We have analysed growth and the major clinical manifestations of 144 patients (89 males, 55 females) with Noonan syndrome from two West German centres. Size at birth was normal in both sexes. In both males and females, the mean height followed along the 3rd per centile until puberty, but decreased transiently due to an approximately 2 year delay in onset of puberty. Final height approaches the lower limits of normal at the end of the 2nd decade of life. The mean adult height was found to be (n = 20) 162.5 cm in males and (n = 13) 152.7 cm in females, respectively. Smoothed means and standard deviations for height were derived. These data may be used for the statistical evaluation of height of Noonan syndrome patients. Except for mental retardation and microcephaly, which are more frequent in males, the relative frequencies of minor anomalies and malformations were found to be similar in both sexes. The characteristic non-cyanotic heart defects in the Noonan syndrome do not appear to have a major influence on growth. The auxological data were compared with those in the Ullrich-Turner syndrome.

In this study SLIT was accompanied by a significant placebo effect. Efficacy of treatment could only be seen in children with severe clinical symptoms and this became clinically marked after 3 years of therapy.

Patients with CAH and complete virilization have a high risk of being diagnosed late. There are major problems and uncertainties of the patients' families and the treating physicians concerning gender assignment. Gender identity is disturbed in some patients. In addition, multiple surgical procedures are necessary and short stature as well as central precocious puberty might be important to avoid late sequelae. While some surgical interventions are probably unavoidable, most of these issues could be resolved with an early diagnosis. Thus, especially for these patients, a neonatal screening programme for CAH would be of paramount importance.

The effects of different doses of oestrogens in constitutionally tall girls were evaluated in two centres for paediatric endocrinology. In one centre, 38 girls were treated with a high oestrogen dose of 0.3 to 0.5 mg ethinyloestradiol (EE) daily. In the other, 44 girls received a comparably low dose of 0.1 mg EE per day. Height prediction (HP), chronological age (CA), and height at the onset of treatment were comparable in both groups. Although the duration of treatment was significantly longer in those receiving the low dose, the cumulative oestrogen dose was still significantly lower. The dose of EE had no effect on final height reduction (high dose group: 4.9 +/- 2.6 cm, low dose group: 5.1 +/- 2.4 cm). Final height was more reduced in both groups when treatment was started at an early bone age (BA) (less than or equal to 13 years). No serious side effects were observed in either group, however weight gain was more pronounced in girls receiving the higher dose. We conclude that treatment of constitutionally tall girls with low doses of oestrogens is equally effective in reducing the final height as the usually administered high doses. The lowest effective dose has to be determined in a randomized, prospective clinical trial.

In vitro measurements were carried out to study the aggregation of erythrocytes in 33 children and young adolescents, three older adolescents, and 38 adults with type I diabetes. The aggregate formation of erythrocytes in stasis was increased in adult patients with both "good" and "poor" metabolic control when compared to control values. The aggregation of red cells in all children and the younger adolescents, both those under good and poor metabolic control, did not statistically differ from those of controls. Each of the three older adolescents showed an increased aggregate formation of their erythrocytes compared to controls. All adult patients under poor metabolic control exhibited various stages of diabetic retinopathy as shown by fluorescence angiography. Only one child under poor metabolic control showed an early stage of retinopathy (stage I according to Malone). The three older adolescents showed an increased aggregate formation of their erythrocytes. These patients exhibited also stage I of retinopathy. We suggest that the increased aggregation of erythrocytes of both adult and older adolescent patients may be one of the reasons for the development of diabetic retinopathy. Moreover, the normal erythrocyte aggregation of the diabetic children and younger adolescents could help to explain the rare occurrence of microangiopathies in childhood diabetes.

Hypothyroid goiter is a rare but well recognized complication following long term administration of iodine containing expectorants and disinfectants in children. Only few reports exist on iodine-induced hypothyroidism after a single injection of the iodized radiopaque dye Lipiodol. A 15-year-old boy with previously normal thyroid function is described who developed hypothyroid goiter within six weeks following bipedal lymphography. Urinary iodine excretion was extremely elevated up to 18 mg/day while serum concentrations of total thyroxine were below the euthyroid range and thyrotropin levels were elevated. After oral L-thyroxine treatment the goiter disappeared. Thyroid function remained normal when treatment was discontinued after five months although iodine excretion was still 50 times higher (2.5 mg/day) than in normal age matched children. The observed alterations of the thyroid gland were caused by a long lasting Wolff-Chaikoff effect with a delayed adaptation to high iodide concentrations.

Clinical, cytological, serological and endocrinological examinations were performed on 50 previously untreated children and adolescent with goitre. Three girls were found to have the cytological picture of Hashimoto's thyroiditis, in one instance associated with Turner's syndrome and hyperthyroidism. Two other girls presented with hyperthyroidism. In the remaining 45 patients thyroid aspirates and thyroid hormone levels were normal. The girl with Turner's syndrome, hyperthyroidism and Hashimoto's thyroiditis had thyroglobulin antibodies. It is the authors' experience that the diagnosis of Hashimoto's thyroiditis can be made only by needle biopsy of the thyroid. If iodination of cooking and table salt were to become widespread in the Federal Republic of Germany, and increased incidence of Hashimoto's thyroiditis is to be expected amount children with goitre.

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