title: Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease.
abstract: An 11 year-old girl, whose condition was diagnosed as juvenile-type autosomal recessive polycystic kidney disease (ARPKD) at five years of age, presented with chest pain and dyspnea that had developed suddenly two months previously. Two-dimensional echocardiography, Doppler study and cardiac catheterization confirmed pulmonary hypertension. The underlying mechanism of the diagnosis was not defined. Two and a half months after the onset of symptoms, the patient died of pulmonary hypertensive crisis. Careful regular checks of cardiopulmonary status using two-dimensional echocardiography and Doppler should be considered for the early detection of pulmonary hypertension even in an asymptomatic patient with juvenile-type ARPKD.
authors.affiliation: Department of Pediatrics, Seoul National University College of Medicine, Korea.
authors.forename: J
authors.initials: J
authors.lastname: Huh
authors.forename: C I
authors.initials: CI
authors.lastname: Noh
authors.forename: J Y
authors.initials: JY
authors.lastname: Choi
authors.forename: Y S
authors.initials: YS
authors.lastname: Yun
authors.forename: Y
authors.initials: Y
authors.lastname: Choi
authors.forename: J K
authors.initials: JK
authors.lastname: Seo
mesh_terms: D001706:Biopsy; D002648:Child; D015150:Echocardiography, Doppler; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging; D008103:Liver Cirrhosis / Q000150:complications / Q000473:pathology; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications* / Q000000981:diagnostic imaging