2008
DOI: 10.1111/j.1440-1746.2008.05312.x
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Cholestatic liver disease in long‐term infantile nephropathic cystinosis

Abstract: Long-term infantile nephropathic cystinosis can be associated with a form of sclerosing cholangitis, which can respond to therapy with ursodeoxycholic acid.

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Cited by 15 publications
(4 citation statements)
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“…Hepatomegaly without liver failure is a common finding in cystinosis, it is a result of cytokine reaction to cystine crystals and collagen deposition without accompanying fibrosis and bridging necrosis [1,5,[13][14][15], which is also common in our group, hepatomegaly was found in three quarters of patients and change in liver pattern in more than half of the patients. Cystine accumulation in liver also causes secretion of vasoactive substances that results in vasoconstriction and portal hypertension [1,5,13,14]. More than half of our patients had splenomegaly and also had increased portal vein diameter leading to possible diagnosis of portal hypertension however no sign of portal hypertension was found in endoscopic studies.…”
Section: Discussionmentioning
confidence: 94%
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“…Hepatomegaly without liver failure is a common finding in cystinosis, it is a result of cytokine reaction to cystine crystals and collagen deposition without accompanying fibrosis and bridging necrosis [1,5,[13][14][15], which is also common in our group, hepatomegaly was found in three quarters of patients and change in liver pattern in more than half of the patients. Cystine accumulation in liver also causes secretion of vasoactive substances that results in vasoconstriction and portal hypertension [1,5,13,14]. More than half of our patients had splenomegaly and also had increased portal vein diameter leading to possible diagnosis of portal hypertension however no sign of portal hypertension was found in endoscopic studies.…”
Section: Discussionmentioning
confidence: 94%
“…Although opthalmic and endocrine system complications are seen frequently, many other systems are affected also like gastrointestinal and muscular systems [ 1 , 4 , 6 , 12 ]. In terms of gastrointestinal system invovement, hepatomegaly is a common finding usually without liver dysfunction [ 13 , 14 ]. Cystine crystal accumulation activates Kupffer cells and secreted vasoactive substances cause vasoconstriction in microcirculation resulting in portal hypertension [ 13 – 15 ].…”
Section: Introductionmentioning
confidence: 99%
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“…1418,1419 In two patients, long-term infantile nephropathic cystinosis was associated with a form of sclerosing cholangitis, which responded to therapy with UDCA. 1420 Liver biopsy specimens showed severe accumulation of cystine, predominantly localized in Kupffer cells, together with morphological features of sclerosing cholangitis. One patient showed changes compatible with sclerosing cholangitis on MRI.…”
Section: Congenital Hyperammonaemia Syndromes and Urea Cycle Disordersmentioning
confidence: 99%