Hemophilia

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Is Hemophilia ? Present Concepts of Hemophilia

By ALFREDO PAVLOVSKY, M.D. I F WE CONSIDER the classical definitions of hemophilia, which is described as an “hemorrhagic hereditary disease, of recessive character, linked to sex, surifered by males and transmitted by females, distinguished by a delayed (‘lottinig time arid with a characteristic hemorrhagic clinical picture,” we must admit that -some of these concepts has-c recently required m...

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Hemophilia ? Hemophilia : Specific Entity or Syndrome ?

TUI)IES OF HEMOPHILIA have, ins the past-, helped greatly to solve the mansy intricacies of the blood coagulation mechanism. More recently, howeser, they have also altered our s-cry (‘onscept of the disease arid stnggested that, ins-stead of a svehl defined entity, hemophilia ins fact represents a syndrome siit.h many different pathogensetic mechanisms. The questions “ i’hat. is hemophihia?” is...

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Hemophilia Pseudotumor.

Hemophilic pseudotumor or “blood cysts”, described by Starker1 are a rare complication of Hemophilia, observed in only 1-2% of patients with severe disease,2 consisting of extravasated clotted blood surrounded by a fibrous capsule. It affects bones, lungs, and abdomen and stomach wall. Morbidity occurs by compression on surrounding structures including bone destruction, muscle and skin necrosis...

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Recent Advances in Hemophilia Gene Therapy

Heterogeneous loss of function mutations at F8 and F9 genes causes X-linked recessive bleeding disorders, hemophilia A (HA) and hemophilia B (HB), respectively. HA is clinically indistinguishable from HB and accounts for more than 80% of hemophilia cases; the former affects 1/5000 and the latter 1/25000 male births worldwide. In Iran, it is estimated that around 4300 HA and 900 HB patients are ...

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A study was made of the clotting defect and the course of the malady in a group of male dogs with an inherited, sex-linked bleeding disease. The clotting defect is characterized by a prolonged clotting time and a delayed prothrombin utilization, and is corrected by the addition either of thromboplastin or of normal plasma. A plasma protein fraction, fraction I, also corrects the defect. The def...

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ژورنال

عنوان ژورنال: The Boston Medical and Surgical Journal

سال: 1897

ISSN: 0096-6762,1533-4406

DOI: 10.1056/nejm189703111361002