119 results on '"Teller WM"'
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2. Pränatale Diagnostik bei hereditären Stoffwechselkrankheiten
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Teller Wm
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Text mining ,business.industry ,Medicine ,Prenatal diagnosis ,General Medicine ,business ,Bioinformatics - Published
- 1975
3. Thin layer chromatography of urinary acid glycosaminoglycans as screening procedure for mucopolysaccharidoses
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Teller Wm and Ziemann A
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Male ,Adolescent ,Endocrinology, Diabetes and Metabolism ,Urinary system ,Clinical Biochemistry ,Biochemistry ,Glycosaminoglycan ,Endocrinology ,Intellectual Disability ,Methods ,Humans ,Child ,Glycosaminoglycans ,Chromatography ,Chemistry ,Heparin ,Sulfates ,Biochemistry (medical) ,General Medicine ,Mucopolysaccharidoses ,Thin-layer chromatography ,Female ,Chromatography, Thin Layer ,Chondroitin ,Retinitis Pigmentosa ,Carbohydrate Metabolism, Inborn Errors - Published
- 1969
4. Andrea Prader Award. Clinical Steroidology. Coming of age.
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Teller WM
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- Female, Gas Chromatography-Mass Spectrometry, Germany, History, 20th Century, Humans, Male, Societies, Medical, Awards and Prizes, Endocrinology history, Steroids blood, Steroids urine
- Abstract
The determination of steroid concentrations in urine and plasma is an indispensable part of the diagnostic work-up in endocrinology. Since the description of the color reaction for determination of 17-ketosteroids by Zimmermann in 1935, a vast amount of work has been invested in the specialty of clinical chemistry, which we called 'clinical steroidology'. Until the early fifties, color reactions for block determinations of C19 and C21 steroids in urine and plasma were the strongholds of steroid clinical chemistry. Around 1952, paper chromatography, and later on column chromatography were added, which afforded fractionation and individual determinations of steroids in body fluids. The final achievement and almost absolute quantification of almost 50 steroid metabolites ('profile') in urine was achieved by capillary glass column chromatography-mass spectrometry (GC-MS), which came into clinical usage around the mid-eighties. For estimation of plasma steroids radioimmunoassays (RIA) were introduced during the sixties. A whole body of physiology and pathology was erected on the basis of steroid RIAs. Recent investigation, however, revealed that steroid RIAs yield unreliable results at low concentrations due to cross reactions and interferences by impurities of samples. The newly (nineties) developed method of isotope dilution/GC-MS produced highly accurate values of small amounts of steroids and became the gold standard of plasma steroid determinations. Thus 'clinical steroidology' has certainly come of age.
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- 1998
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5. Combined pituitary deficiencies of growth hormone, thyroid stimulating hormone and prolactin due to Pit-1 gene mutation: a case report.
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Holl RW, Pfäffle R, Kim C, Sorgo W, Teller WM, and Heimann G
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- Arginine, DNA Mutational Analysis, Growth Disorders metabolism, Growth Hormone deficiency, Growth Hormone metabolism, Humans, Infant, Male, Pituitary Hormones metabolism, Prolactin deficiency, Prolactin metabolism, Thyrotropin deficiency, Thyrotropin metabolism, Transcription Factor Pit-1, Tryptophan, DNA-Binding Proteins genetics, Growth Disorders genetics, Homeodomain Proteins genetics, Pituitary Hormones deficiency, Point Mutation, Transcription Factors genetics
- Abstract
Unlabelled: A child exhibited postnatal obstipation and icterus together with severe growth failure during the 1st year of life, a small facial skull and a prominent forehead. Endocrine work-up established the diagnosis of combined pituitary deficiencies of growth hormone, TSH and prolactin. Subsequently, the Pit-1 gene was analysed in the patient and both parents. A single point mutation was detected in exon 6 of the child: a C to G transversion on one allele, causing arginine in position 271 to be substituted by tryptophan (R271 W). This position is known as a "hot spot" for mutations. The inheritance is autosomal-dominant, as the mutated gene product interferes with DNA-binding of the wild-type protein. In contrast, other mutations in the PIT-1 gene are inherited in an autosomal-recessive mode., Conclusion: Diagnosing Pit-1 gene mutations as a rare cause of combined pituitary deficiency is important both for genetic counselling as well as for predicting the future course in the patient (spontaneous puberty, no glucocorticoid substitution necessary during stress periods).
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- 1997
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6. Insulin resistance with altered secretory kinetics and reduced proinsulin in cystic fibrosis patients.
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Holl RW, Wolf A, Thon A, Bernhard M, Buck C, Missel M, Heinze E, von der Hardt H, and Teller WM
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- Adolescent, Blood Glucose metabolism, C-Peptide blood, Cohort Studies, Cystic Fibrosis blood, Diabetes Mellitus, Type 1 blood, Diabetes Mellitus, Type 1 epidemiology, Female, Glucose Tolerance Test, Humans, Insulin metabolism, Male, Reference Values, Time Factors, Blood Glucose analysis, Cystic Fibrosis complications, Diabetes Mellitus, Type 1 complications, Insulin blood, Insulin Resistance physiology, Proinsulin blood
- Abstract
Background: Impaired glucose tolerance and secondary diabetes are frequent in older patients with cystic fibrosis (CF), associated with increased frequency of infections and reduced life expectancy. Studies on the pathophysiology of islet cell secretion in CF are a prerequisite for a scientifically based therapeutic approach., Methods: Oral glucose tolerance tests were performed in 71 patients (14.2 +/- 0.5 years; mean +/- SE) and 56 control subjects (16.5 +/- 0.9 years). Glucose, insulin, C-peptide, and proinsulin were measured every 30 min., Results: Glucose tolerance in CF patients was classified as normal (NGT, n = 48), impaired (IGT, n = 14), or diabetic (DM, n = 9). Even in CF patients with NGT, blood glucose was significantly elevated at 30, 60, and 90 min of the test. Surprisingly, the secretory responses of insulin and C-peptide were not reduced in CF patients with IGT or DM compared with both healthy controls or CF patients with normal glucose tolerance. However, peak insulin concentration was reached at 90 min in CF-IGT or CF-DM patients compared with 30 min in controls. The ratio of glucose to insulin, an indicator of insulin resistance, increased in CF patients with progression of carbohydrate intolerance. Proinsulin was significantly reduced in all CF patients compared with controls (p < 0.001; Wilcoxon's rank sum test)., Conclusions: In CF patients with impaired glucose tolerance or diabetes, integrated insulin release is not diminished, indicating that insulin resistance is likely to contribute to hyperglycemia in CF patients with IGT or DM. Reduced proinsulin levels in CF patients are compatible either with enhanced conversion of proinsulin to insulin in compensation for reduced beta-cell mass, or enhanced clearance of proinsulin.
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- 1997
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7. Proposed definitive methods for measurement of plasma testosterone and 17 alpha-hydroxyprogesterone.
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Wudy SA and Teller WM
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- Humans, Methods, 17-alpha-Hydroxyprogesterone blood, Testosterone blood
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- 1997
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8. [Body composition in type 1 diabetes mellitus. Bio-impedance measurements in 274 diabetic children, adolescents and young adults].
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Bartz J, Sulzbach U, Heinze E, Teller WM, and Holl RW
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- Adolescent, Adult, Body Mass Index, Body Weight, Child, Cohort Studies, Electric Impedance, Female, Humans, Male, Reference Values, Sex Factors, Body Composition, Diabetes Mellitus, Type 1 metabolism
- Abstract
Background and Objective: Weight gain is a common undesirable side effect of insulin treatment in type 1 diabetics. This study tested the assumption that this is due to an increase in fat mass., Patients and Method: Bioelectric impedance measurements were performed on 157 young male and 117 female diabetics (age 17.6 +/- 4.9 years; diabetes duration 8.9 +/- 5.7 years) and the fat-free mass (FFM) calculated according to the equation of Schfer et al. The data of the diabetics were compared with those of healthy controls and normal values published by Barlett et al., Results: The average weight of the diabetic cohort was 2.0 +/- 0.7 kg higher than in the reference groups, adjusted for sex and age. FFM was higher by 2.9 +/- 0.7 kg in diabetics than in the healthy cohort of Barlett et al (P < 0.005), being equally high in males and females (+2.9 +/- 0.7 kg and 2.9 +/- 0.6 kg, respectively). But compared with the values in metabolically normal controls the percentage fat proportion was lower in the diabetics than the controls, but not significantly (-1.3 +/- 0.6%). Weight gain was greater in females than males (+3.8 +/- 0.9 kg vs +1.2 +/- 0.9 kg, P < 0.05). After correcting for age, there was a partial correlation between good metabolic control and FFM in males. The form of treatment had no effect on body composition., Conclusion: These data indicate that weight gain in young diabetics is due not to an increase in fatty tissue but in muscle mass. This is probably the result of peripheral hyperinsulinism combined with hyperglycaemia.
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- 1997
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9. [Diagnosis of the adrenogenital syndrome caused by 11beta-hydroxylase deficiency using gas chromatographic-mass spectrometric analysis of the urinary steroid profile].
