EFFECT OF ESTROGEN GESTAGEN REPLACEMENT THERAPY ON LIVER-ENZYMES IN PATIENTS WITH ULLRICH-TURNER SYNDROME

The absence of breast development and the prevention of osteoporosis i n Ullrich-Turner syndrome (UTS) require oestrogen/gestagen substitutio n therapy. In 8 out of 35 (23%) patients with UTS treated with conjuga ted equine oestrogens and cyclically with norethisterone acetate, the serum liver enzymes increased to conspicuous levels (AST 35; 20-73 U/l , ALT 92; 37-141 U/l, GGT 77; 25-227 U/l, [median; min-maxi]). These f indings were compared with those in 41 tall girls who received a six-f old larger dose of conjugated equine oestrogens for the reduction of f inal height. None of these 41 girls showed abnormal serum liver enzyme levels. The conspicuous rise in serum liver enzyme levels occurred in the majority of the UTS patients before norethistherone acetate was a dded to the oestrogen replacement therapy. No essential morphological equivalent was found in liver sonography and biopsy studies. During th e follow up the elevated serum liver enzyme levels showed reversibilit y when medication was temporarily discontinued and either a slow decre ase or a steady state after therapy was continued. Conclusion Patients with UTS on oral oestrogen replacement therapy are more susceptible t o develop increased serum liver enzyme levels as compared with eukaryo tic females treated with the same oestrogen preparation for other diso rders. As the underlying pathomechanism is unknown and adverse long-te rm effects cannot be ruled Out, avoiding the portal vein and using the transdermal application of oestrogen may represent a viable solution to the problem.