6.

Leukemia
A. Overview
Leukemia is a malignancy (cancer) of blood cells. In leukemia, abnormal blood
cells are produced in the bone marrow. Usually, leukemia involves the production
of abnormal white blood cells -- the cells responsible for fighting infection.
However, the abnormal cells in leukemia do not function in the same way as
normal white blood cells. The leukemia cells continue to grow and divide,
eventually crowding out the normal blood cells. The end result is that it becomes
difficult for the body to fight infections, control bleeding, and transport oxygen.
There are different types of leukemia, based upon how quickly the disease
develops and the type of abnormal cells produced. Leukemia is called an acute
leukemia if it develops rapidly. Large numbers of leukemia cells accumulate very
quickly in the blood and bone marrow, leading to symptoms such as tiredness,
easy bruising, and susceptibility to infections. Acute leukemia requires fast and
aggressive treatment.
There are around 54,000 new cases of leukemia each year in the U.S. and about
24,000 deaths due to leukemia. Leukemia makes up about 3% of all new cancer
cases.
Chronic leukemias develop slowly over time. These leukemias may not cause
specific symptoms at the beginning of their course. If left untreated, the cells may
eventually grow to high numbers, as in acute leukemias causing similar
symptoms.
Leukemias are further classified as myeloid or lymphoid, depending upon the
type of white blood cell that makes up the leukemia cells. A basic understanding
of the normal development of blood cells is needed to understand the different
types of leukemia. Normal blood cells develop from stem cells that have the
potential to become many cell types. Myeloid stem cells mature in the bone
marrow and become immature white cells called myeloid blasts. These myeloid
blasts further mature to become either red blood cells, platelets, or certain kinds
of white blood cells. Lymphoid stem cells mature in the bone marrow to become
lymphoid blasts. The lymphoid blasts develop further into T or B lymphocytes,
special types of white blood cells. Myeloid leukemias are made up of cells that
arise from myeloid cells, while lymphoid leukemias arise from lymphoid cells.
Knowing the type of cell involved in leukemia is important in choosing the
appropriate treatment.
Common types of leukemia

The four most common types of leukemia are acute lymphocytic leukemia,
chronic lymphocytic leukemia, acute myeloid leukemia, and chronic myeloid
leukemia.
Acute lymphocytic leukemia (ALL, also known as acute lymphoblastic
leukemia) is the most common type of leukemia in children, but it can also
affect adults. In this type of leukemia, immature lymphoid cells grow rapidly in
the blood. It affects over 6,000 people per year in the U.S.
Acute myeloid leukemia (AML, also called acute myelogenous leukemia)
involves the rapid growth of myeloid cells. It occurs in both adults and
children and affects about 18,000 people each year in the U.S.
Chronic lymphocytic leukemia (CLL) is a slow-growing cancer of lymphoid
cells that usually affects people over 55 years of age. It is estimated to affect
about 16,000 people in the U.S. every year. It almost never occurs in children
or adolescents.
Chronic myeloid leukemia (CML, also known as chronic myelogenous
leukemia) primarily affects adults and occurs in about 6,000 people every
year in the U.S.
B. Manifestations and Diagnosis
Many of the symptoms of childhood leukemia can have other causes as well, and
most often these symptoms are not caused by leukemia. Still, if your child has
any of them, its important to have your child seen by a doctor so the cause can
be found and treated, if needed.
The symptoms of leukemia are often caused by problems in the childs bone
marrow, which is where the leukemia begins. As leukemia cells build up in the
marrow, they can crowd out the normal blood cell-making cells. As a result, a
child may not have enough normal red blood cells, white blood cells, and blood
platelets. These shortages show up on blood tests, but they can also cause
symptoms. The leukemia cells might also invade other areas of the body, which
can also cause symptoms.
Symptoms from low red blood cell counts (anemia): Red blood cells carry oxygen
to all of the cells in the body. A shortage of red blood cells can cause:

Tiredness (fatigue)
Weakness
Feeling cold
Feeling dizzy or lightheaded
Headaches
Shortness of breath
Pale skin

Symptoms from low white blood cell counts:

Infections can occur because of a shortage of normal white blood cells.

Children with leukemia can get infections that dont seem to go away or may
get one infection after another. Although children with leukemia often have
high white blood cell counts because they have so many leukemia cells,
these cells dont protect against infection the way normal white blood cells do.
Fever is often the main sign of infection. But some children might have a
fever without having an infection.

