Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease.
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.
Huh
J
J
Department of Pediatrics, Seoul National University College of Medicine, Korea.
Noh
C I
CI
Choi
J Y
JY
Yun
Y S
YS
Choi
Y
Y
Seo
J K
JK
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