Hemophilia

one of a group of inherited bleeding disorders that cause

abnormal or exaggerated bleeding and poor blood clotting.
Hemophilia A and B are inherited in an X-linked recessive
genetic pattern and are therefore much more common in
males.

This pattern of inheritance means that a given gene on the

X chromosome expresses itself only when there is no normal
gene present.
Hemophilia A is caused by a mutation in the gene for factor
VIII, so there is deficiency of this clotting factor. Hemophilia
B (also called Christmas disease) results from a deficiency
of factor IX due to a mutation in the corresponding gene.

A subgroup of hemophilia B :Leyden phenotype, which is

characterized by a severe hemophilia in childhood that
improves at puberty.

A condition referred to as hemophilia C involves a

deficiency of clotting factor XI. It is also not inherited in an Xlinked manner and affects persons of both sexes.

Hemophilia has been called the Royal Disease because

Queen Victoria, Queen of England from 1837 to 1901, was a
carrier.

Hemarthrosis (bleeding into the joints) is

characteristic of hemophilia. The knees and ankles are
most often affected. The bleeding causes distension of
the joint spaces, significant pain, and over time, can
be disfiguring. Over time, joint destruction occurs, and
joint replacement surgeries can be required.
Bleeding into the muscles may occur
with hematoma formation (compartment syndrome).
Bleeding from the mouth or nosebleeds may occur.
Bleeding after dental procedures is common, and
oozing of blood from the gums may occur in young
children when new teeth are erupting.
Bleeding from the gastrointestinal tract can lead
to blood in the stool.(melena)
Bleeding from the urinary tract can lead to blood in
the urine(hematuria).
Intracranial hemorrhage (bleeding into the brain or
skull) can lead to symptoms such as nausea, vomiting,
and/or lethargy.
Increased bleeding after surgery or trauma is
characteristic of hemophilia.

Diagnose:

Hepatitis A
is a virus, or infection, that causes liver disease and
inflammation of the liver. Viruses can cause sickness.
The liver

removes harmful chemicals from your blood

fights infection

helps digest food

stores nutrients and vitamins

stores energy

Mode of transmission: hepatitis A through contact with an

infected person's stool. This contact could occur by

family history
series of blood tests can identify which part or
protein factor of the blood clotting mechanism is
defective if an individual has abnormal bleeding
episodes
the prothrombin time (PT) and activated partial
thromboplastin time (aPTT)

causes haemophilia:
Treatment:
caused by a genetic mutation. The mutations involve genes
that code for proteins that are essential in the blood clotting
process. The bleeding symptoms arise because blood
clotting is impaired.
The process of blood clotting involves a series of complex
mechanisms, usually involving 13 different proteins
classically termed I through XIII and written with Roman
numerals. If the lining of the blood vessels becomes
damaged, platelets are recruited to the injured area to form
an initial plug. These activated platelets release chemicals
that start the clotting cascade, activating a series of 13
proteins known as clotting factors. Ultimately, fibrin is
formed, the protein that crosslinks with itself to form a mesh
that makes up the final blood clot. The protein involved with
hemophilia A is factor VIII (factor 8) and with hemophilia B is
factor IX (factor 9).
Specific sites and types of bleeding are discussed below.

Replacement therapy is replacement of the blood

clotting factors. It is infusing the clotting factor
concentrates into a vein, much like a blood
transfusion.
mild cases of hemophilia A are sometimes treated
with the drug desmopressin, also known as
DDAVP. This drug stimulates release of more
clotting factor by the body. It is administered either
slowly through the intravenous route (IV) or
occasionally, in nasal spray form.
Acetaminophen (Tylenol and others) is often
given for pain relief.

eating food made by an infected person who didn't

wash his or her hands after using the bathroom
drinking untreated water or eating food washed in
untreated water
placing a finger or object in your mouth that came into
contact with an infected person's stool
having close personal contact with an infected person,
such as through sex or caring for someone who is ill

You cannot get hepatitis A from

being coughed or sneezed on by an infected person

sitting next to an infected person

hugging an infected person

A baby cannot get hepatitis A from breast milk.

Manifestation:

feeling tired

muscle soreness

upset stomach

fever

loss of appetite

stomach pain

diarrhea

dark-yellow urine

light-colored stools

yellowish eyes and skin, called jaundice

Symptoms of hepatitis A can occur 2 to 7 weeks after

coming into contact with the virus. Children younger than
age 6 may have no symptoms. Older children and adults
often get mild, flulike symptom

Diagnosed:

A blood test will show if you have hepatitis A

Treatment:
Hepatitis A usually gets better in a few weeks without
treatment. However, some people can have symptoms for
up to 6 months.
The hepatitis A vaccine is given in two shots. The second
shot is given 6 to 12 months after the first shot.

always wash your hands with warm, soapy water after

using the toilet or changing diapers and before fixing
food or eating
use bottled water for drinking, making ice cubes, and
washing fruits and vegetables when you are in a
developing country

tell your doctor and your dentist if you have hepatitis A

Avoid drinking alcohol, which can harm the liver

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