Symptoms from low blood platelet counts: Platelets in the blood normally help
stop bleeding. A shortage of platelets can lead to:
Easy bruising and bleeding
Frequent or severe nosebleeds
Bleeding gums
Bone or joint pain: This pain is caused by the buildup of leukemia cells near the
surface of the bone or inside the joint.
Swelling of the abdomen (belly): Leukemia cells can collect in the liver and
spleen, making them bigger. This might be noticed as a fullness or swelling of the
belly. The lower ribs usually cover these organs, but when they are enlarged the
doctor can often feel them.
Loss of appetite and weight loss: If the spleen and/or liver get big enough, they
can press against other organs like the stomach. This can make the child feel full
after eating only a small amount of food, leading to a loss of appetite and weight
loss over time.
Swollen lymph nodes: Some leukemias spread to lymph nodes. Swollen nodes
may be seen or felt as lumps under the skin in certain areas of the body (such as
on the sides of the neck, in underarm areas, above the collarbone, or in the
groin). Lymph nodes inside the chest or abdomen can also swell, but these can
only be seen on imaging tests, such as CT or MRI scans.
In infants and children, lymph nodes often get bigger when they are fighting an
infection. An enlarged lymph node in a child is much more often a sign of
infection than leukemia, but it should be checked by a doctor and followed
closely.
Coughing or trouble breathing: Some types of leukemia can affect structures in
the middle of the chest, such as lymph nodes or the thymus (a small organ in
front of the trachea, the breathing tube that leads to the lungs). An enlarged
thymus or lymph nodes in the chest can press on the trachea, causing coughing
or trouble breathing. In some cases where the white blood cell count is very high,
the leukemia cells can build up in the small blood vessels of the lungs, which can
also cause trouble breathing.

Swelling of the face and arms: The superior vena cava (SVC), a large vein that
carries blood from the head and arms back to the heart, passes next to the
thymus. An enlarged thymus may press on the SVC, causing the blood to back
up in the veins. This is known as SVC syndrome. It can cause swelling in the
face, neck, arms, and upper chest (sometimes with a bluish-red skin color). It can
also cause headaches, dizziness, and a change in consciousness if it affects the
brain. The SVC syndrome can be life-threatening, and needs to be treated right
away.
Headache, seizures, vomiting: A small number of children have leukemia that has
already spread to the brain and spinal cord when they are first diagnosed. This
can lead to symptoms such as headache, trouble concentrating, weakness,
seizures, vomiting, problems with balance, and blurred vision.
Rashes, gum problems: In children with acute myelogenous leukemia (AML),
leukemia cells may spread to the gums, causing swelling, pain, and bleeding. If it
spreads to the skin, it can cause small, dark spots that look like common rashes.
A collection of AML cells under the skin or in other parts of the body is called a
chloroma or granulocytic sarcoma.
Extreme fatigue, weakness: A rare but very serious consequence of AML is
extreme tiredness, weakness, and slurring of speech. This can occur when very
high numbers of leukemia cells cause the blood to become too thick and slow the
circulation through small blood vessels of the brain.
C. Disease Causation
The exact cause of leukemia is not known, but it is thought to involve a
combination of genetic and environmental factors. Leukemia cells have acquired
mutations in their DNA that cause them to grow abnormally and lose functions of
typical white blood cells. It is not clear what causes these mutations to occur.
One type of change in the cells' DNA that is common in leukemias is known as a
chromosome translocation. In this process, a portion of one chromosome breaks
off and attaches to a different chromosome. One translocation seen in almost all
cases of CML and in sometimes in other types of leukemia is an exchange of
DNA between chromosomes 9 and 22, which leads to what is known as the
Philadelphia chromosome. This creates an oncogene (cancer-promoting gene)
known as BCR-ABL. This change in DNA is not inherited but occurs sometime in
the life of the affected individual.
Most cases of leukemia are not believed to be hereditary, but certain genetic
mutations and conditions can be passed along to offspring that increase the
chances of developing leukemia. A condition known as Li-Fraumeni syndrome is
characterized by an inherited mutation in a tumor suppressor gene known as
TP53, and individuals with this condition have an increased risk of leukemia and
other cancers.

D. Medical Management
The treatment a child will undergo is based on the child's age, overall health,
medical history, their tolerance for certain medications, procedures, and
therapies, along with the parents' opinion and preference.[citation needed]
Chemotherapy is a treatment that uses drugs to interfere with the cancer cells
ability to grow and reproduce. Chemotherapy can be used alone or in
combination with other therapies. Chemotherapy can be given either as a pill to
swallow orally, an injection into the fat or muscle, through an IV directly into the
bloodstream, or directly into the spinal column.[citation needed]
A stem cell transplant is a process by which healthy cells are infused into the
body. A stem-cell transplant can help the human body make enough healthy
white blood cells, red blood cells, or platelets, and reduce the risk of lifethreatening infections, anemia, and bleeding. It is also known as a bone-marrow
transplant or an umbilical-cord blood transplant, depending on the source of the
stem cells. Stem cell transplants can use the cells from the same person, called
an autologous stem cell transplant or they can use stem cells from other people,
known as an allogenic stem cell transplant. In some cases, the parents of a child
with childhood leukemia may conceive a saviour sibling by preimplantation
genetic diagnosis to be an appropriate match for the HLA antigen.
E. Nursing Management
Leukemia is usually treated by a hematologist-oncologist. These are doctors who
specialize in blood disorders and cancer. The treatment depends on the type and
stage of the cancer. Some forms of leukemia grow slowly and dont need
immediate treatment. However, treatment for leukemia usually involves one or
more of the following:

