Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease.
10.3346/jkms.1999.14.4.451
- Author:
June HUH
1
;
Chung Il NOH
;
Jung Yun CHOI
;
Yong Soo YUN
;
Yong CHOI
;
Jeong Kee SEO
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Korea.
- Publication Type:Case Report
- Keywords:
Hypertension, pulmonary;
Kidney, polycystic, autosomal recessive;
Liver cirrhosis
- MeSH:
Biopsy;
Case Report;
Child;
Echocardiography, Doppler;
Fatal Outcome;
Female;
Human;
Hypertension, Pulmonary/ultrasonography;
Hypertension, Pulmonary/complications*;
Liver Cirrhosis/pathology;
Liver Cirrhosis/complications;
Polycystic Kidney, Autosomal Recessive/ultrasonography;
Polycystic Kidney, Autosomal Recessive/complications*
- From:Journal of Korean Medical Science
1999;14(4):451-454
- CountryRepublic of Korea
- Language:English
-
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.
