반복되는 복통과 고혈압을 동반한 급성 간헐성 포르피린증 1예
Acute Intermittent Porphyria Presented with Recurrent Abdominal Pain and Hypertension
박미란, 서정기, 고재성, 장주영, 양혜란,
소속 상세정보
박미란 ( Park Mi-Ran )
서울대학교 의과대학 소아과학교실
서정기 ( Seo Jeong-Kee )
서울대학교 의과대학 소아과학교실
고재성 ( Ko Jae-Sung )
서울대학교 의과대학 소아과학교실
장주영 ( Chang Ju-Young )
서울대학교 의과대학 소아과학교실
양혜란 ( Yang Hye-Ran )
서울대학교 의과대학 소아과학교실
KMID : 0816120110140010081
Abstract
Acute intermittent porphyria (AIP) is a rare disorder characterized biochemically by the increased excretion of porphyrins and porphyrin precursors, including delta-aminolevulinic acid (ALA) and porphobilinogen (PBG). AIP has variable clinical manifestations, such as acute abdominal pain, vomiting, nausea, constipation, peripheral neuropathy, seizures, tachycardia, and hypertension. A 16-year-old girl presented with recurrent abdominal pain, vomiting, hypertension, seizures, hypercholesterolemia, and red urine. AIP was confirmed by clinical features and increased 24-hour urine ALA and PBG. AIP should be considered in the differential diagnosis of patients who have abdominal pain, hypertension, and seizures when the results of all other tests are normal.
키워드
Acute intermittent porphyria; Recurrent abdominal pain; Hypertension; Hypercholesterolemia; Red urine
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