Severe liver disease in early childhood due to fibrinogen storage and de novo gamma375Arg-->Trp gene mutation.

Francalanci, Paola; Santorelli, Filippo M; Talini, Ilaria; Boldrini, Renata; Devito, Rita; Camassei, Francesca Diomedi; Maggiore, Giuseppe; Callea, Francesco · J Pediatr · 2006

case_report · Level V

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Abstract

We report hypofibrinogenemia and massive hepatic storage of fibrinogen in a child with cryptogenic chronic liver disease. Fibrinogen gene analysis revealed a de novo Aguadilla (c.1201C>T; p.Arg375Trp) mutation. This mutation should be considered in childhood hypofibrinogenemia associated with chronic liver disease.

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