Chemotherapy uses drugs to kill leukemia cells. Depending on the type of

leukemia, you may take either a single drug or a combination of different
drugs.
Radiation therapy uses high-energy radiation to damage leukemia cells and
inhibit their growth. Radiation can be applied to a specific area or to your
entire body.
Stem cell transplantation replaces diseased bone marrow with healthy bone
marrow, either your own (autologous transplantation) or from a donor
(allologous transplantation). This procedure is also called a bone marrow
transplant.
Biological or immune therapy uses treatments that help your immune system
recognize and attack cancer cells.
Targeted therapy uses medications that take advantage of vulnerabilities in
cancer cells. For example, imatinib (Gleevec) is a targeted drug that is
commonly used against CML.

7. Hemophilia
A. Overview
Hemophilia is a rare disorder in which your blood doesn't clot normally
because it lacks sufficient blood-clotting proteins (clotting factors). If you
have hemophilia, you may bleed for a longer time after an injury than you
would if your blood clotted normally.
Small cuts usually aren't much of a problem. The greater health concern
is deep bleeding inside your body, especially in your knees, ankles and
elbows. That internal bleeding can damage your organs and tissues, and
may be life-threatening.
Hemophilia is an inherited (genetic) disorder. There's no cure yet. But with
proper treatment and self-care, most people with hemophilia can maintain
an active, productive lifestyle.
B. Manifestations and Diagnosis
Signs and symptoms of hemophilia vary, depending on your level of
clotting factors. If your clotting-factor level is mildly reduced, you may
bleed only after surgery or trauma. If your deficiency is severe, you may
experience spontaneous bleeding.
Signs and symptoms of spontaneous bleeding include:

Unexplained and excessive bleeding from cuts or injuries, or after surgery or

dental work
Many large or deep bruises
Unusual bleeding after vaccinations
Pain, swelling or tightness in your joints
Blood in your urine or stool
Nosebleeds without a known cause
In infants, unexplained irritability
Emergency signs and symptoms of hemophilia include:

Sudden pain, swelling and warmth in large joints, such as knees, elbows,
hips and shoulders, and in your arm and leg muscles
Bleeding from an injury, especially if you have a severe form of hemophilia
Painful, prolonged headache
Repeated vomiting
Extreme fatigue
Neck pain
Double vision

C. Disease Causation
When you bleed, your body normally pools blood cells together to form a
clot to stop the bleeding. The clotting process is encouraged by certain
blood particles (platelets and plasma proteins). Hemophilia occurs when
you have a deficiency in one of these clotting factors.
Hemophilia is inherited. However, about 30 percent of people with
hemophilia have no family history of the disorder. In these people
hemophilia is caused by a genetic change (spontaneous mutation).

There are several types of hemophilia. They are classified according to

which clotting factor is deficient:
Hemophilia A, the most common type, is caused by insufficient clotting factor
VIII.
Hemophilia B, the second most common type, is caused by insufficient
clotting factor IX.
Hemophilia C, in which signs and symptoms are often mild, is caused by
insufficient clotting factor XI.
D. Medical Management
Treatment of hemophilia depends on the type and severity, with the goal
of preventing complications associated with excessive bleeding. Factor
replacement therapy is the standard of care.
Your child will require factor replacement therapyinfusions (injections
into a vein) of clotting factors VIII or IXwhich can prevent or stop
bleeding and promote healing. These infusions will be needed:
after a bleeding episode
before, during or after surgery and routine procedures, including dental
work
as a preventive measure (prophylaxis) in the case of severe hemophilia
Factor replacement therapy stops bleeding and reduces the risk of
complications. Children receive infusions of synthetic clotting factors to
replace the factors missing from their blood. Children with hemophilia A
receive factor VIII infusions, while patients with hemophilia B receive
factor IX infusions.
If your child has mild hemophilia A, he may also receive a synthetic
hormone called desmopressin, or DDAVP. This can be administered as a
nasal spray or intravenously (through an IV).
Some patients may develop an inhibitor to clotting proteins and will
require hemophilia treatment with other clotting proteins, such as
activated factor VII.