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Wudy SA, Homoki J, Wachter UA, and Teller WM
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- Adolescent, Adult, Child, Child, Preschool, Cortodoxone metabolism, Desoxycorticosterone metabolism, Diagnosis, Differential, Female, Humans, Infant, Male, Adrenal Hyperplasia, Congenital diagnosis, Adrenal Hyperplasia, Congenital etiology, Adrenal Hyperplasia, Congenital urine, Gas Chromatography-Mass Spectrometry, Steroids urine
- Abstract
Problem and Objective: In 5-7% of all cases of congenital adrenogenital hyperplasia (AGH) there is 11 beta-hydroxylase deficiency (11 beta-HM). Its clinical picture is characterised by hyperandrogenism and, in some cases, arterial hypertension. The diagnosis of the enzyme deficiency depends on a reliable method of analysing the hormone in plasma and urine. As little is known and data often contradictory about the pattern of urinary steroid excretion in 11 beta-HM, these steroid metabolites were measured by a highly specific method., Patient and Method: The pattern of urinary excretion of steroids was determined by gas chromatography and mass spectrometry (GC/MS) in 16 children and adults (11 males, 5 females: mean age 9(8)/12 [2/12-20(3)/12] years) with 11 beta-HM., Results: In all patients there was greatly increased excretion of tetrahydrated (TH) and hexahydrated (HH) metabolites of 11-desoxycortisol (S) and desoxycorticosterone (DOC). The excretion of THS and THDOC was extremely increased in all patients. The metabolites 5 alpha-THS as well as 20 alpha- and 20 beta-isomers of HHS, not normal found in healthy persons, were present in 15 patients (94%), while the 20 alpha- and 20 beta-isomers of 5 alpha-HHS were demonstrated in 14 (88%). For the first time, 20 alpha- and 20 beta-isomers of 5 alpha-HHS were shown to be typical urinary steroid metabolites in 11-HM. The excretion of cortisol metabolites is typically decreased in 11 beta-HM. No corticosterone metabolites were found., Conclusion: The urinary steroid excretion pattern, measured by GC/MS, is a noninvasive, highly specific and nonselective method in the differential diagnosis of abnormal steroid metabolism.
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- 1997
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10. [Gas chromatography-mass spectrometry determination of plasma 5 alpha-androstane-3 alpha,17 beta-diol and 5 alpha-androstane-3 alpha,17 beta-diol glucuronide in children with premature and normal puberty ].
- Author
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Wudy SA, Homoki J, and Teller WM
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- Child, Diagnosis, Differential, Female, Humans, Male, Puberty, Precocious etiology, Reference Values, Sexual Maturation physiology, Adrenal Hyperplasia, Congenital, Androstane-3,17-diol analogs & derivatives, Androstane-3,17-diol blood, Gas Chromatography-Mass Spectrometry, Puberty, Precocious enzymology
- Abstract
Using stable isotope dilution/gas chromatography-mass spectrometry, we determined the plasma concentrations of 5 alpha-androstane-3 alpha,17 beta-diol (AD) and 5 alpha-androstane-3 alpha,17 beta-diol-glucuronide (ADG) in 20 patients with premature pubarche (16 patients with idiopathic premature pubarche, 4 patients with late onset 21-hydroxylase deficiency) and in 55 healthy children with Tanner stages P1 to P4. No differences between sexes were found in healthy children with Tanner stages P1 and P2. Patients with idiopathic premature pubarche (median, range, nmol/1: 0.22; 0.12-0.31) or late onset 21-hydroxylase deficiency (0.27; 0.23-0.29) had higher plasma AD concentrations than healthy prepubertal children (0.09; 0.00-0.17). Regarding ADG, patients with idiopathic precocious puberty (1.35; 0.25-4.74) or late onset 21-hydroxylase deficiency (4.01; 3.50-4.58) had also higher plasma concentrations than healthy prepubertal children (0.35; 0.00-0.75). Thus, AD and ADG, which both represent end metabolites of peripheral androgen metabolism, can be regarded as markers of androgenicity. Steroid analysis by mass spectrometry is recommended, whenever uncertainties of immunological determinations are to be avoided.
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- 1996
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11. Mitogenic and antiadipogenic properties of human growth hormone in differentiating human adipocyte precursor cells in primary culture.
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Wabitsch M, Braun S, Hauner H, Heinze E, Ilondo MM, Shymko R, De Meyts P, and Teller WM
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- Adipocytes cytology, Adolescent, Adult, Cell Differentiation drug effects, Cell Division drug effects, Cells, Cultured, Child, Child, Preschool, Female, Glucose metabolism, Human Growth Hormone metabolism, Humans, Infant, Iodine Radioisotopes, Lipolysis drug effects, Middle Aged, Mitogens metabolism, Protein Binding, Stem Cells drug effects, Adipocytes drug effects, Human Growth Hormone pharmacology, Mitogens pharmacology
- Abstract
Children with GH deficiency have enlarged fat cells but a reduced number of fat cells compared with healthy children. After treatment with human GH (hGH) both fat cell volume and number are shifted toward normal. To clarify the role of hGH in fat cell formation in human adipose tissue, we investigated the effect of hGH on the proliferation and the differentiation of cultured human adipocyte precursor cells obtained from five children and 10 adults. In a chemically defined serum-free medium treatment of adipocyte precursor cells with hGH led to an increase in IGF-I production and a stimulation of cell proliferation, which could be blocked by a MAb raised against human IGF-I. hGH dose-dependently reduced the number of differentiating cells and suppressed the expression of glycerol-3-phosphate dehydrogenase (GPDH), a marker of adipose differentiation. No significant differences in the hGH effects on proliferation and differentiation capacities were seen between cultures obtained from children and adults. In newly differentiated adipocytes, hGH inhibited glucose uptake and lipogenesis, and stimulated lipolysis. Scatchard analysis of hGH competition experiments using 125I-labeled hGH yielded a linear plot with an apparent Kd of 1.08 nM and an estimated number of 7000 hGH receptors per cell. These data suggest that hGH is able to enlarge the human adipocyte precursor pool via induction of IGF-I synthesis but exhibits a direct antiadipogenic activity. hGH is also able to reduce fat cell volume by reducing lipogenesis and increasing lipolysis.
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- 1996
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12. 5 alpha-androstane-3 alpha, 17 beta-diol and 5 alpha-androstane-3 alpha, 17 beta-diol-glucuronide in plasma of normal children, adults and patients with idiopathic hirsutism: a mass spectrometric study.
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Wudy SA, Wachter UA, Homoki J, and Teller WM
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- Adolescent, Adult, Aging blood, Child, Child, Preschool, Female, Gas Chromatography-Mass Spectrometry, Humans, Male, Osmolar Concentration, Reference Values, Sex Characteristics, Androstane-3,17-diol analogs & derivatives, Androstane-3,17-diol blood, Hirsutism blood
- Abstract
We investigated the developmental patterns of 5 alpha-androstane-3 alpha, 17 beta-diol (AD) and 5 alpha-androstane-3 alpha, 17 beta-diol-glucuronide (ADG) in plasma of normal children and adults of both sexes and in patients with idiopathic hirsutism using a physicochemical method: high-resolution gas chromatography/mass spectrometry (HRGC/MS). In children below the age of 11 years, AD and ADG increased with age showing no differences between sexes (mean +/- SD, nmol/l): normal subjects 3-6 years: AD in females 0.08 +/- 0.03, in males 0.07 +/- 0.03; ADG in females 0.15 +/- 0.05, in males 0.14 +/- 0.04; normal subjects 7-10 years; AD in females 0.17 +/- 0.03, in males 0.17 +/- 0.07; ADG in females 0.59 +/- 0.12, in males 0.47 +/- 0.14. Thereafter, AD and ADG showed a greater increase in males (normal subjects 11-15 years: AD in females 0.24 +/- 0.06, in males 0.41 +/- 0.14; ADG in females 1.47 +/- 0.36, in males 3.36 +/- 1.22). In adults, plasma levels did not overlap between females and males (AD in females 0.24 +/- 0.07, in males 0.99 +/- 0.31; ADG in females 2.32 +/- 0.68, in males 13.01 +/- 3.05). 5 alpha-Androstane-3 alpha, 17 beta-diol-glucuronide discriminated better between sexes than AD. In idiopathic hirsutism, mean plasma concentrations of AD and ADG were higher than those of healthy females (ages 11-15 years: AD 0.31 +/- 0.10, ADG 3.48 +/- 2.00; ages > 16 years: AD 0.44 +/- 0.27, ADG 6.46 +/- 3.11), but 54% of patients had normal plasma concentrations of AD and 29% had normal ADG values. Thus, ADG reflected androgenicity better than AD. However, both metabolites were imperfect markers of androgenicity in idiopathic hirsutism. Therefore, our findings do not support the concept of increased 5 alpha-reductase activity in all patients with idiopathic hirsutism.
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- 1996
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13. Semilente-insulin at bedtime is superior to NPH-insulin for the suppression of the dawn-phenomenon in adolescents with type-I-diabetes.
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Holl RW, Teller WM, and Heinze E
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- Adolescent, Cross-Over Studies, Diabetes Mellitus, Type 1 blood, Female, Humans, Injections, Subcutaneous, Male, Prospective Studies, Time Factors, Blood Glucose metabolism, Diabetes Mellitus, Type 1 drug therapy, Hypoglycemic Agents therapeutic use, Insulin, Isophane therapeutic use, Insulin, Long-Acting therapeutic use
- Abstract
The dawn-phenomenon causes high fasting glucose values in IDDM patients during puberty. Even a bedtime injection of intermediate-acting insulin does not reliably suppress glucose rises during the morning hours. We therefore examined whether Semilente, an amorphous zinc insulin with kinetics different from NPH insulin, is better suited to alleviate the dawn-phenomenon in adolescent patients with long-standing diabetes. This prospective study included 15 adolescent patients (age 15.5 +/- 0.4 years; mean +/- SE) well beyond the remission phase of diabetes (mean duration: 7.5 +/- 0.8 years). On an inpatient basis, blood glucose profiles following bedtime injections of NPH or semilente insulin were compared, using a sequential cross-over design for intra-patient comparison. Fasting blood glucose was significantly lower following bedtime injections of Semilente (183 +/- 21 mg/dL [10.2 +/- 1.1 mmol/L]) compared to nights where NPH had been injected (235 +/- 22 mg/dL [13.1 +/- 1.2 mmol/L]). In addition, the morning postprandial blood glucose was significantly improved. The frequency of nocturnal hypoglycemia was not different, and the dose of Semilente insulin was slightly lower compared to the dose of NPH-insulin injected. For adolescent IDDM patients with suboptimal metabolic control due to a marked dawn-phenomenon, with high fasting glucose concentrations despite a bedtime injection of NPH insulin, bedtime injection of Semilente insulin may result in reduced fasting hyperglycemia on the next morning.
