Patient with adult‐onset type <scp>II</scp> citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation
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- Ko‐ichi Tazawa
- Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine Matsumoto
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- Masahide Yazaki
- Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine Matsumoto
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- Kazuhiro Fukushima
- Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine Matsumoto
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- Satoshi Ogata
- Department of Hepato‐Biliary‐Pancreatic Surgery Japanese Red Cross Medical Center Tokyo
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- Masatoshi Makuuchi
- Department of Hepato‐Biliary‐Pancreatic Surgery Japanese Red Cross Medical Center Tokyo
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- Katsuo Morita
- Department of Gastroenterology Dokkyo Medical University Mibu Japan
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- Hideyuki Hiraishi
- Department of Gastroenterology Dokkyo Medical University Mibu Japan
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- Yoshimi Iwasaki
- Department of Gastroenterological Surgery Dokkyo Medical University Mibu Japan
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- Junji Kita
- Department of Gastroenterological Surgery Dokkyo Medical University Mibu Japan
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- Keiichi Kubota
- Department of Gastroenterological Surgery Dokkyo Medical University Mibu Japan
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- Shu‐ichi Ikeda
- Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine Matsumoto
書誌事項
- 公開日
- 2013-04-26
- 資源種別
- journal article
- 権利情報
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- http://onlinelibrary.wiley.com/termsAndConditions#vor
- DOI
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- 10.1111/j.1872-034x.2012.01098.x
- 公開者
- Wiley
この論文をさがす
説明
<jats:p>We report a 51‐year‐old female patient with adult‐onset type <jats:styled-content style="fixed-case">II</jats:styled-content> citrullinemia (<jats:styled-content style="fixed-case">CTLN2</jats:styled-content>) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 years, paying special attention to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of <jats:styled-content style="fixed-case">CTLN2</jats:styled-content> was made by <jats:styled-content style="fixed-case">DNA</jats:styled-content> analysis of the <jats:italic><jats:styled-content style="fixed-case">SLC25A13</jats:styled-content></jats:italic> gene and treatment with conservative therapies was begun, including a low‐carbohydrate diet and supplementation with arginine and sodium pyruvate. However, despite these treatments, frequent attacks of encephalopathy occurred with markedly elevated plasma ammonia levels. While we were apprehensive regarding the risk of recurrence of somatostatinoma due to immunosuppressive therapy after liver transplantation, the patient was in a critical condition with <jats:styled-content style="fixed-case">CTLN2</jats:styled-content> and it was decided to perform living‐donor liver transplantation using a graft obtained from her son. Her postoperative clinical course was uneventful and she has had an active life without recurrence of somatostatinoma for 2 years. This is the first case of <jats:styled-content style="fixed-case">CTLN2</jats:styled-content> with somatostatinoma. As the condition of <jats:styled-content style="fixed-case">CTLN2</jats:styled-content> patients with rapidly progressive courses is often intractable by conservative therapies alone, liver transplantation should be considered even after surgery for malignant tumors in cases with neither metastasis nor recurrence.</jats:p>
収録刊行物
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- Hepatology Research
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Hepatology Research 43 (5), 563-568, 2013-04-26
Wiley
