Bernard-Soulier syndrome

Bernard-Soulier syndrome
(BSS) an autosomal recessive disorder characterized by giant platelets with membranes lacking glycoprotein Ib, the probable receptor for plasma von Willebrand factor; this keeps the platelets from binding the factor, which is necessary for their adhesion to the subendothelial surfaces of blood vessels. Symptoms include mild to moderate mucocutaneous and visceral hemorrhaging, purpura, and prolonged bleeding time. Called also giant platelet s. See also thrombasthenia and von Willebrand disease.

Medical dictionary. 2011.

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  • Bernard-Soulier syndrome — Bernard Soulier syndrome. См. синдром гигантских тромбоцитов. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • Bernard-Soulier syndrome — Infobox Disease Name = Bernard Soulier syndrome Caption = DiseasesDB = 1356 ICD10 = ICD10|D|69|1|d|65 ICD9 = ICD9|287.1 ICDO = OMIM = 231200 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 230 MeshID = D001606 Bernard Soulier syndrome (BSS),… …   Wikipedia

  • Bernard-Soulier syndrome (giant platelet syndrome) — The Bernard Soulier syndrome is a primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls. This is a crucial aspect of the process of forming a blood clot, and as a result of this… …   Medical dictionary

  • Syndrome, giant platelet (Bernard-Soulier syndrome) — This condition is a primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls and as a result of this problem there is abnormal bleeding. The giant platelet syndrome usually presents in …   Medical dictionary

  • Bernard-Soulier syndrome — Ber·nard Sou·lier syndrome (bār nahrґ s l yaґ) [Jean Alfred Bernard, French hematologist, 1907–2006; Jean Pierre Soulier, French hematologist, 1915–2003] see under syndrome …   Medical dictionary

  • Giant platelet syndrome (Bernard-Soulier syndrome) — This condition is a primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls and as a result of this problem there is abnormal bleeding. The giant platelet syndrome usually presents in …   Medical dictionary

  • Bernard-Soulier syndrome — Genetic deficiency in platelet membrane glycoprotein Ib (CD42) ; platelets aggregate normally (cf Glanzmann s thrombasthenia) but do not stick to collagen of sub endothelial basement membrane …   Dictionary of molecular biology

  • Bernard-Soulier-Syndrom — Klassifikation nach ICD 10 D69.1 Thrombozytopathie …   Deutsch Wikipedia

  • Syndrome, Bernard-Soulier (giant platelet syndrome) — This is a primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls. As a result of this problem there is abnormal bleeding. Bernard Soulier syndrome usually presents in the newborn… …   Medical dictionary

  • Síndrome de Bernard-Soulier — Clasificación y recursos externos CIE 10 D69.1 CIE 9 287.1 …   Wikipedia Español

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