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  • 1
    Electronic Resource
    Electronic Resource
    Harnsteroidprofile bei Cushing Syndrom und Tumoren der Nebennierenrinde (1987)
    Springer
    Journal of molecular medicine 65 (1987), S. 719-726 
    Details
    ISSN: 1432-1440
    Keywords: Cushing's syndrome ; Adrenocortical tumors ; Urinary steroid profiles ; Screening
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The analysis of 24-h excretion profiles of urinary steroids in 18 patients suffering from Cushing's syndrome or adrenocortical tumors revealed typical patterns when compared to 37 healthy control persons, 24 patients with obesity, and 6 patients with hirsutism. The validation of eight criteria — increased excretion of free cortisol, 6β-hydroxycortisol, 20α-dihydrocortisol, 11β-hydroxyandrosterone, and 3β-hydroxy-5-en steroids, decreased ratio of tetrahydrocortisone (THE) to tetrahydrocortisol (THF), and increased ratios of THF to allotetrahydrocortisol (a-THF) and metabolites of androgens (AM) to metabolites of cortisol (CM) — afforded reliable detection of disorders in steroid biosynthesis. The analysis of urinary steroid profiles can therefore be recommended as a screening procedure in patients with clinical symptoms of disorders in steroid production and/or metabolism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01736807
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  • 2
    Electronic Resource
    Electronic Resource
    Contributions of age, gender and insulin administration to weight gain in subjects with IDDM (1998)
    Springer
    Diabetologia 41 (1998), S. 542-547 
    Details
    ISSN: 1432-0428
    Keywords: Keywords Body mass index ; puberty ; metabolic control ; insulin dose ; intensified insulin therapy.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Overweight in insulin-dependent diabetes mellitus (IDDM) has been repeatedly reported, especially in girls during adolescence. Potential pathophysiologic factors include tight metabolic control, insulin dose, treatment regimen, puberty and genetics. A standardized data-base from all IDDM patients treated at our institution was evaluated. IDDM patients with hypothyroidism or celiac's disease as well as all records from the first year of diabetes were excluded, resulting in a total of 427 patients (2454 patient-years) available for analysis. BMI and SD-score for BMI based on the Zurich longitudinal growth study were evaluated. Standardized BMI was higher in pubertal children ( + 1.07 ± 0.06) compared to prepubertal children ( + 0.68 ± 0.07; p 〈 0.002). This increase was present both for boys and girls. Increasing overweight during puberty was found irrespective of the age at diagnosis of diabetes (prepubertal or pubertal). The daily dose of insulin and the long-term metabolic control had only a minor impact on the development of overweight. In contrast, in pubertal children, SDS-BMI was significantly higher in patients on intensified insulin regimens (3 or 4 daily injections) compared to patients with 2 injections (p 〈 0.05). These data demonstrate that both boys as well as girls with IDDM develop overweight during puberty. Multiple injection therapy, not daily dose of insulin or the level of metabolic control achieved, was the main predictor of weight gain. This finding may be explained by increased caloric intake due to the flexibility allowed by intensified treatment. [Diabetologia (1998) 41: 542–547]
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001250050944
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  • 3
    Electronic Resource
    Electronic Resource
    Turner syndrome: final height, glucose tolerance, bone density and psychosocial status in 25 adult patients (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 11-16 
    Details
    ISSN: 1432-1076
    Keywords: Key words: Turner syndrome – Glucose tolerance – Hyperinsulinaemia – Height – Weight – Psychosocial adjustment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The information available on the medical and psychosocial status of patients with Turner syndrome beyond the paediatric age group is scarce. We therefore studied 25 unselected women with cytogenetically proven Turner syndrome (age 20 – 50 years), who never received any growth-promoting therapy, and ten control women (25 – 48 years). In addition to anthropometric measurements, an oral glucose tolerance test was performed, auto-antibodies to endocrine tissues were studied, bone mineral density of the forearm was measured by single photon densitometry, and information about the psychosocial distress of the patients was obtained. Adult height averaged 148.7±1.1 cm (mean±SE), which was 16 cm below the mean of adult women from a similar background. In Turner patients, final height correlated significantly with mid-parental height (final height=0.67×MPH+32.1; r=0.69). Body mass index was increased in Turner patients (25.6±1.3 kg/m2) compared to controls (21.4±0.6; P〈0.006). Six patients (25%) had impaired glucose tolerance or overt diabetes mellitus (one patient). Insulin release was augmented but delayed in the Turner group, and the area under the insulin stimulation curve was correlated to body mass index (r=+0.54, P〈0.01). Thyroid antibodies were detected in nine patients (37.5%). On average, bone density of the forearm was only marginally reduced compared to the age-dependent normal range. All women were employed, while only one of the Turner women was married. As a group, the subjects expressed greater distress due to infertility compared to short stature. These data demonstrate a high degree of impaired glucose tolerance and hyperinsulinism in adult Turner women, which – together with increased body fat – potentially increase the cardiovascular risk for these patients. In contrast, early osteoporosis as well as subjective dissatisfaction with attained height seem to be of secondary importance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02000780
