1.To understand the etiology and the pathogenesis of HDN ?

Etiology - Caused by blood type incompatibility between mother and fetus. Pathogenesis :1. Hemolysis of ABO blood type incompatibility can occurred at first fetus. 2. Hemolysis of Rh blood type incompatibility occurred at second or later fetus.

2. Clinical manifestations of HDN ?

Clinical manifestations :1Jaundice 2Anemia 3Hepatosplenomegaly 4Kernicterus

3. Diagnosis and the treatment of HDN ?

Diagnosis :1Hemolysis of ABO blood type incompatibility: The antibody release test result is positive usually. 2Hemolysis of Rh blood type incompatibility The direct Coombs test is positive usually.

Treatments:
Premature treatments : Premature delivery:

If labor is induced, fetal lung maturity must be determined using the lecithin/sphingomyelon (L/S) ratio (thin layer chromatography) to avoid respiratory distress syndrome Plasmapheresis : For the pregnant mother unsuitable the premature delivery. Intrauterine blood transfusion: If fetal hydrops or fetal Hb<80g/l,and the lungs are still not mature.

 Neonatal treatments: Phototherapy Exchange Transfusions Pharmacological agents Other treatments : Prevention of hypoglycemia,hypothermia.

To correct hypoxia,anemia,edema and heart failure.

4. Definition :Billirubin encephalopathy

Neurologic syndrome resulting from the deposition of

unconjugated bilirubin in nucleus basalis.

Main cause due to hemolytic disease. Caused by any other disease with unconjugated hyperbilirubinemia.

5. Clinical manifestations and diagnosis of Bilirubin Encephalopathy ?

Warning stage: lasts about 12-24hrs. Spasm stage: lasts about 12-48hrs. Convalescent stage: lasts about 2weeks.

Sequelae stage

Warning stage of BE
Lethargy Low response Sucking weakness Weak sucking and moro reflexing Vomiting Reduced muscle tone Severe jaundice

Spasm stage
Convulsion

Opisthotonos and fever

Increased muscle tone Apnea Stiffly extending his of her arms inward rotation with fists clenched or even opisthotonos

Convalescent stage
Nursing and reaction improvement

Reduction of seizure frequency

Opisthotonos gradually disappeared Muscle tone gradually recovered

Sequelae stage:BE of tetralogy

Athetosis

Limitation of eyes movement

Hearing impairment Enamel dysplasia

6. Therapy of BE ?
Phototherapy Plasma or albumin Exchange transfusion Enzyme inducer: phenobarbital Original diseases treatment The other symptomatic treatment

7. How do we avoid the bilirubin encephalopathy for HDN infants?

Declining jaundice quickly is necessary 4 HDN infants 2 avoid BE . Phototherapy Exchange transfusions

8. whats the characteristics of physiologic jaundice The characteristics of physiologic jaundice : 1Jaundice appears at 2~3 days. 2Full-term neonates jaundice declines to adult levels within 14d. Prematures jaundice usually delays to 3~4 wk of age. 3Bilirubin level of blood serum < 222~257 mol/L 4Conjugated bilirubin level of blood < 34 mol/L 5Bilirubin level of blood serum increases every day < 85mol/L. 6General condition of the baby is well.

9. Whats the characteristics of pathologic jaundice?

The characteristics of pathologic jaundice :1Appear too early 2Degree too severe 3Duration too long 4Progress too rapid 5Again appeared after it subsided

10. Treatment of neonatal jaundice ?

Phototherapy Light source : spectral outputs 420 500 nm Exchange transfusions : Remove antibody Remove bilirubin Correct anemia

11. Differences between physiologic jaundice and pathologic jaundice ?

Refer to slide 16- 17.

12. Indications & Complications of Phototherapy ?

Phototherapy :
Lipid unconjugated bilirubin water- soluble isomeride

- Light source: Spectral outputs 420 to 500nm

Side effects of phototherapy :

a. Diarrhea

b. Fever
c. Skin rash d. Bronze baby syndrome

(Conjugated bilirubin > 4mg/dl)

e. Riboflavin reduction f. Dehydration and Hypocalcemia

13. Diagnosis test of HDN ?

Sensitized erythrocytes and blood group antibodies determination:
Coombs test : Red blood cells sensitized.

Antibody releasing test : Sensitivity test to detect sensitized erythrocytes.

Blood type antibody test in the infant serum. Blood type antibody test in the maternal serum.

