3%) had NAFLD, 13 (22.4%) had chronic cryptogenic liver disease, 5 (8.6%) had AIH, 5 had nodular regenerative hyperplasia (NRH) of the liver,
4 (6.8%) had CDG, 2 (3.44%) had congenital hepatic fibrosis, and 2 (3.44%) had Klippel-Trénaunay-Weber syndrome with hepatic vascular malformations. Furthermore, celiac disease, chronic hepatitis C, Alagille syndrome, and sclerosing cholangitis were each present in a single case. The remaining six patients were recruited after familial screening and did not carry any mutation according to the molecular analysis of ATP7B. Ku-0059436 in vivo Liver function tests and other routine laboratory data were obtained with standard methods. The ceruloplasmin concentration in serum was measured by radial immunodiffusion (NOR-Partigen Coeruloplasmin, Behring, Marburg, Germany; normal range = 20-60 mg/dL).12 Urine samples (basal urinary click here copper and urinary copper after PCT) were collected in an acid-washed, plastic, metal-free container. PCT urinary copper was evaluated after patients ingested 500 mg of D-penicillamine at time zero and again at
12 hours while 24-hour urinary copper collection progressed.13 Copper levels in urine were determined by flame atomic absorption spectrophotometry as previously described.14 Liver biopsy was performed by the Menghini technique with a disposable biopsy set (Hepafix, Braun, Melsungen, Germany). Copper levels in dried liver tissue were determined by flame atomic absorption spectroscopy according to Kingston and Jassie15 (normal cAMP range = 6-50 μg/g of dry weight). All slides were examined by the same pathologist, and lesions were evaluated according to the recommendations of Batts and Ludwig.16 For the molecular analysis of the ATP7B gene, DNA extraction and polymerase chain reaction were carried out with the standard methods by Dr.
Georgios Loudianos (Ospedale Regionale per le Microcitemie, Cagliari, Italy). With single-strand conformational polymorphism and sequencing methods, patients were analyzed for the 12 exons (5, 6, 8, 10, and 12-19) on which most mutations reside according to previous studies of the Italian continental population. DNA samples not completely characterized by the first step of analysis or those found to have a new missense mutation were further analyzed for the remaining exons of the ATP7B gene by single-strand conformational polymorphism and sequencing analysis.17 Continuous variables (ceruloplasmin, urinary copper, and liver copper) were presented as numbers of patients, means, medians, and standard deviations, whereas discrete variables (clinical manifestations at presentation and the presence or absence of KF rings) were presented as percentages. Normally distributed continuous variables were presented as means and standard deviations and were compared between groups by analysis of variance with post hoc testing (Scheffe’s test).
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