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- 1996
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14. Biological effects of human growth hormone in rat adipocyte precursor cells and newly differentiated adipocytes in primary culture.
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Wabitsch M, Heinze E, Hauner H, Shymko RM, Teller WM, De Meyts P, and Ilondo MM
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- Adipocytes cytology, Adipocytes enzymology, Animals, Antibodies, Monoclonal immunology, Antibodies, Monoclonal pharmacology, Cell Differentiation drug effects, Cell Differentiation physiology, Cell Division drug effects, Cell Division physiology, Cells, Cultured, Corticosterone pharmacology, Dose-Response Relationship, Drug, Glucose pharmacokinetics, Glycerolphosphate Dehydrogenase analysis, Humans, Insulin-Like Growth Factor I immunology, Iodine Radioisotopes, Lipid Metabolism, Lipolysis physiology, Male, Rats, Rats, Wistar, Stem Cells cytology, Stem Cells enzymology, Thymidine metabolism, Tritium, Adipocytes drug effects, Growth Hormone pharmacology, Stem Cells drug effects
- Abstract
The effects of human growth hormone (hGH) on proliferation and differentiation of primary adipocyte precursor cells isolated from rat epididymal fat pads were studied under serum-free culture conditions. hGH markedly reduced the formation of new fat cells and the expression of glycerophosphate dehydrogenase activity, a marker enzyme of adipose differentiation, in a dose-dependent manner. To find an explanation for this inhibitory effect, we investigated the action of GH on (1) cell proliferation and on (2) lipid accumulation, the latter in the absence and presence of corticosterone. In undifferentiated cells, 5 nmol/L hGH increased both cell number and [3H]-thymidine incorporation (1.3- and 2.6-fold over basal, respectively). This effect was mediated by insulin-like growth factor-I (IGF-I), since hGH stimulated IGF-I production in undifferentiated cells by 12-fold and addition of an anti-IGF-I monoclonal antibody (IGF-I MAb) abolished the mitogenic effect of hGH but did not prevent hGH-induced suppression of adipose differentiation. In developing fat cells, hGH significantly reduced cellular 2-deoxyglucose uptake and glucose incorporation into lipids. In addition, hGH exhibited a lipolytic action in the presence of insulin and triiodothyronine. These effects were not prevented by IGF-I MAb. Specific binding of [125I]-hGH to precursor cells increased significantly during adipose conversion. In differentiated cells Scatchard analysis yielded linear plots with an apparent Kd of 0.16 nmol/L and 8,400 sites per cell. Taken together, these data show that hGH reduces adipose conversion in primary cultures of rat adipocyte precursor cells while promoting cell proliferation through an increase in IGF-I production.
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- 1996
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15. Expression of osteoblastic markers in cultured human bone and fracture callus cells.
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Mörike M, Schulz M, Nerlich A, Koschnik M, Teller WM, Vetter U, and Brenner RE
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- Adult, Alkaline Phosphatase metabolism, Bone and Bones embryology, Bone and Bones metabolism, Bony Callus metabolism, Cells, Cultured, Collagen metabolism, Cyclic AMP metabolism, Fetus, Humans, Osteoblasts metabolism, Osteocalcin biosynthesis, Parathyroid Hormone pharmacology, Bone and Bones cytology, Bony Callus cytology, Osteoblasts cytology
- Abstract
We compared the expression of osteoblastic markers in cultured human cells isolated from fracture calluses of various histological states of development with that in cells from adult and fetal bone. Adult osteoblasts and all callus cells produced almost exclusively type I collagen, whereas fetal osteoblasts produced also considerable amounts of type III collagen in vitro. 1,25-Dihydroxyvitamin D3 induced the synthesis of osteocalcin in all bone and callus cells but to varying extents. Fetal bone cells and early-stage callus cells synthesized less than 10% the amount of osteocalcin produced by adult bone cells. Late-stage callus cells produced intermediate levels of osteocalcin. Fetal bone cells and early-stage callus cells responded to parathyroid hormone with a less pronounced increase in intracellular cAMP than did adult bone cells. Late-stage callus cells showed the best response to parathyroid hormone. The activity of alkaline phosphatase was highest in fetal bone cells. These observations show that cells isolated from fetal bone and from fracture callus tissues express a pattern of markers clearly relating them to the osteoblastic lineage. On the basis of the different patterns of osteoblastic markers expressed in vitro we conclude that functionally distinct subtypes of osteoblasts do exist in different mineralized tissues and at different developmental stages.
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- 1995
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16. Assessment of hypothalamic-pituitary-adrenocortical axis function in dexamethasone treated very low birth weight infants by a single dose metyrapone test and gas chromatographic mass spectrometric determination of urinary steroids.
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Wudy SA, Homoki J, Haack D, Jung D, Sorgo W, Teller WM, and Bartman P
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- Bronchopulmonary Dysplasia drug therapy, Cortodoxone analogs & derivatives, Cortodoxone urine, Gas Chromatography-Mass Spectrometry, Humans, Hydrocortisone urine, Infant, Newborn, Adrenal Cortex physiology, Dexamethasone therapeutic use, Hypothalamus physiology, Infant, Very Low Birth Weight, Metyrapone, Pituitary Gland physiology
- Abstract
In order to assess hypothalamic-pituitary-adrenocortical axis function, we conducted low and single oral dose metyrapone tests (35 mg/kg) in dexamethasone treated very low birth weight infants with bronchopulmonary dysplasia (n = 12). The responses to metyrapone of tetrahydro-11-deoxycortisol (THS) and cortisol metabolites were analyzed by gas chromatography and mass spectrometry in 24-h urinary specimens. For comparative reasons, morning plasma 11-deoxy-cortisol and cortisol were measured by radioimmunoassay before and after metyrapone. No side effects of metyrapone were observed in our patients. In 5 of 12 patients, no urinary THS could be stimulated after metyrapone and most of the other patients had small increases in urinary THS. These findings suggest suppressed or strongly impaired hypothalamic-pituitary-adrenocortical axis function in most patients. While the concentrations of plasma 11-deoxycortisol showed little variation, those of plasma cortisol were grossly different from the respective urinary values. We recommend steroid analysis in 24-h urinary specimens by gas chromatography and mass spectrometry, because urinary steroids provide more information and the highly specific analytical technique is independent of phenomena such as cross reactivity or matrix effects. The low and single oral dose metyrapone test in combination with urinary steroid analysis by gas chromatography and mass spectrometry therefore provides a noninvasive, convenient and safe means of evaluating the integrity of the hypothalamic-pituitary-adrenocortical axis in very low birth weight infants.
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- 1995
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17. The role of growth hormone/insulin-like growth factors in adipocyte differentiation.
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Wabitsch M, Hauner H, Heinze E, and Teller WM
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- Adipocytes metabolism, Animals, Cell Differentiation physiology, Humans, Insulin-Like Growth Factor I pharmacology, Stem Cells cytology, Adipocytes cytology, Growth Hormone physiology, Insulin-Like Growth Factor I physiology
- Abstract
Growth of the adipose tissue results from both the enlargement of mature adipocytes and the formation of new adipocytes from adipocyte precursor cells. The differentiation process of adipocyte precursor cells is controlled by a variety of hormones. Clinical observations indicate that growth hormone (GH) and insulin-like growth factor I (IGF-I) are able to influence the growth of the adipose organ. Recent in vitro studies using cultures of clonal and primary adipocyte precursor cells have elucidated the role of GH and IGF-I in adipocyte differentiation. From the studies it can be concluded that GH is able to enlarge the pool of adipocyte precursor cells capable of differentiating into mature adipocytes, which occurs under the control of other adipogenic hormones. However, due to its metabolic action, GH is also able to reduce the volume of mature adipocytes and thus the net result of its biological action is aimed at reducing body fat. IGF-I stimulates the differentiation process by inducing critical cell divisions of adipocyte precursor cells necessary for their differentiation. IGF-I, which is known to be regulated by GH and several nutritional factors, may exert its effects in the adipose tissue in an autocrine/paracrine and endocrine way. This review summarizes the results of recent studies investigating the role of GH and IGF-I in adipocyte differentiation.
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- 1995
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18. 17 alpha-hydroxyprogesterone, 4-androstenedione, and testosterone profiled by routine stable isotope dilution/gas chromatography-mass spectrometry in plasma of children.
- Author
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Wudy SA, Wachter UA, Homoki J, and Teller WM
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- 17-alpha-Hydroxyprogesterone, Adolescent, Child, Child, Preschool, Female, Humans, Indicator Dilution Techniques, Infant, Infant, Newborn, Isotopes, Male, Radioimmunoassay, Androstenedione blood, Gas Chromatography-Mass Spectrometry methods, Hydroxyprogesterones blood, Testosterone blood
- Abstract
Using stable isotope dilution/gas chromatography-mass spectrometry (ID/GC-MS), a physicochemical method, we have profiled the plasma steroids 17 alpha-hydroxyprogesterone, 4-androstenedione, and testosterone in normal children of various age groups. Comparison of our values with those obtained by direct immunologic assays and those using an extraction or purification step showed that immunoassays in general overestimate steroid concentrations. This was especially true for plasma samples in the neonatal period and was most expressed for the concentrations of 17 alpha-hydroxyprogesterone. Our study demonstrated the applicability of ID/GC-MS to routine clinical steroid analysis. The application of ID/GC-MS is recommended whenever problems from matrix effects or cross-reactivity are likely to arise or suspicious results by immunoassays need to be rechecked.
- Published
- 1995
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19. Reduced pancreatic insulin release and reduced peripheral insulin sensitivity contribute to hyperglycaemia in cystic fibrosis.
