{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1360004235757634816.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1111/j.1872-034x.2012.01098.x"}},{"identifier":{"@type":"URI","@value":"https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1872-034X.2012.01098.x"}},{"identifier":{"@type":"URI","@value":"https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1872-034X.2012.01098.x"}},{"identifier":{"@type":"PMID","@value":"23617280"}}],"resourceType":"学術雑誌論文(journal article)","dc:title":[{"@value":"Patient with adult‐onset type <scp>II</scp> citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation"}],"description":[{"type":"abstract","notation":[{"@value":"<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>"}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1380861288037040385","@type":"Researcher","foaf:name":[{"@value":"Ko‐ichi Tazawa"}],"jpcoar:affiliationName":[{"@value":"Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine  Matsumoto"}]},{"@id":"https://cir.nii.ac.jp/crid/1420001326212727680","@type":"Researcher","personIdentifier":[{"@type":"KAKEN_RESEARCHERS","@value":"70372513"},{"@type":"NRID","@value":"1000070372513"},{"@type":"NRID","@value":"9000001488771"},{"@type":"NRID","@value":"9000412637331"},{"@type":"NRID","@value":"9000001476585"},{"@type":"NRID","@value":"9000413473725"},{"@type":"NRID","@value":"9000411811732"},{"@type":"NRID","@value":"9000351078783"},{"@type":"NRID","@value":"9000022065069"},{"@type":"NRID","@value":"9000001488829"},{"@type":"NRID","@value":"9000021888792"},{"@type":"NRID","@value":"9000021432692"},{"@type":"NRID","@value":"9000379610229"},{"@type":"NRID","@value":"9000001476797"},{"@type":"NRID","@value":"9000261675994"},{"@type":"NRID","@value":"9000412604346"},{"@type":"NRID","@value":"9000257859516"},{"@type":"NRID","@value":"9000020737093"},{"@type":"NRID","@value":"9000242155161"},{"@type":"NRID","@value":"9000021925488"},{"@type":"NRID","@value":"9000021330096"},{"@type":"NRID","@value":"9000411515084"},{"@type":"NRID","@value":"9000415323665"},{"@type":"NRID","@value":"9000402771823"},{"@type":"NRID","@value":"9000021889627"},{"@type":"NRID","@value":"9000412320118"},{"@type":"NRID","@value":"9000412601804"},{"@type":"NRID","@value":"9000412604114"},{"@type":"NRID","@value":"9000000562281"},{"@type":"NRID","@value":"9000240524982"},{"@type":"NRID","@value":"9000020284922"},{"@type":"NRID","@value":"9000021405931"},{"@type":"NRID","@value":"9000409987637"},{"@type":"NRID","@value":"9000259296279"},{"@type":"NRID","@value":"9000021490239"},{"@type":"NRID","@value":"9000411514321"},{"@type":"RESEARCHMAP","@value":"https://researchmap.jp/read0116212"}],"foaf:name":[{"@value":"Masahide Yazaki"}],"jpcoar:affiliationName":[{"@value":"Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine  Matsumoto"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634944","@type":"Researcher","foaf:name":[{"@value":"Kazuhiro Fukushima"}],"jpcoar:affiliationName":[{"@value":"Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine  Matsumoto"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634825","@type":"Researcher","foaf:name":[{"@value":"Satoshi Ogata"}],"jpcoar:affiliationName":[{"@value":"Department of Hepato‐Biliary‐Pancreatic Surgery Japanese Red Cross Medical Center  Tokyo"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634826","@type":"Researcher","foaf:name":[{"@value":"Masatoshi Makuuchi"}],"jpcoar:affiliationName":[{"@value":"Department of Hepato‐Biliary‐Pancreatic Surgery Japanese Red Cross Medical Center  Tokyo"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634959","@type":"Researcher","foaf:name":[{"@value":"Katsuo Morita"}],"jpcoar:affiliationName":[{"@value":"Department of Gastroenterology Dokkyo Medical University  Mibu Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634949","@type":"Researcher","foaf:name":[{"@value":"Hideyuki Hiraishi"}],"jpcoar:affiliationName":[{"@value":"Department of Gastroenterology Dokkyo Medical University  Mibu Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634821","@type":"Researcher","foaf:name":[{"@value":"Yoshimi Iwasaki"}],"jpcoar:affiliationName":[{"@value":"Department of Gastroenterological Surgery Dokkyo Medical University  Mibu Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634963","@type":"Researcher","foaf:name":[{"@value":"Junji Kita"}],"jpcoar:affiliationName":[{"@value":"Department of Gastroenterological Surgery Dokkyo Medical University  Mibu Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1380004235757634816","@type":"Researcher","foaf:name":[{"@value":"Keiichi Kubota"}],"jpcoar:affiliationName":[{"@value":"Department of Gastroenterological Surgery Dokkyo Medical University  Mibu Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1380861288037040384","@type":"Researcher","foaf:name":[{"@value":"Shu‐ichi Ikeda"}],"jpcoar:affiliationName":[{"@value":"Department of Medicine (Neurology and Rheumatology) Shinshu University School of Medicine  Matsumoto"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"13866346"},{"@type":"EISSN","@value":"1872034X"}],"prism:publicationName":[{"@value":"Hepatology Research"}],"dc:publisher":[{"@value":"Wiley"}],"prism:publicationDate":"2013-04-26","prism:volume":"43","prism:number":"5","prism:startingPage":"563","prism:endingPage":"568"},"reviewed":"false","dc:rights":["http://onlinelibrary.wiley.com/termsAndConditions#vor"],"url":[{"@id":"https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1872-034X.2012.01098.x"},{"@id":"https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1872-034X.2012.01098.x"}],"createdAt":"2013-04-26","modifiedAt":"2023-10-15","project":[{"@id":"https://cir.nii.ac.jp/crid/1040282257236376576","@type":"Project","projectIdentifier":[{"@type":"KAKEN","@value":"25461275"},{"@type":"JGN","@value":"JP25461275"},{"@type":"URI","@value":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-25461275/"}],"notation":[{"@language":"ja","@value":"FAPにおける低分子薬剤のアミロイド線維沈着抑制機序の解明と根治治療への応用"},{"@language":"en","@value":"Elucidation