ISSN:1663-2818    

DOI:10.1159/000191285

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000317375

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000317376

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000317377

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000317378

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Systematic screening for congenital hypothyroidism in the neonate constitutes a major progress in the prevention of mental retardation, as the condition occurs in 1/4,000 newborns and necessarily results in brain damage if not properly detected and treated during the first days of life. Screening and diagnostic and therapeutic procedures are discussed, as well as outcome and prognosis of the affected infants. Primary thyroid-stimulating hormone screening is almost universally recommended. Early therapy (within 14 days) with appropriate doses of thyroxine (about 10 μg/kg/day) will prevent any brain damage even in case of evidence of fetal hypothyroidism, as thyroxine of maternal origin will reach the fetus and largely protect him. Neonatal thyroid screening is also a particularly sensitive monitoring tool in the evaluation of the effects and of the correction of iodine deficiency at the population level

ISSN:1663-2818    

DOI:10.1159/000185485

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

A total of 195 children (117 males and 78 females) with idiopathic growth hormone deficiency (IGHD), treated with growth hormone (GH) for at least 1 year before puberty onset and who had completed treatment to adult height, were selected from the KIGS database for study of growth during puberty. Spontaneous and induced puberty started at 13.8 and 14.9 years in boys and at 12.9 and 13.7 years in girls, respectively. Duration of GH treatment and height gained prepubertally were greater when puberty was induced; prepubertal catch up growth (expressed as a percentage of the difference between target height and height at start of GH) was greater when puberty was induced in boys (59% induced vs. 45% spontaneous, p < 0.001), and in girls (72% induced vs. 53.9% spontaneous, p < 0.01). Final height was attained at 17.8 and 19.2 years in boys and at 16.0 and 17.0 years in girls following spontaneous and induced puberty, respectively. Final heights were greater after induced puberty compared with spontaneous puberty in boys (171.3 vs. 166.0 cm, p < 0.001) and in girls (157.0 vs. 155.0 cm, n.s.). Target height was also significantly greater in boys with spontaneous puberty (172.2 cm vs. induced = 174.2 cm) as compared to girls (spontaneous = 158 cm vs. induced = 160 cm). Duration of pubertal growth was longer in boys compared to girls (3.6 vs. 3.0 years, p < 0.001) and was negatively correlated with age, height, and distance from target height at onset of puberty, but was not correlated with the dose of GH. Catch-up growth during puberty (expressed as a percentage of the difference between target height and height at puberty onset) after induced and spontaneous puberty was 87.9% and 80.8% (not significant) in boys and 66.4% and 75.5% (not significant) in girls. Total pubertal growth (TPG) (cm) was inversely correlated with prepubertal growth by simple linear regression. Multiple linear regression analysis indicated 5 independent predictors of TPG accounting for 78% of the variability, namely sex (boys grew more), distance of target height from height at onset of puberty (+), dose of GH at onset of puberty (+), age at onset of puberty (-), and age at end of growth (+).

ISSN:1663-2818    

DOI:10.1159/000185487

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

To investigate any association between changes in lower leg length (LLL) and urinary growth hormone (uGH) excretion, 4 prepubertal children supplied daily 12-hour overnight urine samples and had daily knemometry performed for 4 weeks. The mean daily and weekly LLL velocity (LLLV) of the group was 0.08 mm/day (95% CI 0.01-0.18) and 0.52 mm/week (range 0.38-0.78), respectively. The mean uGH excretion was 8.9 ng/l (CI 3.7-13.3), and the mean intrasubject coefficient of variation of uGH was 55% (range 32-93). The tallest subject who also had the highest LLLV excreted the least amount of uGH (mean 3.7 ng/l, CI 2.9-4.5). No temporal relationship was evident between daily uGH excretion and LLL changes. There was no evidence of any association between amount of uGH excreted and LLLV. There remains some doubt on the usefulness of uGH measurement as the sole predictor of normal GH production.

ISSN:1663-2818    

DOI:10.1159/000185489

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000317381

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

A sporadic case of multiple endocrine neoplasia type I with coexisting insulinoma and hyperparathyroidism was investigated in vivo and in vitro. The insulinoma was localized by somatostatin receptor scintigraphy and these receptors were functionally active. Octreotide administration decreased the basal insulin and glucagon secretion by 90 and 46%, respectively. Immunocytochemistry of the insulinoma tissue was positive for insulin, chromogranin A and neuropeptide Y. The insulinoma cells were also isolated and cultured in vitro. Incubation experiments revealed that a low glucose concentration (1 mmol/l) was sufficient to increase cytosolic free calcium and to produce a maximal glucose-induced insulin release. Northern blot analysis of RNA obtained from the tumor showed a high abundance of the low Km glucose transporter GLUT1 but no transcript for the high Km glucose transporter GLUT2. The abnormal distribution of glucose transporters probably relates to the abnormal glucose sensing of insulinoma cells, and explains their sustained insulin secretion at low glucose concentrations. Whether these abnormalities share a pathogenetic link with the presence of functionally active somatostatin receptors remains to be elucidated.

ISSN:1663-2818    

DOI:10.1159/000185490

出版商:S. Karger AG    

年代:1997

数据来源: Karger