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The absence of breast development and the prevention of osteoporosis in Ullrich-Turner syndrome (UTS) require oestrogen/gestagen substitution therapy. In 8 out of 35 (23%) patients with UTS treated with conjugated 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-max]). These findings were compared with those in 41 tall girls who received a six-fold larger dose of conjugated equine oestrogens for the reduction of final 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 added to the oestrogen replacement therapy. No essential morphological equivalent was found in liver sonography and biopsy studies. During the follow up the elevated serum liver enzyme levels showed reversibility when medication was temporarily discontinued and either a slow decrease or a steady state after therapy was continued. CONCLUSION. Patients with UTS on oral oestrogen replacement therapy are more susceptible to develop increased serum liver enzyme levels as compared with eukaryotic females treated with the same oestrogen preparation for other disorders. As the underlying pathomechanism is unknown and adverse long-term effects cannot be ruled out, avoiding the portal vein and using the transdermal application of oestrogen may represent a viable solution to the problem.

Note from the Editors. Although Abstract. Our previous examinations had shown that 9 of 13 premature infants with severe respiratory distress had hypothyroid T4-values. On the basis of these results a prospective study was initiated. Every second neonate born after less than 37 weeks gestation or weighing less than 2200 g and admitted to our intensive care unit since Janary 1979 received a prophylactic dose of 25 gg L-Thyroxine and 5 lag Tri-iodothyronine daily. Five of the patients inadvertently did not receive the drug and were included in the non-treated group which thus numbered 55. Both groups were nearly identical with regard to gestational age, birth weight and Apgar score.In the treated group of 45 infants three (= 6.6%) died. In the untreated group of 55 infants 16 (= 29%) died. The probability that the different mortality in the two groups was due to chance alone is less than 0.5% 0f2-test: P< 0.005). In 14 of the 55 non-treated patients transient hypothyroidism developed. Five patients with transient hypothyroidism and 2 patients with low T4-values without a TSH-increase were treated with thyroid hormone after ascertainment of their serum thyroxine levels and six survived.The analysis of the prophylactically treated cases showed that the dosage of 25 lag L-Thyroxine and 5 lag Tri-iodothyronine in critically ill infants (i.e., those who were mechanically ventilated or had sepsis) was rarely sufficient to produce normal serum thyroxine levels. In these children thyroxine usually rose to * Corresponding author normal levels only when they had passed the acute stage of the disease. It therefore seems advisable to double the dose of thyroid hormone during the acute stage of the disease.

Two sibs, whose parents are first cousins, had diabetes mellitus with hyperinsulinism, insensitive insulin receptors, and acanthosis nigricans. Both patients had pigmentary retinopathy, secondary cataracts, labyrinthine deafness, mental retardation, and cerebral atrophy. They were disproportionately short with relatively broad hands and feet and slightly coarse face. The young woman had secondary amenorrhea and polycystic ovaries and the boy gynecomastia and hypergonadotrophic hypogonadism. This appears to be the second family with a new autosomal recessive disorder differing from Alström syndrome by the presence of mental retardation and absence of renal insufficiency. Impaired insulin receptor binding and polycystic ovaries are described as part of this syndrome.