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  • 1
    Electronic Resource
    Electronic Resource
    Spontaneous growth in turner syndrome: Evidence for a minor pubertal growth spurt (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 283-287 
    Details
    ISSN: 1432-1076
    Keywords: Turner syndrome ; Growth ; Growth velocity ; Growth chart
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Spontaneous growth of 141 untreated girls with Turner syndrome was analysed. Of the patients 25% were born prematurely; their weight and height were normal when compared to prematurely born healthy infants. However, birth weight and height was significantly retarded in Turner patients born at term. A curve for height and growth velocity for the age range 0–16 years was constructed with a sensitive statistical method. By use of a mathematical model equations were created for calculating z-scores and the related percentiles for the height of individual patients at given age. median height of 18 untreated patients at 18 years was 143.8 cm. Analysis of growth velocity revealed a minor but significant growth spurt at the age of 12.5 years. This growth spurt was also detectable in patients without signs of spontaneous puberty and occurred later in patients with 45,X0 karyotype. Bone age progression was linear up to the age of 7.5 years and decelerated thereafter.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072230
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  • 2
    Electronic Resource
    Electronic Resource
    Treatment of patients with Ullrich-Turner syndrome with conventional doses of growth hormone and the combination with testosterone or oxandrolone: Effect on growth, IGF-I and IGFBP-3 concentrations (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 437-444 
    Details
    ISSN: 1432-1076
    Keywords: Turner syndrome ; Growth hormone ; Anabolic steroids ; IGF-I, IGFBP-3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thirty-nine girls with Ullrich-Turner syndrome (UTS) (median age 9.5 years) were treated with growth hormone (GH) with either 12 or 18 IU/m2 per week for 12 months followed by combination therapy with either oxandrolone (Ox) (0.0625 mg/kg/day po) or low-dose testosterone (T) (5 mg im every 2 weeks). Growth velocity improved significantly after 12 IU/m2 per week (6.4±1.7 cm/year vs 4.0±1.3 cm/year, x±SD,P〈0.001) and 18 IU/m2 per week of GH (6.5±1.3 cm/year vs 4.5±1.4 cm/year,P〈0.001). Ox, but not T was effective in maintaining growth velocity during the 2nd year of therapy (6.9±1.3 vs 5.3±1.5 cm/year). Basal insulin-like growth factor-I (IGF-I) concentrations were in the lower normal range and increased significantly in patients treated with 18 IU/m2 per week (357±180 ng/ml vs 160±84 ng/ml) and 12 IU/m2 per week (273±121 ng/ml vs 140±77 ng/ml). IGF-I concentrations increased further after addition of Ox (533±124 ng/ml,P〈0.001) or T (458±158,P〈0.05). IGFBP-3 concentrations were in the upper normal range before therapy and increased only moderately in both GH dosage groups. However, IGF binding protein-3 (IGFBP-3) concentrations were not affected by additional Ox or T treatment. Conclusions 1. Conventional GH doses are effective in increasing growth velocity in UTS, especially, when combined with Ox. This additive effect is not evident when GH is combined with low dose T. 2. Changes in growth velocity are accompanied by an increase of the IGF-I/IGFBP-3 ratio. 3. Ox obviously acts by increasing IGF-I levels independent of the GH status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02029351
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  • 3
    Electronic Resource
    Electronic Resource
    Treatment of patients with Ullrich-Turner syndrome with conventional doses of growth hormone and the combination with testosterone or oxandrolone: effect on growth, IGF-I and IGFBP-3 concentrations (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 437-444 
    Details
    ISSN: 1432-1076
    Keywords: Key words Turner syndrome ; Growth hormone ; Anabolic steroids ; IGF-I ; IGFBP-3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thirty-nine girls with Ullrich-Turner syndrome (UTS) (median age 9.5 years) were treated with growth hormone (GH) with either 12 or 18 IU/m2 per week for 12 months followed by combination therapy with either oxandrolone (Ox) (0.0625 mg/kg/day po) or low-dose testosterone (T) (5 mg im every 2 weeks). Growth velocity improved significantly after 12 IU/m2 per week (6.4 ± 1.7 cm/year vs 4.0 ± 1.3 cm/year, x ± SD, P 〈 0.001) and 18 IU/m2 per week of GH (6.5 ± 1.3 cm/year vs 4.5 ± 1.4 cm/year, P 〈 0.001). Ox, but not T was effective in maintaining growth velocity during the 2nd year of therapy (6.9 ± 1.3 vs 5.3 ± 1.5 cm/year). Basal insulin-like growth factor-I (IGF-I) concentrations were in the lower normal range and increased significantly in patients treated with 18 IU/m2 per week (357 ± 180 ng/ml vs 160 ± 84 ng/ml) and 12 IU/m2 per week (273 ± 121 ng/ml vs 140 ± 77 ng/ml). IGF-I concentrations increased further after addition of Ox (533 ± 124 ng/ml, P 〈 0.001) or T (458 ± 158, P 〈 0.05). IGFBP-3 concentrations were in the upper normal range before therapy and increased only moderately in both GH dosage groups. However, IGF binding protein-3 (IGFBP-3) concentrations were not affected by additional Ox or T treatment. Conclusions 1. Conventional GH doses are effective in increasing growth velocity in UTS, especially, when combined with Ox. This additive effect is not evident when GH is combined with low dose T. 2. Changes in growth velocity are accompanied by an increase of the IGF-I/IGFBP-3 ratio. 3. Ox obviously acts by increasing IGF-I levels independent of the GH status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02029351
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  • 4
    Electronic Resource
    Electronic Resource
