ترومبوسيتوپني ترومبوتيك مادرزادي در پسري نه ساله با شش سال تاخير تشخيصي

فتحی, افشین and زارع نوقابی, جواد and صالح زاده, فرهاد (1394) ترومبوسيتوپني ترومبوتيك مادرزادي در پسري نه ساله با شش سال تاخير تشخيصي. The Southeast Asian Journal of Case Report and Review ــ 4 (3). pp. 1804-1810. شاپا 1090-2319


Official URL: http://www.scopemed.org/?sec=j_index&jid=83&lng


Congenital Thrombotic Thrombocytopenic Purpura (CTTP) in Nine years old boy with Six years delayed in diagnosis

English Abstract

Congenital thrombotic thrombocytopenic purpura (CTTP) is a rare disease due to mutations in genes that reduce the production of ADAMTS 13 (Metalloproteinease). Metalloproteinease deficiency causes large multimeres of Von Willebrand factor VWF not going to broken and result in anemia and thrombocytopenia (Microangiopatic hemolytic anemia) with renal involvement. The case is a 9 years old boy who suffers from anemia, thrombocytopenia since age three with some courses of hematuria, BUN and creatinine rising period. He had different diagnosis such as ITP (Immune thrombocytopenic purpura), Fanconi anemia, autoimmune hemolytic anemia, Evans syndrome and has been taken various immunosuppressive therapy and frequent blood transfusion. After 6 years following HUS like crisis and due to congenital deficiency of ADAMs13 and normal antibody against it he was diagnosed as a CTTP and treated with regular injections FFP .

Item Type:Article
زبان سند : انگلیسی
نویسنده اول :افشین فتحی
نویسنده مسئول :جواد زارع نوقابی
نویسنده :فرهاد صالح زاده
Additional Information:Indexed in: Google Scholar, Index Copernicus, OAJI
کلیدواژه ها (انگلیسی):ADAMTS13, congenital Thrombotic thrombocytopenic purpura, thrombocytopenia
Subjects:WS Pediatrics
Divisions:Faculty of Medicine > Department of Pediatrics , Community Medicine
ID Code:6890
Deposited By: Ms Zeynab Imani
Deposited On:05 Jun 1394 07:46
Last Modified:29 Sep 1394 10:59

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