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  • 1
    Digitale Medien
    Digitale Medien
    Endocrine Abnormalities in Thalassemia (1990)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 612 (1990), S. 0 
    Details
    ISSN: 1749-6632
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Allgemeine Naturwissenschaft
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1749-6632.1990.tb24317.x
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  • 2
    Digitale Medien
    Digitale Medien
    IN VITRO LEYDIG CELL ASSAY: EFFECT OF PITUITARY HORMONES AND PLASMA OF DIFFERENT ORIGIN ON TESTOSTERONE PRODUCTION (1982)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 383 (1982), S. 0 
    Details
    ISSN: 1749-6632
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Allgemeine Naturwissenschaft
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1749-6632.1982.tb23222.x
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  • 3
    Digitale Medien
    Digitale Medien
    Recombinant human insulin-like growth factor I (rhIGF I) reduces hyperglycaemia in patients with extreme insulin resistance (1991)
    Springer
    Diabetologia 34 (1991), S. 675-679 
    Details
    ISSN: 1432-0428
    Schlagwort(e): Type A insulin resistance ; acanthosis nigricans ; insulin-like growth factor I ; insulin receptor ; IGF I-administration
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The syndrome of type A insulin resistance is encountered in young women and is characterized by glucose intolerance or frank diabetes mellitus, endogenous hyperinsulinism, insensitivity to insulin administration, acanthosis nigricans and virilization. The insulin resistance is due to reduced cellular insulin binding because of a lack of or defective binding sites and/or because the interaction with the tyrosine kinase of the β-subunit is hindered. This study was undertaken to find out whether hyperglycaemia in these patients may be influenced by the administration of recombinant human insulin-like growth factor I which exerts insulin like effects through the insulin receptor as well as the type 1 insulin-like growth factor I receptor. Recombinant human insulin-like growth factor I was intravenously administered in two subsequent doses of 100 μg/kg body weight to three women with type A insulin resistance. An immediate but slow fall of blood glucose was observed. The glucose disappearance rate was 28.0 μmol/min, i.e. considerably lower than that seen in healthy subjects. The markedly elevated insulin and C-peptide levels fell in a parallel manner to blood glucose but not to normal levels. The results show that recombinant human insulin-like growth factor I, presumably by reacting with the type 1 insulin-like growth factor receptor, can normalize serum glucose levels in patients with severe insulin resistance at least for several hours. We suggest that the potential of recombinant human insulin-like growth factor I to control hyperglycaemia in type A insulin resistant patients should be explored in more depth.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00400998
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  • 4
    Digitale Medien
    Digitale Medien
    Preclinical and manifest diabetes mellitus in young patients with friedreich's ataxia: no evidence of immune process behind the islet cell destruction (1989)
    Springer
    Diabetologia 32 (1989), S. 378-381 
    Details
    ISSN: 1432-0428
    Schlagwort(e): Type 1 (insulin-dependent) diabetes mellitus ; Friedreich's ataxia ; HLA ; first phase insulin secretion ; islet cell antibodies
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Friedreich's ataxia is known to be associated with diabetes mellitus in up to 20% of the patients. However, type, development and course of diabetes mellitus are not well characterised. We report on 3 patients (2 female and 1 male, age 13–20 years) with the combination of Friedreich's ataxia and diabetes mellitus. Diabetes mellitus was characterised as follows: (1) it was strictly insulin-dependent and ketosis-prone, (2) the average insulin requirement was 1 U/kg body weight, (3) the HLA haplotype was not typical of Type 1 (insulin-dependent) diabetes mellitus, (4) there were no positive immune parameters typical of Type 1 diabetes at the clinical onset of diabetes mellitus and (5) there was no remission. To evaluate a preclinical phase as in common autoimmune Type 1 diabetes, i.v. glucose tolerance tests (0.5 g glucose/kg body weight) were performed in 8 patients with Friedreich's ataxia without diabetes mellitus. Seven patients had normal early phase insulin response. In contrast, the glucose disappearance rate was slow in 4 and normal in 3 patients. One of the 8 patients showed a prediabetic metabolic state: the early-phase insulin response was abolished and the glucose disappearance rate was abnormal. The results suggest that diabetes in Friedreich's ataxia is caused by a loss of islet cells similar to common Type 1 diabetes but without HLA-association and without serologic evidence for autoimmune destruction of the islet cells.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00277262
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  • 5
    Digitale Medien
    Digitale Medien
    Guidelines for neonatal screening programmes for congenital hypothyroidism (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 974-975 
    Details
    ISSN: 1432-1076
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01957217
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  • 6
    Digitale Medien
    Digitale Medien
    A physiological mode of puberty induction in hypogonadal girls by low dose transdermal 17β-oestradiol (1990)
    Springer
    European journal of pediatrics 150 (1990), S. 86-91 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Transdermal oestradiol ; Induction of puberty ; Hypogonadal girls ; Turner syndrome ; Serum E1 and E2
