Inherited ADAMTS13 deficiency: unique presentation and treatment.

Inherited ADAMTS13 deficiency: unique presentation and treatment. Research Abstract Details 

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Inherited ADAMTS13 deficiency: unique presentation and treatment. Abstract Text:

Hayley Born,Antoinette Peters,Robert Ettinger,

A 3-year-old male presented with severe thrombocytopenia and microangiopathic hemolytic anemia in conjunction with severe bilateral otitis media. After laboratory analysis, a diagnosis of inherited ADAMTS13 deficiency was proven. Rather than treating with prophylactic fresh frozen plasma, to date the patient has been successfully treated with single-donor, directed plasma infusions in response to early signs of relapse. It may be reasonable to consider observational and reactive care rather than prophylactic care in some cases of inherited ADAMTS13 deficiency.

Inherited ADAMTS13 deficiency: unique presentation and treatment. Publishing Authors By Initials

H Born,A Peters,R Ettinger,

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Inherited ADAMTS13 deficiency: unique presentation and treatment. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Pediatric blood & cancer

VOLUME: 50

Page Numbers: 956-7

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ISSN: 1545-5017

DAY: 2

MONTH: May

YEAR: 2008

Inherited ADAMTS13 deficiency: unique presentation and treatment. Information

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LANGUAGE: eng

NlmUniqueID: 101186624

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Grant and Affiliation Information for Inherited ADAMTS13 deficiency: unique presentation and treatment.

AFFILIATION: Department of Pediatric Hematology/Oncology, Gundersen Lutheran Health System, La Crosse, Wisconsin, USA.

Country: United States

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MEDLINETA: Pediatr Blood Cancer

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