During the course of treating the hemophilia, your childs doctor will
recommend:
giving routine immunizations subcutaneously (under the skin) in the
muscle to prevent deep muscle bleedsthis is important for your
pediatrician to know
avoiding aspirin and ibuprofen, as well as products containing them, since
they have been linked to bleeding problems
getting frequent follow-up care, including regular assessment of the joints;
children who experience bleeding into the joints may require physical
therapy.
E. Nursing Management

Provide emotional support, and listen to the patients fears and concerns.
If the patient has surface cuts or epistaxis, apply pressure.
Give the deficient clotting factor or plasma, as ordered.
Apply cold compress or ice bags and raise the injured part.
To prevent recurrence of bleeding, restrict activity for 48 hours after bleeding
is under control.
Control pain with an analgesics, as ordered.
If the patient cant tolerate activities because of blood loss, provide rest
periods between acivities.
To restore joint mobility, if ordered, begin range of motion exercises at least
48 hours after the bleeding is controlled.
Watch for signs and symptoms of decreased tissue perfusion such as
restlessness, anxiety, confusion, pallor, cool and clammy skin, chest pain,
decreased urine output.
Tell the patient to avoid heavy lifting and using power tools because they risk
of injury that can result in serious bleeding problem.
Advise the patient to notify the doctor immediately after even a minor injury
Teach the patient the importance of protecting his veins for lifelong therapy.
8. Tonsiltis and Adenoiditis
A. Overview
Tonsillitis is an infection of the tonsils that causes inflammation. It's most
common in children aged 3 to 7, who have larger tonsils than adults and
older children. The tonsils are made of lymphatic tissue. Their job is to
produce antibodies that fight infection. Ironically, such tissue is quite
prone to becoming infected itself. Tonsillitis can be caused by viral or
bacterial infections.
Many cases of tonsillitis never reach the doctor's office. However, it is
estimated that 15% of all visits to family doctors are because of tonsillitis.

Tonsillectomy (tonsil removal) has always been the most common

childhood operation, but it is considered a last resort for tonsillitis these
days.
B. Manifestations and Diagnosis
The main symptom of tonsillitis is sore throat, but since the throat and
ears share the same nerves, the pain is often felt in the ears. The pain is
usually worse when swallowing. Very young children may not complain of
a sore throat but may simply refuse to eat.

Other symptoms can include:

fever
general ill feeling
tender lymph nodes in the neck
headaches
vomiting
C. Disease Causation
There are three common causes of tonsillitis:
Group A streptococcus, which are common bacteria that cause throat
infections in 1 in 5 people, including adults. Many people have no
symptoms but they can still transmit the bacteria. Group A streptococcus
can also cause strep throat. It's also capable under some circumstances
of causing more serious illness, such as rheumatic fever.
Various respiratory viruses, especially cold and flu viruses, account for
most cases of tonsillitis. These infections can be sometimes milder than
bacterial infections, but it is often difficult to tell the difference between a
viral and bacterial infection.
Infectious mononucleosis, caused by the Epstein-Barr virus, can also
produce symptoms of tonsillitis, especially in children.

D. Medical Management
If you have tonsillitis, you should rest and stay well hydrated. You can
take acetaminophen* or ibuprofen to ease symptoms, but acetylsalicylic
acid (ASA) should be avoided in children with viral infections, as it can
lead to Reye's syndrome, a very dangerous condition that affects many
organs, particularly the brain and liver.
When bacteria are causing the infection, your doctor will likely prescribe
an antibiotic. Most doctors will not prescribe antibiotics until tests confirm
that bacteria are the cause. However, people with 3 out of following 4
characteristic symptoms may be treated with antibiotics "up-front" (before

culture results are known): fever, discharge from the tonsils, no cough,
and tender lymph nodes.
E. Nursing Management

To stop the spread of infection of the said disease, the nurse must healthteach:
Wash his or her hands thoroughly and frequently, especially after using
the toilet and before eating
Avoid sharing food, drinking glasses, water bottles or utensils
Replace his or her toothbrush after being diagnosed with tonsillitis
Health the following to prevent the spread of a bacterial or viral infection
to others:
Keep your child at home when he or she is ill
Ask your doctor when it's all right for your child to return to school
Teach your child to cough or sneeze into a tissue or, when necessary, into
his or her elbow
Teach your child to wash his or her hands after sneezing or coughing
Adenoiditis

A. Overview
Adenoids are a mass of tissue that, along with your tonsils, help keep you
healthy by trapping harmful germs that pass through the nose or mouth.
Your adenoids also produce antibodies to help your body fight infections.
Unlike tonsils, which can be easily seen by opening your mouth, you
cannot see the adenoids. A doctor has to use a small mirror or special
instrument with a light to see the adenoids. Sometimes X-rays may be
taken to see them more clearly.
While adenoids play an important role in keeping a person healthy, as you
get older, adenoids become less important, because your body is able to
fight infection in other ways. In fact, adenoids often get smaller around
age 5 or 6 and virtually disappear by the teen years.
Even though adenoids help filter out germs from your body, sometimes
they can get overwhelmed by bacteria and become infected. When this
happens they also get inflamed and swollen. This condition is called
adenoiditis. It is most commonly seen in children, but sometimes affect
adults.

