摘要:

The diagnosis of nesidioblastosis was established in a 9-month-old male child with a history of recurrent convulsive seizures and hypoglycemia. After unsuccessful subtotal pancreatectomy, treatment was started with the long-acting somatostatin derivative Sandostatin® (Octreotide, Sandoz) at a dosage of 25 µg t.i.d. spaced between carbohydrate-enriched meals. With this regime, blood glucose was maintained at the low normal range and seizures ceased. During a 30-month observation period, growth velocity and weight progression were well within the predicted limits. A 24-hour hormone profile recorded at the end of the observation period revealed the following: (1) failure to improve blood glucose with carbohydrate-enriched food due to reactive hyperinsulinemia; (2) hyperglycemic reaction after administration of Sandostatin caused by a reduction of plasma insulin; this effect was particularly marked during sleep; (3) low mean GH, decreased spiking frequency and reduced area covered by the nocturnal peaks by recognized standards, and (4) normal somatomedin C levels for age. Interpretation of growth hormone (GH) data is hindered by the lack of pertinent information from the patient’s age group. Recording of normal growth progression in the case illustrated here can only be explained by the capability of a reduced GH secretory rate to maintain full biological activity as shown by the normal plasma level of somatomedin C. Indeed, recent evidence has been provided elsewhere for normal growth progression in the presence of low GH secretion, although other factors unrelated to this hormone may also be operative at this early age. Further reports concerning the treatment of non-GH-dependent conditions with somatostatin derivatives will certainly contribute to the better understanding of the mechanisms governing growth in the postnatal per

ISSN:1663-2818    

DOI:10.1159/000181907

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

We compared the final adult height (FH) of patients with classic constitutional delay of growth and puberty with their target height (TH) and with the height prediction by the Bayley-Pinneau method (BP). 20 patients and their parents were included in our study: 6 females (mean age 19.1 years) and 14 males (mean age 20.6 years). No significant difference could be detected between TH, FH and BP prognosis. This is in contrast to recent studies using height data partly obtained by self-estimation. We measured our patients and their parents ourselves and were accurately able to calculate their genetically determined TH. This proceeding could explain our results. Our study shows that adolescents with true constitutional delay do not need treatment and that height prediction seems to be accurate.

ISSN:1663-2818    

DOI:10.1159/000181908

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

We document a male infant with congenital panhypopituitarism as detected at birth, in whom the adenohypophysis was totally absent by magnetic resonance imaging and all the anterior pituitary hormones were undetectable. His neurohypophysis was, by contrast, identified ectopically at the median eminence and antidiuretic hormone was appropriately secreted.

ISSN:1663-2818    

DOI:10.1159/000181909

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

22 girls with Turner syndrome aged 10.8 ± 2.4 years with bone age 8.58 ± 1.32 years, randomized in two groups, were treated for 3 years with either growth hormone (GH), 0.1 U/kg daily (group A), or GH, 0.1 U/kg, plus oxandrolone, 0.06 mg/kg (group B). This resulted in a sharp increase in growth rate for the first year of treatment, followed in the second and third years by a growth rate near to the normal mean for age. The growth velocity was better in group B, the difference being significant during the first year only. After 3 years, the predicted adult height had increased by 2.1 cm as a mean in group A and by 4.5 cm in group B, with important individual variations, resulting in a gain of at least 3 cm in 3/10 patients of group A and 9/12 of group B. No metabolic or other side effects occurred. These 3-year data confirm that GH improves the predictable height in Turner girls. They suggest that it may be useful for at least 3 years and that adding a small dose of oxandrolone for 2 years in girls aged more than 8 years could be of good practice. However, earlier and more protracted treatment with GH has to be studied with the hope to better improve the predictable adult heigh

ISSN:1663-2818    

DOI:10.1159/000181910

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

Twelve female volunteers from Berlin and 9 from Stockholm, all using a contraceptive pill (30 µg ethinyl estradiol and 150 µg levonorgestrel), received an intramuscular injection of estriol (E3; 1 mg in oil) on day 5 of withdrawal bleeding. Blood samples were collected at increasing time intervals during 4 weeks. Three months later, on day 5 of their withdrawal bleeding, 6 women were given intramuscularly (in oil) estriol 3,17-dipropionate (E3-prop) and 15 women estriol 3,17-dihexanoate (E3-hex). The doses were equivalent to 5 mg of estriol, i.e. 6.94 and 8.90 mg, respectively. Blood samples were collected during a period of 9 weeks. Estriol was analyzed by radioimmunoassay in all plasma samples. The average half-life of E3 ranged from 1.5 to 5.3 h after the administration of E3. It was 12.7 h and between 187 and 221 h after the administration of E3-prop and E3-hex, respectively. The average areas under the curve (in nmol 1-1·h) of E3 were between 82.5 and 161 after the administration of E3-prop or E3-hex, and between 27.1 and 37.9 when E3 had been given. As E3 was administered in a 5-fold lower dose than the esters, the areas under curve appeared to be comparable. Thus, the total exposure to E3 seemed to be almost independent of the type of E3 derivatization, while the time and intensity of exposure were very differe

