摘要:

It has been suggested recently that in congenitally hypothyroid infants with organification defect there is a maternal-fetal transfer of thyroxine (T4). The present study was conducted to evaluate how effective the maternal-fetal transfer is and whether the maternal T4 can prevent intrauterine hypothyroidism. The clinical, laboratory and radiological data on 271 full-term infants with persistent primary congenital hypothyroidism, detected by the national screening program, were used to assess the degree of in utero hypothyroidism. For 6 out of 50 athyroid infants, two pretreatment blood samples spotted on filter paper were available for calculating the T4 disappearance rate. Most infants with agenesis of the thyroid had very low T4 and very high levels of thyroid-stimulating hormone compared to infants with ectopic thyroid. In the athyroid infants the initial T4 declined to low and undetectable levels. Bone maturation was significantly delayed while the clinical symptomatology was more prominent in the athyroid congenital hypothyroid infants, as compared with the ectopic thyroid infants. In conclusion, there is some maternal-fetal transfer of T4. However, this transfer is insufficient to suppress the fetal levels of thyroid-stimulating hormone and prevent intrauterine hypothyroidism.

ISSN:1663-2818    

DOI:10.1159/000182686

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Twenty-five short children (6F, 19M) with intrauterine growth retardation were treated with daily subcutaneous injections of biosynthetic human growth hormone for 4 years. The treatment was commenced at a chronological age of 6.3 years (range 2.1-9.8). Eighteen of the patients had major dysmorphic signs of Russell-Silver syndrome. During the first year they were randomised into two groups treated with either 15 or 30 IU of growth hormone/m2/week. The higher dose was administered to all the children after the first year of the trial. After the initial increase in growth velocity SDS (greater in those treated with the higher dose regimen), there was a progressive decrease although with values significantly higher than pretreatment levels (p < 0.02) in both groups. However there was no improvement in height SDS for bone age after 4 years. The triceps and skinfold thickness showed a decrease during the first years followed by a gradual increase. The body mass index improved during the 4 years although showing no difference between the two treatment groups. There was no alteration in thyroid function or metabolic indices (glucose or lipid homeostasis) during the study.

ISSN:1663-2818    

DOI:10.1159/000182687

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We investigated the relationship between urinary growth hormone (u-GH) and spontaneous 24-hour plasma GH secretion in 15 acromegalic patients. To measure u-GH, we have developed a method based on concentrating the sample by centrifugal ultraflltration and then performing an immunoradiometric assay using commercially available reagents. u-GH correlated well with the integrated concentration of plasma GH (r = 0.66, p < 0.02). Additionally, u-GH excretion in acromegalic patients was significantly higher than in the control group (190 ± 100 vs. 3.89 ± 0.56 pg/min, mean ± SEM, p < 0.001). Immunoreactive u-GH showed the same elution pattern in Sephadex G-75 as standard or labeled hGH, proving that the substance measured in urine is authentic GH. In conclusion, u-GH appears to be a simple, noninvasive and inexpensive test for evaluating GH secretion in active acromega

ISSN:1663-2818    

DOI:10.1159/000182688

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

In order to assess the value of urinary growth hormone (GH) as a reflection of central GH release, 42 prepubertal children with short stature and without organic disease were studied. A nocturnal GH profile and L-dopa and GH-releasing hormone (GHRH) tests were performed. Urinary GH was measured by means of a direct immunoradiometric method we have developed, using two monoclonal antibodies. Nocturnal urinary GH values correlated positively with plasma GH values expressed as the area under the curve (r = 0.76; p = 0.0001) or mean peak amplitude (r = 0.73; p = 0.0001). Also, urinary GH values correlated positively with peak plasma GH levels during the GHRH test (r = 0.64; p = 0.001). In contrast, no correlation was observed between peak plasma GH and urinary GH during the L-dopa test (r = 0.29; p = 0.11). This suggests a specific but as yet undetermined effect of L-dopa on urinary GH secretion.

ISSN:1663-2818    

DOI:10.1159/000182689

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Ten girls with early puberty secondary to cranial irradiation as a part of the treatment for acute lymphoblastic leukaemia (ALL) were treated with either gonadotropin-releasing hormone analogue (GnRHa) and human growth hormone (GH) (8 girls) or with GnRHa alone (2 girls). After 4 years of treatment, height SDS for bone age was improved in the group who received combined treatment (from -0.97 to +0.07, p < 0.001), in contrast to the 2 patients who received GnRHa alone in whom height standard deviation scores for bone age decreased (from -1.10 to -1.33). Sitting height in all patients was relatively shorter than leg length, and there was no significant alteration during the 4 years of treatment.

ISSN:1663-2818    

DOI:10.1159/000182690

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

So far, few studies have addressed the regulation of GH and GH-dependent growth factors in adult patients with Turner syndrome. We therefore studied a group of 25 genetically proven patients with Turner syndrome (age 20-50 years) and 10 control women (25-48 years). Turner patients were significantly shorter (148.7 ± 1.1 cm vs. 169.1 ± 2.3 cm; mean ± SE; p < 0.0001) and more overweight [body mass index (BMI)] 25.6 ± 1.3 vs. 21.4 ± 0.6 in controls; p < 0.01). No significant differences were present when the integrated GH response to stimulation with arginine and the serum levels of GH-binding protein (GH-BP), IGF-I, IGF-II and binding protein 3 for IGFs (IGFBP-3) were compared between the two groups. However, more detailed analysis revealed significant abnormalities of the somatotropic axis in Turner patients. Pituitary GH secretion was negatively and serum GH-BP positively related to the degree of overweight in normal patients. In Turner patients, no such relationship was present, while IGF-II significantly increased with BMI. IGFBP-3 was positively related to adult height in normal women but not in Turner patients. While serum testosterone values did not affect any of the somatotropic parameters measured, there was a previously unreported, inverse relation between serum estradiol and GH-BP in controls but not in Turner patients. While adult patients with Turner syndrome do not display endocrine features of GH insufficiency, a detailed analysis reveals several abnormalities of the interrelation between anthropometric parameters, sex steroids and the pituitary-somatomedin

