J Korean Med Sci.  1999 Aug;14(4):451-454. 10.3346/jkms.1999.14.4.451.

Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease

• Huh J
• Noh CI
• Choi JY
• Yun YS
• Choi Y
• Seo JK

• KMID: 1731322
• DOI: http://doi.org/10.3346/jkms.1999.14.4.451

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.

Keyword

Hypertension, pulmonary; Kidney, polycystic, autosomal recessive; Liver cirrhosis

MeSH Terms

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*