can present with numerous infections, diarrhea and failure to
thrive.
. Although such patients present with more serious bacterial and fungal infections.
. Hepatomegaly and splenomegaly may be found.
. Laboratory studies reveal: leukocytosis, thrombocytopenia and anemia. Other
findings may include hyperuricemia,hyponatremia, elevated LDH and hypokalemia.
. Acute lymphoblastic leukemia:
______________________________
. Acute lymphoblastic leukemia is the most common leukemia in children.
. The first symptoms are nonspecific: anorexia, irritability and lethargy.
. Patients may have a history of viral respiratory infection or exanthem from which
they do not fully recover.
. On physical examination, there may be pallor, hepatosplenomegaly, petechiae,
and/or lymphadenopathy.
. Diagnosis of leukemia is suggested by presence of anemia, thrombocytopenia and
blast cells on peripheral blood smear, but is confirmed by B.M. examination.
. Lymphoblasts lack peroxidase positive granules but often contain cytoplasmic
aggregates of periodic acid Schiff (PAS) positive material.
. Immunostaining for terminal deoxynucleotidyltransferase (TdT) is positive in more
than 95% of patients TdT is expressed only by pre B and pre T lymphoblasts.
. Myeloblasts on the other hand contain peroxidase positive material.
. Acute myeloid leukemia (AML):
______________________________
. Seen more commonly in adults.
. It can present similarly to ALL with anemia, thrombocytopenia and high WBC
count.
. The main differentiating and diagnostic factor is demonstration of greater than
25% of myeloblasts in the bone marrow.
. Chronic lymphocytic leukemia (CLL):
__________________________________
. usually presents in elderly patients.
. with fatigue, lymphadenopathy and splenomegaly.
. Labs show lymphocyte count of greater than 5000/mm3, made of matureappearing cells.
. Hodgkins' disease (HD):
_________________________
. The most common presenting sign is Painless, firm, cervical or supraclavicular
adenopathy.
. Depending on the extent and location of nodal and extranodal disease, there
maybe symptoms and signs of airway obstruction, pleural or pericardial effusion,
hepatocellular dysfunction, or bone marrow infiltration.
. Although signs and symptoms are similar to ALL, presence of lymphoblasts makes
ALL most probable.
. Burkitt lymphoma:
___________________
. It is a neoplasm of mature B cells. It is associated with the Epstein-Barr virus
infection.
. Most patients present with either a mass involving the mandible or abdominal
viscera.
. High mitotic index is typical. Histological examination shows characteristic "starry
sky appearance".
. It is a very aggressive tumor but responds well to the high dose chemotherapy.
. Myelodysplastic syndromes (MOS):
__________________________________
. They are clonal stem cell disorders which may progress to acute leukemias.
. These are usually seen in elderly patients, and are characterized by pancytopenia.
. Henoch-Schonlein Purpura:
___________________________
. Is an lgA-mediated vasculitis of small vessels, affecting children more than adults.
. Many cases follow an upper respiratory tract infection.
. The clinical manifestations: a classic tetrad of rash, arthralgias, abdominal pain,
and renal disease.
. The rash is typically purpuric and distributed symmetrically over the lower legs,
buttocks and arms.
. Arthralgias most commonly affect the knees and ankles, these symptoms are
always transient, and there is no permanent damage to the joints.
. GIT symptoms most commonly colicky abdominal pain (mostly due to local
vasculitis) that is frequently associated with vomiting,
. Two common pathologies which should be ruled out emergently are GI bleeding
and intussusception ( a surgical emergency, and is treated with air/barium enema).
. Renal involvement relatively mild disease characterized by asymptomatic
hematuria & proteinuria with a normal or slightly elevated plasma creatinine;
however,
. More marked findings may occur including the nephrotic syndrome, hypertension,
& acute renal failure (treatment includes steroids and monitoring of renal function).
. Other organs as the lung and central nervous system, may be involved.
. Confirmation of the diagnosis requires evidence of tissue deposition in the skin or
kidney of lgA by immunofluorescence microscopy.
N.B.:
1- Minimal change disease: electron microscopy uasually shows Podocyte fusion in
the glomerulous.
2- Membranous nephropathy: electron microscopy shows Glomerular basement
thickening & is considered the most common cause of adult nephrotic syndrome.
3- focal segmental glomerulosclerosis: electron microscopy shows Focal and
segmental sclerosis.
4- Goodpasture's syndrome: electron microscopy shows Linear deposition of lgG on
the basement membrane is seen.
. Lymphadenitis in children:
_____________________________
. Cervical lymphadenopathy is common in children.
. Lymphadenitis is diagnosed when the lymph node becomes tender and
erythematous in addition to being enlarged.
. Lymphadenopathy maybe acute or subacute/chronic, and unilateral or bilateral.
. Acute, unilateral lymphadenitis in children is usually caused by bacterial infection.
. Staphylococcus aureus is the most common followed by group A streptococcus.
. Patients with bacterial lymphadenitis are usually less than 5 years old and
nontoxic appearing.
. The affected lymph node is tender, warm, erythematous, and usually 3 to 6 cm in
size.
. In some cases, the infection can progress to induration and fluctuance.
N.B.: Other causes of unilateral lymphadenitis:
1- Tularemia: caused by Francisella tularensis, can present with acute unilateral
cervical lymphadenopathy Affected children.
. usually have fever, chills, headache, and malaise in addition to the
lymphadenopathy.
. In addition, tularemia is a zoonosis and presents after contact with an infected
animal (e g rabbits, hamsters, or blood-sucking arthropods).
2- Peptostreptococcus is an anaerobic bacteria that can cause acute, unilateral
lymphadenitis However,
it is usually seen in older children with a history of periodontal (gum) disease.
3- Nontuberculous mycobacteria (most commonly Mycobacterium aviumintracellulare)
. are one cause of unilateral subacute-chronic lymphadenopathy Affected children
are usually less than 5 years old and
. present with firm, nontender lymphadenopathy that is usually less than 4 cm in
size.
. The skin over the lymph node often thins and develops a violaceous color.
. Fever and tenderness are unusual with this infection.
4- Epstein-Barr virus usually presents with bilateral subacute-chronic
lymphadenopathy along with
systemic symptoms such as fever, pharyngitis, hepatosplenomegaly and
lymphocytosis.
. GeneticB-cell deficiencies:
_____________________________
. Patients with genetic B-cell deficiencies begin to develop recurrent infections after
passing 6 months (when the newborn's levels of maternal antibodies drop).
. The deficient humoral immune response in these patients impairs the body's
ability to destroy encapsulated organisms.
. Hence, recurrent sinopulmonary infections with H. influenzae and S. pneumoniae
are common.
. Lack of lgA also predisposes to Giardia infection.
. The serum concentrations of lgG, lgA, lgM and lgE may also be decreased, but
there is no absence or decrease in the number of circulating B cells.
. Furthermore, there is no sexual predominance in CVID.
. Transient hypogammaglobulinemia (THI):
________________________________________
. Characterized by decreased lgG levels, normal lgA levels, and variable lgM levels.
. These immunoglobulin levels all normalize by 6-11 months of age.
. lgA deficiency:
_________________
. Selective lgA deficiency is characterized by decreased lgA levels (less than
50mcg/ml) with normal serum concentrations of other immunoglobulins,
although lgG2 subclass deficiency may occur.
. There is no other evidence of any immune defects.
. Patients commonly present with respiratory, gastro intestinal, and genitourinary
infections + transfusion reaction.
. Recurrent sinopulmonary (Streptococcus & Hemophilus) and GI infections
(Giardia), a consequence of lgA's role in mucosal barrier protection.
. Patients with lgA deficiency may also form antibodies to lgA, predisposing them to
anaphylactic transfusion reactions.
. DiGeorge syndrome:
____________________
. DiGeorge syndrome is an example of a contiguous gene syndrome in which
neighboring genes on a chromosome are deleted.
. DiGeorge syndrome is specifically caused by the microdeletion of 22q11.
. The underlying pathology is a defect in the development of the 3rd and 4th
pharyngeal pouches, leading to: cyanotic heart disease,
. craniofacial skeleton anomalies (low-set ears and micrognathia), absent thymus,
absent parathyroid glands, recurrent infections, or cognitive impairment.
. Absence of the parathyroid glands result in hypocalcemia; therefore, the patient's
calcium levels must be monitored during surgery.
. Prolonged QT intervals may be caused by hypocalcemia.
. Severe calcium deficiency can lead to laryngeal spasm, convulsions (a neonate
with hypocalcemic seizures), and cramping.
. Thymic aplasia and consequent T cell deficiency, these children are susceptible to
fungal infections.
. Other congenital defects are esophageal atresia, anomalies of the great vessels,
mandibular hypoplasia.
. Chronic granulomatous disease (CGD):
_______________________________________
. Is a defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme
complex (impaired oxidative metabolism within the phagocytes) leading to:
. Recurrent and uncontrolled infections with catalase-positive organisms, as S.
aureus, Serratia marcescens, Burkholderia cepacia, Klebsiella, and Aspergillus sp.
. Patients are not susceptible to catalase-negative organisms (Streptococcus
pyogenes, Streptococcus pneumoniae, and Haemophilus influenzae).
==================================================
========= Pulmonary
==================================================
=====================================
. Pseudomonas Aeruginosa :
________________________
. Is a common cause of severe pulmonary infection in patient with cystic fibrosis
(CF).
. So the best treatment for a patient with severe exacerbation of cystic fibrosis is
combination of aminoglycosides (Tobramycine) and antipseudomonas penicillines
(piperacillin).
.N.B.: Staphylococcal aureus is a frequent cause of mild pulmonary infection in
patient with cystic fibrosis SO;
when pulmonary infection is mild (cough and mucous production only) oral antistaphylococcal antibiotic is preferred (Dicloxacillin).
.N.B.:
- Mothers with gestational diabetes mellitus (GDM) usually give birth to large
babies with the most common complications:
- Clavicular fracture which is usually greenstick fracture and heal rapidly without
complications.
. The characteristic laboratory findings of:
____________________________________________
1) Iron deficiency anemia are: decreased serum iron, decreased percent of
saturation (serum iron/ TIBC) & increased TIBC.
2) Sideroblastic anemia: is characterized by increased serum iron levels and normal
TIBC.
3) In dimorphic anemia: two distinct forms of red cells are circulating
4) Megaloblastic anemia is characterized by an elevated MCV, elevated MCH, and
normal MCHC.
5) Anemia of chronic disease is associated with decreased TIBC.
. Cystic fibrosis:
________________
. Is an autosomal recessive disorder that is more common in Caucasian people.
