Acute Intermittent Porphyria with Relapsing Acute Pancreatitis and Unconjugated Hyperbilirubinemia Without Overt Hemolysis

Kobza, K.; Gyr, K.; Neuhaus, Konstantin; Gudat, F

doi:10.1016/s0016-5085(76)80462-3

Cited by 14 publications

(1 citation statement)

References 11 publications

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“…A few cases of acute intermittent porphyria (AIP) complicated by pancreatitis have been reported. [8][9][10] Shiraki et al 10 have reported a patient with AIP who developed acute pancreatitis during an episode of AIP. In that patient, the serum amylase, GOT, and bilirubin levels were increased after the cessation of abdominal pain, as seen in our patient.…”

Section: Discussionmentioning

confidence: 99%

Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

Komatsu

Shimosegawa

Uchi

et al. 2000

Journal of Gastroenterology

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A case of erythropoietic protoporphyria associated with severe hepatic dysfunction and acute pancreatitis is reported. The patient, a 33-year-old man, was admitted to our hospital complaining of upper abdominal pain, nausea, and vomiting of 3 days' duration. Laboratory tests on admission demonstrated liver dysfunction, anemia, and thrombocytopenia. On the third hospital day, the intensity of the upper abdominal pain increased, concomitantly with elevated levels of serum amylase. Ultrasonography and computed tomography scanning revealed a slightly enlarged pancreas. During this episode, he also complained of various neurological symptoms, including reduced mental alertness, weakness of extremities, constipation, profound sweating, and urinary retention. Porphyrin studies demonstrated markedly elevated erythrocyte and fecal protoporphyrin levels. Laparoscopic findings obtained after the attack subsided were compatible with porphyric liver cirrhosis. We therefore concluded that neurologic disorders and acute pancreatitis could develop in patients with erythropoietic protoporphyria with severe liver dysfunction.

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