We have compared the growth and the body composition in children with Prader-Willi syndrome (PWS) with and without growth hormone treatment (recombinant GH 0.1 IU/kg/day) after a 1-y period. Twenty-nine prepubertal children with PWS, with mean body mass index (BMI) SDS of 2.2, and 10 (control) healthy obese children with mean BMI SDS of 5.6, underwent 24-h frequent blood sampling. Both PWS and control obese children had low and similar GH levels (0.7 microg/l +/- 0.4SD). Serum IGF-I levels, however, were significantly lower in children with PWS (-1.5SDS +/- 0.8SD vs -0.2SDS +/- 0.8SD). The 29 PWS children were randomized into 2 groups of 15 and 14 subjects for GH treatment and no treatment, respectively. Height velocity increased from -1.9SDS to + 6.0SDS in the treated group (p < 0.001) and decreased from -0.1SDS to -1.4SDS in the control PWS group during the study year. BMI decreased significantly for the treated group (+3.0SDS to +2.0SDS). Relative fat mass decreased significantly, while fat-free mass increased (p < 0.001) for the treated group. No significant changes were noticed in body composition in the control PWS group. In conclusion, the low spontaneous 24-h GH secretion, regardless of body weight, and the exceptional response to growth hormone treatment together with the finding of low IGF-I levels suggest that growth hormone deficiency is a common feature of PWS, as a result of hypothalamic dysfunction. Treatment with growth hormone is beneficial for the majority of PWS children.

The reliability of gray-scale ultrasonography in diagnosing torsion of the testicular appendages was studied in a series of 54 boys with acute non-traumatic scrotal pain. All boys were operated upon, and the appendages extirpated irrespective of their appearance at exploration; the final diagnosis was based on histological examination. Forty-two boys had appendicular torsion, 2 had testicular torsion and 10 had other diagnoses. Using the sign of appendicular torsion--an echogenic extratesticular structure situated between the head of the epididymis and the upper pole of the testis--as the criterion, 37 displayed a true positive, 9 a true negative, 3 a false positive and 5 a false negative diagnosis. Thus, the sensitivity was 88%, the specificity 75% and the positive predictive value 93% respectively. The echogenic mass varied in size from 3 to 17 mm in diameter. In 34 of the 42 cases of appendicular torsion extratesticular fluid was present, and 19 patients showed enlargement of the head of the epididymis. It is concluded that gray-scale sonography is an accurate and valuable tool in diagnosing torsion of the testicular appendages.

We have compared the growth and the body composition in children with Prader‐Willi syndrome (PWS) with and without growth hormone treatment (recombinant GH 0.1 IU/kg/day) after a 1‐y period. Twenty‐nine prepubertal children with PWS, with mean body mass index (BMI) SDS of 2.2, and 10 (control) healthy obese children with mean BMI SDS of 5.6, underwent 24‐h frequent blood sampling. Both PWS and control obese children had low and similar GH levels (0.7 /ng/l ± 0.4 SD). Serum IGF‐I levels, however, were significantly lower in children with PWS (‐1.5 SDS ± 0.8 SD vs ‐0.2 SDS ±0.8 SD). The 29 PWS children were randomized into 2 groups of 15 and 14 subjects for GH treatment and no treatment, respectively. Height velocity increased from ‐1.9 SDS to + 6.0 SDS in the treated group (p < 0:001) and decreased from ‐0.1 SDS to ‐1.4 SDS in the control PWS group during the study year. BMI decreased significantly for the treated group (+3.0 SDS to + 2.0 SDS). Relative fat mass decreased significantly, while fat‐free mass increased (p < 0:001) for the treated group. No significant changes were noticed in body composition in the control PWS group. In conclusion, the low spontaneous 24‐h GH secretion, regardless of body weight, and the exceptional response to growth hormone treatment together with the finding of low IGF‐I levels suggest that growth hormone deficiency is a common feature of PWS, as a result of hypothalamic dysfunction. Treatment with growth hormone is beneficial for the majority of PWS children.

Lindgren AC, Hagenas L, Miiller J, Blichfeldt S, Rosenborg M, Brismar T, Ritzdn EM, in collaboration with the Swedish National Growth Hormone Advisory Group. Effects of growth hormone treatment on growth and body composition in Prader-Willi syndrome: a preliminary report. Acta Paediatr 1997; Suppl423:60-2. Stockholm. ISSN 0803-5326 A controlled, randomized study was conducted to assess the effect of growth hormone (GH) treatment on growth, body composition and behaviour in prepubertal children (3-12 years of age) with Prader-Willi syndrome. GH treatment was given to one group of 15 patients (group A) at a dose of 0.1 IU/kg/day for 2 years. The second group (group B; n = 12) was not treated for the first year and was then given GH at a dose of 0.2 IU/kg/day for the second year. All patients had low 24-hour levels of GH and insulin-like growth factor I before GH treatment. Height velocity SDS increased from -1.9 2.0 to 6.0 f 3.2 during the first year of GH treatment in group A, and from -1.4 f 1.2 to 10.1 f 3.9 in the second year of the study in group B. When GH treatment was stopped, height velocity declined dramatically. Height SDS followed a similar pattern. GH treatment reduced the percentage body fat and increased the muscle area of the thigh. Isometric muscle strength was also increased. In addition, GH treatment appeared to have psychological and behavioural benefits, which were reversed after cessation of treatment. It was concluded that GH treatment improves growth, body composition and behaviour in children with Prader-Willi syndrome. 0 Prader-Willi syndrome, growth hormone treatment, growth, body composition, behaviour