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Holl RW, Heinze E, Wolf A, Rank M, and Teller WM
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- Adolescent, Adult, Case-Control Studies, Diabetes Complications, Female, Glucose Tolerance Test, Humans, Insulin blood, Insulin Secretion, Male, Pancreas physiopathology, Receptor, Insulin, Time Factors, Cystic Fibrosis complications, Diabetes Mellitus blood, Hyperglycemia etiology, Insulin metabolism, Insulin Resistance
- 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 male, 9 female, age 15-29 years) and 17 healthy control subjects (8 male, 9 female, 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 x 10(-4) l/min/pmol; control group: 1.95 +/- 0.25; P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
- Published
- 1995
20. Diagnosis and management of laryngeal obstruction in childhood pachyonychia congenita.
- Author
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Wudy SA, Lenders H, Pirsig W, Mohr W, and Teller WM
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- Airway Obstruction complications, Airway Obstruction physiopathology, Epithelium physiopathology, Female, Humans, Infant, Keratosis complications, Keratosis physiopathology, Laryngeal Diseases physiopathology, Laryngoscopy, Larynx surgery, Larynx ultrastructure, Nail Diseases complications, Nail Diseases physiopathology, Voice Disorders complications, Laryngeal Diseases complications, Laryngeal Diseases diagnosis, Larynx physiopathology
- Abstract
Pachyonychia congenita is a genetic syndrome of epithelial dysplasia. In infants and young children, laryngeal involvement can present a life threatening complication: obstruction of the patient's airway due to leukokeratosis can lead to severe respiratory distress. This report concentrates on the diagnosis and successful microsurgical management of laryngeal obstruction in the first female case, a 19-month-old girl with pachyonychia congenita.
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- 1995
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21. Serum and urinary steroids in girls with precocious pubarche and/or hirsutism due to mild 3-beta-hydroxysteroid dehydrogenase deficiency.
- Author
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Sólyom J, Halász Z, Hosszú E, Gláz E, Vihko R, Orava M, Homoki J, Wudy SA, and Teller WM
- Subjects
- 17-alpha-Hydroxyprogesterone, Adolescent, Adrenal Hyperplasia, Congenital, Adrenocorticotropic Hormone, Child, Female, Hirsutism blood, Hirsutism urine, Humans, Hydroxyprogesterones blood, Hydroxyprogesterones metabolism, Hydroxyprogesterones urine, Puberty, Precocious blood, Puberty, Precocious urine, Steroids blood, Steroids urine, 3-Hydroxysteroid Dehydrogenases deficiency, Hirsutism metabolism, Puberty, Precocious metabolism, Steroids metabolism
- Abstract
To obtain data on the correlation of serum and urinary steroids in nonclassical 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency, 9 girls with precocious pubarche and 33 adolescent girls with mild to severe hirsutism were studied. Urinary steroid profiles were analyzed by capillary gas chromatography. Serum 17-OH-pregnenolone (17-OHPreg) and 17-OH-progesterone (17-OHP) were determined by RIA after column-chromatographic separation. One out of 9 girls with precocious pubarche and 4/33 girls with hirsutism had elevated ratios of 17-OHPreg to 17-OHP after ACTH stimulation in serum and elevated urinary excretion of 5-ene steroids under basal conditions. These patients were defined to have decreased adrenal 3 beta-HSD activity. Basal and ACTH-stimulated serum 17-OHPreg levels in patients with mild 3 beta-HSD deficiency overlapped those of healthy controls and peripubertally virilized female patients without enzyme deficiency. Post-ACTH 17-OHPreg/17-OHP ratios in serum discriminated patients with and without 3 beta-HSD deficiency using a cutoff value of 13 instead of mean + 2 SD for age-related control values (6.7 and 11.6 for girls with Tanner stage II-III and IV-V, respectively). Sums of urinary 5-ene steroids in patients with 3 beta-HSD deficiency overlapped those in patients without enzyme deficiency. Results showed that an abnormal post-ACTH serum 17-OHPreg/17-OHP ratio may not be associated with elevated urinary 5-ene steroid excretion, and vica versa. In conclusion, patients with simultaneous elevation of post-ACTH serum 17-OHPreg/17-OHP ratio and basal urinary 5-ene steroid excretion are supposed to have mild 3 beta-HSD deficiency.
- Published
- 1995
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22. Longitudinal analysis of somatic development in paediatric patients with IDDM: genetic influences on height and weight.
- Author
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Holl RW, Heinze E, Seifert M, Grabert M, and Teller WM
- Subjects
- Age Determination by Skeleton, Body Mass Index, Child, Child, Preschool, Diabetes Mellitus, Type 1 genetics, Female, Humans, Infant, Longitudinal Studies, Male, Nuclear Family, Reference Values, Body Height genetics, Body Weight genetics, Diabetes Mellitus, Type 1 physiopathology
- Abstract
Normal growth and development, as well as the prevention of overweight, are major goals in the treatment of paediatric patients with insulin-dependent diabetes mellitus (IDDM). We therefore evaluated longitudinally the anthropometric measurements of height and weight, as well as bone age, in an unselected group of 389 patients with IDDM treated at one institution. In order to identify genetic influences on these parameters, height and weight were determined in 186 unaffected siblings and 177 pairs of parents. At diagnosis, patients were slightly taller than average (median z score: +0.37). During the subsequent course of diabetes, age-adjusted heights decreased progressively for the first 9 years, catching up again after more than 10 years of diabetes. Bone ages were progressively retarded with increasing duration of diabetes. In 76 patients of 18 years or older, median z-score for height was +0.30, not different from their unaffected siblings (median z-score: +0.22). The correlation with midparental height was identical for diabetic and nondiabetic siblings (r = 0.43). In contrast, children with diabetes were significantly heavier (z-score for weight: +0.74 compared to +0.34 in unaffected siblings; p < 0.002). Obesity developed primarily during and after puberty. We conclude that: 1) during the course of diabetes, longitudinal growth is temporarily reduced and maturation is delayed in children with diabetes compared to unaffected siblings. However, this effect of diabetes is transient and small compared to genetic influences on height in an individual child. 2) As a group, children with IDDM become significantly overweight, which is likely to increase the cardiovascular risk during adulthood.
- Published
- 1994
- Full Text
- View/download PDF
23. Successful prenatal treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
- Author
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Wudy SA, Homoki J, and Teller WM
- Subjects
- Adult, Amniocentesis, Dexamethasone adverse effects, Female, Humans, Infant, Newborn, Pedigree, Pregnancy, Adrenal Hyperplasia, Congenital drug therapy, Dexamethasone therapeutic use, Fetal Diseases
- Abstract
A mother carrying a fetus affected with 21-hydroxylase deficiency received prenatal treatment with dexamethasone (0.5 mg, tid, p.o.) started from the very beginning of the 8th week of gestation. Prenatal diagnosis had to rely on amniocentesis with karyotyping and steroid hormone determination, because HLA and DNA data from the deceased index case or direct molecular genetic techniques were not available. The pre- and postnatal diagnosis of 21-hydroxylase deficiency was based on mass spectrometric determination of 17-hydroxyprogesterone. Dexamethasone was discontinued for 5 days prior to amniocentesis. Monitoring of cortisol, dehydroepiandrosterone-sulphate and oestriol in maternal plasma revealed suppressed maternal and fetal adrenal glands throughout pregnancy. Plasma dexamethasone levels confirmed excellent maternal compliance. At term, an eutrophic girl with normal female genitalia was delivered. The diagnosis of 21-hydroxylase deficiency and salt loss was confirmed postnatally. Regarding the side-effects of dexamethasone, the benefit/risk ratio was in favour of prenatal dexamethasone therapy.
- Published
- 1994
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24. In vitro expression of osteoblastic markers in cells isolated from normal fetal and postnatal human bone and from bone of patients with osteogenesis imperfecta.
- Author
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Mörike M, Schulz M, Brenner RE, Bushart GB, Teller WM, and Vetter U
- Subjects
- Alkaline Phosphatase metabolism, Biomarkers, Bone and Bones cytology, Bone and Bones embryology, Cell Differentiation, Cell Division, Cells, Cultured, Child, Child, Preschool, Electrophoresis, Polyacrylamide Gel, Fetus, Humans, Isoenzymes metabolism, Osteoblasts cytology, Osteocalcin biosynthesis, Osteogenesis Imperfecta embryology, Parathyroid Hormone physiology, Phenotype, Reference Values, Bone and Bones metabolism, Collagen metabolism, Osteoblasts metabolism, Osteogenesis Imperfecta metabolism
- Abstract
We studied the expression of osteoblastic markers in cultured cells isolated from the bone of 15 patients with different clinical forms of osteogenesis imperfecta (OI) and of seven fetal and postnatal controls. Cultured bone cells of ten OI patients produced abnormal collagen type I. Similar to controls, OI bone cells produced predominantly collagen type I with traces of collagen types III and V. The 1,25(OH)2 vitamin D3-stimulated synthesis of osteocalcin, a specific osteoblastic marker protein, was similar in OI bone cells and age-matched controls. Bone cells from fetal controls and from patients with the perinatal lethal OI type II produced less osteocalcin than bone cells from postnatal controls and surviving OI patients. OI bone cells responded to parathyroid hormone (PTH) by increased production of cAMP similar to controls. Bone cells from fetal controls and from OI type II donors showed a decreased response to PTH. Activity of the bone-liver-kidney isoenzyme alkaline phosphatase (AP) was detected in all control and OI bone cells. The expression of all osteoblastic markers was similar in bone cells producing abnormal collagen type I. These observations show that OI bone cells in vitro express a pattern of osteoblastic markers similar to age-matched control bone cells indicating that osteoblastic differentiation is not altered by the underlying defects of collagen type I metabolism in OI bone cells.