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  • 4
    Electronic Resource
    Electronic Resource
    Reduced pancreatic insulin release and reduced peripheral insulin sensitivity contribute to hyperglycaemia in cystic fibrosis (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 356-361 
    Details
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Diabetes ; Insulin secretion ; Insulin resistance ; Minimal model
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Traditional opinion holds that patients with cystic fibrosis (CF) develop impaired glucose tolerance or diabetes due to insulinopenia caused by fibrosis of the pancreas. However, studies on the dynamics of insulin secretion and peripheral insulin action have yielded confliciting results. We studied 18 patients with CF (9 ♂, 9 ♀, age 15–29 years) and 17 healthy control subjects (8 ♂, 9 ♀, 20–32 years). Oral glucose tolerance tests and combined i.v.-glucose-tolbutamide-tests were performed on separate days in fasting subjects. Bergman's “Minimal Model” was used to quantitate both peripheral insulin sensitivity (SI) and insulin-independent glucose disposal (glucose effectiveness; SG). Based on National Diabetes Data Group criteria, 4 patients were classified as diabetic 922%; CF-DM), 3 patients (17%) had impaired glucose tolerance (CF-IGT) while glucose metabolism was normal in 11 patients (61%; CF-NGT). Irrespective of the degree of glucose tolerance, the insulin response to oral glucose was not reduced but delayed, up to 60 min in the CF-IGT/DM group. First-phase insulin release (0–10 min) after i.v.-glucose was significantly lower in CF patients (29% of healthy controls;P〈0.0001), with no difference between the CF-NGT and CF-IGT/DM groups. Insulin release following tolbutamide injection was only marginally reduced in CF patients (64% of controls). In contrast, SI was significantly reduced in the subgroup of CF patients with abnormal glucose metabolism (CF-IGT/DM: 0.97±0.16·10−4 l/min/pmol; control group: 1.95±0.25;P〈0.05). Conclusion The early insulin release is reduced in response to i.v.-glucose, while in the oral glucose tolerance test, insulin secretion is quantitatively preserved, but delayed. Reduced peripheral insulin sensitivity is a major factor for impaired glucose tolerance and diabetes mellitus in CF patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072102
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  • 5
    Electronic Resource
    Electronic Resource
    Adenylosuccinase deficiency: an inborn error of purine nucleotide synthesis (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 126-131 
    Details
    ISSN: 1432-1076
    Keywords: Adenylosuccinase deficiency ; Purine metabolism ; Succinylpurines ; Mental retardation ; Autism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Clinical and biochemical data are presented on eight children with adenylosuccinase deficiency. This newly discovered inborn error of purine metabolism is characterized by an accumulation in body fluids of succinyladenosine (S-Ado) and succinylaminoimidazole carboxamide riboside (SAICA riboside), the dephosphorylated derivatives of the two substrates of adenylosuccinase. Six living children (three boys and three girls) and one deceased sibling displayed severe psychomotor retardation. Epilepsy was documented in five cases, autistic features in three, and growth retardation associated with muscular wasting in a brother and sister. In the cerebrospinal fluid, plasma and urine of these patients, the S-Ado/SAICA riboside ratio was between 1 and 2. In striking contrast, the eighth patient (a girl) was markedly less mentally retarded. Most noteworthy, the S-Ado/SAICA riboside ratio in her body fluids was around 5, suggesting that her milder psychomotor retardation was causally linked to this higher ratio. Adenylosuccinase deficiency was demonstrated in the liver of all seven living children, in the kidney of three patients in whom the enzymatic activity was measured, and in the muscle of three patients, including the two with muscular wasting. In fibroblasts of the six severely retarded patients, adenylosuccinase activity was reduced to approximately 40% of normal; in the patient with the higher S-Ado/SAICA riboside ratio, it reached only 6% of normal. The clinical heterogeneity of adenylosuccinase deficiency justifies systematic screening for the enzyme defect in unexplained neurological disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445919
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  • 6
    Electronic Resource
    Electronic Resource
    Reduced pancreatic insulin release and reduced peripheral insulin sensitivity contribute to hyperglycaemia in cystic fibrosis (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 356-361 
    Details
    ISSN: 1432-1076
    Keywords: Key words Cystic fibrosis ; Diabetes ; Insulin secretion ; Insulin ; resistance ; Minimal model
