title: Pleuropulmonary blastoma in a child with autosomal-recessive polycystic kidney disease.
abstract: Autosomal-recessive polycystic kidney disease (ARPKD) is a developmental disorder known to affect both the kidneys and the liver. Renal involvement results in progressive renal insufficiency and hypertension, while hepatic involvement can result in portal hypertension and cholangitis. Pulmonary abnormalities relate mainly to pulmonary insufficiency in those patients who present as neonates. We present a unique case of a child with ARPKD found to have a cystic lesion of the lung. Upon surgical resection, a pathological diagnosis of pleuropulmonary blastoma (PPB) was made. There are no previous reports in the literature describing the association of these two entities. Knowledge of this potential association is important in the clinical management of these children and may open new avenues of genetic research.
authors: Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Canada. jeffrey.traubici@sickkids.ca
authors: Jeffrey
authors: J
authors: Traubici
authors: Gino R
authors: GR
authors: Somers
authors: Simon C
authors: SC
authors: Ling
authors: Rachel J
authors: RJ
authors: Pearl
authors: Paul C
authors: PC
authors: Nathan
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications*; D018202:Pulmonary Blastoma / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed