KMID : 0374920040250010133
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Inje Medical Journal 2004 Volume.25 No. 1 p.133 ~ p.139
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Acute intermittent porphyria presenting paroxysmal hypertension
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Shin Jeong-Ah
Lee Byoung-Kwon Byun Young-Sup Lee Ji-Seon Goh Chong-Won Kim Byung-Ok Lee Kun-Joo
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Abstract
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Acute intermittent porphyria is a disorder that results from partial porphobilinogen deaminase deficiency and accumulation of overproducted porphyrin and its precusors. This makes variable manifestations such as abdominal pain, nausea, vomiting, contipation, peripheral neuropathy, seizure, psychotic symtoms, respiratory failure, tachycardia, and hypertension. When acute paroxysmal hypertension presents, it is difficult to differentiate from pheochromocytoma.
We experienced a case of young female with acute intermittent porphyria presenting paroxysmal recurrent headache, palpitation, chest discomfort and paroxysmal hypertension. Initially blood pressure was 160/100 mmHg and checked high paroxysmally on follow up. Rhythm was sinus tachycardia. She did not complain any other specific symptoms.
Although her clinical manifestations were suspicious for pheochromocytoma, laboratory findings were not compatible to it. In searching other causes of secondary hypertension mimickng ph´ëchromocytoma, elevated urine porphobilinogen level was detected and we could make diagnosis of acute intermittent porphyria. If AIP was not beared in mind as a cause of secondary hypertension, diagnosis would be delayed more
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KEYWORD
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acute intermittent porphyria, secondary hypertension
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