B. Manifestations and Diagnosis

Symptoms of adenoiditis can vary depending on what is causing the
infection, but may include:

sore throat

stuffy nose
swollen glands in the neck
ear pain and other ear problems
When the nose is stuffy, breathing through it can be a challenge. Other
symptoms of adenoiditis related to nasal congestion include:

breathing through the mouth

speaking with a nasal sound, as if you are speaking with a pinched nose
difficulty sleeping
snoring or sleep apnea (a condition where you stop breathing for a short
amount of time during sleep)

C. Disease Causation
Adenoiditis can be caused by a bacterial infection, such as infection with
the bacteria Streptococcus. It can also be caused by a number of viruses,
including Epstein-Barr virus, adenovirus, and rhinovirus.
D. Medical Management
If a bacteria caused your adenoiditis, your doctor may prescribe
antibiotics. The use of antibiotics often proves successful in treating
inflamed adenoidal tissue. If a virus caused your adenoiditis, your doctor
will put you on a treatment plan that is specific to the virus.

Surgery to remove your adenoids may also be an option. This is called

adenoidectomy. Surgery is used to remove adenoids that:
don't get better with antibiotics
have recurring infections
exist alongside an underlying health issue, such as cancer or a tumor of
the throat and neck
cause breathing and swallowing problems

E. Nursing Management

Promote rest
Increase fluid intake
Warn saline gargle
Analgesics as ordered
Antimicrobial as ordered.
Surgery: Tonsillectomy/ Adenoidectomy (indicated if tonsillitis recurs 5 to 6 times
a year).

9. AGN
A. Overview

Acute glomerulonephritis (GN) comprises a specific set of renal diseases

in which an immunologic mechanism triggers inflammation and

proliferation of glomerular tissue that can result in damage to the

basement
membrane,
mesangium,
or
capillary endothelium.
Glomerulonephritis (GN) is inflammation of the glomeruli, which are
structures in your kidneys that are made up of tiny blood vessels. These
knots of vessels help filter your blood and remove excess fluids. If your
glomeruli are damaged, your kidneys will stop working properly and you
can go into kidney failure. Glomerulonephritis is a serious illness that can
be life-threatening and requires immediate treatment. The condition is
sometimes called nephritis. There can be both acute (sudden)
glomerulonephritis
and
chronic
(long-term
or
recurring)
glomerulonephritis.
B. Manifestation and Diagnosis

Acute GN
Early symptoms of acute GN include:
puffiness in the face (edema)
urinating less often
blood in your urine (dark, rust-colored urine)
extra fluid in your lungs, causing coughing
high blood pressure
Chronic GN
The chronic form of glomerulonephritis can creep up without any
symptoms. There may be slow development of symptoms similar to the
acute form. Some symptoms include:

blood or excess protein in your urine, which may be microscopic and

show up in urine tests
high blood pressure
swelling in ankles and face (edema)
frequent nighttime urination
bubbly or foamy urine (from excess protein)
abdominal pain
frequent nosebleeds
Diagnoses
The first step in diagnosis is to have a urinalysis test. A routine physical
exam for another condition can also lead to the discovery of GN. Blood
and protein in the urine during urinalysis are important markers for the
disease.

C. Disease Causation
Acute GN can be a response to an infection such as strep throat or an
abscessed tooth. It may be due to problems with your immune system
overreacting to the infection. This can go away without treatment. If it
doesnt go away, prompt treatment is necessary to prevent long-term

damage to your kidneys. Illnesses that have been known to trigger acute
GN include:

strep throat
systemic lupus erythematosus (SLE), which is also called lupus
Goodpastures syndrome, which is a rare autoimmune disease in which
antibodies attack your kidneys and lungs
amyloidosis, which occurs when abnormal proteins that can cause harm
build up in your organs and tissues
Wegeners granulomatosis, which is a rare disease that causes
inflammation of the blood vessels)
polyarteritis nodosa, which is a disease in which cells attack arteries

Chronic GN
The chronic form of GN can develop over several years with no or very
few symptoms. This can cause irreversible damage to your kidneys and
ultimately lead to complete kidney failure.
A genetic disease can sometimes cause chronic GN. Hereditary nephritis
occurs in young men with poor vision and poor hearing.
Immune diseases may also cause chronic GN. A history of cancer may
also put you at risk. Having the acute form of GN may make you more
likely to develop the chronic form later on. Exposure to some hydrocarbon
solvents may increase the risk of chronic GN.
Chronic GN doesnt always have a clear cause. Twenty-five percent of
people with the condition have no history of kidney disease.
D. Medical Management
Glomerulonephritis can lead to nephrotic syndrome, where you lose large
amounts of protein in your urine. This leads to a lot of fluid and salt
retention in your body. You can develop high blood pressure, high
cholesterol, and swelling throughout your body. Treatment for high blood
pressure

Keeping your blood pressure under control is key to protecting your

kidneys. To control your high blood pressure and slow the decline in
kidney function, your doctor may prescribe one of several medications,
including:
Diuretics
Angiotensin-converting enzyme (ACE) inhibitors
Angiotensin II receptor blockers
Corticosteroids treat this condition. Eventually, nephrotic syndrome will
lead to end-stage renal disease if it doesnt come under control.
In general, the goal of treatment is to protect your kidneys from further
damage. For acute glomerulonephritis and acute kidney failure, dialysis
can help remove excess fluid and control high blood pressure.