. The cause is a defect in the CFTR (Cystic fibrosis transmembrane conductance
regulator) protein, which leads to impaired chloride transport in tissues
. and consequent multi-system involvement, particularly:
(1) increased salt content in sweat gland secretions.
(2) thick, viscous secretions in the lungs, pancreas, reproductive tract, and liver.
. Young child presents with recurrent upper respiratory tract infection, persistent
nasal discharge and bilateral nasal polyps.
. Also can be present with recurrent upper respiratory tract infections,
bronchiectasis and failure to thrive.
. The patient may have pulmonary manifestations, which begins as a persistent,
productive cough, and progresses to obstructive airway disease.
__________________________________________________
. Should be suspected in all term and post-term infants with cyanosis with or
without fetal distress.
. Persistence of fetal circulatory pattern of right-to-left shunting through the PDA
and foramen ovale after birth is due to very high pulmonary vascular resistance.
. The associated hypoxia is universal and unresponsive to 100% oxygen.
. The chest-x ray may be normal or may show parenchymal opacification in the
chest, depending on the etiology.
. Although the clinical presentation of PPHN is indistinguishable from HMD, the xray findings can differentiate.
. Meconium aspiration syndrome:
_______________________________
. Usually occurs in term or post-term infants, either in utero or more often with the
first breath.
. Thick & particulate meconium is aspirated into lungs, resulting in small airway
obstruction & consequent respiratory distress,that present within the 1st hour of
birth.
. Partial obstruction of some airways may lead to pneumothorax or
pneumomediastinum.
. Patchy infiltrates, coarse streaking of both lung fields, increased anteroposterior
diameter, and flattening of the diaphragm characterize the typical chest-x ray
. Intracranial hemorrhage:
___________________________
. In neonates classically presents as periods of apnea, pallor or cyanosis, poor
suckling, abnormal eye signs, high-pitched, shrill cry, muscular twitching,
convulsions, decreased muscle tone or paralysis, metabolic acidosis, shock, and a
decreased hematocrit.
. The fontanel may be tense and bulging.
. The condition is rarely present at birth and is diagnosed on the basis of the
history, clinical manifestations, and trans-fontanel cranial (CT).
. Bronchiolitis:
________________
. is defined as the first episode of wheezing associated with an upper respiratory
tract infection.
. The infection is usually caused by respiratory syncytial virus.
. It affects 50% of children in the 1st 2 years of life, especially those who are prone
to airway reactivity, & there is increased incidence of asthma later in life.
. The WBC count is normal, and the chest x-ray may show air trapping or
atelectasis.
. The treatment is supportive care with humidified oxygen and bronchodilators.
. Ribavirin may be administered in high- risk children.
==================================================
================ Rheumatology
==================================================
=============================
. Septic Arthritis:
___________________
. Septic arthritis can occur following skin or upper respiratory tract infections.
. Arthrocentesis is both diagnostic and therapeutic, and is mandatory in all
suspected cases.
. Blood cultures and synovial fluid cultures must be obtained prior to administering
the antibiotics.
. Empiric antibiotic therapy ( IV nafcill in or IV cefazolin) should be started with
pending synovial fluid cultures.
. Clubfoot (talipes equinovarus):
______________________________
. Presents with equinus and varus of the calcaneum and talus, varus of the midfoot,
and adduction of the forefoot.
. This is a common foot deformity and may be congenital, teratologic, or positional
(in utro).
. Initial treatment involves, stretching and manipulation of the foot, followed by
serial plaster casts, malleable splints, or taping.
. Because conservative treatment corrects the majority of cases, so Surgical
treatment is indicated if conservative management
gives unsatisfactory results, and is preferably performed between 3 and 6 months
of age.
. Remember that the treatment of clubfoot should be started immediately.
. Spondylolisthesis:
____________________
. Is a developmental disorder characterized by a forward slip of vertebrae (usually
L5 over S1 ) that usually manifests in preadolescent children.
. In the typical clinical scenario, back pain, neurologic dysfunction (e g , urinary
incontinence), and
a palpable "step-off" at the lumbosacral area are present if the disease is severe.
. Hemophilic arthropathy:
_________________________
. patients with coagulopathies (Hemophilia) --> Recurrent hemarthroses may then
lead to a joint injury called "hemophilic arthropathy".
. Although the exact mechanism underlying the joint damage is not known, iron
deposition and synovial thickening with fibrosis are characteristic.
. Iron deposition and cytokines released from the blood stimulate synovial
proliferation, fibrosis and cartilage injury.
. Growing pains:
________________
. Common in children between the ages of two and twelve.
. It is usually poorly localized and common in the legs below the knees and
bilateral.
. It awakens the child at night and responds to reassurance, massages or
analgesics.
. In all cases, it resolves in the morning.
. The diagnosis of growing pain is a diagnosis of exclusion, all the physical and
laboratory findings are negative.
. X- rays are not required if the physical exam is unremarkable.
. All such children are observed, and parents are assured that this condition will
resolve with time.
. Transient synovitis:
______________________
. Is a frequent cause of hip pain in children, typically occurring in male children
ages 3-10 years old.
. The cause of transient synovitis is unknown, but the condition usually follows mild
trauma or viral infection.
. Transient inflammation of the joint synovium leads to pain and decreased range of
motion.
. Patients usually have a history of a recent viral infection and present with a limp
and/or unilateral hip or knee pain A low grade fever may be present, but
patients are generally afebrile.
. On examination, the affected hip will be flexed, slightly abducted, and externally
rotated, There is mild limitation of motion.
. The patient may complain of pain with palpation or passive motion.
. Laboratory findings characteristic of transient synovitis include a slightly elevated
white blood cell count and erythrocyte sedimentation rate.
. Four clinical criteria have been shown useful in differentiating septic arthritis from
transient synovitis;
white blood cell count > 12,000/mm3; temperature > 39C (102F); erythrocyte
sedimentation rate > 40 mm/h; and refusal to bear weight.
. If at least three of these four criteria are met, further work-up is indicated to rule
out septic arthritis.
. Radiographs of the hip should be done to exclude bony lesions and Legg-CalvePerthes disease.
. Widening of the joint space or an enhanced pericapsular shadow may be seen
with transient synovitis.
. Additional work-up is not usually necessary, unless the diagnosis is uncertain.
. Treatment consists of rest and non-steroidal anti-inflammatory medications
(NSAIDs).
. Most patients experience resolution of symptoms within several days, although
transient synovitis may contribute to the development of arthritis later in life.
. Compartment syndrome:
_______________________
. supracondylar humerus fractures may be complicated by neurovascular injury or
compartment syndrome.
. Compartment syndrome is characterized by severe pain, pallor, poikilothermia,
paresthesias, and the late findings of pulselessness and paralysis.
. Patients with predisposing injuries should be continually monitored for the
development of compartment syndrome, particularly as swelling from the injury
increases.
. Initial treatment includes removal of any bandages, measurement of
compartment pressures, and emergent orthopedic evaluation for possible
fasciotomy.
. N.B.:
1- The United States Preventive Services Task Force (USPSTF) recommends vision
screening for children aged 0-5 years,
primarily to identify those with strabismus, amblyopia, and refractive errors.
Early diagnosis and treatment are being emphasized, because poor visual acuity
resulting from the above conditions may impair
a child's future academic performance and selfimage,and may even lead to
blindness.
2- Lead toxicity causes microcytic anemia and permanent neurologic damage, so
identification of affected patients is desirable at an early stage.
Risk factors for environmental lead exposure include minority race or ethnicity,
low socioeconomic status, pre-1950 housing,
occupational exposure from parents, and recent immigration.
- The USPSTF recommends no screening for asymptomatic children aged 1-5 years
at average (no) risk of lead toxicity.
3- The Centers for Disease Control (CDC) recommend beginning the meningococcal
vaccination series between 11-12 years.
Children who are at high risk of the disease (i.e , asplenic) may be vaccinated as
early as 2 years of age.
4- The CDC recommends administering the rotavirus vaccine between 2 and 8
months.
first dose should be administered between 6 and 14 weeks of age Rotavirus.
vaccination should not be initiated for infants aged 15 weeks and older.
The final dose should not be given beyond 8 months of age.
. Hand-foot syndrome or dactylitis:
____________________________________
. Is the earliest manifestation of vaso-occlusion in sickle cell anemia, so needs a
complete workup for previously asymptomatic sickle cell patients.
. Patients usually present at the age of 6 months to 2 years with an acute onset of
pain and symmetric swelling of the hands and feet.
. Although only soft tissue swelling is initially seen, fever is sometimes present.
. The pathophysiology of dactylitis involves vascular necrosis of the metacarpals
and metatarsals, which may be seen on plain radiographs as osteolytic lesions.
. Ewing's sarcoma:
__________________
. A highly malignant tumor that is found in the lower extremity more than the upper
extremity.
. The most common sites are the metaphysis and diaphysis of the femur, followed
by the tibia and humerus.
. It is very aggressive and metastasizes early to the lungs and lymph nodes.
. Patients are typically white males in their first or second decade of life.
. Presents with pain and swelling for weeks or months, Erythema and warmth of the
local area are sometimes seen.
. Septic arthritis:
___________________
. Like osteomyelitis in a child, a septic joint is usually hematogenous in origin and
frequently follows an upper respiratory tract infection.
. The most common organisms causing septic arthritis in children are
Staphylococcus aureus and streptococcus.
. It is characterized by severe pain, leukocytosis and an elevated ESR.
. The typical patient generally refuses to walk, and keeps the affected limb
externally rotated (the external rotation position allows for maximal pus
accumulation).
. Ultrasound guided aspiration of the synovial fluid should be performed
immediately.
. The synovial fluid white cell count of more than 100,000/ul indicates a definite
septic arthritis.
. An x-ray is often normal.
. Empiric antibiotics: in infants a combination of antistaphylococcal agent (nafcillin
or vancomycin) and third generation cephalosporin and
for children over age 5 years antistaphylococcal agent (nafcillin or vancomycin)
alone is a reasonable empiric antibiotic therapy.
. Septic joint in a child is a true surgical emergency and needs immediate drainage.
. A delay of even 4-6 hours can lead to avascular necrosis of the femoral head.
. Lyme arthritis:
_________________
. occurs as a result of untreated Lyme infection. It is caused by Borrelia burgdorferi,
a spirochete carried by the deer ticks.
. It spreads via blood to various areas of the body, including the joints.
. The early symptoms are mild and are easily overlooked, arthritis is the hallmark of
late disease.
. It tends to involve the knee in most patients Warmth, swelling from effusion and
restriction of movements differentiate it from arthralgias, which occur early in the
disease.
. The first episode occurs within six months of having erythema migrans, the
episodes gradually disappear in about ten years, if left untreated.
