Congenital hepatic fibrosis: a very uncommon cause of pancytopenia in children |
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Authors: | Trizzino Antonino Farruggia Piero Russo Delia D'Angelo Paolo Tropia Serena Benigno Vincenzo Tarantino Giuseppe Di Marco Vito Aricò Maurizio |
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Institution: | Onco Ematologia Pediatrica, Ospedale dei Bambini G. Di Cristina, Palermo, Italy. triznino@hotmail.com |
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Abstract: | The disease presentation of autosomal recessive polycystic kidney disease (OMIM #263200, ARPKD) is highly variable and includes polycystic kidneys, pulmonary hypoplasia, and congenital hepatic fibrosis. The authors report an unusual case of ARPKD presenting with hepatosplenomegaly and cytopenia mimicking acute leukemia. |
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