UNUSUAL TYPE OF CUSHING'S SYNDROME (CS) IN ADOLESCENTS

4 patients (If, 3m, 15–18 yrs) presented with clinical CS. Urinary 170HC were increased (16.9 mg/24 h), but classic criteria excluded CS (2 mg dexamethasone suppression, normal plasma F and diurnal rhythm). Urinary THE, THF-allo THF and cortolone determined by gas chromatography on capillary column were slightly increased (sum = 11.4 mg/m2/24h), but β-cortolone was normal. Free urinary F was increased (206 μg/m2/24h). In 1 case, the metabolism of exogenous F and E was studied. i.v.F and p.o.E resulted in increased THF and THE respectively, as in controls. The pattern after p.o.F, however, was different : THE and THF remained low, but β-cortolone increased, while THF increased in controls. Phenobarbital reduced all steroids in the patient. In 3 cases, serum LDH was found to be high, suggesting impaired liver function. It is concluded that CS in these cases might be due not to increased F production, but to altered F catabolism, possibly in the liver.