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Pediatric Hematology and Oncology, 2005 Gastrointestinal angiodysplasia in association with Bernard-Soulier syndrome has been previously described in adults. The authors report on a 14-year-old boy presenting with massive upper gastrointestinal bleeding due to a large gastric angiodysplasia ...
Zühre Kaya, Deniz Aslan
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Bernard–Soulier syndrome in pregnancy
International Journal of Laboratory Hematology, 2006Bernard-Soulier syndrome (BSS) is a rare autosomal recessively inherited bleeding disorder. Pregnancy in patients with BSS is characterized by ante-, intra-, or postpartum haemorrhage, which may be delayed and severe. There is no consensus in the management of BSS in pregnancy and so far only 16 pregnancies in nine patients have been described.
L A Parapia
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Blood, 1998
IN 1948, BERNARD AND SOULIER described a young male patient with a severe bleeding disorder that was characterized by a prolonged bleeding time, thrombocytopenia, and extremely large platelets.[1][1] They termed the disorder “la dystrophie thrombocytaire-hemorragipare congenitale.” Since ...
J A, López +3 more
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IN 1948, BERNARD AND SOULIER described a young male patient with a severe bleeding disorder that was characterized by a prolonged bleeding time, thrombocytopenia, and extremely large platelets.[1][1] They termed the disorder “la dystrophie thrombocytaire-hemorragipare congenitale.” Since ...
J A, López +3 more
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2018
Bernard-Soulier syndrome (BSS) is a rare mostly autosomal recessive platelet function disorder that is characterized by variable bleeding tendency, macrothrombocytopenia, impaired platelet agglutination in response to ristocetin, and defect in the glycoprotein (GP) Ib-IX-V complex.
Bahare Ghasemi, Akbar Dorgalaleh
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Bernard-Soulier syndrome (BSS) is a rare mostly autosomal recessive platelet function disorder that is characterized by variable bleeding tendency, macrothrombocytopenia, impaired platelet agglutination in response to ristocetin, and defect in the glycoprotein (GP) Ib-IX-V complex.
Bahare Ghasemi, Akbar Dorgalaleh
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BERNARD SOULIER SYNDROME IN PREGNANCY
GLOBAL JOURNAL FOR RESEARCH ANALYSIS, 2021Bernard-Soulier syndrome is an inherited platelet disorder, transmitted in an autosomal recessive pattern. Thrombocytopenia and large defective platelets are characteristics, often presents early with bleeding symptoms, such as epistaxis, ecchymosis, menometrorrhagia, and gingival or gastrointestinal bleeding.
Malini Sukayogula +2 more
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Trends in Cardiovascular Medicine, 1997
The Bernard-Soulier syndrome (BSS) is a rare inherited disorder of platelet function due to absence or markedly decreased expression of the platelet GpIb-V-IX receptor. This receptor plays a critical role in hemostasis by initiating platelet adhesion and subsequent activation at the site of vessel injury.
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The Bernard-Soulier syndrome (BSS) is a rare inherited disorder of platelet function due to absence or markedly decreased expression of the platelet GpIb-V-IX receptor. This receptor plays a critical role in hemostasis by initiating platelet adhesion and subsequent activation at the site of vessel injury.
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Bernard Soulier syndrome in pregnancy: a systematic review
Haemophilia, 2010Summary. Bernard Soulier syndrome (BSS) is a rare disorder of platelets, inherited mainly as an autosomal recessive trait. It is characterised by qualitative and quantitative defects of the platelet membrane glycoprotein (GP) Ib‐IX‐V complex. The main clinical characteristics are thrombocytopenia, prolonged bleeding time and the presence of giant ...
Edward Tuddenham, Rezan A Kadir
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1999
Das Bernard-Soulier-Syndrom (BSS) ist eine seltene, autosomal rezessiv vererbte Thrombozyto-pathie und durch das Vorkommen von morphologisch veranderten, ungewohnlich grosen, sogenannten „Riesenplattchen“ bei gleichzeitig bestehender Thrombozytopenie charakterisiert. Betroffene Patienten leiden unter einer lebenslangen Blutungsneigung.
K. J. Clemetson, R. E. Scharf
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Das Bernard-Soulier-Syndrom (BSS) ist eine seltene, autosomal rezessiv vererbte Thrombozyto-pathie und durch das Vorkommen von morphologisch veranderten, ungewohnlich grosen, sogenannten „Riesenplattchen“ bei gleichzeitig bestehender Thrombozytopenie charakterisiert. Betroffene Patienten leiden unter einer lebenslangen Blutungsneigung.
K. J. Clemetson, R. E. Scharf
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Successful pregnancy outcome in Bernard–Soulier syndrome
Journal of Obstetrics and Gynaecology Research, 2005AbstractBernard–Soulier syndrome is a rare bleeding disorder, and there is scant literature on the pregnancy outcome in women with this syndrome. Due to the bleeding tendency in this disease, pregnant woman may have ante‐, intra‐ or postpartum complications.
Alka Kriplani
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Obstetric complications in a patient with Bernard-Soulier syndrome
American Journal of Obstetrics and Gynecology, 1991We describe the obstetric complications and management of a patient with Bernard-Soulier syndrome. Severe bleeding at the time of delivery and delayed postpartum hemorrhage were prominent features of her pregnancies. Further complicating this woman's pregnancies was the development of antibodies to platelet glycoprotein IB/IX, leading to neonatal ...
Thomas S Kickler +2 more
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