Acta paediatrica Scandinavica. Supplement最新文献

Boys with idiopathic GH deficiency, treated with hGH and entering spontaneous puberty, have an onset of puberty and peak height velocity (PHV) at a late chronological age but normal bone age. PHV occurs at G3 with testicular volumes of 6-12 ml. The size of PHV and the height gain after G2 are similar to those of a normal delayed adolescent. In contrast, idiopathic GH deficient girls have an onset of puberty and PHV nearer to a normal chronological age and at an early bone age. PHV occurs at B2 and its size and the height gain after B2 are similar to those of normal girls. The length of time of pubertal growth is shorter in both GH deficient boys and girls. Very late induction of puberty in idiopathic GH deficient boys results in psychosocial damage and in bodily disproportion. It is suggested that induction of puberty be considered no later than 14.5 years in boys and 13.5 years in girls with the use of low-dose sex steroids. The decision to induce puberty should be taken to avoid psychosocial problems and be independent of proof of associated gonadotrophin deficiency. In GH deficient girls with early puberty, therapies to delay puberty may be considered. There are theoretical grounds for increasing the GH dose given during puberty, but present dose-response studies fail to include controls for important biological variables and are so far inconclusive. Cost-effectiveness is an important consideration. Increasing the frequency of injections probably improves the growth effect for a given dose of GH.

In this French study with recombinant somatropin, the stimulation of growth in 32 prepubertal (age 10.0 +/- 3.5 years; mean +/- SD) and 19 pubertal (age 14 +/- 1.5 years) GH deficient children was compared; the stimulation of growth was similarly good in the two groups. The height velocity SD scores increased from -2.5 +/- 1.7 and -0.9 +/- 1.5 to 2.2 +/- 1.9 and 1.6 +/- 1.6 in prepubertal and pubertal children, respectively. Expressed as cm/year, these correspond to increases from 3.2 +/- 1.3 cm/year and 4.1 +/- 1.2 cm/year to 8.1 +/- 1.5 cm/year and 8.6 +/- 1.9 cm/year in the prepubertal and pubertal patients, respectively. Safety and tolerance were good and the immunogenicity of Genotonorm was low.

Treatment of precocious puberty of central origin is aimed at controlling the development of sexual characteristics and improving final height. Increased growth rate is one of the major clinical symptoms, accompanied by an even more marked advance in bone age. Medroxyprogesterone acetate and cyproterone acetate have provided almost satisfactory control of pubertal characteristics, but with accompanying adrenal insufficiency. The data with regard to growth and bone maturation are contradictory. LHRH analogues have recently become available, and provide good control of gonadotrophin secretion. In a series of 21 cases (13 girls, 8 boys), a significant decrease in growth rate was achieved in both sexes with an LHRH analogue, with a significant increase in the height age/bone age ratio; control of gonadal secretions was also obtained. These results are only preliminary, but provide hope that the final height of these children will be improved.

In this follow-up study 497 twins from 335 twin deliveries were examined between the ages of 12-20 years to ascertain late effects of perinatal complications and mild neurological abnormalities on mental health, by means of questionnaires filled in by the parents and by the twins themselves. One third of the twins had not suffered from any perinatal complications, while one third had been small for gestational age, one third had been born prematurely and one third had suffered from respiratory disorders. Neonatal hypoglycaemia had been found in 22% and hyperbilirubinaemia in 7% of the twins. Perinatal complications had occurred in almost all those twins who later underwent inpatient psychiatric treatment. Most perinatal problems were reflected in the twin's dependency on his co-twin, showing in particular a cumulative effect when occurring simultaneously. Those twins who are currently more dependent learned to walk and speak later than the non-dependent ones, and their EEG background activity of ten years ago, at the age of 2-10 years, was more abnormal than that of the non-dependent ones, indicating brain organic, i.e. probably perceptual, factors enhancing the development of inter-twin dependency. The dependent twins were more submissive and had the most frequent feelings of inferiority. The neurological, psychological and family interactional factors that cause a twin to distrust himself and depend on his co-twin are also discussed in this study.

Linear growth was analysed in 438 boys and 483 girls aged from 6 years to 17 years. Annual records of each subject were fitted by using a cubic spline function. Parameters characterizing the growth process, such as peak height velocity (PHV), age at PHV, and age at onset of the pubertal growth spurt (PGS), were calculated directly from the estimated curves. The mean age at PHV was 11.2 years in girls and 13.0 years in boys. The mean PHV was 8.7 cm/year in girls and 10.3 cm/year in boys. Age at PHV showed a significant negative correlation with PHV and total pubertal gain, and a significant positive correlation with height at onset of the PGS, age at onset of the PGS and height at PHV. When subjects were divided into three groups (early, average and late pubertal maturation) according to the age at PHV, no significant difference in mean final height was seen between these groups, though a significant difference in height was observed at 6 years of age. When the results were adjusted for the difference in height at 6 years, the age at PHV showed a significant positive correlation with final height. It is concluded that age at PHV influences the final height.

Mother's depression during postpartum is rather common and it has an impact on the mother-child relationship. As breast-feeding is an important situation for the early mother-child interaction, the impact of mother's depression on her breast-feeding and nursing attitudes and experiences was decided to be studied. In this pilot study 119 healthy primipara mothers filled in Beck's Depression Inventory, breast-feeding and childrearing attitude scales and other questionnaires. The prevalence of depression was 8%. The depressed mothers had more difficulties during breast-feeding than other mothers. The attitudes of the depressed mothers were more positive during pregnancy, but more negative during breast-feeding than those of the non-depressed mothers. The results might be clinically meaningful, although we need more detailed information about the correlations between depression and breast-feeding experiences.

Cystic fibrosis (CF) is a chronic genetic disease mainly affecting the exocrine glands. Its main clinical symptoms are: abnormal production of mucus which blocks the airways, pancreatic insufficiency and increased sweat electrolytes. In the present investigation a series of enzymes and metabolites--mainly of carbohydrate metabolism--was investigated in biological fluids from CF homozygotes, CF heterozygotes and healthy controls under different conditions. CF homozygotes--and to a lesser degree CF heterozygotes--had increased activity of ribonuclease, and increased concentrations of electrolytes and lactate in their saliva and urine at rest. Saliva of CF patients also had augmented levels of protein. When healthy persons were submitted to anaerobic effort, the activity of ribonuclease and the concentrations of protein, electrolytes and lactate increased in their saliva, and thereby, mimicked the values found in the saliva of cystic fibrosis patients at rest. An abnormal response to a sucrose load was found in both CF-homozygotes and CF-heterozygotes. Greater increase in both glucose and lactate concentrations in the blood as well as a more rapid clearance of these metabolites was observed after the sucrose intake. A possible cause for these findings could be a disturbed carbohydrate metabolism in CF. Therefore, enzymes and metabolites connected to the metabolic pathway of glucose were investigated in CF fibroblasts and in animal models for CF. An increased activity of glycolytic enzymes in CF fibroblast were shown. Similar increases in activities of glycolytic enzymes were found in cells of submandibular glands of rats that were killed in a state of induced metabolic acidosis. These cells also showed increased protein and mucus contents, and elemental changes similar to those observed in fibroblasts of cystic fibrosis patients. Contrary to this, decreased activities of the enzymes of the glycolytic pathway were found in the submandibular gland of chronically reserpinized rats. The end-products of glycolysis, pyruvate and lactate, were also decreased, whereas the concentration of phosphoenolopyruvate and creatinphosphate were increased, possibly causing acidosis in the gland. Thus a disturbed glycolytic pathway in CF cells and a decreased intracellular pH might play an important role in the pathogenesis of this disease.