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Traditional opinion holds that patients with cystic fibrosis (CF) develop impaired glucose tolerance or diabetes due to insulinopenia caused by fibrosis of the pancreas. However, studies on the dynamics of insulin secretion and peripheral insulin action have yielded conflicting results. We studied 18 patients with CF (9 ♂, 9 ♀, age 15–29 years) and 17 healthy control subjects (8 ♂, 9 ♀, 20–32 years). Oral glucose tolerance tests and combined i.v.-glucose-tolbutamide-tests were performed on separate days in fasting subjects. Bergman's "Minimal Model" was used to quantitate both peripheral insulin sensitivity (SI) and insulin-independent glucose disposal (glucose effectiveness; SG). Based on National Diabetes Data Group criteria, 4 patients were classified as diabetic (22%; CF-DM), 3 patients (17%) had impaired glucose tolerance (CF-IGT) while glucose metabolism was normal in 11 patients (61%; CF-NGT). Irrespective of the degree of glucose tolerance, the insulin response to oral glucose was not reduced but delayed, up to 60 min in the CF-IGT/DM group. First-phase insulin release (0–10 min) after i.v.-glucose was significantly lower in CF patients (29% of healthy controls; P 〈 0.0001), with no difference between the CF-NGT and CF-IGT/ DM groups. Insulin release following tolbutamide injection was only marginally reduced in CF patients (64% of controls). In contrast, SI was significantly reduced in the subgroup of CF patients with abnormal glucose metabolism (CF-IGT/DM: 0.97 ± 0.16 · 10–4 l/min/pmol; control group: 1.95 ± 0.25; P 〈 0.05). Conclusion The early insulin release is reduced in response to i.v.-glucose, while in the oral glucose tolerance test, insulin secretion is quantitatively preserved, but delayed. Reduced peripheral insulin sensitivity is a major factor for impaired glucose tolerance and diabetes mellitus in CF patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050303
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  • 7
    Electronic Resource
    Electronic Resource
    Turner syndrome: Final height, glucose tolerance, bone density and psychosocial status in 25 adult patients (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 11-16 
    Details
    ISSN: 1432-1076
    Keywords: Turner syndrome Glucose tolerance ; Hyperinsulinaemia ; Height ; Weight Psychosocial adjustment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The information available on the medical and psychosocial status of patients with Turner syndrome beyond the paediatric age group is scarce. We therefore studied 25 unselected women with cytogenetically proven Turner syndrome (age 20–50 years), who never received any growth-promoting therapy, and ten control women (25–48 years). In addition to anthropometric measurements, an oral glucose tolerance test was performed, auto-antibodies to endocrine tissues were studied, bone mineral density of the forearm was measured by single photon densitometry, and information about the psychosocial distress of the patients was obtained. Adult height averaged 148.7±1.1 cm (mean±SE), which was 16 cm below the mean of adult women from a similar background. In Turner patients, final height correlated significantly with mid-parental height (final height=0.67×MPH+32.1;r=0.69). Body mass index was increased in Turner patients (25.6±1.3 kg/m2) compared to controls (21.4±0.6;P〈0.006). Six patients (25%) had impaired glucose tolerance or overt diabetes mellitus (one patient). Insulin release was augmented but delayed in the Turner group, and the area under the insulin stimulation curve was correlated to body mass index (r=+0.54,P〈0.01). Thyroid antibodies were detected in nine patients (37.5%). On average, bone density of the forearm was only marginally reduced compared to the agedependent normal range. All women were employed, while only one of the Turner women was married. As a group, the subjects expressed greater distress due to infertility compared to short stature. These data demonstrate a high degree of impaired glucose tolerance and hyperinsulinism in adult Turner women, which—together with increased body fat—potentially increase the cardiovascular risk for these patients. In contrast, early osteoporosis as well as subjective dissatisfaction with attained height seem to be of secondary importance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02000780
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  • 8
    Electronic Resource
    Electronic Resource
    Reduced secretion of gastric inhibitory polypeptide in turner patients with impaired glucose tolerance (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 339-342 
    Details
    ISSN: 1432-1076
    Keywords: Glucose tolerance ; Turner syndrome ; Insulin ; GIP
