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6533b838fe1ef96bd12a3e34

RESEARCH PRODUCT

Congenital Hepatic Fibrosis

Giuseppe TarantinoV BenignoSerena TropiaDelia RussoPiero FarruggiaPaolo D'angeloAntonino TrizzinoAricò MaurizioVito Di Marco

subject

Liver CirrhosisMalePathologymedicine.medical_specialtyAdolescentPancytopeniaHepatosplenomegalyurologic and male genital diseasesPulmonary hypoplasiahemic and lymphatic diseasesmedicineHumanscytopeniaPolycystic Kidney Autosomal RecessivesplenomegalyCytopeniaAcute leukemiapolycystic kidney diseasebusiness.industryHematologymedicine.diseasePancytopeniaeye diseasesfemale genital diseases and pregnancy complicationsAutosomal Recessive Polycystic Kidney DiseaseOncologyDisease PresentationPediatrics Perinatology and Child HealthCongenital hepatic fibrosismedicine.symptomTomography X-Ray ComputedbusinessHepatomegaly

description

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.

yearjournalcountryeditionlanguage
2005-10-12Journal of Pediatric Hematology/Oncology
https://doi.org/10.1097/01.mph.0000184577.46458.7e
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