- Published
- 1993
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25. Familial occurrence of agonadism and multiple internal malformations in phenotypically normal girls with 46,XY and 46,XX karyotypes, respectively: a new autosomal recessive syndrome.
- Author
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Kennerknecht I, Sorgo W, Oberhoffer R, Teller WM, Mattfeldt T, Negri G, and Vogel W
- Subjects
- Abnormalities, Multiple diagnosis, Diagnosis, Differential, Female, Heart Defects, Congenital genetics, Hernia, Umbilical genetics, Humans, Karyotyping, Lung abnormalities, Phenotype, Pulmonary Artery abnormalities, Syndrome, X Chromosome, Y Chromosome, Abnormalities, Multiple genetics, Genes, Recessive, Genitalia, Female abnormalities
- Abstract
We report on 2 phenotypic sisters, one with 46,XY; the other with 46,XX. The 2 girls had similar related internal malformations, including agonadism, hypoplasia of the right pulmonary artery, hypoplasia of the right lung, isolated dextrocardia with complex vitium cordis, and diaphragmatic hernia (only sib 1) or omphalocele (only sib 2). This combination of malformations did not fit into any of the previously described syndromes. For this syndrome we suggest the acronym PAGOD ([hypoplasia of the] pulmo, and pulmonary artery, agonadism, omphalocele/diaphragmatic defect, dextrocardia). The occurrence of a basically similar set of malformations in 2 unlike sex is interpreted as evidence for autosomal recessive inheritance. The different gonosomal status excludes the Y chromosome as a responsible factor. The peculiar finding of a 46,XX sex chromosome constitution combined with agonadism and an intact urogenitral tract emphasizes the concept of secondary regression of Wolffian and Müllerian structures. The associated malformations of mesodermal structures can be interpreted as midline defects. We suggest that, from the developmental field perspective, secondary regression of midline structures including the gonadal anlage explains the pathogenesis reasonably well.
- Published
- 1993
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26. [Osteogenesis imperfecta in childhood and adolescence].
- Author
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Brenner RE, Schiller B, Pontz BF, Lehmann H, Teller WM, Spranger J, and Vetter U
- Subjects
- Activities of Daily Living classification, Adolescent, Adult, Bone and Bones physiopathology, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Male, Motor Skills physiology, Osteogenesis Imperfecta classification, Osteogenesis Imperfecta rehabilitation, Body Height physiology, Osteogenesis Imperfecta physiopathology
- Abstract
Background: 209 patients with osteogenesis imperfecta type I, III and IV were studied to provide data on the natural course of the disease until the end of the second decade., Results: Weight and length at birth were normal in most cases of type I and IV, markedly reduced in type III. Fractures and deformities at birth were most frequent in type III. High fracture rates occurred in type III and IV up to 8-10 years of age. Skeletal deformities developed primarily in the lower extremities and the spine, again most frequently in type III. Radiological features were--besides osteopenia--Wormian bones of the skull, pseudoarthroses, deformity of the pelvis, popcorn epiphyses (most frequent in type III) and hyperplastic callus (most frequent in type IV). Longitudinal growth of patients with type I was in the lower normal range, while patients with type III and IV developed marked growth deficiency. Motor performance was not severely impaired in most cases of type I; however, all type III patients and 71% of type IV patients were confined to a wheelchair in later life., Conclusion: As defined, type I patients had a mild clinical course until early adulthood. Type III and IV represented a spectrum of severely affected patients. Although type IV patients were less affected at birth, their postnatal course in some respects resembled that of type III.
- Published
- 1993
27. Munchausen syndrome.
- Author
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Teller WM
- Subjects
- Humans, Eponyms, Munchausen Syndrome
- Published
- 1993
- Full Text
- View/download PDF
28. Proliferation and collagen biosynthesis of osteoblasts and chondrocytes in short rib syndrome type beemer.
- Author
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Brenner RE, Nerlich A, Kirchner F, Mörike M, Terinde R, and Teller WM
- Subjects
- Cartilage cytology, Cell Division drug effects, Cells, Cultured, Collagen biosynthesis, Electrophoresis, Polyacrylamide Gel, Female, Growth Hormone pharmacology, Growth Plate pathology, Humans, Infant, Newborn, Insulin-Like Growth Factor I pharmacology, Insulin-Like Growth Factor II pharmacology, Osteoblasts metabolism, Short Rib-Polydactyly Syndrome etiology, Short Rib-Polydactyly Syndrome metabolism, Transforming Growth Factor beta pharmacology, Short Rib-Polydactyly Syndrome pathology
- Abstract
We report on a case of lethal short-limbed skeletal dysplasia with extremely short ribs, median cleft upper lip and palate, malrotation of intestine, lung hypoplasia with bilateral segmentation defect, atrial septum defect, union of distal urethra and vagina, and complex brain malformations. Based on radiological criteria and the pattern of associated abnormalities a short rib syndrome without polydactyly (Type Beemer) was diagnosed. Morphologically, the growth plate showed a reduced proliferation zone and an enlarged zone of hypertrophic cartilage. In addition, islands of persistent hypertrophic cartilage were present even in the metaphysis. In monolayer cell cultures supplemented with 10% fetal calf serum proliferation was normal in articular chondrocytes, reduced in costal chondrocytes, and elevated in osteoblasts from the patient. Clonal growth of costal and articular chondrocytes in methylcellulose could be stimulated normally by insulin-like growth factor-I (IGF-I), IGF-II, and human growth hormone (hGH). However, the response to transforming growth factor beta 1 (TGF-beta 1) was markedly elevated in articular chondrocytes of the patient compared to those of 3 fetal controls. Quantitative collagen synthesis in both osteoblasts and chondrocytes from the patient did not differ significantly from that of controls. Osteoblasts synthesized predominantly collagen I and minor amounts of collagen III, chondrocytes synthesized primarily collagen II. All collagen chains including CNBr-peptides of collagen II showed normal migration in PAA gel electrophoresis.
- Published
- 1993
- Full Text
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29. Defective collagen fibril formation and mineralization in osteogenesis imperfecta with congenital joint contractures (Bruck syndrome).
- Author
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Brenner RE, Vetter U, Stöss H, Müller PK, and Teller WM
- Subjects
- Abnormalities, Multiple physiopathology, Ankle Joint abnormalities, Calcification, Physiologic, Contracture congenital, Contracture physiopathology, Elbow Joint abnormalities, Humans, Infant, Newborn, Knee Joint abnormalities, Male, Osteogenesis Imperfecta pathology, Osteogenesis Imperfecta physiopathology, Syndrome, Abnormalities, Multiple metabolism, Collagen biosynthesis, Contracture metabolism, Osteogenesis Imperfecta metabolism
- Abstract
We describe a male patient with osteogenesis imperfecta (OI) who was born with contractures of the knee, elbow and ankle joints. During the first 4 years he suffered from recurrent fractures. He has white sclerae, mild dentinogenesis imperfecta, multiple wormian bones, severe scoliosis and short stature. Morphological analysis of cortical bone revealed typical characteristics of OI including varying width of the osteoid, swollen mitochondria and a dilated endoplasmic reticulum of the osteoblasts. Collagen fibrils of the osteoid had a varying diameter, a feature not found in typical OI patients. Analysis of compact bone showed that the size of apatite crystals and the extractability of collagen with pepsin were markedly elevated compared to controls and other OI type III and IV patients. Lysyl hydroxylation of collagen from the organic bone matrix and the electrophoretic mobility of collagen alpha 1(I)- and alpha 2(I)-chains were normal. Our results provide evidence that this patient belongs to a subtype of OI. The biochemical studies indicate that the underlying defect involves defective fibril-formation of collagen type I leading to an altered mineralization of bone.
- Published
- 1993
- Full Text
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30. Different regulation of clonal growth by transforming growth factor-beta 1 in human fetal articular and costal chondrocytes.
- Author
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Brenner RE, Nerlich A, Heinze E, Vetter U, and Teller WM
- Subjects
- Cartilage cytology, Cartilage, Articular cytology, Cartilage, Articular drug effects, Cell Division drug effects, Clone Cells cytology, Clone Cells drug effects, Fetus cytology, Growth Hormone pharmacology, Humans, Insulin-Like Growth Factor I pharmacology, Insulin-Like Growth Factor II pharmacology, Cartilage drug effects, Transforming Growth Factor beta pharmacology
- Abstract
The variable affection of rib and limb growth in human skeletal dysplasias suggests the presence of site-specific regulatory mechanisms for chondrocyte proliferation. We therefore studied the clonal growth of normal human costal and articular chondrocytes from the same four fetuses (15 to 30 wk of gestation) in a semisolid medium (0.8% methylcellulose) with a basal supplementation of 5% heat-inactivated FCS. IGF-I[0.3-12.5 ng/mL (0.04-1.6 nmol/L)], IGF-II [0.3-12.5 ng/mL (0.04-1.7 nmol/L)], and hGH [0.5-25 ng/mL (0.02-1.1 nmol/L)] stimulated clonal growth of articular and costal chondrocytes without site-specific difference. In contrast, a significant difference was found for transforming growth factor-beta 1, which proved to be a potent growth factor for fetal articular chondrocytes but did not stimulate or only minimally stimulated fetal costal chondrocytes [p < 0.05 for 0.3 ng/mL (0.01 nmol/L) TGF-beta 1 and p < 0.01 for 1.25 ng/mL (0.05 nmol/L) TGF-beta 1 using paired t test]. Preincubation with an IGF-I receptor antibody (alpha IR-3) completely prevented the proliferative effect of IGF-I, IGF-II, and hGH, indicating that hGH acts via autocrine or paracrine induction of IGF. The antibody partly reduced TGF-beta 1 action on articular chondrocytes [p < 0.05 for 0.3 ng/mL (0.01 nmol/L) TGF-beta 1, NS for 1.25 ng/mL (0.05 nmol/L) TGF-beta 1 using paired t test]. These results indicate that TGF-beta 1 is involved in the regulation of human fetal growth and has a different effect in ribs and limbs.
