caa a22 4500 475785134 CHVBK 20180406123655.0 cr unu---uuuuu 170329e20000201xx s 000 0 eng 10.1007/s004310050038 doi (NATIONALLICENCE)springer-10.1007/s004310050038 Serum liver enzymes in Turner syndrome [Elektronische Daten] [D. Larizza, M. Locatelli, L. Vitali, C. Viganò, V. Calcaterra, C. Tinelli, M. G. Sommaruga, A. Bozzini, R. Campani, F. Severi] Increased serum concentrations of liver enzymes are sometimes observed, in the absence of clinical symptoms of liver disease, in patients with Turner syndrome. The purpose of this study was to evaluate, in our Turner patients, serum liver enzyme levels and to find a cause for their increase. In 70 Turner patients, serum AST, ALT, GGT levels were evaluated every 6 months during a period of 0.8-21.9 years. In patients in whom increased values of liver enzymes were found, serological markers for infectious hepatitis, serum hepatitis C virus RNA and virus genotype, IgG and IgA antibodies to gliadin and endomysium, coeruloplasmin, copper, α1-antitrypsin, total proteins and electrophoresis, IgG, IgA, IgM, fibrinogen, prothrombin, alkaline phosphatase, creatine kinase and total and direct bilirubin were also determined. Antinuclear, anti-smooth muscle and anti-liver-kidney microsome antibodies together with antithyroglobulin and anti-thyroid peroxidase antibodies were determined in all patients and in 166 age-matched female controls. In 22 patients, increased liver enzymes were observed, not related to karyotype. Follow-up showed that the hepatic disorder did not worsen with the time. Serological markers of hepatitis C virus were positive in three patients. When the serum liver enzyme increase was first observed in the other 19 patients with high enzyme levels (group A), 14 patients had never been submitted to hormonal treatment, 4 were on oestrogen/gestagen treatment and 1 was being treated with both growth hormone and oestrogen. Coeliac disease, α1-antitrypsin deficiency and Wilson disease were ruled out by appropriate investigations. In 8/19 group A patients, antinuclear and/or anti-smooth muscle antibodies were present versus 6/48 of patients with normal liver enzymes (group B). Thyroid antibodies were found in 8/19 patients in group A and in 13/48 in group B. Weight excess SDS was significantly higher in Turner girls with liver enzyme increase. Ultrasonography, performed in 17 patients of group A, showed mild hepatomegaly in 4 and increased echogenicity with fatty infiltration in 6. Conclusion Hepatic abnormalities in Turner syndrome are not progressive. Oestrogen should not be considered the main cause of increased liver enzymes in Turner syndrome since most of our patients with this finding had not been previously treated with oestrogens. An auto-immune pathogenesis might be considered in some cases, whereas the association with weight excess seems the most frequent cause of liver disorder in Turner syndrome. Springer-Verlag Berlin Heidelberg, 2000 Key words Turner syndrome nationallicence Liver enzymes nationallicence Oestrogen therapy nationallicence Weight excess nationallicence Larizza D. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Locatelli M. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Vitali L. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Viganò C. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Calcaterra V. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Tinelli C. Biometric Unit, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut Sommaruga M. G. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut Bozzini A. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut Campani R. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut Severi F. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut https://doi.org/10.1007/s004310050038 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004310050038 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Larizza D. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut NATIONALLICENCE 700 1- Locatelli M. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut NATIONALLICENCE 700 1- Vitali L. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut NATIONALLICENCE 700 1- Viganò C. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut NATIONALLICENCE 700 1- Calcaterra V. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut NATIONALLICENCE 700 1- Tinelli C. Biometric Unit, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut NATIONALLICENCE 700 1- Sommaruga M. G. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut NATIONALLICENCE 700 1- Bozzini A. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut NATIONALLICENCE 700 1- Campani R. Institute of Radiology, IRCCS Policlinico S. Matteo, Pavia, Italy, IT aut NATIONALLICENCE 700 1- Severi F. Department of Paediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia, Italy Tel.: +39-382-502885; Fax: +39-382-527976, IT aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer