Definition:
Hemorrhagiparous thrombocytic dystrophy is commonly called Bernard-Soulier syndrome named after Dr. Jean Bernard and Jean Pierre Soulier. It is a severe bleeding disorder caused by a deficiency of glycoprotein Ib (GpIb), which is important in clot formation.
Diagnosis:
Physicians must be aware of the patient's medical history particularly about bleeding and bleeding illness in the family. The patient undergoes a physical exam and laboratory tests, and examined for signs of bleeding. The disorder is usually confirmed in a specialist laboratory with a specialization in this illness.
Treatment:
Patients should be aware of certain medications to avoid such as aspirin, which can interfere with platelet function. Dental hygiene is also a vital issue. Heavy periods during a woman's menstruation cycle can result in low iron, which must be treated. Severe bleeding episodes usually require blood or platelets transfusion.
Symptoms and Signs:
The patient suffers from a prolonged bleeding time, and has a low platelet count and large platelets.
Causes:
The person experiences prolonged bleeding due to the absence of the glycoprotein Ib-IX-V complex that causes platelets to stick. A child may also inherit the abnormal gene that causes this syndrome from each parent. However, the disease is rarely inherited from just one parent who carries the abnormal gene.
:
hemorrhagiparousthrombocyticdystrophy
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