All issues > Volume 31(2); 1988

Original Article

J Korean Pediatr Soc. 1988;31(2):253-257. Published online February 28, 1988.

A Case of Acute Hepatic Porphyria.

Young Cheal Han¹, Sin Hang Joo¹, Jin Han Kang¹, Byung Churl Lee¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

Received: July 16, 1987;

Abstract

Acute hepatic porphyria may be an inborn error of metabolism characterized biochemically by the excessive hapatic over-production and urinary excretion of porphyrin precursors and clinically by episode of acute neuro-visceral dysfunction. Acute porphyria is very rare in childhood. We experienced a case of acute hepatic porphyria in childhood age, confirmed by characterized clinical features of neuro-visceral dysfunction and biochemical findings in blood and urine (Watson- Schwartz test). A brief review of related literature also is presented.

Keywords :Acute hepatic porphyria