. In United States, Lyme disease is prevalent in: Connecticut, Rhode Island, New
York, Pennsylvania, New Jersey, Delaware, Maryland and Wisconsin.
. Slipped capital femoral epiphysis (SCFE):
___________________________________________
. is a common hip disorder seen in adolescents.
. SCFE occurs when the capital femoral epiphysis displaces from the femoral neck.
. Boys > girls, and the usual age is 10 to 16 years old.
. Obesity is a significant risk factor, additional risk factors: hypothyroidism,
panhypopituitarism, and growth hormone deficiency or supplementation.
. Children with endocrine issues are more likely to have bilateral disease and to
present at an earlier age.
. Commonly present with an insidious onset of knee or hip pain and a limp, usually
without a history of trauma.
. Sometimes, minor trauma can increase the pain and bring the patient to medical
attention.
. Patients tend to hold the hip in passive external rotation and exhibit decreased
internal rotation, abduction, and flexion.
. Diagnosis is made with plain radiographs of the hip (AP and frog leg lateral views),
which show the posteriorly and inferiorly displaced femoral head.
. Treatment: emergent orthopedic consultation and surgical fixation at the current
degree of slippage to avoid the risk of avascular necrosis.
. Supracondylar fractures:
__________________________
. are the most common fractures in the pediatric population.
. The most common complication is entrapment of the brachial artery, resulting in
loss of the radial artery pulse.
. therefore, all patients with a supracondylar fracture must have their radial pulse
checked.
. After reduction of the fracture, the radial pulse must be reassessed.
. Duchenne muscular dystrophy:
______________________________
. should be suspected in a child under the age of 5 who presents with proximal
muscle weakness (difficulty climbing stairs),
Gower's sign (use his hands to "walk up" his legs in order to assume an upright
position), diminished reflexes, and pseudohypertrophy of the calf muscles.
. Mental retardation.
. Cardiomyopathy.
. Serum CK (and aldolase)levels are used for screening the muscular dystrophies.
. Muscle biopsy can confirm the diagnosis in most cases.
. The gold standard is genetic studies, which is required in atypical cases.
. Prognosis: most of patients are wheelchair bound by age of 12 years.
. Becker muscular dystrophy:
____________________________
. Similar to Duchenne muscular dystrophy but:
. After age of 5 years (usually around 12 y).
. Milder than Duchenne muscular dystrophy.
. Caulf pain with exercise.
. Normal or near normal mentality.
. Prognosis: patients are able to walk untile early childhood.
. Nursemaid's elbow:
____________________
. occurs when infants or children are lifted or pulled by the hand or arm.
. This pulling injury occurs when the radial head slips through parts of the annular
ligamen.
. The child typically keeps the hand in a pronated position, and refuses (cries out in
pain) attempted fo rearm supination
. Rotating the hand and forearm to a supinated position with pressure over the
radial head usually reduces the annular ligament with an audible click, and
restores full, normal use of the extremity.
==================================================
========== GIT
==================================================
================================
. Congenital abdominal wall defects:
____________________________________
. Diagnosis Congenital abdominal wall defects can be made antenatally with
maternal serum-AFP measurements and ultrasonography.
. The two types of abdominal wall defects are described in the table below:
* Gastroschisis:
---------------. Bowel protrudes through a defect on the right side of the umbilical cord.
. Bowel is not covered by a protective membrane.
. Bowel looks "angry" and matted.
. Not typically associated with any other abnormalities outside the GI tract.
. If the diagnosis was made antenatally, management would begin with planned
spontaneous vaginal delive ry at a tertiary neonatal surgical unit,
. Caesarean section is performed only in the presence of the usual obstetric
indications.
. After delivery, the next step in management is to immediately wrap the exposed
bowel with sterile saline dressings and
. Cover with plastic wrap in order to prevent insensible heat and large fluid losses.
. The neonate is then maintained in a thermo-neutral environment.
. Further steps in the management of gastroschisis include inserting an orogastric
tube (to decompress the stomach), establishing peripheral intravenous access (to
provide fluids), and administration of broad-spectrum antibiotics.
. Definitive treatment is surgical; however, surgery should be preceded by the
above measures.
. Primary closure is successful in 70% of neonates, but if it is unsuccessful, staged
closure with Silastic silo can be used.
* Omphalocele:
-------------. Intra-abdominal contents protrude through the umbilical ring The abdominal
herniat ion usually includes small bowel and may include large bowel and liver.
. Bowel is covered by an amnioperitoneal membrane.
. Can be associated with other congenital abnormalities ( e g , heart, kidney).
. The management of omphalocele is similar to that of gastroschisis:
. Delivery is also planned at a tertiary care center, and caesarean section is
reserved for the usual obstetric indications,
(This is based on the lack of evidence that caesarean delivery improves the
outcome of patients with uncomplicated omphalocele ).
. Immediate care of the newborn with omphalocele also involves, sterile wrapping
of the bowel,insertion of an orogastric tube,
stabilizing the airway (to ensure adequate ventilation), and establishing peripheral
intravenous access.
. Small defects (< 2 cm) can be repaired with primary closure, but most defects will
require a staged procedure with Silastic silo.
. Umbilical hernia:
___________________
. Is due to an imperfect closure or weakness of the umbilical ring, it is especially
common in low birth weight, female, and black infants.
. It appears as a soft swelling covered by skin that protrudes during crying,
coughing or straining, and can be reduced easily through the fibrous ring at the
umbilicus.
. The hernia consists of omentum or portions of the small intestine.
. Most umbilical hernias disappear spontaneously by 1 year of age.
. Surgery is advised if the hernia persists to the age of 3-4 years, exceeds 2 cm in
diameter, causes symptoms, becomes strangulated,
or progressively enlarges after the age of 1 to 2 years.
. Necrotizing enterocolitis:
____________________________
. Suspect necrotizing enterocolitis in any preterm or low birth weight infant with
fever, vomiting, abdominal distension, bloody diarrhea, leukocytosis & pneumatosis
intestinalis.
. These symptoms usually occur after the introduction of formula feeding.
. The radiologic finding of pneumatosis intestinalis (i.e. intramural air) in infants is
diagnostic.
. Increased gastric residual volume in a preterm neonate is highly suspicious for
necrotizing enterocolitis.
. The condition usually presents at 3-10 days after birth, and is due to bowel wall
injury resulting from perinatal asphyxia.
. Preterm neonates are at high risk for other complications such as hyaline
membrane disease,
which presents with respiratory distress and ground glass appearance of the lungs
on chest x- rays.
N.B:
____
. Trachea-esophageal fistula: presents with excessive drooling, choking, coughing,
and cyanosis due to aspiration of feedings.
. Duodenal atresia: Bilious vomiting without abdominal distention is the cardinal
sign for duodenal atresia.
. Meconium aspiration syndrome: Clinical features of meconium aspiration
syndrome include thick, greenish amniotic fl uid
in the mouth and throat at birth, accompanied by cyanosis and respiratory
distress.
. Pyloric stenosis is characterized by projectile nonbilious vomiting and palpation of
an olive-shaped mass immediately after the infant has vomited.
. Celiac disease:
_________________
. The presence of dermatitis herpetiformis and chronic non-bloody diarrhea in a
child of 12-15 months is suggestive of celiac disease.
. The child has Kwashiorkor-like clinical features associated with dermatitis
herpetiformis (erythematous vesicles symmetrically,
distributed over the extensor surfaces of elbows and knees).
. This scenario is suggestive of celiac disease, which is also called gluten-sensitive
enteropathy.
. It is due to abnormal hypersensitivity to gluten and presents characteristically at
12-15 months of age, when gluten-containing foods
such as wheat, rye or barley are introduced into the child's diet.
. Pyloric stenos:
_________________
. Is a common congenital anomally typical presentation is non-bilious projectile
vomiting which gradually becomes more frequent and
forceful in a 4-8 week old infant Pertinent physical findings are a palpable
abdominal mass and visible peristaltic waves in the upper abdomen.
. Hypokalemic alkalosis occurs due to the frequent episodes of vomiting.
. Abdominal ultrasound is the most useful tool for confirming the diagnosis, and
shows a thick hypoechogenic ring in the pyloric region.
. Additionally, the loss of large volumes of fluid via vomiting leads to contraction
alkalosis due to the action of aldosterone.
. In order to compensate for the metabolic alkalosis, the respiratory system
responds via hypoventilation to create a secondary respiratory acidosis.
. i.e. have a primary metabolic alkalosis with respiratory compensation.
. This is characterized by a plasma pH greater than 7.45, PaC02 greater than 40
mm Hg and HC03- greater than 24 mEq/L.
. Pyloric stenosis is treated su rgically; however, surgery should be undertaken only
after the infant's hydration status and electrolyte levels have been stabilized.
. Meckel's diverticulum:
________________________
. Typically presents with painless melena in 2 to 3-year-old children.
. It is present in 2-3% of the population, and is the most common anomaly of the
GIT.
. It results from the failure of the vitelline duct to obliterate during the fetal
development.
. Heterotropic gastric tissue may be present in the diverticulum, which results in
ulcerations and bleeding.
. The diagnosis of a Meckel's diverticulum is best made with technetium-99m
pertechnetate scanning (uptake by heterotopic gastric mucosa).
.N.B.:
1- Peptic ulcer disease may present in preschool children with epigastric pain
related to meals with a positive family history.
2- Anal fissure is a painful condition associated with constipation it may occur in
infancy, and presents with blood streaked stools.
. Duodenal atresia usually presents with bilious vomiting a few hours after the first
feeding.
. It is usually associated with other congenital anomalies, and can be a feature of
Down's syndrome.
. Midgut volvulus:
___________________
. usually presents in a child less than one month old with bilious vomiting,
abdominal distension and passage of bloodstained stools.
. Volvulus is associated with malrotation of the gut, and can be complicated by
perforation and peritonitis.
. lntussusception:
__________________
. is the most common cause of intestinal obstruction in the first two years of life.
. The classic presentation: is an infant with colicky abdominal pain, vomiting, and
red currant jelly stools.
. The currant jelly stools are caused by bowel ischemia, which leads to bleeding and
mucus production.
. Between episodes of abdominal pain, the child appears well.
. On physical examination, the abdomen is usually soft, nontender, and
nondistended, A sausage-shaped mass is often palpated.
. An abdominal radiograph may show air fluid levels due to obstruction.
. An ultrasound commonly shows a targetoid lesion in which one bowel segment is
nested inside another bowel segment.
. An air contrast enema is both diagnostic and therapeutic in the majority of cases.
. Emergent laparotomy is indicated for the treatment of intussusception when air
contrast enema is unsuccessful.