- Published
- 1993
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- View/download PDF
31. Determination of dehydroepiandrosterone sulfate in human plasma by gas chromatography/mass spectrometry using a deuterated internal standard: a method suitable for routine clinical use.
- Author
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Wudy SA, Wachter UA, Homoki J, and Teller WM
- Subjects
- Adult, Child, Dehydroepiandrosterone blood, Dehydroepiandrosterone standards, Dehydroepiandrosterone Sulfate, Deuterium, Evaluation Studies as Topic, Female, Gas Chromatography-Mass Spectrometry standards, Gas Chromatography-Mass Spectrometry statistics & numerical data, Humans, Radioimmunoassay, Reference Standards, Sensitivity and Specificity, Dehydroepiandrosterone analogs & derivatives, Gas Chromatography-Mass Spectrometry methods
- Abstract
A capillary gas chromatographic-mass spectrometric method for the determination of dehydroepiandrosterone sulfate (DHEA-S) in human plasma is described. [7,7-2H2]DHEA-S is used as internal standard. After desulfation by methanolysis, heptafluorobutyryl derivatives are prepared, and selected ion monitoring of characteristic fragment ions--m/z 270 for DHEA and m/z 272 for [7,7-2H2]DHEA--carried out. Requiring small amounts of plasma, the rapid, convenient work-up and the application of bench-top GC/MS instrumentation proved our method to be suited for routine clinical use in adults and children. The method needs no complex corrections for isotope contributions and provides good accuracy and precision. Comparative values of samples assayed by our GC/MS procedure and by a direct RIA indicated that the RIA overestimates the true concentration.
- Published
- 1993
- Full Text
- View/download PDF
32. Defective stimulation of proliferation and collagen biosynthesis of human bone cells by serum from diabetic patients.
- Author
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Brenner RE, Riemenschneider B, Blum W, Mörike M, Teller WM, Pirsig W, and Heinze E
- Subjects
- Adolescent, Adult, Blood Proteins physiology, Bone Diseases, Metabolic etiology, Carrier Proteins blood, Cell Division, Cells, Cultured, Child, Child, Preschool, Diabetes Mellitus, Type 1 metabolism, Diabetes Mellitus, Type 1 physiopathology, Female, Humans, Insulin-Like Growth Factor Binding Proteins, Insulin-Like Growth Factor I analysis, Insulin-Like Growth Factor II analysis, Male, Osteoblasts cytology, Osteoblasts metabolism, Radioimmunoassay, Blood Proteins pharmacology, Bone and Bones cytology, Bone and Bones metabolism, Collagen biosynthesis, Diabetes Mellitus, Type 1 blood
- Abstract
We studied the influence of fasting serum from nine insulin-dependent diabetic children and adolescents under insufficient metabolic control on normal human bone cells in vitro compared with serum from eight sex- and age-matched controls. Cell number 24 h after plating was significantly less under diabetic serum, indicating impaired cell attachment, spreading and initiation of cell proliferation. Cell number after five days was reduced by 1% diabetic serum, while higher serum concentrations had diverging effects on osteoblast proliferation. Collagen synthesis of human osteoblasts was significantly reduced by 8% diabetic serum compared to 8% control serum, while synthesis of non-collagenous proteins was not affected. Duration of diabetes (several weeks up to 12 years) had no influence on these parameters. The serum from one patient, which was studied a second time under excellent metabolic control three months later, however, had lost its inhibitory influence on collagen synthesis of osteoblasts. The pattern of the interstitial collagen types I, III and V was not altered by diabetic serum. These results indicate that defective regulation of proliferation and collagen synthesis of osteoblasts by components present in human diabetic serum may be an important factor in the development of diabetic osteopenia. The negative influence might be explained in part by reduced levels of IGF-I and elevated levels of IGF binding protein-1 in the diabetic sera.
- Published
- 1992
- Full Text
- View/download PDF
33. Collagen metabolism in cultured osteoblasts from osteogenesis imperfecta patients.
- Author
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Mörike M, Brenner RE, Bushart GB, Teller WM, and Vetter U
- Subjects
- Adolescent, Adult, Cells, Cultured, Child, Child, Preschool, Collagen biosynthesis, Collagen isolation & purification, Electrophoresis, Polyacrylamide Gel, Female, Fibroblasts metabolism, Fractures, Bone etiology, Humans, Macromolecular Substances, Male, Osteogenesis Imperfecta physiopathology, Procollagen isolation & purification, Procollagen metabolism, Proline metabolism, Reference Values, Skin metabolism, Tritium, Collagen metabolism, Osteoblasts metabolism, Osteogenesis Imperfecta metabolism
- Abstract
Collagen produced in vitro by bone cells isolated from 19 patients with different forms of osteogenesis imperfecta (OI) was analysed. Clinically, four patients were classified as OI type I, 10 patients as OI type III and five patients as OI type IV. Bone cells of 12 of the 19 OI patients produced structurally abnormal type I collagen. Electrophoretically uniformly slower migrating collagen type I alpha-chains were found in one case of OI type I, in seven cases of OI type III and in one case of OI type IV; two cultures of OI type III produced two different populations of collagen type I alpha-chains, and one culture of OI type IV showed reduction-sensitive dimer formation of alpha 1(I) chains, resulting from the inadequate incorporation of a cysteine residue into the triple helical domain of alpha 1(I). Quantitative analysis of collagen metabolism led to the distinction of two groups of cultured OI osteoblasts. In osteoblasts of OI type I, mainly production of collagen was decreased, whereas secretion, processing and pericellular accumulation of (pro)collagen type I was similar to that in control osteoblasts. In contrast, in osteoblasts of OI types III and IV, production as well as secretion, processing and pericellular accumulation of (pro)collagen type I were significantly decreased. Low levels of type I collagen were found irrespective of the presence or absence of structural abnormalities of collagen type I in all OI types.
- Published
- 1992
- Full Text
- View/download PDF
34. Androgen metabolism assessment by routine gas chromatography/mass spectrometry profiling of plasma steroids: Part 1, Unconjugated steroids.
- Author
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Wudy SA, Wachter UA, Homoki J, Teller WM, and Shackleton CH
- Subjects
- 17-alpha-Hydroxyprogesterone, Androstane-3,17-diol blood, Androstenedione blood, Child, Dehydroepiandrosterone blood, Deuterium, Dihydrotestosterone blood, Female, Humans, Hydroxyprogesterones blood, Male, Testosterone blood, Androgens blood, Gas Chromatography-Mass Spectrometry
- Abstract
Using gas chromatography/mass spectrometry we have developed a method for the simultaneous determination of six plasma steroids: testosterone, 4-androstenedione, 17 alpha-hydroxyprogesterone, 5 alpha-androstane-3 alpha,17 beta-diol, 5 alpha-dihydrotestosterone, and dehydroepiandrosterone. For each analyte, a deuterium-labeled internal standard was used for quantification. Due to the high isotopic purity of our standards, no complex corrections for isotope contributions were necessary. The procedure provides a sensitive and specific technique with good accuracy and precision.
- Published
- 1992
- Full Text
- View/download PDF
35. Urinary excretion of 17-hydroxypregnanolones in patients with different forms of congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency.
- Author
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Homoki J, Sólyom J, Wachter U, and Teller WM
- Subjects
- Adolescent, Child, Child, Preschool, Chromatography, Gas, Female, Humans, Infant, Newborn, Male, Pregnanes urine, Virilism urine, 17-alpha-Hydroxypregnenolone urine, Adrenal Hyperplasia, Congenital enzymology, Adrenal Hyperplasia, Congenital urine
- Abstract
To improve diagnostic criteria in different (classical salt-wasting (SW), classical simple virilizing (SV) and non classical late onset (LO)) forms of congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency, we investigated the urinary excretion of 17-hydroxypregnanolones (17OH-PO(5 beta) and (5 alpha)), 15 beta-hydroxypregnanolone(15 beta OH-PO), pregnanetriol(PT) and 11-oxo-pregnanetriol (11-O-PT) compared to hydrocortisone metabolities. During the 1st month of life newborn infants with CAH-SW excreted from barely detectable to very large amounts of 17OH-PO(5 beta), 15 beta OH-PO and PT, and, in 12 of 14 cases, also 11-O-PT in their urines. From the 1st to the 28th day of life, cortisol metabolites were virtually absent in urines of CAH-SW infants. This was in contrast of 36 healthy newborn infants. We measured the excretion of 17OH-PO(5 alpha) in children with CAH of whom 19 patients with CAH-SV had a median 17OH-PO(5 alpha) excretion of 1110 micrograms/day (range: 152-5515). In 21 patients with CAH-LO, median excretion of 17OH-PO(5 alpha) was 294 micrograms/day (range: 66-1273). Besides the conventional metabolites of 17-hydroxyprogesterone (17OH-PO(5 beta), PT and 11-O-PT), no 17OH-PO(5 alpha) was detected in the urines of 14 patients with precocious pubarche, in 14 patients with virilization of unknown origin and in 94 healthy children of comparable age. The ratio of 17OH-PO(5 alpha) to tetrahydrocortisone (THE) discriminated between CAH-SV and CAH-LO from the 1st to the 18th year of age.(ABSTRACT TRUNCATED AT 250 WORDS)
- Published
- 1992
- Full Text
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36. Gonadal agenesis in a 46,XY female with multiple malformations and positive testing for the sex-determining region of the Y chromosome.
