A case of adult-onset type II citrullinemia induced by hospital diet

A 47-year-old Japanese man was first admitted to our hospital for 8 days because of an asthma attack. After discharge he changed his diet. On the 12th day after his discharge, he was re-admitted to our hospital because he exhibited transient loss of consciousness with flapping tremor. His plasma amm...

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Veröffentlicht in:Clinical journal of gastroenterology 2011-02, Vol.4 (1), p.28-33
Hauptverfasser: Ueda, Akira, Okada, Kazuhiko, Takahara, Terumi, Iwasa, Keiichi, Shinagawa, Kazuko, Entani, Akio, Inatsuchi, Syuji, Ando, Takayuki, Fujinami, Haruka, Tajiri, Kazuto, Tokimitsu, Yoshiharu, Ogawa, Kohei, Miyazono, Takayoshi, Miyazaki, Takako, Murakami, Jun, Hosokawa, Ayumu, Yasumura, Satoshi, Minemura, Masami, Kudo, Takahiko, Sugiyama, Toshiro
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container_end_page 33
container_issue 1
container_start_page 28
container_title Clinical journal of gastroenterology
container_volume 4
creator Ueda, Akira
Okada, Kazuhiko
Takahara, Terumi
Iwasa, Keiichi
Shinagawa, Kazuko
Entani, Akio
Inatsuchi, Syuji
Ando, Takayuki
Fujinami, Haruka
Tajiri, Kazuto
Tokimitsu, Yoshiharu
Ogawa, Kohei
Miyazono, Takayoshi
Miyazaki, Takako
Murakami, Jun
Hosokawa, Ayumu
Yasumura, Satoshi
Minemura, Masami
Kudo, Takahiko
Sugiyama, Toshiro
description A 47-year-old Japanese man was first admitted to our hospital for 8 days because of an asthma attack. After discharge he changed his diet. On the 12th day after his discharge, he was re-admitted to our hospital because he exhibited transient loss of consciousness with flapping tremor. His plasma ammonia level was extremely high (245 μg/dL; normal,
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After discharge he changed his diet. On the 12th day after his discharge, he was re-admitted to our hospital because he exhibited transient loss of consciousness with flapping tremor. His plasma ammonia level was extremely high (245 μg/dL; normal, &lt;90 μg/dL), suggesting hepatic encephalopathy. He underwent intravenous administration of branched-chain amino acids (Aminoleban ® ) and oral administration of lactulose and kanamycin sulfate; however, the hyperammonemia did not improve. Analysis of the amino acids and citrin gene led to the diagnosis of adult-onset type II citrullinemia (CTLN2). Following this diagnosis, the carbohydrate content of his diet was mildly restricted. As a result, his plasma ammonia level markedly improved (ammonia, 40–60 μg/dL) and he became symptom-free without any medication. CTLN2 is a metabolic disorder characterized by increased plasma concentrations of citrulline and ammonia, which occurs by the failure of compensatory mechanisms associated with diet. Here, we report a case of a patient for whom a change in eating habits during his hospitalization disturbed his compensatory mechanism resulting in clinical CTLN2, which was reversed with an appropriate diet.</description><identifier>ISSN: 1865-7257</identifier><identifier>EISSN: 1865-7265</identifier><identifier>DOI: 10.1007/s12328-010-0188-3</identifier><identifier>PMID: 26190618</identifier><language>eng</language><publisher>Japan: Springer Japan</publisher><subject>Abdominal Surgery ; Case Report ; Colorectal Surgery ; Gastroenterology ; Hepatology ; Medicine ; Medicine &amp; Public Health ; Surgical Oncology</subject><ispartof>Clinical journal of gastroenterology, 2011-02, Vol.4 (1), p.28-33</ispartof><rights>Springer 2010</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c320t-77e545bc322c3b6992f85e30bc0d9b78ea3bc54ca9f276c8796f6df44f5375293</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s12328-010-0188-3$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s12328-010-0188-3$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,777,781,27905,27906,41469,42538,51300</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26190618$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ueda, Akira</creatorcontrib><creatorcontrib>Okada, Kazuhiko</creatorcontrib><creatorcontrib>Takahara, Terumi</creatorcontrib><creatorcontrib>Iwasa, Keiichi</creatorcontrib><creatorcontrib>Shinagawa, Kazuko</creatorcontrib><creatorcontrib>Entani, Akio</creatorcontrib><creatorcontrib>Inatsuchi, Syuji</creatorcontrib><creatorcontrib>Ando, Takayuki</creatorcontrib><creatorcontrib>Fujinami, Haruka</creatorcontrib><creatorcontrib>Tajiri, Kazuto</creatorcontrib><creatorcontrib>Tokimitsu, Yoshiharu</creatorcontrib><creatorcontrib>Ogawa, Kohei</creatorcontrib><creatorcontrib>Miyazono, Takayoshi</creatorcontrib><creatorcontrib>Miyazaki, Takako</creatorcontrib><creatorcontrib>Murakami, Jun</creatorcontrib><creatorcontrib>Hosokawa, Ayumu</creatorcontrib><creatorcontrib>Yasumura, Satoshi</creatorcontrib><creatorcontrib>Minemura, Masami</creatorcontrib><creatorcontrib>Kudo, Takahiko</creatorcontrib><creatorcontrib>Sugiyama, Toshiro</creatorcontrib><title>A case of adult-onset type II citrullinemia induced by hospital diet</title><title>Clinical journal of gastroenterology</title><addtitle>Clin J Gastroenterol</addtitle><addtitle>Clin J Gastroenterol</addtitle><description>A 47-year-old Japanese man was first admitted to our hospital for 8 days because of an asthma attack. 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After discharge he changed his diet. On the 12th day after his discharge, he was re-admitted to our hospital because he exhibited transient loss of consciousness with flapping tremor. His plasma ammonia level was extremely high (245 μg/dL; normal, &lt;90 μg/dL), suggesting hepatic encephalopathy. He underwent intravenous administration of branched-chain amino acids (Aminoleban ® ) and oral administration of lactulose and kanamycin sulfate; however, the hyperammonemia did not improve. Analysis of the amino acids and citrin gene led to the diagnosis of adult-onset type II citrullinemia (CTLN2). Following this diagnosis, the carbohydrate content of his diet was mildly restricted. As a result, his plasma ammonia level markedly improved (ammonia, 40–60 μg/dL) and he became symptom-free without any medication. CTLN2 is a metabolic disorder characterized by increased plasma concentrations of citrulline and ammonia, which occurs by the failure of compensatory mechanisms associated with diet. Here, we report a case of a patient for whom a change in eating habits during his hospitalization disturbed his compensatory mechanism resulting in clinical CTLN2, which was reversed with an appropriate diet.</abstract><cop>Japan</cop><pub>Springer Japan</pub><pmid>26190618</pmid><doi>10.1007/s12328-010-0188-3</doi><tpages>6</tpages></addata></record>
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subjects Abdominal Surgery
Case Report
Colorectal Surgery
Gastroenterology
Hepatology
Medicine
Medicine & Public Health
Surgical Oncology
title A case of adult-onset type II citrullinemia induced by hospital diet
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