. However, laparotomy becomes the treatment of choice if the child presents with
peritoneal signs.
. The most common type of intussusception is ileocolic.
. Hirschsprung's disease:
________________________
. usually presents with failure to pass meconium within the first 24 hours.
. In this disease, an aganglionic segment of the colon causes the intestinal
obstruction.
. Cyclical vomiting:
__________________
. Recurrent self-limiting episodes of vomiting and nausea in children in the absence
of any apparent cause.
. The etiology of this condition is unclear; however, its incidence is high in children
whose parents have a history of migraine headaches.
. Complications that may arise from the condition are anemia and dehydration.
. Treatment consists of anti-emetics and reassurance of the parents.
. Infantile gastroesophageal reflux disease (GERD):
___________________________________________________
. The child typically has frequent vomiting, but maintains adequate weight gain.
. Symptoms usually begin in the first few months of life, peak around 4 months, and
generally resolve by 1 year
. Conservative therapy is first-line management in these infants, and make sure
that the feedings are the appropriate volume and are given at regular intervals.
. Breast milk or formula can be thickened with rice cereal, which can decrease the
frequency of vomiting.
. The infant should also be positioned to help decrease intraabdominal pressure by
keeping the child's head elevated or laying the child prone when awake.
. Parents should be reassured that most children will have resolution of their
symptoms by 12 months.
. If infants develop respiratory symptoms due to GERO such as stridor, apnea, or
failure to thrive, a more detailed evaluation is necessary including
. an upper gastrointestinal series, pH probe monitioring, or endoscopy.
. These children may require pharmacotherapy if conservativetreatments do not
improve their symptoms.
. N.B:
- Batteries lodged in the esophagus on x-ray should be removed immediately under
endoscopic guidance to prevent mucosal damage and esophageal ulceration.
- Batteries located distal to the esophagus pass uneventfully in most cases and
need only to be observed with stool examination and/or follow up x-rays to confirm
excretion.
. Hemolytic-uremic syndrome (HUS):
__________________________________
. Caused by a toxin released from Escherichia coli.
. The toxin enters the systemic circulation and injures the endothelial cells in the
kidney.
. When there is progressive elevation of BUN and creatinine, the chances for
improvement of the kidneys' function is poor.
. When the kidneys are damaged, the mortality is 5-10%.
. Turcot's syndrome:
____________________
. It is autosomal recessive and mainly occurs in teens.
. An association between brain tumors (primarily medulloblastomas and gliomas) &
FAP (Familial Adenomatous polyposis) or HNPCC (Hereditary nonpolyposis colorectal
cancer).
. Gardner's syndrome:
_____________________
. is autosomal dominant where colonic polyps are seen with prominent
extraintestinal lesions.
. Includes: desmoid tumors, sebaceous or epidermoid cysts, lipomas, osteomas
(especially mandible), supernumerary teeth & juvenile nasopharyngeal
angiofibroma.
. Peutz-Jeghers syndrome:
_________________________
==================================================
============= Neonatology
==================================================
===================
. The Apgar score is measured in newborns at 0 and 5 minutes of life. It has five
components, and is scored as follows:
A) Color of the newborn
----------------------0 body and extremities are blue/pale
1 body is pink and extremities are blue
2 body and extremities are pink.
B) Heart rate
------------0 heart shows no activity
1 HR <100 beats/min
2 HR > 100 beats/min
C) Reaction to nasal stimulation:
--------------------------------0 no response to stimulation
1 grimace
2 active cough.
D) Tone/ Activity:
-----------------O limp
1 some flexion of extremities
2 active flexion of extremities.
E) Respirations:
---------------0 completely absent
1 slow and irregular
2 good respiratory effort.
. Breast milk:
______________
. The protein in human milk is 70% whey and 30% casein, and the protein content
is highest at birth and decreases over the first month of life.
. Whey is more easily digested than casein and helps to (improve gastric
emptying).
. Human milk also contains lactoferrin, lysozyme, and secretory immunoglobulin A
proteins that confer improved immunity to the infant.
. The main carbohydrate in both human milk and standard infant formulas is
lactose.
. Although calcium and phosphorus content is significantly lower in human milk
when compared to formula, these minerals are better absorbed from human milk.
. Breast milk has an inadequate supply of vitamin D and exclusively breast-fed
infants must receive supplemented vitamin D.
. Breast feeding:
_________________
. Mostly located at the base of the spine, lower back, and buttocks, it can also be
seen in other parts such as the shoulders, arms, wrists, legs, ankles or abdomen.
. The face, palms and soles are usually spared
N.B.:
- Cafe-au-lait spots are well-demarcated, tan or light brown flat lesions that can
vary in number, size, and distribution.
. This finding may indicate neurofibromatosis if the diameter is larger than 0.5 mm
and if more than 5 lesions are found.
- Cutis Marmorata appears as a lace-like pattern on the skin in response to cold or
stress.
. It can persist in some diseases such as Down's syndrome and trisomy 18.
- A salmon patch is a flat salmon-colored lesion commonly seen over the glabella,
eyelids, and neck.
. It is a vascular lesion that usually disappears in early childhood.
. Indications of the evaluation of neonatal jaundice include:
_____________________________________________________________
1. Conjugated hyperbilirubinemia (> 2 mg/dL)
2. Jaundice that appears in the first 24 - 36 hours of life.
2. Serum bilirubin rising at a rate faster than 5 mg/dl/24 hours.
3. Serum bilirubin greater than 12 mg/dl in full-term (especially in the absence of
risk factors) or 10 - 14 mg/dl in preterm infants.
4. Jaundice persists after 10 -14 days of life.
5. The presence of signs or symptoms.
N.B.: Conjugated hyperbilirubinemia (> 2 mg/dL):
- Association with light colored stools, hepatomegaly, and direct (conjugated)
hyperbilirubinemia.
- Is indicative of neonatal cholestasis and impaired hepatic excretion of bilirubin,
either by extrahepatic obstruction or liver cell injury.
- With conjugated neonatal hyperbilirubinemia, priority should be given to
conditions that require prompt diagnosis and treatment, such as
sepsis, endocrinopathy (hypothyroidism). and nutritional hepatotoxicity caused by
metabolic diseases (galactosemia, tyrosinemia).
- After these diagnoses are ruled out, the final step is to differentiate biliary atresia
from neonatal hepatitis.
. Breast-milk jaundice:
_______________________
. Appears in the second week of life; however, the hyperbilirubinemia is indirect
(unconjugated).
. The unconjugated bilirubin levels may rise as high as 10-30 mg/dl Levels drop
rapidly as soon as breastfeeding is stopped,
and the infant is given milk formula for 1-2 days.
. After 2-3 days the breastfeeding can be resumed, and usually there is no return of
the hyperbilirubinemia.
. Although it is a benign condition in some instances, phototherapy may be
indicated.
. Physiologic jaundice:
_______________________
. Consists of moderate unconjugated hyperbilirubinemia (< 12-14 mg/dL) appears
after the first 24 hours of life and resolves before the end of the first week.
. It is a benign condition and is more common in preterm infants, infants of diabetic
mothers, and infants of Asian or Native American descent.
. Crigler-Najjar and Gilbert's syndrome:
________________________________________
. Are inherited deficiencies of UDP-glucuronyl transferase that result in
unconjugated hyperbilirubinemia.
. In Gilbert's syndrome, the deficiency is mild, and patients are asymptomatic due
to the mild indirect hyperbilirubinemia.
. In Crigler-Najjar syndrome, the enzyme is absent; patients present early in life,
and die within their first few years.
. Erythroblastosis fetalis:
___________________________
. is characterized by unconjugated hyperbilirubinemia and anemia.
. The diagnosis is suggested by a positive Coombs' test.
. Care of a neonate:
____________________
. Early neonatal care in an uncomplicated pregnancy include:
. initial physical assessment, removal of airway secretions, drying the infant &
keeping him warm, & early preventive measures (gonococcal ophthalmia
prevention, vitamin K supplementation).
==================================================
========================= Endocrinology & Genetics
==================================================
=========
. Precocious pubarche (Puberty):
_________________________________
. It is very important to differentiate between precocious puberty that is caused by
premature activation of the hypothalamus-pituitary-gonad (HPG) axis,
and precocious pseudo-puberty that is caused by a gonadotropin-independent
process, typically an excess of sex steroids.
. AV canal defects may be partial or complete, but both need early surgical
correction to prevent pulmonary hypertension.
. Patients with Down's syndrome have a high predilection for duodenal atresia.
. Other GIT anomalies: Hirschsprung's disease, Esophageal atresia, Pylo ric
stenosis, Malrotation of the bowel.
. Atlantoaxial instability is commonly seen in Down syndrome, and most commonly
occurs due to excessive laxity in the posterior transverse ligament, which causes
increased mobility between the atlas (C1) and the axis (C2).
. Symptoms of atlantoaxial instability: behavioral changes, torticollis, urinary
incontinence, and vertebrobasilar symptoms as dizziness, vertigo, and diplopia.
. Also upper motor neuron symptoms such as leg spasticity, hyperreflexia, a
positive Babinski sign, and clonus.
. Patients with Down syndrome are normally hypotonic, and they may remain
hypotonic or have increased tone due to atlantoaxial instability.
. It is diagnosed by lateral radiographs of cervical spine in flexion, extension & in a
neutral position Open mouth radiographs can also visualize the odontoid.
. Treatment: surgical fusion of the first cervical vertebrae (C1) to the second (C2).
. Marfan syndrome:
__________________
. MSF is an autosomal dominant disorder that results from the mutations of the
fibrill in-1 (FBN1) gene.
. It presents with tall stature, long and emaciated extremities, arachnodactyly,
hypermobility of the joints, upward lens dislocation, and aortic root dilation.
. Homocystinuria:
_________________
. An autosomal recessive disease caused by cystathionine synthase deficiency.
. Her features are characteristic of Marfan's syndrome (tall stature, long emaciated
extremities, arachnodactyly, hyperlaxity of the skin and joints).
. But her clinical presentation of a stroke is highly indicative of homocystinuria.
. Remember: Marfan's features + thromboembolic events = classic homocystinuria.
. Thromboembolic events at any age, due to the pathologic changes in the vessel
walls and increased adhesiveness of the platelets (commonly cerebral vessels).
. Lens dislocation (ectopia lentis): In Marfan's syndrome, the lens is dislocated
upward, and in homocystinuria, it is dislocated downward.
. Elevation of both homocysteine and methionine in body fluids confirm the
diagnosis.
. The initial treatment: high doses of Vitamin B6.
. Restriction of methionine along with supplementation of cysteine is used for
patients not responsive to Vitamin B6 therapy.
