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HEMOPHILIA

Created by : Muhanmmad Fahryzal


FK UNISSULA 2012

DEFINITION & TERMINOLOGY


Hemophilia (heem-o-FILL-ee-ah) is a rare
bleeding disorder in which the blood doesn't
clot normally.
http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/

DEFINITION CONT.
Hemophilia is a genetic disorderusually
inheritedof the mechanism of blood
clotting. Depending on the degree of the
disorder present in an individual, excess
bleeding may occur only after specific,
predictable events (such as surgery, dental
procedures,
or
injury),
or
occur
spontaneously, with no known initiating event
http://medical-dictionary.thefreedictionary.com/hemophilia

DEFINITION CONT.
Haemophilia(/himfli/;
also
spelledhemophiliainNorth America, from
theGreekhaima 'blood' andphilia
'love'[1]) is a group ofhereditarygenetic
disordersthat impair the body's ability to
controlbloodclotting orcoagulation, which
is used to stop bleeding when ablood
vesselis broken.
https://en.wikipedia.org/wiki/Haemophilia

CAUSE & EFFECT

f you have inherited hemophilia, you're born


with the disorder. It's caused by a defect in
one of the genes that determine how the
body makes blood clotting factor VIII or IX.
These genes are located on the X
chromosomes (KRO-muh-somz).
Chromosomes come in pairs. Females have
two X chromosomes, while males have one X
and one Y chromosome. Only the X
chromosome carries the genes related to
clotting factors.

CLASSIFICATION

HEMOPHILIA A
Haemophilia Ais a recessive X-linked genetic
disorder involving a lack of functional
clotting Factor VIII and represents 80% of
haemophilia cases.

HEMOPHILIA B
Haemophilia Bis a recessive X-linked genetic
disorder involving a lack of functional
clotting
Factor
IX.
It
comprises
approximately 20% of haemophilia cases

HEMOPHILIA C
Haemophilia
Cis
anautosomalgenetic
disorder (i.e.notX-linked) involving a lack of
functional clottingFactor XI. Haemophilia C
is
not
completely
recessive,
asheterozygousindividuals
also
show
increased bleeding.

SIGN & SYMPTHOM

EXCESSIVE BLEEDING
Signs of external bleeding may include:
Bleeding in the mouth from a cut or bite or from
cutting or losing a tooth
Nosebleeds for no obvious reason
Heavy bleeding from a minor cut
Bleeding from a cut that resumes after stopping
for a short time

CONT.
Signs of internal bleeding may include:
Blood in the urine (from bleeding in the kidneys
or bladder)
Blood in the stool (from bleeding in the
intestines or stomach)
Large bruises (from bleeding into the large
muscles of the body)

BLEEDING IN THE JOINTS


Bleeding in the knees, elbows, or other joints
is another common form of internal bleeding
in people who have hemophilia. This
bleeding can occur without obvious injury.

BLEEDING IN THE BRAIN

Long-lasting, painful headaches or neck pain


or stiffness
Repeated vomiting
Sleepiness or changes in behavior
Sudden weakness or clumsiness of the arms
or legs or problems walking
Double vision
Convulsions or seizures

PATHOPHYSIOLOGY

First, the blood vessel constricts to limit the


volume of blood that is lost.
Second, circulating platelets form a plug at
the site of injury.
Finally, the blood undergoes coagulation.

TREATMENT

You or your child can get quicker treatment


when bleeding happens. Early treatment
lowers the risk of complications.
Fewer visits to the doctor or emergency
room are needed.
Home treatment costs less than treatment in
a medical care setting.
Home treatment helps children accept
treatment and take responsibility for their
own health.

PREVENTION & EDUCATION

People who have hemophilia should avoid


situations that might cause bleeding. They
should be careful about dental care so they
won't need to have teeth extracted. People who
have hemophilia should also avoid certain drugs
that can aggravate bleeding problems:
Aspirin
Heparin
Warfarin

Certain analgesics, such as nonsteroidal antiinflammatory drugs (eg, Tylenol and Ibuprofen )

CONT.

Genetic counselors can make a detailed


family history. They will discuss risks and
available testing options.

PUSTAKA LIST

"History of Bleeding Disorders." What Are Bleeding


Disorders?. 2006. National Hemophilia Foundation. 17 Dec
2008
Biggs R, Douglas AS, MacFarlane RG, Dacie JV, Pitney WR,
Merskey C, O'Brien JR (1952)."Christmas disease: a
condition previously mistaken for haemophilia".Br Med
J2(4799): 1378
82.doi:10.1136/bmj.2.4799.1378.PMC2022306.PMID1299
7790.
^Michael Price (8 October 2009)."Case Closed: Famous
Royals Suffered From Hemophilia".ScienceNOW Daily News.
AAAS. Retrieved 9 October 2009.
^Evgeny I. Rogaev et al. (8 October 2009)."Genotype
Analysis Identifies the Cause of the "Royal Disease"".
Science. Retrieved 9 October 2009.

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