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Chronic Pancreatitis Associated with Adult-Onset Type II Citrullinemia: Clinical and Pathologic Findings
- Shu-ichi Ikeda, MD;
- Shigeyuki Kawa, MD;
- Yo-ichi Takei, MD;
- Kanji Yamamoto, MD;
- Hisashi Shimojo, MD;
- Ken-ichi Tabata, MD;
- Keiko Kobayashi, PhD; and
- Takeyori Saheki, MD
- From Shinshu University School of Medicine, Matsumoto 390-8621, Japan; Saku General Hospital, Usuda 384-0393, Japan; and Kagoshima University; Kagoshima 890-8520, Japan.
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TO THE EDITOR:
Background: Adult-onset type II citrullinemia is a urea cycle disorder attributed to a deficiency of argininosuccinate synthetase in the liver (1). A mutation of the citrin gene is responsible for the development of this disease (2, 3). Clinically, patients have recurrent episodes with hepatic encephalopathy, and some patients with this disease seem to have had previous pancreatitis (4).
Objective: To clarify the clinicopathologic …