. Glucose-6-phosphatase deficiency:
___________________________________
. is also known as type I glycogen storage disease and Von-Gierkes' disease.
. As its name suggests, this condition is caused by deficient glucose-6-phosphatase
in the liver, kidneys, and intestinal mucosa.
. The typical patient, is 3-4 months of age with hypoglycemia, lactic acidosis,
hyperuricemia, and hyperlipidemia.
. Hypoglycemic seizures may occur.
. The characteristic features: a doll-like face (fat cheeks), thin extremities, short
stature, and a protuberant abdomen (due to the enlarged liver and kidneys).
. The spleen and heart are normal.
==================================================
========================= Nephrology
==================================================
===============
. Systemic Lupus erythematosus:
_________________________________
. Positive anti-Smith antibodies and/or anti-double stranded DNA antibodies is
specific and confirmatory for the diagnosis of systemic lupus erythematosus
. Transient proteinuria:
________________________
. A urine dipstick can be positive in up to 10% of school-aged children. Proteinuria
in children can be transient (intermittent), orthostatic, or persistent.
. Transient proteinuria is the most common cause of proteinuria and can be caused
by fever, exercise, seizures, stress, or volume depletion.
. Orthostatic proteinuria is very common in adolescent boys and is defined as
increased protein when the patient is in an upright position
that returns to normal when the patient is recumbent.
. If the urinalysis shows no hematuria and is otherwise normal, the urine dipstick
should be repeated on at least two additional specimens.
. If these subsequent tests are negative for protein, the diagnosis is transient
proteinuria that are usually benign conditions that require no further evaluation.
. Posterior urethral valves:
____________________________
. are the most common congenital obstructive urethral lesion in males.
. There is midline lower abdominal mass probably represents a distended bladder.
. The finding of a distended bladder indicates that the obstructive lesion is distal to
the bladder neck.
. Posterior urethral valves are abnormal folds in the posterior urethral wall (distal
prostatic urethra) that obstruct urine flow out of the bladder.
. Affected infants may develop hydronephrosis, azotemia, and failure to thrive.
. A voiding cystourethrogram (VCUG) is the diagnostic test of choice.
. Bladder exstrophy:
____________________
. Results from a ventral defect of the urogenital sinus, which causes separation of
the pubic rami, external rotation of the hips,
and separation of the rectus abdominis muscles.
. The resulting ventral midline defect allows protrusion of a malformed bladder.
. This condition is associated with epispadias, recurrent urinary tract infections, and
urinary incontinence.
. Hypospadias:
______________
. is a congenital abnormality where the penile urethra opens on the ventral surface
of the penis rather than at the tip.
. It results from incomplete fusion of the urethral folds on the ventral penis.
. urachus:
__________
. is a tubular extension of the allantois that extends from the bladder to the
umbilicus.
. It is normally obliterated during fetal development A patent urachus can result in
an urachal fistula, cyst or sinus.
. None of these conditions would interfere with urine drainage from the bladder.
. An omphalomesenteric duct cyst:
_________________________________
. is a focal failure of vitelline duct obliteration.
. Such a vitelline cyst may cause a small midline mass deep to the umbilicus.
. The cyst could be attached to the umbilicus and the wall of the ileum by vitelline
remnants and may cause small intestinal volvulus.
. Cryptorchidism:
_________________
. is a fa ilure of one or both testes to descend from the abdomen through the
inguinal canal(s) into the scrotum.
. Wilms tumor (nephroblastoma):
_______________________________
. Wilms tumor is usually diagnosed between the ages of two and five years.
. It is the most common primary renal neoplasm of childhood.
. 80% of the cases have an asymptomatic abdominal mass that is usually detected
by the mother/caretaker while bathing the child.
. The mass may be bilateral, symptoms may include hypertension, hematuria,
abdominal pain, and vomiting.
. In a few cases, it may present with lung metastases.
. It is associated with Beckwith-Wiedemann and Denys-Drash syndromes.
. The treatment is nephrectomy.
. If treated in the early stage, majority of the patients have a long-term survival.
. It arises from metanephros, which is the embryologic precursor of the renal
parenchyma.
. Neuroblastoma (NBL):
______________________
. Neuroblastoma, which is the most common extracranial solid tumor of childhood.
. The median age at diagnosis is 2 years.
. The tumor arises from neural crest cells, which are also the precursor cells of the
sympathetic chains and adrenal medulla.
. For this reason, NBL may arise from the adrenal gland or any location along the
paravertebral sympathetic chains.
. The most common site involved is the abdomen, either from the adrenals or
retroperitoneal ganglia.
. The mass is usually firm and nodular in consistency Calcifications and
hemorrhages are seen on plain x- ray and CT scan.
. Up to 70 % of patients have metastatic disease at time of presentation, and the
most common metastatic sites are: long bones, skull, bone marrow, liver, L.N. &
skin.
. The levels of serum and urine catecholamines and their metabolites (i.e. HVA and
VMA) are usually elevated;
. however, patients do not present with fainting spells, sweating, palpitations and
hypertension, as in pheochromocytoma.
. Renal tubular acidosis (RTA):
_______________________________
. Is a normal anion gap metabolic acidosis caused by a defect in the ability of the
renal tubules to reabsorb bicarbonate or excrete hydrogen.
. There are three types of RTA:
1. Type 1 or distal RTA occurs due to a defect in hydrogen ion secretion.
These patients are acidotic, hypokalemic, and have an elevated urinary pH In
children, type 1 RTA is often a genetic disorder.
Patients commonly develop nephrolithiasis.
2. Type 2 RTA is caused by decreased bicarbonate reabsorption in the proximal
tubule. Fanconi syndrome is a common cause in children.
3. Type 4 RTA is caused by a defect in the sodium/potassium exchange in the distal
tubule which results in hyperkalemic, hyperchloremic acidosis.
In children, obstructive uropathy, renal disease, or multicystic dysplastic kidneys
are common causes.
. Renal tubular acidosis can present as growth failure and should be considered in
the differential diagnosis for failure to thrive.
. Screening labs will show a low bicarbonate level with an increase in chloride,
producing a normal anion gap metabolic acidosis.
. Minimal change disease:
_________________________
. The most common cause of nephrotic syndrome in patients younger than 16 years
old.
. Light microscopy and immunofluorescence does not usually reveal any change in
kidney architecture.
. Electron microscopy shows diffuse effacement of foot processes of podocytes.
. Minimal change disease is a highly steroid-sensitive condition and is the most
common cause of nephrotic syndrome in children.
. For these reasons, empiric steroid therapy is indicated in any child with a clinical
presentation suggestive of nephrotic syndrome
. Fibromuscular dysplasia:
_________________________
. The most common cause of secondary hypertension in children is fibromuscular
dysplasia.
. It is responsible for approximately 20% of all cases of renal hypertension.
. Aside from children, fibromuscular dysplasia is also generally seen in
premenopausal women (or women less than 50 years old).
. Physical examination reveals a hum or bruit in the costovertebral angle due to
well-developed collaterals.
. The right renal artery is more affected than the left.
. Angiography typically shows a "string of beads" pattern to the renal artery.
. Vesicoureteral reflux (VUR):
______________________________
. Is the retrograde flow of urine from the bladder to the ureter and renal pelvis.
. Reflux is a risk factor for UTI & Repeated attacks of UTI can lead to progressive
renal scarring,
which is the major cause of end stage renal disease and hypertension in children.
. It is a risk factor for UTI as it facilitates the transport of bacteria from bladder to
the upper urinary tract,
. this explains why VUR is the most likely cause of acute UTI in children.
. VUR is present in 35-40 o/o of children with UTI.
. The diagnosis is made with voiding cystourethrogram (VCUG) followed by renal
imaging.
. Diagnosis of VUR is best made with a voiding cystourethrogram or a radionuclide
cystogram (RNC).
. Renal ultrasonography is less sensitive in detecting reflux nephropathy.
. The American Academy of Pediatrics recommends that all children aged 2 to 24
months with a first UTI should undergo a VCUG or RNC to detect the presence VUR.
. N.B:
- Chronic pyelonephritis is characterized by focal parenchymal scarring and
blunting of calices on IVP.
- Hydronephrosis is seen on IVP as dilation of the collecting system including the
calyces, pelvis and ureter, depending on the level of obstruction.
- Ureteropelvic obstruction would also lead to hydronephrotic changes in the kidney
with IVP showing the level of obstruction as a constriction in the flow of dye.
. Acute pyelonephritis:
_______________________
. usually present with fever, chills, nausea, vomiting and flank or suprapubic pain.
. Physical examination shows costovertebral angle tenderness.
. Urinalysis shows bacteriuria and pyuria.
. Therapy should be started with empiric antibiotics after withdrawal blood & urine
for culture and sensitivity and before its results are available.
. Severe disease with systemic manifestations warrants intravenous (IV) antibiotics.
. If patient is vomiting and hypotensive; therefore, IV antibiotics should be
administered.
==================================================
======================= Infection
==================================================
===================
. Measles (rubeola):
____________________
. It is caused by Paramyxovirus.
. Characterized by a prodrome of non-productive cough, coryza, non-purulent
conjunctivitis, followed by
. Koplik's spots (pathognomonic): red spots with bluish specks over the buccal
mucosa, opposite the premolar tooth & sometimes on the inner conjunctivae &
vaginal mucosa
. And maculopapular rash initially appearing on the face then spread to involve the
entire body.
. Lab. findings: leukopenia, lymphopenia and Proteinuria may be seen.
. Diagnosis is mostly clinical and can be supported by a fourfold rise in
hemagglutination inhibition antibody titer.
. Leukopenia (T-cell cytopenia) and thrombocytopenia can be seen with measles
infection.
. Vitamin A has been shown to reduce the morbidity and mortality rates of patients
with measles through immune enhancement.
. It also helps the gastrointestinal and respiratory epithelium to regenerate.
. N.B.: - Atypical measles:
- Occurs in persons who have previously received inactivated measles vaccine
(was available in the 1960's), (the measles vaccine available now is a live one).
- This form is potentially life-threatening, and characterized by atypical rash (not
maculopapular), the absence of Koplik spots, arthritis,
hepatitis and lung involvement Edema of the hands and feet may occur.
. Rubella:
__________
. characterized by low-grade fever, lymphadenopathy (sub-occipital and posterior
auricular) and rash.
. The rash is erythematous, maculopapular and classically begins on the face,
spreading subsequently down the body.
. congenital rubella infection:
_______________________________
. Transmitted via the placenta, the causative organism is Toga virus.
. Maternal infection manifests after an incubation period of 14 to 21 days with mild
symptoms such as rash, arthralgias and generalized lymphadenopathy.