The only long-term therapies for end-stage kidney disease are kidney
dialysis and kidney transplant. When a transplant isn't possible, often
because of poor general health, dialysis is the only option.
E. Nursing Management
Treatment of acute poststreptococcal glomerulonephritis (PSGN) is
mainly supportive, because there is no specific therapy for renal disease.
When acute glomerulonephritis (GN) is associated with chronic infections,
the underlying infections must be treated. As a nurse, you can health
teach the patient about his/her medical condition.

Salt restriction during the acute phase to control edema and volumerelated hypertension
Blood pressure monitoring at periodic intervals
Ongoing long-term monitoring of patients with persistent urinary
abnormalities and elevated blood pressure
Consideration of protein restriction and angiotensin-converting enzyme
(ACE) inhibitors (in patients who show evidence of persistent
abnormalities or in those who develop late evidence of progressive
disease)
Early antibiotic treatment of close contacts

10. RHD
A. Overview
Rheumatic (pronounced roo-MAT-ik) heart disease is a condition in which the heart
was damaged by rheumatic fever. Typically, this long-term damage occurs to the
mitral valve, aortic valve or both. This damage may cause the valve to leak or
become narrowed over time.

Usually, the symptoms of rheumatic heart disease show up 10 to 20 years after the
original illness. The mitral valve (between the left atrium and left ventricle) is usually
more affected than the aortic valve (between the left ventricle and aorta).
Rheumatic heart disease can be prevented by preventing rheumatic fever.
B. Manifestations and Diagnosis
Children with rheumatic heart disease may not have any clear symptoms.
If your child has aortic or mitral valve abnormalities due to rheumatic fever, they may
have symptoms related to these valve problems. Some symptoms that may suggest
a problem with these valves include being short of breath, particularly with activity or
when lying down.
Children with myocarditis or pericarditis may have chest pain or swelling.
Your child may also have other effects of rheumatic fever, like joint pain and
inflammation.

To diagnose this condition, your doctor will ask about any recent strep infections
(strep throat) or sore throat episodes, examine your child and use a stethoscope to
listen to their heart. In children with rheumatic heart disease, doctors can often hear a
heart murmur.
During the exam, your childs doctor will look for signs of inflammation in your childs
joints.
The doctor will ask for details about your childs symptoms, their health history and
your family health history. Your doctor may order a throat culture or a blood test to
check for strep throat or signs of a recent strep infection, as well as performing other
blood tests.
Your child will also need tests that provide information about how their heart looks
and works. These may include a chest X-ray, echocardiography, electrocardiogram or
MRI (magnetic resonance imaging) of the heart.

C. Disease Causation
Rheumatic fever is thought to result from an inflammatory autoimmune
response. Rheumatic fever only develops in children and adolescents
following group A beta-hemolytic streptococcal pharyngitis, and only
streptococcal infections of the pharynx initiate or reactivate rheumatic
fever. Medical therapy is directed at eliminating the group A streptococcal
pharyngitis (if still present), suppressing inflammation from the
autoimmune response, and providing supportive treatment for congestive
heart failure.
The treatment and prevention of group A streptococcal pharyngitis
outlined here is based on the current recommendations of the Committee
on Infectious Disease (American Academy of Pediatrics). See the
Medscape Reference article Pediatric Pharyngitis.
Penicillin V is the drug of choice for treatment of group A streptococcal
pharyngitis. Ampicillin or amoxicillin may be used instead of penicillin V
but have no microbiologic advantage. Tetracyclines and sulfonamides
should not be used to treat group
A streptococcal pharyngitis. For recurrent group A streptococcal
pharyngitis, a second 10-day course of the same antibiotic can be
repeated. Alternate drugs include narrow-spectrum cephalosporins,
amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides for
10 d. As many as 15% of patients allergic to penicillin are also allergic to
cephalosporins.
D. Medical Management
Medical therapy is directed at eliminating the group A streptococcal
pharyngitis (if still present), suppressing inflammation from the
autoimmune response, and providing supportive treatment for congestive
heart failure. The treatment and prevention of group A streptococcal
pharyngitis outlined here is based on the current recommendations of the