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract There is a well documented increase in the incidence of abnormal glucose tolerance in patients with Turner syndrome. To elucidate the pathophysiology of this phenomenon, we studied the serum concentrations of gastric inhibitory polypeptide (GIP) — as probably the most important hormonal factor of the entero-insular axis — in relation to impaired glucose tolerance in this syndrome. Oral glucose tolerance tests were performed in 12 Turner patients with simultaneous determination of plasma glucose, insulin and GIP. An impaired glucose tolerance (iGT) was found in four patients with a chronological age between 12.3 and 14.9 years. These patients were compared with found Turner patients of similar age and weight and a normal glucose tolerance (nGT). The highest insulin level occurred 90 min after stimulation in the patients with iGT compared to 30 min in the nGT group. Interestingly, the total areas under the insulin curves were not different. Stimulated plasma GIP concentrations and the areas under the GIP curves wer significantly lower in iGT compared to nGT patients. A disturbed entero-insular axis might contribute to the delayed — rather than diminished — release of insulin in patients with Turner syndrome and impaired glucose tolerance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01955936
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  • 9
    Electronic Resource
    Electronic Resource
    Development of height and weight in children with diabetes mellitus: Report on two prospective multicentre studies, one cross-sectional, one longitudinal (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 258-262 
    Details
    ISSN: 1432-1076
    Keywords: Diabetes mellitus ; Growth ; Height ; Weight ; Metabolic control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Optimal regimen for insulin therapy should lead to normal longitudinal growth and weight gain in children with diabetes mellitus. However, reports published so far indicate that this goal of paediatric diabetology is currently not achieved in a considerable number of patients. In a cross-sectional sample of 89 children with insulin dependent diabetes mellitus (IDDM) for more than 3 years, we found the relation of height to weight to be significantly different compared to 102 healthy school children of similar age. Using bivariate analysis, body shape in these children with diabetes was shifted towards small and obese (P〈0.05) compared to control children. We subsequently initiated a longitudinal study and followed children from the onset of diabetes for the following 3 years, recording height, weight and bone age as well as glycosylated haemoglobin and daily insulin requirement. At diagnosis, height SDS was identical in children with IDDM (+0.04±0.10) compared to control children (−0.07±0.10; M±SE), while weight SDS was −0.26±0.10 in children with diabetes (controls: +0.01±0.1). Bone age was identically retarded in newly diagnosed IDDM children (−0.73±0.12 SDS) and in our control group of children from the same regional background (−0.50±0.12; n.s.). In this group of children with diabetes mellitus followed prospectively, height to weight relationship differed from controls after 2 and after 3 years of the disease (P〈0.05). At 2 years, body size in children with diabetes was shifted towards taller and heavier compared to controls, while at 3 years, the relation of height to weight was even more abnormal with increased obesity but a reduction of standardized height. This is the same relation encountered in the cross-sectional sample of children with a duration of diabetes beyond 3 years. These data demonstrate that even modern insulin therapy does not guarantee normal development of height and weight in children with IDDM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072224
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  • 10
    Electronic Resource
    Electronic Resource
    Silastic catheters for home antibiotic therapy in patients with cystic fibrosis (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 209-211 
    Details
    ISSN: 1432-1076
    Keywords: Key words Cystic fibrosis ; Silastic catheter ; Intravenous antibiotic home therapy ; Pseudomonas aeruginosa infection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Repeated 14-day courses of intravenous antibiotic therapy for patients with cystic fibrosis (CF), who have been colonized with Pseudomonas aeruginosa (PA), is one currently accepted treatment. Conventional intravenous cannulas for antibiotic delivery often have a short line life leading to frequent venipunctures. Therefore we used silastic catheters as a peripheral venous access. Silastic catheters (15 cm, 0.6 mm diameter) were inserted 10 cm into a cubital vein in 15 patients with CF (age 5–32 years) for 20 antibiotic courses. After the antibiotic infusion the catheter was flushed with 200 U heparin (2 ml Vetren). In all patients the antibiotic therapy was delivered as a home therapy. In 15 antibiotic courses the silastic catheter could be continuously used for 14 days. One patient with methicillin resistant Staphylococcus aureus received antibiotic therapy for 54 consecutive days using the same silastic catheter. The catheter had to be removed in four courses: once because of thrombophlebitis with local inflammation, once because of burning pain during infusion and occlusion twice. In one case the patient removed his catheter because of technical problems. No other serious side effects occurred. Ten patients had previously received intravenous antibiotics at least once. The median line life of the last used conventional peripheral cannula of all patients was 4 days versus 14 days with the use of the silastic catheter (P 〈 0.005). All patients preferred the silastic catheter to other venous access. Conclusion Because of the long line life and easy handling, silastic catheters may be an alternative venous access to perform home antibiotic therapy in patients with CF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050585
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