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KMID : 0366219750100010063
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Korean Journal of Hematology
1975 Volume.10 No. 1 p.63 ~ p.67
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A Case Presentation of Acute Intermittent Porphyria
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박경남(朴炅南)/Kyung Nam Park
조성운(趙誠雲)/이방헌(李邦憲)/김명재(金明宰)/이정균(李禎均)/김종설(金鐘卨)/Sung Wun Cho/Bang Hun Lee/Myung Jae Kim/Chung Kyun Lee/Chong Suhl Kim.
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Abstract
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Acute intermittent porphyria is characterized biochemically by hepatic overproduction
of porphyrin precursors (ALA and PBG) and clinically by acute visceral and neurological
dysfunction. The authors experienced a case of acute intermittent porphyria and
presented briefly with review of literatures.
A 30-year-old Korean woman was admitted to the Department of General Surgery,
with chief complaints of severe abdominal pain, nausea and vomiting of 9 days'
duration. On the 5th hospital day tingling sensation on both extremities was noticed and
on the 6th hospital day she was transferred to the Department of Internal Medicine
because of three occasions of convulsive seizure following neurotic complaints.
The acute attacks were accompained with hypertension. The Watson-Schwars test
was strongly positive. Liver function tests were within normal limits except slightly
increased B.S.P.retention.
Both demerol (50㎎) and chloropromazine(50㎎) showed of little beneficial effect, while
chloral hydrate (10㎎) per rectum could affect rapid relief of acute symptoms. The
therapy with gluose infusion as well as rectal administration of chloral hydrate yield the
correction of electrolyte disturbance, improvement of gastrointestinal symptoms and
remarkable amelioration of psychotic manifestations.
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