Committee on Infectious Disease (American Academy of Pediatrics). See

the Medscape Reference article Pediatric Pharyngitis.
Penicillin V is the drug of choice for treatment of group A streptococcal
pharyngitis. Ampicillin or amoxicillin may be used instead of penicillin V
but have no microbiologic advantage. Tetracyclines and sulfonamides
should not be used to treat group A streptococcal pharyngitis. For
recurrent group A streptococcal pharyngitis, a second 10-day course of
the same antibiotic can be repeated. Alternate drugs include narrowspectrum
cephalosporins,
amoxicillin-clavulanate,
dicloxacillin,
erythromycin, or other macrolides for 10 d. As many as 15% of patients
allergic to penicillin are also allergic to cephalosporins.
E. Nursing Management
Treatment will involve getting rid of all of the residual group A strep
bacteria and treating and controlling the symptoms. This can include any
of the following:
Antibiotics
Your doctor will prescribe antibiotics and might prescribe a long-term
treatment to prevent it from occurring again. This treatment can last up to
five years.
Anti-Inflammatory Treatment
Anti-inflammatory treatments include pain medications that are also antiinflammatory, such as aspirin or naproxen. Doctors may also prescribe a
corticosteroid to reduce inflammation.
Anticonvulsant Medications
Your doctor might prescribe an anticonvulsant if involuntary movements
become too severe.
Bed Rest
Your doctor will also recommend bed rest and restricted activities until the
major symptoms like pain and inflammation have passed. Strict bed rest
will be recommended for a few weeks to a few months if the fever has
caused heart problems.
ARTICLES:
http://vector.childrenshospital.org/2015/03/five-new-developments-in-hemophilia/
Ellis Neufeld, MD, PhD, is a hematologist at Dana-Farber/Boston Childrens Cancer
and Blood Disorders Center.
From new longer-acting drugs to promising gene therapy trials, much is changing in
the treatment of hemophilia, the inherited bleeding disorder in which the blood does

not clot. Hemophilia Awareness Month comes at a time of both progress and
remaining challenges.
1. Many more treatment products are being introduced, including some that last
longer.

People with hemophilia lack or have defects in a factora blood protein that helps
normal clots form. Of the approximately 20,000 people with hemophilia in the U.S.,
about 80 percent have hemophilia A, caused by an abnormally low level of factor VIII,
and most of the rest have hemophilia B, caused by abnormally low levels of factor IX.
Many patients with severe hemophilia give themselves prophylactic IV infusions of
the missing factor to prevent bleeding (which otherwise can lead to crippling joint
disease when blood seeps into the joint and enzymes released from blood cells
erode the cartilage).

Hemophilia factors traditionally have such a short half-life that we tend to treat
patients every other day with factor VIII and twice a week with factor IX. The first two
longer-lasting products came onto the market within the past year, and more are on
the way. So now, with factor IX, it is possible to get an infusion just once a week and
not bleed. This is really changing how we think about the disease. So far, the longeracting factor VIII products are not yet long-lasting enough to make as dramatic a
difference in the frequency of infusions. And creating really long-acting factors
remains a challenge.

2. Other new products are coming to market as factors go off patent.

The expiration of patents opens up a field that was limited to a few products as
recently as 2014. Some companies are considering making bio-similarsgeneric-like
products for complex protein moleculesfor the more expensive factors.

Meanwhile, clinicians are trying to cut through the hype that often accompanies the
introduction of new products to help patients understand whats actually happening. I
am about to lead an observational study for the American Thrombosis and
Hemostasis Network that will follow patients as they switch to the newer products and
evaluate how well the products perform in terms of safety and how well they prevent
bleeds. Were trying to take this kind of observational study out of the exclusive
hands of drug companies, which conduct proprietary studies of their particular
products, and instead collect data that cuts across brands.

3. Gene therapy is the next big thing.

Gene therapy is progressing much faster for factor IX than for factor VIII, because the
factor VIII gene is so physically large that it doesnt fit perfectly into the vector that
delivers gene therapy. In the case of factor IX, however, the vector can be delivered

through an IV infusion. It then travels directly to the liver, which is where the factor is
produced. The therapy appears to be very safe, according to early results published
in 2011 and updated in 2014. Although it doesnt work for everybody, researchers are
getting to the point where they believe they can reliably turn severe hemophilia into
moderate or mild disease. If they can really turn severe hemophilia into mild
hemophilia with one IV infusion, it would completely change the whole field of
hemophilia, making factor prophylaxis a thing of the past. The gene therapy trials are
starting with adults; therapy will be more difficult in children because the added gene
would get diluted by the growing liver.

4. New regimens require less frequent prophylactic infusions, even with less longlasting products.

Traditionally, U.S. clinicians had patients infuse themselves two or three times a week
to boost the missing factor to one percent of their blood, under the theory that this
was the threshold needed to prevent bleeds. Canadian researchers showed you can
start treating only once a week, and a number of U.S. centers are now following this
regimen. If it prevents bleeding, then the patient stays on a once-a-week regimen
even if his factor level is below one percent. If it doesnt prevent bleeding, then the
frequency of infusion is increased. We now often use this regimen with our young
children. If once a week works, a home care nurse can come in and give an IV
instead of surgically inserting a port. It also helps us learn what the patient really
needs.