- Author
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Sorgo W, Gortner L, Bartmann P, Streb HP, Oberhoffer R, Teller WM, Zachmann M, Heymer B, Graf M, and Lattermann U
- Subjects
- Abnormalities, Multiple pathology, Abnormalities, Multiple physiopathology, Base Sequence, Blotting, Northern, Blotting, Southern, Female, Growth, Hormones blood, Hormones urine, Humans, Infant, Male, Molecular Sequence Data, Polymerase Chain Reaction, Steroids blood, Steroids urine, Urinary Bladder abnormalities, Vagina abnormalities, Zinc Fingers genetics, Abnormalities, Multiple genetics, Genitalia abnormalities, Sex Determination Analysis, Y Chromosome
- Abstract
A full-term 46,XY female newborn presented with respiratory failure due to a right-sided diaphragmatic hernia. During surgical repair, exploration revealed isolated dextrocardia and hypoplasia of the right lung. Neither gonads nor wolffian or müllerian structures could be palpated. Cardiac catheterization demonstrated defects of the ventricular septum, hypoplasia of the right pulmonary artery, persistence of the left vena cava superior and a patent ductus arteriosus. Anthropometric data were normal at birth, but fell below the 3rd percentile during follow-up. Body proportions displayed a predominance of the upper compared to the lower segment. Endocrine studies indicated no defect of steroid biosynthesis and no functional gonadal tissue. Using genetic analyses of various loci within the testis-determining region of the Y chromosome, a mutation could not be detected. The patient died from pneumonia at the age of 19 months. Postmortem examination confirmed the diagnosis of gonadal agenesis.
- Published
- 1991
- Full Text
- View/download PDF
37. [Conservative strategies in treating thyroid diseases in children].
- Author
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Homoki J and Teller WM
- Subjects
- Child, Diagnosis, Differential, Humans, Thyroid Diseases etiology, Thyroid Function Tests, Thyroid Diseases drug therapy
- Abstract
In the Federal Republic of Germany disorders of the thyroid gland are among the most common chronic diseases of childhood and adolescence. In Munich 30% of schoolchildren, in Göttingen 16% of children 6 to 16 years of age have goiters. Iodine deficiency is the most important etiological reason for the development of euthyroid goiter, which is encountered in more than 80% of children and adolescents suffering from thyroid disorders. Lymphocytic chronic thyroiditis (Hashimoto) causes goiters which may be eu-, hypo- but also hyperthyroid. Thyrotoxicosis (M. Basedow, Grave's Disease) in childhood occurs mainly in pubertal girls. The frequency of congenital hypothyroidism in West Germany is 1:3,800 neonates. The postnatally acquired hypothyroidism in childhood is as rare as hyperthyroidism caused by toxic thyroid adenoma. 27% of nodular goiters in childhood are due to Thyroid carcinoma. The diagnostic procedure of evaluation of thyroid disorders in childhood includes family and past personal history, palpatory findings, ultrasonography and the determination of TSH and thyroid hormone plasma concentrations. The aims of conservative management of thyroid diseases during childhood and adolescence are 1st normalisation of size of the thyroid gland, 2nd achievement and maintenance of euthyroid function.(ABSTRACT TRUNCATED AT 250 WORDS)
- Published
- 1990
38. Altered collagen metabolism in osteogenesis imperfecta fibroblasts: a study on 33 patients with diverse forms.
- Author
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Brenner RE, Vetter U, Nerlich A, Wörsdorfer O, Teller WM, and Müller PK
- Subjects
- Cells, Cultured, Child, Child, Preschool, Collagen biosynthesis, Collagen classification, Cyanogen Bromide, Fibroblasts metabolism, Glycosylation, Humans, Infant, Osteogenesis Imperfecta classification, Peptide Fragments isolation & purification, Protein Processing, Post-Translational, Collagen metabolism, Osteogenesis Imperfecta metabolism
- Abstract
The pattern of collagen metabolism was analysed in fibroblast cultures from patients with diverse forms of osteogenesis imperfecta (OI). Generally, OI fibroblasts show an insufficient collagen synthesis which is most obvious in patients between 2 and 9 years of age during which period control fibroblasts have an elevated collagen synthesis. OI fibroblasts remain on a basal level except for fibroblasts from OI type IV patients which seem to approach normal levels. In addition, OI fibroblasts generally show a slightly increased degradation of newly synthesized collagen which again is most obvious between 2 and 9 years. These differences in collagen degradation, however, only contribute to a minor extent to the lack of net collagen synthesis during early childhood. No correlation could be found between the degree of overmodification of collagen and its degradation since fibroblasts of both OI type I and OI type II have an elevated degradation though only the latter ones produce overmodified collagen molecules. Pulse labelling of collagen with radioactivity labelled sugars was used to distinguish between normal collagen chains or CNBr-derived peptides and those which were overmodified. In all three cases studied (OI II, OI III, OI IV) the entire triple helical domain of alpha 1(I) and alpha 2(I) was overglycosylated. The amount of overmodification, however, was not uniform but rather unique for each patient studied. We assume that the molecular defects in the majority of OI cases may be located in the mechanisms operating on the control of both the age appropriate synthesis of collagen and its degree of post-translational modification.
- Published
- 1990
- Full Text
- View/download PDF
39. 25-Hydroxyvitamin D, 24, 25-dihydroxyvitamin D and 1,25-dihydroxyvitamin D in human cerebrospinal fluid.
- Author
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Balabanova S, Richter HP, Antoniadis G, Homoki J, Kremmer N, Hanle J, and Teller WM
- Subjects
- 24,25-Dihydroxyvitamin D 3, Adult, Female, Humans, Intervertebral Disc Displacement cerebrospinal fluid, Male, Middle Aged, Reference Values, Calcifediol cerebrospinal fluid, Calcitriol cerebrospinal fluid, Dihydroxycholecalciferols cerebrospinal fluid
- Abstract
Samples of CSF and plasma were obtained simultaneously from 46 adult patients who had no endocrine disorders and were undergoing routine diagnostic lumbar puncture because of suspected or proved prolapse of a disc. Concentrations of 25-OHD, 24,25(OH)2D and 1,25(OH)2D were measured. The samples were purified by column chromatography and fractionated by HPLC. In the appropriate fractions the vitamin D metabolites were measured by PBA, and cytoreceptor assay. The results were as follows (median, range in brackets): 25-OHD in CSF 8.3 ng/ml (2.0-24.8), in plasma 14.5 ng/ml (7.0-36.0). 24,25(OH)2D in CSF 1.8 ng/ml (0.3-4.6) and 2.5 ng/ml (0.4-4.7) in plasma. 1.25(OH)2 D in CSF 25.0 pg/ml (2.2-39.0) and 31.0 pg/ml (10.1-55.0) in plasma. The correlations between plasma and CSF concentrations were as follows: 25-OHD r = 0.479 (P less than 0.001); 24,25(OH)2D r = 0.815 (P less than 0.001) and for 1.25(OH)2D r = 0.497 (P less than 0.001). Our findings showed vitamin D metabolites to be present in human CSF.
- Published
- 1984
- Full Text
- View/download PDF
40. Biochemical analysis of bone compacta in osteogenesis imperfecta.
- Author
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Brenner RE, Vetter U, Wörsdorfer O, Nerlich A, Teller WM, and Müller PK
- Subjects
- Collagen analysis, DNA analysis, Humans, Hydroxylation, Minerals analysis, Osteogenesis Imperfecta genetics, Bone and Bones analysis, Osteogenesis Imperfecta metabolism
- Published
- 1988
- Full Text
- View/download PDF
41. Haemoglobin A1c: a predictor for the duration of the remission phase in juvenile insulin-dependent diabetic patients.
- Author
-
Vetter U, Heinze E, Beischer W, Kohne E, Kleihauer E, and Teller WM
- Subjects
- Adolescent, Child, Child, Preschool, Diabetes Mellitus, Type 1 drug therapy, Female, Humans, Insulin therapeutic use, Male, Remission, Spontaneous, Time Factors, Diabetes Mellitus, Type 1 blood, Hemoglobin A analysis
- Abstract
Increased HbA1c concentrations in diabetic patients indicate retrospectively a poor metabolic control during the preceding 2-3 months. In the present study attempts have been made to use the HbA1c concentration at the time of diagnosis as an indicator of the duration of the remission phase in 23 juvenile diabetic children. The regression analysis revealed a significant negative correlation between the initial HbA1c concentrations and the duration of the remission phase defined as no glucose excretion, an insulin requirement of less than 0.5 U/kg/day and detectable serum C-peptide concentration (r =- 0.84, p < 0.001). The results suggest that the initial HbA1c concentration may serve as a useful indicator to predict the duration of the remission phase in juvenile-onset diabetic patients.
- Published
- 1980
- Full Text
- View/download PDF
42. [Urinary steroid profile in Cushing syndrome and in tumors of the adrenal cortex].
- Author
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Homoki J, Holl R, and Teller WM
- Subjects
- Adolescent, Adrenal Cortex Neoplasms diagnosis, Adrenocorticotropic Hormone urine, Adult, Child, Cushing Syndrome diagnosis, Female, Humans, Hydrocortisone urine, Male, 17-Hydroxycorticosteroids urine, 17-Ketosteroids urine, Adrenal Cortex Neoplasms urine, Cushing Syndrome urine
- Abstract
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, 6 beta-hydroxycortisol, 20 alpha-dihydrocortisol, 11 beta-hydroxyandrosterone, and 3 beta-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.
- Published
- 1987
- Full Text
- View/download PDF
43. [Comparison between constitutional developmental retardation and growth hormone deficiency in LH-RH-test].
- Author
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Meder R, Dahlén HG, and Teller WM
- Subjects
- Adolescent, Age Factors, Child, Child, Preschool, Female, Gonadotropins blood, Humans, Male, Methods, Radioimmunoassay, Sex Factors, Gonadotropin-Releasing Hormone, Growth Disorders diagnosis, Growth Hormone deficiency
- Published
- 1976
44. Detection of late onset steroid 21-hydroxylase deficiency by capillary gas chromatographic profiling of urinary steroids in children and adolescents.