14. Pathophysiology of Rh ?
Pathophysiology :-

Red blood cell breakdown

Hyperbilirubinemia
Jaundice Kernicterus Seizures etc.

Anemia
1. Liver

2. Spleen
3. Heart, other organs 4. Hydrops

15. Indications of Exchange Transfusions ?

Indication of transfusions:

one of the follows

a. 20mg/dl (340 mol/L) b. > 4mg/dl,Hgb<120g/L, edema c. 0.7mg/dl/h d. Kernicterus e. Preterm infants

16. Causes of neonatal hypoxic ischemic encephalopathy ?

Causes

17. Pathology & Pathophysiological changes of neonatal HIE ?

Pathophysiology

18. Clinical features of neonatal HIE ?

Muscle tone and deep tendon reflexes Behavioral abnormalities Neonatal reflexes Apnea and seizures Stupor and ventilatory support Ocular motion and pupils Heart rate and blood pressure Outcome

19. Differential diagnosis of neonatal HIE ?

Methylmalonic Acidemia Propionic Acidemia Urea cycle defects Zellweger syndrome Mitochondrial disorders Neuromuscular disorders Brain tumors Developmental defects Infections

20. Treatment of neonatal HIE ?

Medical Care : Initial resuscitation and stabilization Supportive care: mechanical ventilation Perfusion and blood pressure management Fluid and electrolytes management Hyperthermia management Treatment of seizures

Surgical Care Consultations Diet

21. Prognosis of neonatal HIE ?

Lack of spontaneous respiratory effort The presence of seizures Abnormal neurological findings Abnormal EEG background Feeding difficulties Poor head growth

22. Diagnosis criteria of bacterial meningitis ?

Confirmation of the diagnosis: isolation from the CSF of a specific bacteria pathogen by microscopy or a positive culture or rapid antigen-detection test of CSF Gram-stained smear of CSF: identify the causative organism in 70-90% of cases CSF culture: positive in about 80% of cases. Definitive diagnosis, determination of antibiotic sensitivity. PCR: amplifies bacteria DNA(H influenzae, N meningitidis)

23.Symptom of purulent meningitis ?

Fever Headache Vomiting Increased ICP Meningeal Irritation Anorexia Malaise

24.Complication of meningitis ?
1) Subdural effusion 2) Cerebral hyponatremia 3) Ependymitis 4) Hydrocephalus 5) Others : Epilepsy (complications)

25. Treatment of Meningitis

Antibiotic : Penicilln, Ampicillin,ceftriaxone Corticosteroid : Dexamethasone Mannitol General and supportive measure
Maintenance fluid and thermal energy supplement. Fluid administration: 60-80ml/kg.d

26.Complication of epilepsy ?
1.Mental retardation 2.Loss of hearing 3.Impairment of visual area 4.Motor and sensory deficits

27. Tuberculous Meningitis (Manifestation)

Increased Intracranial Pressure Cranial Nerve Injury

Meninges Irritation Signs

Cerebral-Parenchyma Damage

Spinal Cord Damage

Pneumonia (Manifestation)
Tachypnea, Dyspnea, Retractions, Grunting, Nasal flaring, Cyanosis and the presence of rales or rhonchi on physical examination. Lethargy, Poor feeding, Fever, temperature instability, Apnea, Tachycardia, Poor perfusion, Metabolic acidosis

Tuberculosis Symptoms
Perpetual Cough Fever Weight loss

Night sweats
Loss of appetite

Fatigue
Pain while breathing

Diagnosis of TB
Tuberculin skin test Chest X-ray Physical examination Serological (blood) test AFD test (collect sputum to smear and culture it)

TREATMENT FOR ACTIVE TB

A combination of the following four antibiotics are the most commonly used, however are not risk free: rifampicin R
It may cause fever, rashes, liver damage, and in some rare cases has led to death.

isoniazid H
It may cause rash and abnormal liver function tests.

ethambutol E
It may cause Red-green color blindness and loss of vision

pyrazinamide Z
It may cause joint pains, nausea, vomiting, skin rash,, malaise, and fever

When taking antibiotics, overdose and underdose is a problem

Treatment for TB meningitis

Antituberculosis drugs Corticosteroids Decrease intracranial pressure Ventriculoperitoneal shunting

Questions
What is diagnostic criteria of nephrotic syndrome? How to distinguish the differences between simple type and nephritic type? What are causes of the nephrotic syndrome ? What is histology of primary nephrotic syndrome? What are major complications of nephrotic syndrome? How to treat nephrotic syndrome?