5. The biggest challenge: reducing the risk of inhibitor antibodies that keep factors
from working.

If a patients body treats the factor as a foreign protein and makes an antibody that
keeps it from working, its as if he hadnt even been given a dose. We can get rid of
inhibitors in two thirds of patients who get them through Immune Tolerance Induction
by giving enough factor (daily, for months or even years) to confuse the immune
system and make it forget its a foreign protein. About 10 percent of patients,
however, are left with high-titer inhibitors that we cant overcome, which is life-altering
and can be terrible. There are hints from experiments in mice that some of the newer
factors might lower the incidence of inhibitors in people with severe hemophilia. But
mice arent people. If this does turn out to be true in humans, it would be a major
breakthrough.
Critic:
Gene therapy is the therapeutic delivery of nucleic acid polymers into a patient's cells
as a drug to treat disease.
The origins of gene therapy can be traced back to the first live attenuated vaccines in
the 1950s. Although attenuated vaccines do not alter extant human genes, viruses
are RNA polymers with their own genetic code that acts upon human cells, thus live
vaccines can be considered a primitive form of gene therapy, albeit not in the sense
that is generally implied today.

The first attempt at modifying human DNA was performed in 1980 by Martin Cline,
but the first successful and approved nuclear gene transfer in humans was performed
in May 1989. The first therapeutic use of gene transfer as well as the first direct
insertion of human DNA into the nuclear genome was performed by French Anderson
in a trial starting in September 1990.
Between 1989 and July 2015 over 2,200 clinical trials had been conducted.
It should be noted that not all medical procedures that
patient's genetic makeup can be considered gene
transplantation, and organ transplants in general have
foreign DNA into patients.[4] Gene therapy is defined
procedure and the intention of direct therapeutic effects.

introduce alterations to a
therapy. Bone marrow
been found to introduce
by the precision of the

Also according to the researcher, he said that there are new products that are coming
to market. In which many consumer with the said disease will benefit more from the
drug.
http://www.smartparenting.com.ph/news/new-primary-complex-drug-to-be-availablethis-year
Parents know all too well how difficult it is to give medicine to kids. One, some meds,
specifically antibiotics, do not taste very good. Two, they're cranky and the last thing
they want it to have a bitter taste in their mouth.
But, as parents, we do whatever it takes because it's for the best. You try to explain
that it will help them get better, take the pain and discomfort away. But they are kids,
so they'll still put up a struggle.
It's a good thing someone thought of adding flavors to medicine for kids--specifically
to drugs that treat a serious condition as tubercolosis in children.

More from Smart Parenting

How To Deal With Childrens Primary Complex

A new and improved medicine for primary complex, or childhood tuberculosis, is

expected to hit the global market this year. TB Alliance, a non-profit organization
dedicated to the discovery and development of better, faster-acting, and affordable
tuberculosis drugs, announced that the new dissolvable tablet is a fixed-dose
combination (FDC) medicine that combines sweet flavors and correct dosage. This is
the first FDC drug for kids that met the dosage guidelines set by the World Health
Organization (WHO).
The availability of correctly dosed medications will improve treatment for children
everywhere, said Dr. Mel Spigelman, president and CEO of TB Alliance, ahead of
the 46th Union World Conference on Lung Health in Cape Town, South Africa held in
December 2015. This is an important step toward ending the neglect that has
characterized the care of children with TB for far too long.
The new drug is manufactured by Indian pharmaceutical company Mcleods and was
developed through a collaboration of TB Alliance, WHO, and the United States
Agency for International Development (USAID). UNITAID, an international drug
purchasing facility overseen by the WHO, also helped the drug be available in the

market this year. While it's not technically a new drug, it is an improved version of the
drugs rifampicin, isoniazid and pyrazinamide that are currently used to treat
childhood tuberculosis
The WHO's current recommendation for treatment of primary complex spans over a
period of six months. In the first two months, a child with primary complex needs to
take the drugs rifampicin, isoniazid, and pyrazinamide. The next four months entails
continuing medication with rifampicin and isoniazid. Many parents take time to crush
tablets and struggle to properly administer treatment to kids as the drugs come in
complex dosages.
Primary complex usually have no immediate symptoms, and is only diagnosed via a
tuberculin skin test. An article on Smartparenting.com.ph explains that the most
common route of infection is when a person with active TB coughs up the germ and it
is inhaled by a healthy child. The child usually exhibits no symptoms until their
imm,une system declines and the disease becomes active.
Tuberculosis now ranks as the worlds leading infectious disease killer along with HIV.
While childhood tuberculosis is not contagious, the WHO estimates that at least one
million children get primary complex every year. It is treatable, and yet 140,000 die
from the disease because of the difficulty of providing them with the correct
treatment.
Critic:

Discovering new medications and solutions tp the major illnesses nowadays

is indeed a very good news for everyone. it does not only help the sick ones,
but also helps in many aspects of life in the society and the world. this may
help in leading to other interventions and development of new medications
and cure to diseases. having a new cure for Primary complex, which is a
problem majority to pediatrics, is a very good news for them. this may help in
their condition and help them to be free from their illness. discovering
something like this is a good sign for the world of medicine, which means that
they are advancing effectively and fast.