- Author
-
Homoki J, Solyom J, and Teller WM
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Chromatography, Gas, Female, Humans, Infant, Male, Pregnanolone analogs & derivatives, Puberty, Precocious urine, Tetrahydrocortisol urine, Virilism urine, Adrenal Hyperplasia, Congenital urine, Pregnanes urine, Pregnanetriol analogs & derivatives, Pregnanetriol urine, Pregnanolone urine, Steroid Hydroxylases deficiency
- Abstract
Patients suffering from late onset 21-hydroxylase deficiency (LO-CAH) excreted only slightly higher amounts of 17-hydroxypregnanolone (17-OH-PO), pregnanetriol (PT) and 11-oxo-pregnanetriol (11-O-PT) than age-matched healthy controls. To discriminate between LO-CAH and virilization of unknown origin and precocious pubarche, we calculated the following ratios: (1) pregnanetriol to tetrahydrocortisone (PT/THE), (2) the sum of 17-OH-PO, PT and 11-O-PT (OHP-M) to the sum of THE, tetrahydrocortisol (THF) and allotetrahydrocortisol (a-THF) (C-M) and (3) 11-O-PT to C-M. The following patients were studied: 9 patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency-non-salt losing (CAH-NSL), never treated; 8 patients with CAH (NSL/SL: 3/5) off treatment; 10 patients with LO-CAH; 11 patients with virilization of unknown origin (prepubertal/pubertal: 5/6) and 9 patients with precocious pubarche. Healthy individuals and obligatory heterozygote carriers of comparable ages served as controls. LO-CAH showed increased ratios (median (range] of PT/THE: 2.27, (1.15-9.09), OHP-M/C-M: 2.30, (1.24-8.15), and 11-O-PT/C-M: 0.24, (0.13-1.23) compared to healthy individuals and heterozygous carriers: PT/THE 0.28, (0.03-0.57), OHP-M/C-M 0.23, (0.06-0.46) and 11-O-PT/C-M less than 0.01, (less than 0.01-0.06), respectively. The calculation of ratios, rather than absolute amounts seems to allow the detection of LO-CAH in a single spontaneously voided urine specimen. The clinical and measurable hormonal manifestations of LO-CAH occur at the same time.
- Published
- 1988
- Full Text
- View/download PDF
45. Posterior subcapsular cataracts, associated with long-term corticosteroid therapy. Prednisolone versus 6 alpha-fluor-16 alpha-methyl-1-dehydrocorticosterone.
- Author
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Miltényi M, Homoki J, Fazekas AK, Romàn H, and Teller WM
- Subjects
- Adolescent, Child, Child, Preschool, Fluocortolone adverse effects, Humans, Infant, Prednisolone adverse effects, Adrenal Cortex Hormones adverse effects, Cataract chemically induced
- Abstract
The side effects of corticosteroid therapy were studied in 50 children on long-term therapy for nephrotic syndrome or various collagen diseases. The treatment consisted of prednisolone given to 24 children, and Ultralan (6 alpha-fluor-16 alpha-methyl-1-dehydrocorticosterone) given to 15 patients, both in an alternate-day fashion. Both drugs as well as methylprednisolone and dexamethasone were given successively to 11 children. Duration of therapy and doses were equivalent in all patients studied. The patients on Ultralan therapy developed posterior subcapsular cataracts to a significant higher degree (10 out of 15) and more rapidly (after a cumulative dose of 5.0-10.0 g/m2 body surface area on 1-2 years duration of therapy) than on prednisolone monotherapy or on mixed steroid therapy.
- Published
- 1983
46. Human leukemic cells: receptor binding and biological effects of insulin and insulin-like growth factors.
- Author
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Vetter U, Schlickenrieder JH, Zapf J, Hartmann W, Heit W, Hitzler H, Byrne P, Gaedicke G, Heinze E, and Teller WM
- Subjects
- Cell Line, DNA biosynthesis, Glycogen biosynthesis, Humans, Receptors, Somatomedin, Insulin pharmacology, Leukemia metabolism, Receptor, Insulin analysis, Somatomedins pharmacology
- Abstract
Receptor binding and biological effects of insulin and insulin-like growth factors I and II (IGF I/II) were assessed in three human malignant cell lines: a Burkitt-type ALL-cell line, a ANLL-cell line and a Hodgkin's disease-cell line. Insulin receptor binding could be demonstrated in Burkitt-type ALL cells and ANLL cells, whereas no insulin receptor binding was detectable in Hodgkin cells. IGF I and IGF II binding could be demonstrated in all leukemic cells. Insulin stimulated glycogen synthesis in the insulin receptor bearing cell lines. DNA synthesis was stimulated by insulin, IGF I and II. IGF I was more active in stimulating DNA synthesis than IGF II.
- Published
- 1986
- Full Text
- View/download PDF
47. [Breast milk and drugs].
- Author
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Teller WM
- Subjects
- Female, Humans, Infant, Infant, Newborn, Risk Factors, Breast Feeding, Milk, Human metabolism, Pharmacokinetics
- Abstract
This review deals with the transfer of drugs into breastmilk and its possible threat for the infant. The following groups of pharmaca are discussed: antibiotics, antiepileptics, psychotropic drugs, cardiac drugs, diuretics as well as some other compounds. Social drugs and ecological poisons are also considered. Medications which are contraindicated during lactation and may lead to prohibition of nursing an infant have been tabulated. In general it still is valid, that despite of the vast number of possibly interfering substances in breast milk, nursing of the infant remains the best and most reliable means of infant feeding and rearing.
- Published
- 1988
48. Critical analysis of height reduction in oestrogen-treated tall girls.
- Author
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Sorgo W, Scholler K, Heinze F, Heinze E, and Teller WM
- Subjects
- Adolescent, Age Determination by Skeleton, Child, Drug Therapy, Combination, Female, Gigantism genetics, Humans, Body Height drug effects, Estrogens, Conjugated (USP) therapeutic use, Ethinyl Estradiol therapeutic use, Gigantism drug therapy, Lynestrenol therapeutic use
- Abstract
Twenty-one girls with familial tall stature were reevaluated at 18 years of age. Fourteen of them had been treated with high-dose oestrogens (I), while seven girls had not been treated (II). The untreated group is comparable but not a strict control group. Recordings on initiation of the study were: Chronologic age: 12.0 +/- 1.4 (I) versus 13.5 +/- 1.5 years (II; means +/- SD), Bone age: (1) Greulich-Pyle: 11.8 +/- 1.4 (I) versus 13.1 +/- 1.1 years (II), (2) Tanner-Whitehouse II: 12.7 +/- 1.0 (I) versus 13.6 +/- 1.1 years (II). Mean height predictions according to (1) Bayley-Pinneau, (2) Roche-Wainer-Thissen and Tanner (3) with, and (4) without allowance for mid-parent height ranged from 179.4-184 (I) to 175.7-179.5 cm (II). In the treated group there was an average reduction of predicted height of between 2.3 and 6.5 cm, depending on which of the four methods was used. In the untreated group the average differences between calculated and observed mature height varied from 0.2-3.4 cm. The difference in the reduction of predicted height between the treated and untreated groups was significant (P less than 0.05) only with the Bayley-Pinneau method and not with the others. In the treated group highly significant correlations were found between height reduction and the initial chronologic age, bone age and duration of therapy.
- Published
- 1984
- Full Text
- View/download PDF
49. [Growth disorders. Recommendations for a practice-oriented classification].
- Author
-
Teller WM
- Subjects
- Body Height, Child, Diagnosis, Differential, Female, Growth Disorders etiology, Growth Disorders therapy, Humans, Male, Growth Disorders classification
- Abstract
Besides acute illnesses, including allergies, growth disturbances are among the most frequent reasons for parents to consult a physician about their children. The basis of diagnosis is a detailed family and personal history. Actual measurement of the parents is necessary for calculation of the patient's target height. The growth data obtained must be charted on percentile curves. Only in the second line of the diagnostic approach should the physician resort to hormone determinations and X-rays of the left hand and wrist for bone age determination. For practical purposes, growth aberrations may be subdivided into normal variants and pathologic processes. The latter may cause proportionate or disproportionate disturbances. For therapeutic reasons it is also important to know whether the deviation of growth started pre- or postnatally. Many growth disorders that develop postnatally are amenable to therapy. They include the following endocrinopathies associated with short stature: isolated growth hormone deficiency (treatment with hGH), congenital adrenal hyperplasia due to enzyme deficiencies (treatment by replacement of cortisol), idiopathic, iso-sexual precocious puberty (treatment with LHRH agonists), and hypothyroidism (treatment with thyroxine). Patients with Turner syndrome benefit from sex hormones only insofar as secondary sexual characteristics develop: these agents do not promote overall growth. The treatment of patients with tall stature by administration of estrogens/gestagens in high dosages is viewed with increasing scepticism. On the average, only a 4-cm reduction in length can be achieved if patients are treated from the onset of puberty through a bone age of 16 years. All secondary growth disturbances are improved by efficient treatment of the primary, underlying disease entity.
- Published
- 1989
50. IgG therapy in systemic lupus erythematosus--two case reports.
- Author
-
Gaedicke G, Teller WM, Kohne E, Dopfer R, and Niethammer D
- Subjects
- Azathioprine therapeutic use, Child, Cushing Syndrome chemically induced, Female, Humans, Osteoporosis chemically induced, Prednisolone adverse effects, Prednisolone therapeutic use, Rib Fractures etiology, Immunoglobulin G therapeutic use, Lupus Erythematosus, Systemic therapy
- Abstract
Two paediatric patients with systemic lupus erythematosus were treated with immunoglobulin G (IgG). In the first case treatment resulted in regression of the most acute symptoms and a long remission was achieved. In the second patient, who was treated during the chronic stage of the disease, there was no significant effect on the course of the SLE.
- Published
- 1984
- Full Text
- View/download PDF
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