Diagnostic Criteria
1. Nephrotic range proteinuria
proteinuria(>50mg/kg/day) Urine protein 3+ or 4+ by dipstick testing

2. Hypoalbuminaemia
serum albumin less than 25g/l

3. Oedema
The onset is insidious , with swelling around the eye and facial puffiness .Over the next few days ,it gradually increases to involve the extremities and abdomen

4. Hyperlipidemia
Serum cholesterol more than 5.7mmol/l

Difference
1. Nephrotic syndrome is a disease of the kidney while nephritic syndrome is a disease of the glomeruli. Nephritic syndrome is also called glomerulonephritis. 2. Nephrotic syndrome manifests the classic symptoms, such as: edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Nephritic syndrome manifests the same except there is an accompanying blood in the urine. 3. Diagnosis for nephrotic syndrome is a 24-hour urine/protein measurement and lipid profile while a nephritic syndrome involves an ASOT, urine tests, and blood tests.

Main causes of nephrotic syndrome (NS) in children

Primary nephrotic syndrome Secondary nephrotic syndrome (Multisystem diseases ) Henoch-schonlein purpura Systemic lupus erythematosus Hepatitis B Congenital nephrotic syndrome Idiopathic CNS of Finnish type Diffuse mesangial sclerosis Secondary Congenital syphilis Toxoplasmosis Cytomegalovirus

Histopathologic classification
Minimal change diseases (MCD) Pure minimal change diseases MCD with mesangial proliferation (MesPGN) Focal segmental glomerulosclerosis(FSGS) Membranoproliferative glomerulonephritis(MPGN) Membraneous nephropathy(MN)

Major Complications of Nephrotic Syndrome

Infection Hypovolaemia Thrombosis Hyperlipidaemia Acute renal failure Side-effects of drugs Corticosteroids Alkylating agents Cyclosporin A

Management
Cyclophosphamide (CTX)
CTX2-3mg/kg/day po for 8-12weeks ,or iv pulse CTX 0.5/m2

Cyclosporine (CsA)
Dosage of 5-6mg/kg/day to maintain the whole blood trough level at 80 to 120ng/ml

Tacrolimus (FK506)
0.1mg/kg/day in BD doses Trough level maintain at 5-10mcg/l

Mycophnolate mofetil (MMF)

Dosage 250-1200mg/m2/day in 2 doses

Supportive Therapy
To minimize infection ( vaccination and prophylactic antibioticcs ) To minimize risk of thrombosis ( hydration; and avoiding prolonged immobilisation) To maintain nutrition ( high biological value protein intake ) To treat edema and hypertension To treat hypercholesterolaemia (low cholesterol diet and lipid lowering agents) To reduce proteinuria (ACEI or angiotensin 2 receptor blockers) To avoid the use of nephrotoxic drugs

General Symptoms Acute Glomerulonephritis

Fatigue Abdominal pain: more than half Low-grade fever Loss of appetite Nausea Vomiting Headache Hematuria Proteinuria Edema Hypertension

Lab Investigation A.G.

Urinalysis Blood test Blood Chemistry Serological testing Complement level (C3,C4) Renal Ultrasound

Treatment AG
Restriction
Salt Fluid intake

Control of hypertension
Diuretics (Furosemide) Anti hypertensives

Dialysis

1. Inadequate storage: Premature<3mon, Twins or Multiplets, Intrapartum hemorrhage, etc. 2. insufficiency of intake Iron is absorbed 2 to 3 times more efficiently from human milk than from cow's milk / Introduction of Solid Food 3.Growthblood volume increased > Iron intake 4.Disorder of Digestion and Absorption: chronic diarrhea, Repeated infection, Malnutrition 5. Losing too much: Protein allergy, Hookworm, Polyp, Diverticulum

Anemia three types: Anemia of inadequate production Hemolytic anemia

Blood loss

1.blood filmhypochromic and microcytic

2.bone marrow samplingThe bone marrow is hypercellular, with erythroid

hyperplasia,The normoblasts.may have scanty, fragmented cytoplasm with poor hemoglobinization.

3.iron metabolism:

Iron study *serum ferritin(SF) Iron store FEP( sideroblasts Serum iron

ID (<12ng/mL) N N N

IDE N or

IDA