Document Detail


Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation.
MedLine Citation:
PMID:  23617280     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
We report a 51-year-old female patient with adult-onset type II citrullinemia (CTLN2) 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 CTLN2 was made by DNA analysis of the SLC25A13 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 CTLN2 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 CTLN2 with somatostatinoma. As the condition of CTLN2 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.
Authors:
Ko-Ichi Tazawa; Masahide Yazaki; Kazuhiro Fukushima; Satoshi Ogata; Masatoshi Makuuchi; Katsuo Morita; Hideyuki Hiraishi; Yoshimi Iwasaki; Junji Kita; Keiichi Kubota; Shu-Ichi Ikeda
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Hepatology research : the official journal of the Japan Society of Hepatology     Volume:  43     ISSN:  1386-6346     ISO Abbreviation:  Hepatol. Res.     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-04-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9711801     Medline TA:  Hepatol Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  563-8     Citation Subset:  -    
Copyright Information:
© 2013 The Japan Society of Hepatology.
Affiliation:
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto.
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