Acute Necrotizing Pancreatitis Secondary to Acute Intermittent Porphyria: A Rare Clinical Association

We report a rare case of acute necrotizing pancreatitis in a 23-year-old man during an acute attack of acute intermittent porphyria (AIP). Initially presenting with neurovisceral symptoms and hyponatremia, he was diagnosed with AIP based on clinical features and a positive urine porphobilinogen test. Despite symptomatic improvement, he developed severe abdominal pain and elevated pancreatic enzymes, with imaging confirming necrotizing pancreatitis. He required prolonged supportive care, percutaneous drainage, and nutritional support. This case highlights the importance of recognizing pancreatic complications in AIP, as timely diagnosis and multidisciplinary management are crucial for favorable outcomes in this rare but serious association.

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Publication Details

Journal
ACG Case Reports Journal
Published
2025-11-01
DOI
https://doi.org/10.14309/crj.0000000000001910
Primary Topic
Porphyrin Metabolism and Disorders
Type
article
Field-Weighted Citation Impact
0.00
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article

Acute Necrotizing Pancreatitis Secondary to Acute Intermittent Porphyria: A Rare Clinical Association

Mohan Gurjar, Sachin Wali, Varunvenkat M. Srinivasan, Shreyas Gutte et al.
ACG Case Reports Journal
Porphyrin Metabolism and Disorders
article

Acute Necrotizing Pancreatitis Secondary to Acute Intermittent Porphyria: A Rare Clinical Association

Mohan Gurjar, Sachin Wali, Varunvenkat M. Srinivasan, Shreyas Gutte, Wg Cdr Gaurav Pandey
article en

Abstract

We report a rare case of acute necrotizing pancreatitis in a 23-year-old man during an acute attack of acute intermittent porphyria (AIP). Initially presenting with neurovisceral symptoms and hyponatremia, he was diagnosed with AIP based on clinical features and a positive urine porphobilinogen test. Despite symptomatic improvement, he developed severe abdominal pain and elevated pancreatic enzymes, with imaging confirming necrotizing pancreatitis. He required prolonged supportive care, percutaneous drainage, and nutritional support. This case highlights the importance of recognizing pancreatic complications in AIP, as timely diagnosis and multidisciplinary management are crucial for favorable outcomes in this rare but serious association.

ACG Case Reports JournalVol. 12(11)
Sanjay Gandhi Post Graduate Institute of Medical Sciences (IN), Institute of Child Health (IN), Command Hospital Air Force (IN)
Openalex Percentile: Top 68%
Porphyrin Metabolism and Disorders
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