. Since rubella infection confers permanent immunity, only primary infection in the
pregnant woman carries the risk of fetal disease.
. The severity of fetal disease depends on the time of pregnancy when the
transmission took place.
. Infections of early pregnancy are the most severe, and the associated adverse
outcomes include spontaneous abortion and congenital rubella syndrome (CRS).
. If transmission occurs in the first 4 weeks of pregnancy, the risk of developing
CRS is 50%, drops to 1 % if transmission occurs in the third trimester.
. Features of CRS: IUGR, deafness, cardiac malformations (e g , patent ductus
arteriosus, atrial septal defects), microphthalmia,
cataract, retinopathy, hepatosplenomegaly, thrombocytopenia, and CNS
involvement.
. Chronic infection may result in growth retardation, radiolucent bone disease,
jaundice, hepatosplenomegaly, thrombocytopenia and purple skin lesions
("blueberry muffin spots").
. Maternal infection is confirmed by the presence of lgM to rubella virus, or a
fourfold increase in lgG on two serum samples obtained two weeks apart.
. In the infant, the diagnosis is confirmed by the presence of lgM or persistence of
lgG beyond the age of 6 months.
. Prevention of congenital rubella is primarily achieved by administration of rubella
vaccine to all females of childbearing age.
. If the immunologic status of a pregnant woman is unknown, rubella titers should
be obtained in the first trimester.
. Immunization should not be performed in pregnancy because of a theoretical risk
for the fetus, and such women should be advised to avoid anyone with possible
rubella infection.
. classic triad of congenital rubella syndrome (CRS) - sensorineural deafness,
cardiac malformations (e g , PDA and ASD), and cataracts.
. Erythema infectiosum or Fifth disease:
________________________________________
. caused by human parvovirus 819.
. Children have different presentations and develop a rash with a "slapped cheeks"
appearance.
. Fever is not present or very mild in this disease.
. Roseola infantum:
___________________
. Caused by herpes virus 6.
. Characterized by the abrupt onset of high-grade fever, which is then followed by a
maculopapular rash, appearing on the trunk and then spreading peripherally.
. The patient is no longer febrile when the rash develops.
. There are no positive physical signs such as sore throat or lymphadenopathy
during the febrile stage.
. chickenpox (Varicella virus):
_______________________________
. The clinical manifestations of chickenpox in healthy children generally develop
within fifteen days after the exposure.
. Include a prodrome of fever, malaise, or pharyngitis, followed by the development
of a generalized vesicular rash, usually within 24 hours.
. The lesions are commonly pruritic and appear as successive crops of vesicles over
a three to four day period.
. Patients typically have lesions in different stages of development on the face,
trunk and extremities.
. New lesion formation generally stops within four days, and most lesions are fully
crusted by the 6th day in normal hosts.
. Impetigo :
____________
. Is a superficial skin infection with multiple vesiculopustules on the exposed areas
of the face and extremities.
. Is a contagious disease caused by Staphylococcus aureus, Streptococcus, or both.
. It presents as an erythematous macule, which rapidly evolves into vesicles and
pustules.
. The pustules later rupture and leave honey-colored, crusted exudates.
. There are 2 forms of impetigo vesiculo-pustular and bullous types Bullous type is
caused by Staphylococcus.
. These vesiculopustules eventually rupture, and then appear encrusted with a
characteristic golden-yellow color.
. A history of skin trauma or insect bite is common, and local lymphadenopathy can
be present.
. Impetigo is most commonly seen in children, and is associated with poststreptococcal glomerulonephritis.
. The etiologic agent is either group-A beta-hemolytic streptococci (GABS) or S.
aureus, the clinical presentations of these organisms are almost similar.
. Factors predispose to impetigo are warm and humid climate, poverty, crowding,
poor personal hygiene, and carriage of GABS or S . aureus.
. Nasal carriage of Staphylococci can cause recurrent impetigo.
. Topical mupirocin is the treatment of choice or oral erythromycin.
. Kawasaki disease or mucocutaneous lymph node syndrome:
________________________________________________________
. Criteria of Kawasaki's disease:
1. Fever for > 5 days, and
2. Four of the following symptoms:
Bulbar conjunctiva! injection.
Desquamation of the finger and toe tips, indurative edema.
Erythema, fissuring, and crusting of the lips, strawberry tongue, and diffuse
mucosal injection of the oropharynx.
Morbill ifo rm truncal exanthem.
Cervical lymphadenopathy.
. Kawasaki disease is one of the most common causes of generalized vasculitis in
children.
. It is usually self-limited, although it can be fatal because of giant aneurysm
formation, thrombosis or rupture of the coronary arteries, leading to MI.
. 2D echocardiography is used to assess cardiac function and coronary vasculature
(Coronary artery aneurysms are the most serious complication of Kawasaki
disease).
. Stevens-Johnson syndrome:
__________________________
. A severe variant of erythema multiforme.
. Characterized by skin lesions of erythema multiforme (target lesions), followed by
inflammatory bullae of two or more mucous membranes.
. Sometimes, the GI, respiratory, or GU tracts may also be involved.
. Rabies:
_________
. Fatal disease that is transmitted to humans by contact with saliva or nervous
tissue from an infected animal through a bite, open wound, or mucus membrane
contact.
. Bats are a major reservoir for the rabies virus, Bat bites are often so quick and
minor that the victim is not even aware.
. For this reason, any direct contact between bats and humans requires postexposure rabies prophylaxis.
. People bitten by domestic animals suspected of being rabid or not available for
observation, or
by wild carnivores like raccoons, skunks, and foxes should also receive
prophylaxis.
. Rabbits and small rodents like squirrels, chipmunks, and rats are ra rely infected
with rabies,so Post-exposure prophylaxis following bites is not routine.
. Viral meningitis:
___________________
. usually self-limited inflammation of the leptomeninges caused by a viral infection.
. 90% of cases are caused by non-polio enteroviruses, such as echovirus and
coxsackievirus.
. The incidence of viral meningitis decreases with increasing age Infants are most
commonly affected, and disease morbidity and mortality is highest in this group.
. Present with a viral prodrome of constitutional and upper respiratory symptoms
with low-grade fever.
. Over the next 36-48 hours, the patient develops a high fever, headache,
irritability, and nuchal rigidity.
. Focal neurologic signs are absent.
. The patient may also present with seizures. Other symptoms of enteroviral
infection may also be seen such as pharyngitis, rash, or herpangina.
. In viral meningitis, (CSF) will show pleocytosis with lymphocytic predominance,
although neutrophils may predominate early in the course.
. The protein level is normal to slightly elevated and the glucose level is normal,
CSF gram stain will not show any organisms.
. Treatment is supportive; in most patients, symptoms resolve within 7-10 days.
. Viral meningitis can be caused by Epstein-Barr virus, but this is a much less
common cause than the enteroviruses.
. Bacterial meningitis:
_______________________
. caused by Streptococcus pneumoniae or Neisseria meningitidis.
. will have a similar presentation However,
. CSF examination will show an increased cell count with a predominance of
neutrophils, increased protein, and decreased glucose Gram stain will show
bacteria.
. Suspect meningococcemia in a neonate with signs of meningitis and a petechial
rash.
. 75% of patients with Meningococcus meningitis present with a petechial rash that
is prominent on the axilla, wrists, flanks and ankles.
. It appears within 24 hours of the infection, and the patient generally appears sick.
. Even though pneumococcus is the most common organism responsible for
meningitis, it does not cause a rash.
. In an infant with meningococcemia, watch out for Waterhouse-Friderichsen
syndrome, which is
. characterized by a sudden vasomotor collapse and skin rash (large purpuric
lesions on the flanks) due to adrenal hemorrhage Fulminant meningococcemia can
occur
after a meningococcus infection, and approximately 10-20% of infants present
with vasomotor collapse, large petechiae and purpuric lesions.
. The condition carries an almost 100% mortality.
. Lumbar puncture (LP) is the key to establishing the diagnosis of meningitis and
differentiating between viral and bacterial causes.
. The first steps in management: supportive care with oxygen and fluids, a CBC,
and blood cultures.
. In contrast to suspected meningitis in adults, in whom antibiotics are followed by
(CT) scan and LP, children should first have an LP and then antibiotics.
. An LP is quick and easy to obtain in most children, even if they are agitated.
. Herniation is less likely in infants aged <1 year because the fontanelles have not
fully closed.
. This patient should have an LP followed by administration of vancomycin (given
the high incidence of resistant S. pneumoniae) and a third-generation cephalosporin
. If the LP is unsuccessful or cannot be performed, then antibiotics should be given
and the LP obtained as soon as possible.
. Although antibiotics given before LP can affect the yield of (CSF) Gram stain and
culture, pathogens other than meningococcus can usually be
identified in the CSF up to several hours after the administration of antibiotics.
. Blood cultures must be obtained prior to the antibiotics.
. Many experts recommend dexamethasone for all children aged >6 weeks with
pneumococcal meningitis and for those with Haemophilus influenza type b
meningitis
(if given within 1 hour of initial antibiotic therapy) to reduce the incidence of
hearing loss with bacterial meningitis.
. N.B.:
- CSF in tuberculous meningitis also shows a mildly elevated white cell count with
lymphocyte predominance; the protein will be very high and glucose will be low.
However, the presentation tends to be subacute rather than acute.
________
. Parotitis secondary to mumps.
. Orchitis is one of the most frequent complications of mumps, developing in about
20% of cases.
. It is most common in postpubertal young men, ages 15-29. Because less than
15% of orchitis cases of are bilateral, infertility is a rare complication.
. Treatment for mumps is supportive, with application of cold compresses to the
parotid area or testes.
. Other common complications of mumps are aseptic meningitis and encephalitis.
. Eczema herpeticum:
____________________
. is a form of primary herpes simplex virus infection that is usually superimposed on
healing atopic dermatitis lesions after exposure to herpes simplex virus.
. Numerous umbilicated vesicles over the area of healing atopic dermatitis are
typical.
. It is frequently accompanied with fever and adenopathy.
. In infants, the infection may be life-threatening, and acyclovir treatment should be
initiated as soon as possible.
. Erythema toxicum:
___________________
. The healthy appearance of the neonate, the evanescent nature of the rash, and
the distinctive red halo surrounding the lesions support the diagnosis of erythema
toxicum.
. The presence of numerous eosinophils in the pustules is diagnostic.
. Erythema toxicum is a benign, self-limited condition usually found in newborns
after the first 2 days of birth.
. Treatment is not necessary.
.N.B.:
- Milia are small pearly white cysts, and are distinct from the rash of erythema
toxicum.
- Sebaceous hyperplasia presents as little yellowish papules and are commonly
found on the face.