ISSN:1663-2818    

DOI:10.1159/000182691

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The effects of GH therapy on glucose metabolism in 72 Turner patients treated with human GH (HGH) 2, 3 or 4 IU/m2/day for 2 years are reported. OGTTs were performed at 0, 3, 12 and 24 months. The overall frequency of glucose intolerance was 9.7% before therapy and did not change under HGH. No change in HbAlc and fasting glucose values occurred. Integrated blood sugar values in the OGTT (area under the curre) did not change with 2 and 3 IU but were significantly elevated over control after 2 years with 4IU. Insulin secretion was not significantly affected over time with 2 IU, whereas 3 and 4 IU produced significant increases which persisted after 2 years. Results indicate that glucose homeostasis is maintained under GH therapy at the expense of a compensatory increase in insulin secretion which persists at higher GH dosages.

ISSN:1663-2818    

DOI:10.1159/000182692

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Growth hormone-binding protein (GHBP) was studied in 11 girls with true precocious puberty, aged 7.3 ± 0.2 years (mean ± SE), before and after the first 6 months of treatment with luteinizing hormone-releasing hormone analogue D-Trp6-LHRH. The 125I-human GH was incubated with 150 µl of serum, bound and free GH were separated by gel filtration. The levels of GHBP increased significantly from 24.2 ± 1.3 to 28.1 ± 1.9% (p < 0.002, paired t test), more than expected for the normal age-dependent increase. The efficiency of LHRH-A therapy was confirmed by a decrease in growth rate and normalization of clinical and biological parameters. Our data agree with the hypothesis that the pubertal spurt is mediated by a sex-steroid-induced rise in GH concentration, and they suggest that the levels of GHBP may be related to the GH secretion and its variation with treat

ISSN:1663-2818    

DOI:10.1159/000182693

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We describe 5 children, 4 girls, aged 4-14 years with evolving hypothalamic-pituitary dysfunction. They had presenting features, isolated or combined, of polyuria and polydipsia (n = 3), weight gain and hyperphagia (n = 3), and growth failure (n = 1). During periods of 1-5 years per child, the following abnormalities developed: diabetes insipidus (n = 5), osmoreceptor dysfunction (hypernatraemia with absent thirst) (n = 3), hyperprolactinaemia (n = 3), growth hormone (GH) deficiency (n = 4, of whom 3 had normal linear growth), ACTH deficiency (n = 2), TSH deficiency (n = 2) and precocious puberty (n = 1, female). In 2 patients, high-resolution CT scans and MRI showed structural lesions of the hypothalamus 1.5 and 3.5 years after presentation. These were inaccessible and not biopsied. Scans in the remainder were normal. In conclusion, weight gain, impaired thirst, and hyperprolactinaemia were early features of evolving hypothalamic-pituitary dysfunction, and occurred with diabetes insipidus, accompanied by progressive anterior pituitary deficiencies. Pituitary hormone replacement with clinical and neuroradiological surveillance is important in any child with symptoms suggestive of an evolving hypothalamic lesion.

ISSN:1663-2818    

DOI:10.1159/000182694

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We evaluated the GH response to combined administration of pyridostigmine (PD), a cholinergic agonist, and GH-releasing hormone (GHRH) (60 mg PD given orally 60 min before the GHRH bolus) as well as baseline IGF-I concentrations in 10 patients (5 males and 5 females, age 6.0-24 years) with Prader-Labhard-Willi (PLW) syndrome, 8 prepubertal obese children (4 males and 4 females, age 5.6-12.0 years) and 9 prepubertal short normal children (7 males and 2 females, age 8.0-12.8 years). Mean GH responses to PD + GHRH were significantly lower (p < 0.0001) in the PLW patients (13.8 ± 3.3 µg/l) than in the short normal children (52.2 ± 9.0 µg/l) and similar to those of the obese children (14.3 ± 3.2 µg/l). Mean serum IGF-I levels were significantly lower (p < 0.05) in the PLW patients (117.5 ± 26.4 µg/l) than in the obese (329.3 ± 88.0 µg/l) and the short normal children (214.3 ± 38.3 µg/l). Two of the PLW patients had absent GH responses to PD + GHRH associated with subnormal IGF-I concentrations, indicating pituitary GH deficiency. When these 2 cases were excluded from the statistical calculation, mean peak GH responses to PD + GHRH remained significantly lower (p < 0.0001) in the PLW patients (17.1 ± 3.0 µg/l), while their mean serum IGF-I concentrations (143.4 ± 71.5 µg/l) were not significantly different from those of the other two groups. These results indicate that patients with the PLW syndrome have a reduced or absent GH secretory reserve associated in some cases with low levels of IGF-I. Whether these findings are involved in the pathogenesis of their short stature remains

ISSN:1663-2818    

DOI:10.1159/000182695

出版商:S. Karger AG    

年代:1993

数据来源: Karger