ISSN:1663-2818    

DOI:10.1159/000181911

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

Arginine vasopressin (AVP) concentrations were determined in plasma and in cerebrospinal fluid (CSF) in 8 adult male patients suffering from hydrocephalus of various etiologies, before and after intravenous administration of 10 mg metoclopramide. Metoclopramide was able to increase the plasma (2.6 ± 0.2 ng/l in basal conditions and 6.1 ± 0.6 ng/l at 30 min) but not the CSF AVP levels. The results suggest that the neurons which secrete AVP into the CSF may be functionally different from those secreting into the peripheral circulatio

ISSN:1663-2818    

DOI:10.1159/000181912

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

The effect of norethindrone acetate (NET-Ac) and ethinyl estradiol (EE2) on the 3β-hydroxysteroid dehydrogenase (HSD)-delta5-isomerase complex of the human fetal testis was studied by administration of 20 mg NET-Ac and 0.04 mg EE2 p.o on a single day to 4 women, pregnant 10–16 weeks, before abortion was induced, the other 4 patients serving as controls. Testosterone and androstenedione formation from radioactive dehydroepiandrosterone was measured in 8 fetuses by incubation of testicular tissue in vitro. The presence of normal fetal Leydig cells was confirmed by electron microscopy. There was no difference between the enzyme activities of testicles in the experimental and control groups. The findings give values of 3β-HSD-isomerase activity in human fetal testis and suggest that the steroidogenic function of the fetal testis exposed for a short time to normally used contraceptive steroids remains at the same magnit

ISSN:1663-2818    

DOI:10.1159/000181913

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

Epidermal growth factor (EGF) and insulin-like growth factor I (IGF-I) synergistically stimulate placental lactogen (hPL) secretion by placental cells. To understand the mechanism of actions we have investigated a possible heterologous regulatory effect of EGF and IGF-I on each other’s receptors. Pretreatment of the cells with IGF-I had no effect on [125I]-EGF binding or the down-regulation of EGF receptor. Pretreatment of the cells with EGF, concomitantly with IGF-I, had no effect on [125I]-IGF-I binding but it augmented the IGF-I down-regulation of IGF-I receptor. The time required to initiate the IGF-I-induced down-regulation of IGF-I receptor was reduced by 4 h in the presence of EGF. IGF-I-down-regulated decreased (p < 0.05) receptor numbers were further decreased (p < 0.05) in the presence of EGF. These results suggested that the synergistic effect of EGF and IGF-I seen in hPL secretion by placental cells is not due to direct heterologous hormone-receptor interactive effects. However, the effects seen may be due to a differentiating effect of EGF sensitizing the cells for responsiveness to IGF-

ISSN:1663-2818    

DOI:10.1159/000181914

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

There are reports that both interleukin-1 β and interleukin-6 (IL-6) stimulate the release of adrenocorticotropin through stimulation of hypothalamic corticotropin-releasing factor. We established a primary culture system for hypothalamic neurons producing gonadotropin-releasing hormone (GnRH) and examined whether IL-6 stimulated their GnRH secretion. We demonstrated immunohistochemically that some of these neurons contained GnRH-like immunoreactivity. In primary cultures of these GnRH neurons, we found that the calcium ionophore A23187 stimulated GnRH secretion in a dose- and time-dependent manner. These hypothalamic cells secreted IL-6 spontaneously, producing about 10 ng/l in 24 h, and their IL-6 secretion was significantly stimulated by E2 at 10-9 10-8 mol/l. This stimulatory effect was observed within 3 h. IL-6 also stimulated the release of GnRH in a dose- and time-dependent manner, and these effects of IL-6 were significantly blocked by anti-IL-6 antiserum. These results suggest that the central action of IL-6 on the GnRH neurons may be an important physiological event in the hypothalamus

ISSN:1663-2818    

DOI:10.1159/000181915

出版商:S. Karger AG    

年代:1991

数据来源: Karger

ISSN:1663-2818    

DOI:10.1159/000181916

出版商:S. Karger AG    

年代:1991

数据来源: Karger