- Vaccination against hepatitis B decreases the incidence of hepatocellular
carcinoma, especially,
in regions with high levels of hepatitis B infection such as Asia (china) and Africa.
- The vaccination schedule for preterm infants should be conformed to the child's
chronologic age, not the gestational age.
- Because prematurity does not markedly change the immune response to
vaccines, and the risk of infection and complications in preterm infants is greater
than term.
- The exception is that children should be 2 kg prior to receiving the first hepatitis B
vaccine (which is at birth).
- Small for gestational age infants have a weight under the 10th percentile for
gestational age at birth
- May have complications such as hypoxia, polycythemia, hypoglycemia,
hypothermia, and hypocalcemia.
.N.B.:
- Albendazole or mebendazole is the first-line treatment for Enterobius vermicuLaris
infection, Pyrantel pamoate is an alternative.
- Urinalysis is a preliminary investigation that should be performed first in all
patients with suspected renal disease.
- All children with recurrent episodes of nocturnal vulvar itching should be
examined for pinworms and treated empirically with mebendazole.
- Female infants less than 3 months of age sometimes develop vaginal spotting,
discharge or bleeding.
Due to maternal estrogens crossing the placenta to the fetal blood before birth,
causing a pubertal effect in the newborn,
which disappears as soon as the hormone is cleared from the infant's circulation,
so treatment is reassurance of the mother.
. Lyme disease:
_______________
. The causative agent is the spirochete Borrelia burgdorferi, which is transmitted to
humans by the bite of an infected tick of the Ixodes species.
. The first clinical manifestation is the typical annular rash, named erythema
migrans, at the site of the bite.
. The rash may be uniformly erythematous, or it may appear as a target lesion with
central clearing.
. Erythema migrans may be associated with systemic features: fever, myalgia,
headache or malaise.
. Individuals who are exposed to tick-infested areas should wear light-colored
clothing so that ticks can be spotted more easily and removed before getting
attached.
. Wearing long-sleeved shirts and tucking pants into socks or boot tops can help
keep ticks from reaching the skin.
. Applying insect repellents containing DEET to clothes and exposed skin, and
applying permethrin to clothes, which kills ticks on contact, also reduce the risk of
tick attachment
. Tinea corporis:
_________________
. Is a superficial fungal infection mostly seen in hot, humid climates.
. The lesions are pruritic, erythematous, scaly, and have a red ring with central
clearing.
. Exposure to infected animals, people, and public places are major risk factors.
. Tinea corporis is most common in preadolescents.
. It is diagnosed clinically or with a skin scraping and potassium hydroxide
examination.
. Topical antifungals ( Terbinafine) are the preferred therapy.
. Reye syndrome:
________________
. also known as fatty liver with encephalopathy.
. A rare illness seen exclusively in children less than 15 years old who were treated
with salicylates for a viral infection.
. Pathophysiology Reye syndrome is characterized by diffuse mitochondrial injury
that leads to;
. Present with: vomiting, agitation, and irrational behavior, progressing to lethargy,
stupor, and restlessness, convulsions may occur.
. The liver is enlarged but there is no icterus.
. Laboratory findings: hyperammonemia, normal or slightly elevated bilirubin and
alkaline phosphatase, prolonged prothrombin time, hypoglycemia,
and moderate to severe elevations in AST, ALT, and lactate dehydrogenase levels.
. Histologically, extensive fatty vacuolization of the liver without inflammation is
presen
. Biopsy of the liver, kidneys and brain reveals microvesicular steatosis.
. Treatment is supportive
. Aspirin is therefore generally contraindicated in children, except in the treatment
of Kawasaki disease.
.N.B:
- Newborns of mothers with active hepatitis B infection should be passively
immunized at birth with hepatitis B immune globulin (HBIG) followed by
active immunization with recombinant HBV vaccine.
. Infant botulism:
__________________
. C. botulinum is the most common etiologic agent and is usually transmitted
through food, especially honey, which is the carrier in 1/3 of cases.
. In infantile form of botulism, the causative organism gains entry through the food
and produces toxin in the GIT, which subsequently triggers the symptoms.
. It is a protease that blocks acetylcholine release.
. Infants typically present between 2 weeks and 9 months with constipation and
poor feeding.
. This is followed by progressive hypotonia, weakness, loss of deep tendon reflexes,
cranial nerve abnormalities (impaired gag reflex), and respiratory difficulties.
. Signs of autonomic dysfunction such as hypotension and neurogenic bladder can
occur early in the course of the disease.
. Staphylococcal scalded skin syndrome (SSSS):
______________________________________________
. is caused by exfoliative toxin-producing strains of S. aureus.
. It starts with a prodrome of fever, irritability, and skin tenderness, which is
followed by generalized erythema and superficial flaccid blisters.
. A positive Nikolsky sign (gentle lateral pressure on the skin surface adjacent to a
blister causes slipping and detachment of a superficial layer of skin).
. Scaling and desquamation follow, before resolution of the disease process SSSS
usually affects children below age 10.
. but adults with kidney disease or immune compromise may also be affected.
. Cultures from intact bullae are usually sterile, because this is a toxin-mediated
process.
. The goal of treatment is to eliminate any focus of infection with appropriate antistaphylococcal antibiotics & provide supportive wound care of all denuded areas.
==================================================
============== Growth and
development==========================================
=================
. Rehydration after diarrhia:
_____________________________
. Adequate hydration is very important in the management of diarrhea,
replacement of electrolytes is also essential to prevent complications
such as water intoxication and hyponatremia.
. Water intoxication, hyponatremia, and seizures may result if diarrhea is treated
with large amounts of hypotonic or low-solute fluids (e g , water).
. Mild to moderated dehydration secondary to acute diarrhea --> Oral rehydration
therapy with Oral Rehydration Solution (ORS).
. Severe dehydration requires hospitalization and IV therapy.
. A healthy full term baby weight:
__________________________________
. Healthy, full term, and appropriate for gestational age infants may lose up to 10%
of their birth weight in the first week of life.
. The fetus exists in a state of relative total body water and extracellular fluid
excess.
. After birth, excretion of this excess water accounts for the majority of the weight
loss.
. The birth weight should be regained by 10 days of life.
. Signs of Neonate/Infant Dehydration:
--------------------------------------- Dry oral mucosa, lips, and tongue.
- Decreased number of wet diapers.
- No tears present when crying.
- Delayed capillary refill time (>2 seconds).
- Decreased skin turgor.
Managment of dehydration:
------------------------. The initial step in managing children with dehydration is to determine its severity.
. The ideal method of assessing dehydration is to determine the measured change
in weight because 1 kg of acute weight loss equals 1 L of fluid loss.
. A child's weight, however, changes constantly, making it difficult to obtain an
accurate recent "well" weight.
. So, the degree of dehydration often has to be determined by the clinical history
and physical examination & can be divided into:
. Fine Motor: Tow finger pincer grasp & turns several pages of a book at a time.
. Gross Motor: Walks without assistance & - Waves bye & Climbs up on furniture.
. Language: Says 2-3 words & - Says "mama" and "dada" games (can identify each
parent).
. Social: Imitates actions & - Plays reciprocal games (peek-a-boo) & indicates wants.
- Age: 2 year:
-------------. Fine Motor: Builds a twer of 6 cubes.
. Gross Motor: Walks up & down stairs
. Language: 200 word vocabulary & uses 2-word phrases
. Social: Follow 2 step commands.
- Age: 3 years:
---------------. Fine Motor: Copies a circle uses utenisles to feed self & stacks 9 blocks.
. Gross Motor: climbs stairs with alternating feet, rides a tricycle & kicks a ball.
. Language: uses 3 word sentences, stats first name & 3/4 (75%) of speech is
intelligible.
. Social: wash/dry hands, helps with simple household tasks & group play.
- Age: 4 years:
---------------. Fine Motor: Copies a cross, draw a person, begins to use scissors & holds a crayon
with a tripod grasp.
. Gross Motor: Hops on one foot with out losing balance & jumps over objects.
. Language: Counts to 10, tells stories & use lurals and prepositions.
. Social: Cooperative play, has imaginary friends & imitate adults role.
--------------------------------. Language development:
----------------------- Social smile 2 months
- Babbles 6 months
- 2 words, obeys 1-step command 1 year
. When a child is not ready for toilet training, forcing him to sit on the toilet will
make him more reluctant and can make potty training very difficult.
. The best step in the management of a reluctant child is to stop toilet training for
several months and allow the child to become interested on his own.
. Vitamin D deficiency rickets:
_______________________________
. This condition usually presents after several months of being Vitamin D-deficient.
. The typical pathology is - defective mineralization of growing bone or osteoid
tissue
. The typical patients are low-birth weight infants, unsupplemented dark-skinned
infants, infants with inadequate sun exposure, and solely breastfed infants.
. Early manifestations: craniotabes (ping-pong ball sensation over the occiput or
posterior parietal bones), rachitic rosary, and thickening of the wrists and ankles.
. Other findings: Harrison groove and a large anterior fontanelle.
. Diagnosis is confirmed by obtaining the serum calcifediol level (decreased),
alkaline phosphatase level (increased), and
observing the characteristic radiologic changes(cupping and fraying of the distal
ends of long bones, and double contour along the lateral outline of the radius).
. Treatment includes oral Vitamin D administration and adequate sunlight (or
artificial light) exposure.
. The current recommendation to prevent rickets is oral Vitamin D supplementation,
beginning during the first 2 months of life and continuing until early adolescence.
. Sexual developement:
______________________
. Normal sexual behaviors for young children (ages 2-5) include:
. Talking about genitals or reproduction, occasional masturbation, enjoying being
naked, and curiosity about their own and other people's body parts.
. They may play "doctor" with each other or compare their bodies with other
children's bodies.
. In addition, transient cross-dressing is often common in preschool boys.
. Extensive sexual knowledge as well as simulating foreplay or intercourse are
concerning behaviors in a young child and could be indicators of sexual abuse.
. Conduct disorder:
___________________
. Characterized by disruptive behavioral patterns that violate basic social norms for
at least one year in patients less than 18 years old.
. It includes aggressive behavior towards others or animals, behaviors resulting in
property damage, theft, or violations of societal rules.
. Constitutional growth delay:
_______________________________
. Constitutional growth delay is the most common cause of short stature and
pubertal delay in adolescents.
. Affected individuals have a normal birth weight and height, but between 6 months
to 3 years of age, the height growth velocity slows, and they drops percentiles on
their growth curve.
. Around 3 years of age, the child regains a normal growth velocity and follows the
growth curve at the 5th to 10th percentile.
. Puberty and the adolescent growth spurt are delayed, but eventually occur.
