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신경 및 위장관 증세를 동반한 Erythropoietic Protoporphyria 환아 1예
DC Field | Value | Language |
---|---|---|
dc.contributor.author | 박영년 | - |
dc.contributor.author | 정기섭 | - |
dc.date.accessioned | 2017-10-26T06:09:21Z | - |
dc.date.available | 2017-10-26T06:09:21Z | - |
dc.date.issued | 2005 | - |
dc.identifier.issn | 1229-0114 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/150584 | - |
dc.description.abstract | Erythropoietic protoporphyria is a genetic disorder due to a deficiency of ferrochelatase resulting in excessive accumulation and excretion of protoporphyrin. The predominant clinical feature is photosensitivity. Severe hepatic failure occurs in a small percentage of patients, and neurological symptoms are very rare. We report a case of erythropoietic protoporphyria associated with severe hepatic dysfunction and neurological symptoms. A 9-year-old girl presented with severe abdominal pain, nausea, weakness and pain of extremities, and urinary retention. Ultrasonogram and abdominal CT scanning revealed a diffuse infiltrated and enlarged liver. Liver biopsy showed deposition of dense dark brown pigment within the bile, hepatocytes and Kupffer cells. Plus, dense dark brown deposits gave a red birefringent under polarize light. Porphyrin studies demonstrated markedly elevated serum free erythrocyte protoporphyrin. This girl was diagnosed as erythropoietic protoporphyria with severe liver dysfunction and neurological symptoms. | - |
dc.description.statementOfResponsibility | open | - |
dc.language | Korean | - |
dc.publisher | 대한소아소화기영양학회 | - |
dc.relation.isPartOf | Korean Journal of Pediatric Gastroenterology and Nutrition (대한소아소화기영양학회지) | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.subject.MESH | Erythropoietic protoporphyria | - |
dc.subject.MESH | Neurological symptoms | - |
dc.subject.MESH | Liver dysfunction | - |
dc.title | 신경 및 위장관 증세를 동반한 Erythropoietic Protoporphyria 환아 1예 | - |
dc.title.alternative | A Case of Erythropoietic Protoporphyria with Severe Liver Dysfunction and Neurological Symptoms | - |
dc.type | Article | - |
dc.publisher.location | Korea | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pathology (병리학교실) | - |
dc.contributor.department | Dept. of Pediatrics (소아청소년과학교실) | - |
dc.contributor.googleauthor | 김보현 | - |
dc.contributor.googleauthor | 박영년 | - |
dc.contributor.googleauthor | 정기섭 | - |
dc.identifier.doi | OAK-2005-04053 | - |
dc.contributor.localId | A01563 | - |
dc.contributor.localId | A03581 | - |
dc.relation.journalcode | J02095 | - |
dc.identifier.eissn | 2093-5633 | - |
dc.relation.journalsince | 1988~2011 | - |
dc.relation.journalafter | 2012~ Pediatric Gastroenterology, Hepatology & Nutrition | - |
dc.subject.keyword | Erythropoietic protoporphyria | - |
dc.subject.keyword | Neurological symptoms | - |
dc.subject.keyword | Liver dysfunction | - |
dc.contributor.alternativeName | Park, Young Nyun | - |
dc.contributor.alternativeName | Chung, Ki Sup | - |
dc.citation.volume | 8 | - |
dc.citation.number | 2 | - |
dc.citation.startPage | 263 | - |
dc.citation.endPage | 268 | - |
dc.identifier.bibliographicCitation | Korean Journal of Pediatric Gastroenterology and Nutrition (대한소아소화기영양학회지), Vol.8(2) : 263-268, 2005 | - |
dc.date.modified | 2017-05-04 | - |
dc.identifier.rimsid | 44157 | - |
dc.type.rims | ART | - |
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