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KMID : 0191119990140040451
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Journal of Korean Medical Science
1999 Volume.14 No. 4 p.451 ~ p.454
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Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease
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Huh J
Noh CI/Choi JY/Yun YS/Choi Y/Seo JK
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Abstract
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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.
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KEYWORD
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