| Post Anesthetic Porphyrinogenic Acute Neuropathy. |
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Cheung Soo Shin, Yong Taek Nam, Myong Sik Lee |
1Department of Anesthesiology, Yonsei University College of Medicine, Seoul, Korea.
2Department of Neurology, Yonsei University College of Medicine, Seoul, Korea. |
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| Abstract |
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Acute intermittent porphyria(AIP) is a rare hepatic disorder resulting from defects in the synthesis of heme. This occurs in approximately 0.01% of the general population and is even rare in black and orientals. Acute episode of this disease can be triggered by surgery, certain drug, pregnancy, mensturation, fasting and etc. We report a porphyrinogenic neuropathy occured after general anesthesia in a oriental woman who presented with abdominal pain and other typical sign and symptom. 56 year-old female patient was diagnosed as acute appendicitis for 2 days of constipation and abdominal pain and appendectomy was performed under general anesthesia at local clinic. Anesthesia was induced with penthothal and maintained with halothane, and she was recovered from anesthesia without event. After operation she experienced difficult swallowing and generalized weakness which were aggrevated day by day. Therefore she was transferred to our hospital on fourth postoperative day.
Physical examination revealed Grade II motor weakness of Gxtremities and decreased deep tendon reflex. Neostigmine test and Jolly test were done under impression of myasthenia gravis but revealed negative. Guillian-Barre syndrome was difficult to rule out in this patient, but urinalysis revealed increased delta aminolevulinic acid and urine color changed dark under light. Therefore we diagnosed her as AIP.
6 days after appendectomy she complained severe dyspnea with vital capacity 350 ml and arterial blood gas analysis revealed PO2 of 56 mmHg and PCO2 of 44 mmHg under nasal oxygen 5 1/min inhalation. Therefore we diagnosed this peripheral neuropathy as AIP and report here with references. |
| Key Words:
Porphyria; Neuropathy; Anesthesia |
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