of preventive effect of amyloidogenesis by low molecule drugs in FAP"}]}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050845763844424704","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011144197938176","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Liver transplantation and neuroendocrine tumors: lessons from a single centre experience and from the literature review"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570527652736","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Treatment of a citrin‐deficient patient at the early stage of adult‐onset type II citrullinaemia with arginine and sodium pyruvate"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418520150657024","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Orthotopic liver transplantation for urea cycle enzyme deficiency"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699994813732608","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Pyruvate ameliorates the defect in ureogenesis from ammonia in citrin-deficient mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699996209340928","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Somatostatinoma of vater's papilla and of the minor papilla"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981468744510464","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944961634944","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Adult onset type II citrullinemia as a cause of non-alcoholic steatohepatitis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262945136133376","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Primary Liver Carcinoma Exhibiting Dual Hepatocellular-Biliary Epithelial Differentiations Associated With Citrin Deficiency"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419700078592","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitochondrial aspartate glutamate carrier (citrin) deficiency as the cause of adult-onset type II citrullinemia (CTLN2) and idiopathic neonatal hepatitis (NICCD)"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319001177216","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"SLC25A13 gene mutations in Taiwanese patients with non-viralhepatocellular carcinoma"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670320291695232","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Citrin and aralar1 are Ca2+-stimulated aspartate/glutamate transporters in mitochondria"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670320773712128","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Effectiveness of carbohydrate-restricted diet and arginine granules therapy for adult-onset type II citrullinemia: a case report of siblings showing homozygous SLC25A13 mutation with and without the disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951793365156992","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Type II (adult onset) citrullinaemia: clinical pictures and the therapeutic effect of liver transplantation"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233268740066176","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Chronic Pancreatitis Associated with Adult-Onset Type II Citrullinemia: Clinical and Pathologic Findings"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270254902016","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"An autopsy case with adult onset type II citrullinemia showing myelopathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1390282679843063680","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Hepatocellular Carcinoma in a Case of Adult-onset Type II Citrullinemia"},{"@value":"Hepatocellular Carcinoma in a Case of Adult-onset Type 2 Citrullinemia"}]},{"@id":"https://cir.nii.ac.jp/crid/1390282679845899264","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Orthodox Sometimes Generates Paradox"},{"@value":"Risk of Worsened Encephalopathy after Intravenous Glycerol Therapy in Patients with Adult-onset Type 2 Citrullinemia(CTLN2)"},{"@value":"Risk of Worsened Encephalopathy after Intravenous Glycerol Therapy in Patients with Adult-onset Type II Citrullinemia (CTLN2)"}]},{"@id":"https://cir.nii.ac.jp/crid/1390282679847185280","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency"}]},{"@id":"https://cir.nii.ac.jp/crid/1390282680011414784","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Therapeutic approaches for patients with adult-onset type II citrullinemia (CTLN2): effectiveness of treatment with low-carbohydrate diet and sodium pyruvate"},{"@language":"ja","@value":"シンポジウム０２‐５　アカデミア発の創薬・治療研究　成人型シトルリン血症の治療‐低炭水化物食と経口ピルビン酸投与の有効性"}]},{"@id":"https://cir.nii.ac.jp/crid/1390846609822417280","@type":"Article","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"A case of adult-onset type 2 citrullinemia brought on by changes in disease-specific eating habits following resection of a large portion of the small intestine"},{"@language":"ja","@value":"小腸大量切除後に食癖の変化を認めた成人発症II型シトルリン血症の1例"},{"@value":"症例報告 小腸大量切除後に食癖の変化を認めた成人発症Ⅱ型シトルリン血症の1例"},{"@language":"ja-Kana","@value":"ショウレイ ホウコク ショウチョウ タイリョウ セツジョ ゴ ニ ショクヘキ ノ ヘンカ オ ミトメタ セイジン ハッショウ Ⅱ カタ シトルリン ケッショウ ノ 1レイ"}]},{"@id":"https://cir.nii.ac.jp/crid/1572824499129764096","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Somatostatinoma/inhibitory syndrome : a statistical evaluation of 173 reported cases as compared to other endocrinomas"}]}],"dataSourceIdentifier":[{"@type":"CROSSREF","@value":"10.1111/j.1872-034x.2012.01098.x"},{"@type":"KAKEN","@value":"PRODUCT-15080725"},{"@type":"OPENAIRE","@value":"doi_dedup___::92ed81dda681f2b98bcb2ff253e141dc"},{"@type":"CROSSREF","@value":"10.2957/kanzo.61.204_references_DOI_ND78EZI5AUZg5QKJi8Q2fNb88Im"}]}