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Transdermal 17β-oestradiol administration (17β-E2), used mainly in menopausal women, allows a continuous 17β-E2 delivery through the skin into the systemic circulation, avoiding intestinal and hepatic passage. In order to explore whether transdermal 17β-E2 could be used for the induction of puberty, 17β-E2 patches with low dose delivery were administered in nine prepubertal girls with Turner syndrome (bone age 〉10.5 years) for a mean period of 2.2 years. Treatment schedule: 5 μg/day for 6–9 months, 10 μg/day for 6–9 months, 25 μg/day for long-term substitution; addition of cyclic gestagen p.o. after 18–24 months. Breast development started within 3 months of therapy and menstruation occurred after 2 years. Growth rate increased from 3.2 to 5.0 cm/year during the 1st year of therapy, height prediction did not change. Serum oestradiol (E2) and urinary E2 conjugates increased proportionally with 17β-E2 doses, serum oestrone (E1) rose much less. The possibility to imitate time course, clinical events and hormonal changes of normal puberty, the absence of adverse drug reactions and the excellent acceptance and easy mode of application suggest that transdermal 17β-E2 is optimally suited for hormonal substitution in girls with hypogonadism.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02072044
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  • 7
    Digitale Medien
    Digitale Medien
    Pericardial effusion as a sign of acquired hypothyroidism in children with Down syndrome (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 397-398 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Pericardial effusion ; Hypothyroidism ; Down syndrome
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract In three children with Down syndrome and acquired hypothyroidism echocardiography was performed before and after the start ofl-thyroxine treatment. Initial studies revealed pericardial effusions which resolved during treatment suggesting that they were caused by hypothyroidism. The incidence of hypothyroidism in Down syndrome is high, but the diagnosis is often missed for lack of specific clinical criteria. The finding of pericardial effusion by echocardiography may be essential in discovering thyroid dysfunction. The cases illustrate that regular thyroid function tests are important in Down syndrome.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01955895
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  • 8
    Digitale Medien
    Digitale Medien
    Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 890-893 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Key words Growth hormone deficiency ; Prader-Willi syndrome
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract It is well established that insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein-3 (IGFBP-3) and insulin are low in growth hormone deficiency, but due to their dependence on nutrition, they are elevated in healthy obese children. As the presence of growth hormone deficiency in Prader-Labhart-Willi syndrome (PWS) is still controversial, we studied insulin, IGF-I and IGFBP-3 levels in 19 children with PWS (age range 0.5–14.6 years). Serum concentrations of insulin (SDS: −0.7±0.9, P=0.01) and IGF-I (SDS: −0.7±0.8,P=0.002) were low, but IGFBP-3 (SDS: −0.3±1.2, P=0.2) was normal compared to normal weight age-matched children. Since children with PWS are typically obese, insulin, IGF-I and IGFBP-3 levels should be compared to normal obese children who present increased levels of these hormones. In comparison to data of healthy obese children reported in the literature, not only IGF-I, but also IGFBP-3 levels are low and fasting insulin levels even very low, suggesting a growth hormone deficiency.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004310050961
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  • 9
    Digitale Medien
    Digitale Medien
    Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 368-377 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Key words Body composition ; Growth hormone treatment ; Physical performance ; Prader-Willi syndrome ; Quality of life
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m2/week) during 1 year. The children were divided into three groups: group 1: overweight and prepubertal (n = 6, age 3.8–7.0 years); group 2: underweight and prepubertal (n = 3, age 0.6–4.1 years); group 3: pubertal (n = 3, age 9.2–14.6 years). In group 1, height increased from −1.7 SD to −0.6 SD, while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SD to 1.2 SD. Hand length increased from −1.5 SD to −0.4 SD and foot length from −2.5 SD to −1.4 SD. Body fat, measured by dual X-ray energy absorptiometry, dropped by a third, whereas muscle mass increased by a fourth. Physical capability (Wingate test) improved considerably. The children were reported to be much more active and capable. In group 2, similar changes were seen, but weight for height increased, probably because muscle mass increase exceeded fat mass decrease. Changes in group 3 were similar as in group 1, even though far less distinct. Conclusion Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight children.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004310050832
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  • 10
    Digitale Medien
    Digitale Medien
    15β-Hydroxylated steroids may be diagnostically misleading in confirming congenital adrenal hyperplasia suspected by a newborn screening programme (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 928-931 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Congenital adrenal hyperplasia ; Screening ; Diagnosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract In a Swiss screening programme for detection of congenital adrenal hyperplasia (CAH), 27 of over 120,000 newborns examined from 1992 to 1994 were further studied because of persistingly high 17α hydroxyprogesterone (17OHP). Out of 27, 11 were later confirmed to have CAH by specific gas chromatography of urinary steroids and ACTH test at age 3–4 months. Of 27, 11 were born at term (7 confirmed 21-hydroxylase deficiency, one 11β-hydroxylase deficiency). Out of 27, 16 were preterm newborns. Of them, only 2 were confirmed to have CAH (one 21-, one 11β-hydroxylase deficiency). In 3 cases with high 17OHP, but later not confirmed CAH, what appeared to be a pregnanetriolone peak in the gas chromatograms was shown to be 3β, 15β, 17α-pregnenetriol. This compound may be misleading in confirming the diagnosis of CAH. 15β-Hydroxylated compounds occur in fetuses, neonates, and amniotic fluid. Since human tissues do not have l5β-hydroxylating capacity, their origin is unclear. However, since some bacteria (Bacillus megatherium) and mycelial fungi (fusaria) are known to hydroxylate steroids in position 15β, it is likely that this compound is formed by micro-organisms in the enterohepatic circulation of newborns or their mothers.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02282880
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