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Focal lesions in the liver in the course of glycogenosis type I : in CT and MR images

Focal lesions in the liver in the course of glycogenosis ...

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dc.contributor.author Chrzan, Robert [SAP20001365] pl
dc.contributor.author Urbanik, Andrzej [SAP20001269] pl
dc.contributor.author Herman-Sucharska, Izabela [SAP20000105] pl
dc.contributor.author Wojciechowski, Wadim [SAP20002419] pl
dc.date.accessioned 2019-03-27T11:57:16Z
dc.date.available 2019-03-27T11:57:16Z
dc.date.issued 2009 pl
dc.identifier.issn 1733-134X pl
dc.identifier.uri https://ruj.uj.edu.pl/xmlui/handle/item/71540
dc.language eng pl
dc.rights Udzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 4.0 Międzynarodowa *
dc.rights.uri http://creativecommons.org/licenses/by-nc-nd/4.0/pl/legalcode *
dc.title Focal lesions in the liver in the course of glycogenosis type I : in CT and MR images pl
dc.title.alternative Zmiany ogniskowe w wątrobie w przebiegu glikogenozy typu I w badaniu TK i MR pl
dc.type JournalArticle pl
dc.description.physical 93-98 pl
dc.description.additional Bibliogr. s. 98 pl
dc.identifier.weblink http://archiwum.inforadiologia.pl/download/index/idArt/878423.html pl
dc.abstract.en Background: Glycogenosis type I (von Gierke's disease) is an inherited hepatic glycogen storage disease. The main diagnostic criteria are: hepatomegaly, hypoglycemia and hyperlacticacidemia. Its transmission is autosomal recessive. Depending on the intensity of fatty degeneration and amount of glycogen, the liver density may be decreased, normal or increased. Hepatocellular adenomas develop in most patients and malignant degeneration of adenomas into hepatocellular carcinomas has been reported in some cases. Case Report: Two siblings with glycogenosis type I were initially followed up using US. Focal liver lesions detected in US were then followed up in CT and MR and reported as adenomas. However, some lesions enlarged considerably in the course of observation and developed foci of degeneration suspicious for malignant transformation, so were referred for biopsy. FNB repeated several times revealed no malignant transformation. Conclusions: Patients with glycogenosis type I must be periodically screened for malignant transformation of adenoma into hepatocellular carcinoma using biochemical markers and imaging. Suspicion of malignant transformation in imaging may be difficult to verify in cytology/pathology. Surgery/interventional radiology is suggested in every case of a lesion suspicious in imaging. Liver transplantation is recommended as a definite treatment. pl
dc.subject.en glycogenosis pl
dc.subject.en adenoma pl
dc.subject.en computer tomography pl
dc.subject.en magnetic resonance pl
dc.description.volume 74 pl
dc.description.number 4 pl
dc.identifier.eissn 1899-0967 pl
dc.title.journal Polish Journal of Radiology pl
dc.language.container eng pl
dc.date.accession 2019-04-26 pl
dc.affiliation Wydział Lekarski : Zakład Radiologii pl
dc.subtype Article pl
dc.identifier.articleid 878423 pl
dc.rights.original CC-BY-NC-ND; otwarte czasopismo; ostateczna wersja wydawcy; w momencie opublikowania; 0 pl
dc.identifier.project ROD UJ / OP pl


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Udzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 4.0 Międzynarodowa Except where otherwise noted, this item's license is described as Udzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 4.0 Międzynarodowa