Quantitative liver functions in Turner syndrome with and without hormone replacement therapy.

BACKGROUND Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes. AIM To study quantitative liver functions in TS in detail with and without HRT. DESIGN Randomized crossover study with active treatment (HRT in TS and P-pill in controls) or no treatment. SUBJECTS Women with TS (n = 8, age 29.7 +/- 5.6 (mean +/- s.d.) years), verified by karyotype, and age-matched controls (C; n = 8, age 27.3 +/- 4.9 years). METHODS We determined liver enzymes in blood, used the galactose elimination capacity to assess hepatocyte cytosol activity, plasma clearance of indocyanine green to assess excretory function, antipyrine clearance to estimate microsomal activity, and the functional hepatic nitrogen clearance (FHNC) to assess mitochondrial-cytosolic metabolic capacity for conversion of amino-nitrogen. RESULTS Liver enzymes were elevated in untreated TS and reduced by HRT. The hepatic capacities for conversion of galactose, indocyanine green, and antipyrine were normal and did not change by HRT. The FHNC was marginally reduced (untreated TS vs C: 19.4 +/- 5.4 vs 25.2 +/- 7.3 L/h, P = 0.1). FHNC changed slightly with HRT in TS (19.4 +/- 5.4 vs 24.4 +/- 10.2 L/h, P = 0.2). CONCLUSIONS The elevations of liver enzymes in untreated TS are readily suppressed by HRT. Quantitative liver functions in TS are comparable to controls and are not affected by HRT.