Objectives: We aimed to evaluate the factors influencing true adult height (HT) after long-term (from 1987 to 2000) GH treatment in Ullrich -Turner syndrome (UTS) based on modalities conceived in the 1980s. Design: Out of 347 near-adult (.16 years) patients from 96 German centres, whose longitudinal growth was documented within KIGS (Pharmacia International Growth Database), 188 (45, X ¼ 59%; bone age .15 years) were available for further anthropometric measurements. Results: At a median GH dose of 0.88 (10th/90th percentiles: 0.47/1.06) IU/kg per week, a gain of 6.0 (2 1.3/+13) cm above the projected adult height was recorded. Variables were recorded at GH start, after 1 year GH, puberty onset, and last visit on GH therapy. At these visits, the median ages were 11.7, 12.7, 14.2, 16.6 and 18.7 years; and median heights, 0.4, 1.1, 1.7, 1.7 and 1.3 SDS (UTS) respectively. Height gain (DHT) after GH discontinuation was 1.5 cm. Total DHT correlated ðP , 0:001Þ negatively with bone age and HT SDS at GH start, but positively with DHT after the first year, DHT at puberty onset, and GH duration. Final HT correlated ðP , 0:001Þ positively with HT at GH start, first-year DHT, and HT at puberty onset. Body mass index increased slightly ðP , 0:05Þ; with values at start and adult follow-up correlating highly ðR ¼ 0:70; P , 0:001Þ: No major side effects of GH occurred. Conclusions: GH dosages conceived in the 1980s are safe but too low for most UTS patients. HT gain and height are determined by age and HT at GH start. Height gain during the first year on GH is indicative of overall height gain. After spontaneous or induced puberty, little gain in height occurs.

Background: On clinical grounds, arachnoid cysts are usually associated with neurological dysfunction. There is little information concerning their involvement in endocrinological disorders. Patients: The experience in 6 children (birth to 12 years) with hypothalamic-pituitary disturbances secondary to the presence of intracranial arachnoid cysts is reported and the literature is reviewed. Results: Three of our children were diagnosed with isolated hormone abnormalities (2 children with precocious puberty and 1 child with growth hormone, GH deficiency). One child presented the unusual combination of GH deficiency and precocious puberty. The remaining 2 children developed panhypopituitarism associated with diabetes insipidus. Conclusion: Arachnoid cysts may cause a wide spectrum of endocrinological disorders. Periodical and complete follow-up of every patient is recommended.

Elevated plasma homocysteine is a risk factor for cardiovascular disease and a sensitive marker of inadequate vitamin B12 and folate status. We studied 257 pupils (120 boys, 137 girls, aged 6-17 years) and their parents (88 males, 172 females, aged 26-50 years). Our measurements were part of a national Bavarian health and nutrition examination survey evaluating cardiovascular risk factors. A mild hyperhomocysteinemia (Hcys >15 micromol/l) occurred in 7% of the adults, but in none of the children. Men had significantly higher Hcys levels than women (p<0.0001), boys and girls had comparable concentrations. For adults and children, Hcys correlated inversely with vitamin B12 and folate and positively with the lean body mass and creatinine in serum, but not with cystatin C. Genetic and nutritional factors are determinants of Hcys metabolism. The correlation of Hcys and serum creatinine is dependent on the metabolic link between Hcys production and creatine synthesis.

SOD is characterized by optic nerve hypoplasia and a variety of endocrine deficiencies. In addition, forebrain malformations are present in most SOD patients. Hormonal disorders are present in some SOD patients which may be of hypothalamic origin and need to be investigated systematically.

Our retrospective analysis of growth and pubertal development includes 109 children and adults with meningomyelocele (MMC) (52 M, 57 F) aged 3.2-21.0 years (median 8.9 years). Anthropometric data, growth-retarding factors and data on pubertal development were analysed in comparison to the normal population using standards from Prader et al. (1). The results (mean +/- SD) were as follows. Fifty patients (46.8%) had short stature (height SDS for chronological age (SDS CA) < -2). The supine length was influenced by the level of the lesion (height SDS CA: > or = L2 -3.13 +/- 1.62, < or = S2 -0.46 +/- 1.27), ambulatory status, skeletal deformities and pubertal stage. The mean adult height (n = 15, age 16.1-21.0 years) measured 141.3 cm for women (height SDS CA -3.83 +/- 1.79) and 159.2 cm for men (height SDS CA -2.27 +/- 1.81). In 82.6% of the subjects (n = 90), arm spans were within the normal range. Reduced arm spans (SDS < -2) as found in 19 patients (17.4%) with short stature (mean height SDS CA -3.29 +/- 1.29) may be caused by factors other than neurological lesions and skeletal deformities, and require further endocrinological studies. Out of 27 pubertal patients, central precocious puberty was diagnosed in five girls. The stages of puberty in MMC girls developed earlier than expected for the age-related group.