. The child will have a normal growth spurt and reach a normal adult height.
. Bone age radiographs show a bone age that is delayed compared to the
chronological age.
. Separation anxiety:
_____________________
. Separation anxiety is a normal behavior in young children between 9-18 months.
. usually manifests when parents leave the child or at night when the child is put to
bed.
. The child responds by crying, clinging to the parent, and becoming upset
Separation anxiety usually lasts 2-4 months and gradually resolves as the child
begins to
understand that his parents will return.
.The initial step in the management of children with speech delay is an audiology
evaluation especially when there is history of recurrent otitis media.
==================================================
=================== Miscellaneous
==============================================
. Autism:
_________
. patient is usually less than 3 years old and presents with impairment in social
interactions and communication, delayed language development,
repetitive activities, and stereotypical behaviors.
. Treatment include special education and behavioral modification techniques, No
pharmacological agent has been found to be useful in autism.
. Asperger syndrome:
____________________
. These kids are usually more socially aware and communicative. Although patients
may have features of stereotypical movements and
self-injurious behavior, their language development is normal.
. Attention-deficit hyperactivity disorder (ADHD):
__________________________________________________
. Children with ADHD have poor impulse control, inattentiveness, and motor
overactivity.
. Multiple factors contribute to development of ADHD: genetics, pregnancy or birth
complications, maternal drug use, abnormal brain development or traumatic brain
injury,
and psychological stressors.
Talks constantly.
Does not follow instructions with regard to school work chores etc.
Physically active all the time.
Has a hard time organizing tasks ,including fun tasks.
. In addition, features of autism generally appear before age three and social
isolation is more prominent than in hearing impairment.
. Management of a child who has accidentally taken liquid alkali:
---------------------------------------------------------------. The initial management is directed towards maintaining airway patency, The
absence of any added breath sounds and
presence of air movement bilaterally in this patient ensures patent airways.
. Next step, upper GI endoscopy is recommended in the first 24hrs to assess the
extent of the injury and to dictate further management.
. Barium swallow is not recommended due to the associated difficulty in
determining the extent of the injury with this procedure.
. Steroids are not recommended because these may increase the chance of
perforation Furthermore, giving steroids have not been proven to provide ,any
benefit
in preventing strictures in such cases.
. Nasogastric lavage and oral antidotes such as vinegar are not recommended since
these may increase the extent of injury.
. Remember attempting to neutralize the alkali with vinegar or lavage is dangerous!
. Photo-protection in high risk group: (high yield)
______________________________________
. Sun exposure is associated with skin photo-aging and several types of skin
cancer, including melanoma.
. Encouraging photo-protection is the leading preventive measure in skin care, and
sun avoidance is the best method of photo-protection.
. Patients are instructed to avoid outdoor activities in the middle of the day
(between 10 am and 4 pm), which is the period of peak sun radiation intensity.
. Sunscreens are useful adjuncts to photo-protection, but offer insufficient
protection from ultraviolet radiation (UVR) when used alone.
. Overdependence on sunscreens may sometimes even increase or encourage
outdoor exposure Sunscreens should be applied 15-60 min
prior to sun exposure to allow enough time for protective film development.
. Currently, the available data shows little to no protection against melanoma with
the use of sunscreen lotions with SPF 15 - 30;
however, these can protect from non-melanoma skin cancers such as squamous
cell carcinoma.
. Resting under umbrellas decreases UVR exposure by 70%, but does not affect
reflected radiation.
. Shade from trees provides insufficient UVR protection .
. Clothing is considered to be an effective tool for sun protection; however, it does
not supercede sun avoidance.
. Iron poisoning:
________________
. The patient's symptoms and history of spilled multivitamin pills are highly
suggestive of iron poisoning.
. This is one of the most common causes of death by poisoning Elemental iron,
when ingested in large amounts, has a corrosive effect on the GI mucosa,
leading to abdominal pain, nausea, vomiting, diarrhea and hematemesis.
. Iron also accumulates in the mitochondria and tissue, thus impairing energy
production and resulting in cellular damage and systemic toxicity.
. Hypotension then occurs due to the increased capillary permeability and
venodilation.
. The resulting peripheral hypoperfusion and mitochondrial damage lead to
accumulation of lactic and citric acid, and consequent metabolic acidosis.
. Other complications include liver necrosis, coagulopathy, drowsiness, seizures and
coma. Long-term sequelae include gastric scarring and pyloric stenosis.
. Since iron is radio-opaque, the tablets can be seen in the stomach on abdominal
x-ray.
. The diagnosis is confirmed by measuring serum iron levels.
. The treatment depends on the severity of the poisoning: Ipecac syrup to induce
emesis is used in asymptomatic patients with an intact gag reflex.
. Intravenous deferoxamine, an iron chelator, is used in moderate to severe cases
of intoxication.
. Activated charcoal is not effective, and gastric lavage is not recommended in
young children.
. Acute iron intoxicTION:
_________________________
. Is usually the result of a suicide attempt especially if symptoms of depression
(isolative behavior, decreased appetite, poor sleep) are present.
. Acute iron intoxication has five phases:
1- GIT phase --> 30 minutes to six hours after ingestion & is due to direct mucosal
damage, patient experience nausea, vomiting, hematemesis, melena, and abd.
pain.
2- Latent phase --> 6 to 24 hours after ingestion, and is largely asymptomatic.
3- Shock and metabolic --> acidosis may occur 6 to 72 hours following the
ingestion.
4- Hepatotoxicity --> 12 to 96 hours.
5- Bowel obstruction --> secondary to mucosal scarring can develop several weeks
post-ingestion
. The diagnosis of acute iron intoxication is made by checking the serum iron
concentration.
. Levels > 350 mcg/dL are consistent with toxicity.
. Treatment of iron poisoning involves deferoxamine, which binds ferric iron,
allowing urinary excretion.
.N.B: Common poisonings:
-----------------------1- There are no known cases of riboflavin (vitamin 82) poisoning.
2- Acute ingestion of vitamin A causes anorexia, bulging fontanelre (pseudotumor
cerebri), hyperirritability and vomiting.
3- Acute vitamin D ingestion causes anorexia, nausea, vomiting, diarrhea,
headache, polyuria and polydipsia.
4- Vitamin K may cause hyperbilirubinemia in premature infants.
. It derives its name from its similarities to true serum sickness, an immunecomplex mediated hypersensitivity reaction to non-human proteins.
. Serum-sickness like reaction is a clinical diagnosis, and should resolve with
withdrawal of the offending agent; it does not represent a true drug allergy.
. Deficiency of niacin (vitamin B3):
____________________________________
. leads to pellagra, which is characterized by diarrhea, dermatitis, dementia, and if
severe, death.
. Pellagra is common in 3rd world countries where the main diet consists of cereal
or corn, but can be seen in people with bowel disease that interferes with vitamin
absorption.
. Present with GIT complaints (nausea, abdominal pain, or epigastric discomfort)
along with glossitis and watery diarrhea.
. Dermatitis seen in pellagra occurs in sun exposed areas and resembles a sunburn;
it is also typically bilateral and symmetric.
. As the rash progresses, the skin becomes hyperpigmented and thickened.
. Mental status changes can range from poor concentration to irritability,
aggressiveness, and dementia.
. Death can occur in severe niacin deficiency if untreated.
. Treatment of pellagra is niacin replacement.
.N.B:
----1- Deficiency of thiamine (vitamin B1 ) causes beriberi or Wernicke-Korsakoff
syndrome
- These conditions are characterized by neurologic and psychiatric symptoms, and
are often seen in alcoholics or patients who have had weight loss surgery.
2- Deficiency of riboflavin (vitamin B2) can lead to cheilosis, glossitis, seborrheic
dermatitis (often affecting the genital areas), pharyngitis, and
edema and/or erythema of the mouth (Pure riboflavin deficiency is has been
documented in regions of the world with severe food shortages).
3- Pyridoxine (vitamin B6) deficiency causes irritability, depression, dermatitis, and
stomatitis.
- It can also cause an elevated serum homocysteine concentration, which is a
known risk factor for venous thromboembolic disease and atherosclerosis.
4- Deficiency of cyanocobalamin (vitamin 812) causes macrocytic anemia and
peripheral neuropathy.
. Vitamin A deficiency:
________________________________
. Suspect vitamin A deficiency in a 2 or 3-year-old child with impaired adaptation to
darkness, photophobia, dry scaly skin, xerosis conjunctiva,
xerosis cornea, keratomalacia, Bitot spots and follicular hyperkeratosis of the
shoulders, buttocks, and extensor surfaces
. Sudden infant death syndrome (SIDS):
______________________________________
. is the leading cause of mortality in infants aged between 1 month and 1 year in
the United States.
. It is defined as the sudden, unexpected death of an infant that cannot be
explained by the history and a thorough post-mortem examination.
. It is recommended that infants be placed in a supine position while sleeping to
reduce the risk of SIDS.
. Anaphylaxis:
______________
. is an acute, life-threatening, lgE-mediated type I hypersensitivity reaction.
. The most common inciting agents for anaphylaxis are insect envenomation, drugs
and foods.
. Food allergies are the major cause of pediatric outpatient anaphylaxis, and peanut
allergies account for the majority of fatal and near fatal reactions.
. Signs include bronchoconstriction, hypotension and urticaria.
. Intramuscular injection of epinephrine is the first-line treatment for anaphylaxis in
a patient with a patent airway.
. The most effective and potentially life-saving intervention in a patient with known
anaphylaxis is
prescription of self-administrable epinephrine for use upon reexposure to the
inciting allergen.
. Risperidone:
______________
. Is an atypical antipsychotic commonly used to treat schizophrenia and bipolar
disorder.
. It is a dopamine & serotonin antagonist.
. It inhibits dopamine, which leads to elevated serum prolactin levels.
. The hyperprolactinemia causes: oligomenorrhea, amenorrhea, and galactorrhea,
in premenopausal females.
. The resultant side effects can include the breast tenderness, amenorrhea, and
galactorrhea.
. Risperidone has been found to increase prolactin levels to a greater extent than
do many of the other antipsychotics.
. Cradle cap, or seborrheic dermatitis:
________________________________________
. is a common pediatric skin condition.
. This papular, scaly rash tends to affect the eyebrows, nasolabial folds, and scalp.
. Treatment: moisturizers, antifungals, and topical steroids.
. Atopic dermatitis:
____________________
. In infancy presents with pruritus and skin lesions typically distributed
symmetrically over the face, scalp, chest and extensor surfaces of the extremities.
. The diaper region is typically spared.
. Patients with exfoliative dermatitis (erythroderma):
_______________________________________________________
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=====================