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호흡부전과 신경학적 이상을 동반한 Acute Intermittent Porphyria
전대원(Dae Won Jun),최춘식(Choon Shik Choi),최호순(Ho Soon Choi),전용철(Yong Chul Jung),손주현(Joo Hyun Sohn),한동수(Dong Soo Han),윤병철(Byung Chul Yoon),함준수(Joon Soo Hahm),이민호(Min Ho Lee),이동후(Dong Hoo Lee),기춘석(Choon Shu 대한소화기학회 2000 대한소화기학회지 Vol.36 No.3
The acute intermittent porphyria (AIP) is characterized biochemically by increased excretion of porphyrins and porphyrin precursors, S-aminolevulinic acid (ALA) and porphobilinogen (PBG). AIP presents with abdominal pain, mental disturbances and neurologic dysfunction. A 24-year-old woman with a medical history of AIP was admitted to our hospital because of abdominal pain and proximal muscle weakness. She was treated by carbohydrate loading with intravenous glucose. Twenty five days later, she developed tachycardia, fluctuation of blood pressure and syndrome of inappropriate secretion of antidiuretic hormore (SIADH). On 90th day, intubation was required to control her breathing. Complete flaccid paralysis developed, and the patient became comatose. The neurologic effects of acute porphyria are generally reversible, but the clinical course of severe case is complicated with respiratory failure, fluid electrolyte imbalance. AIP should be considered in differential diagnosis of patient with unexplained respiratory failure. (Kor J Gastroenterol 2000;36:413 - 418)