| A Case of Acute Hepatic Porphyria. |
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Young Cheal Han, Sin Hang Joo, Jin Han Kang, Byung Churl Lee |
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Department of Pediatrics, Catholic University Medical College, Seoul, Korea |
| 소아의 급성 간성 포르피리아 1례 |
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한영철, 주신행, 강진한, 이병철 |
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가톨릭의대 의학부 소아과학교실 |
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Received: 16 July 1987 |
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| Abstract |
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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. |
| Key Words:
Acute hepatic porphyria |
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