    Hartnup syndrome, progressive encephalopathy and allo-albuminaemia (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 899-903 
    Details
    ISSN: 1432-1076
    Keywords: Aminoacidopathy ; Allo-albuminaemia ; Encephalopathy ; Hartnup syndrome ; Lethality
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Clinical, biochemical, neuropathological and neurochemical findings in a case of Hartnup syndrome are reported. After initially normal development, the affected girl suffered progressive neuropsychiatric decline with statomotor and mental retardation and intractable seizures and died at the age of 2 years. Postmortem neuropathological and neurochemical investigations showed a combination of extensive neuronal degeneration and cerebral dysmyelination. Pathogenetic hypotheses and the relationship between neuropsychiatric disease and Hartnup syndrome are discussed. Additionally, a fast type bisalbuminaemia present in the girl and her mother is described.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01954126
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  • 5
    Electronic Resource
    Electronic Resource
    Orale Trichloräthylenvergiftung bei einem 4 1/2 Jahre alten Kind (1973)
    Springer
    Archives of toxicology 31 (1973), S. 13-18 
    Details
    ISSN: 1432-0738
    Keywords: Trichlorethylene ; Oral Intoxication ; Child ; Trichloracetic-Acid ; Trichloräthylen-orale ; Vergiftung ; Kind ; Trichloressigsäure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine orale Trichloräthylenvergiftungs eines 4 1/2 Jahre alten Knaben berichtet. Zur Intoxikation kam es, als der Knabe aus einer Fruchtsaftflasche trank, die mit dem „Tri“-haltigen Reinigungsmittel gefüllt war. Die wenige Minuten später einsetzende narkotische Wirkung hielt etwa 3 Std an. Der leichte Verlauf der Vergiftung stand in gutem Einklang mit der aus der Ausscheidungskurve geschätzten Menge von etwa 7,6 g aufgenommenem Trichloräthylen. Ein einfaches chemisches Verfahren zum Nachweis von Chloroform, Trichloräthylen und Tetrachlorkohlenstoff in Fleckputzmitteln wird mitgeteilt.
    Notes: Abstract This report deals with trichlorethylene intoxication in a 4 1/2-year-old boy. The intoxication happened when the boy drank from a fruit juice bottle containing a trichlorethylene detergent. The narcotic reaction, which started after a few minutes, lasted for about 3 h. The mild process of the intoxication is in keeping with the quantity of about 7.6 g trichlorethylene estimated to have been ingested from the quantity of trichloracetic acid excreted. A simple chemical method for the determination of chloroform, trichlorethylene, and carbon tetrachloride in stain removers, based on the Fujiwara-reaction after separation by distillation, is reported.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00330291
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  • 6
    Electronic Resource
    Electronic Resource
    Cytogenetische Untersuchung des peripheren Blutes beim familiären Neuroblastom (1975)
    Springer
    Human genetics 〈Berlin〉 28 (1975), S. 217-220 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Description / Table of Contents: Zusammenfassung Eine Familie mit 2 autoptisch gesicherten Neuroblastomen, einem Ganglioneuroblastom und Hinweisen auf 3 weitere Neuroblastome wurde cytogenetisch untersucht. Anlaß war der von Sandberg et al. (1972) mitgeteilte Befund von double-minute-Chromosomen in Lymphocyten des peripheren Blutes bei einem Neuroblastom. Die Untersuchung sollte klären, ob in dieser “Neuroblastom-Familie” eine chromosomale Aberration als erbliche Vorschädigung die Entstehung von Sympathicustumoren begüngstigt. Es fanden sich keine konstanten Chromosomenanomalien, auch keine double-minute-Chromosomen.
    Notes: Summary A cytogenetic investigation was performed in a family which included 2 individuals with congential neuroblastomas of the suprarenal gland confirmed by autopsy and one with a ganglioneuroblastoma of the thoracic wall as well as 3 other individuals with tumors which probably were also neuroblastomas. The lymphocytes of the peripheral blood of 5 healthy relatives as well as of the child with the treated ganglioneuroblastoma failed to show a constant alteration of chromosomes. In this family, therefore, the suggestion could not be proofed that the very rate familial aggregation of neuroblastomas is caused by a hereditary chromosomal aberration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00278547
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  • 7
    Electronic Resource
    Electronic Resource
    The influence of growth hormone monotherapy and growth hormone in combination with oxandrolone or testosterone on thyroxid hormone parameters and thyrxine binding globulin in patients with Ullrich- Turner syndrome (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 99-103 
    Details
    ISSN: 1432-1076
    Keywords: Key words Ullrich-Turner syndrome  ;  Growth hormone therapy  ;  Thyroid hormone regulation  ;  Insulin-like growth factor I
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Administration of human growth hormone (GH) has yielded conflicting results concerning its role on thyroid function in patients with Ullrich-Turner syndrome. Therefore, we investigated the course of thyroid hormone parameters and thyroxin binding globulin in relation to GH therapy, IGF-I and additional oxandrolone-(Ox) or testosterone (T) treatment in 20 patients with Ullrich-Turner syndrome. During the 1st year the patients received only GH. There was no change in T4, fT4, and TSH levels, T3 increased significantly (P 〈  0.01) after 6 and 12 months, resulting in a higher T3/T4 ratio. TBG (P 〈 0.05) and IGF-I (P 〈 0.01) increased after 6 months and remained elevated at 12 months. A significant positive correlation was found between the change of T4 and TBG after 6 months (r = 0.47, P 〈 0.05) and after 12 months (r = 0.69, P 〈 0.005). Thirteen patients were further investigated after addition of an anabolic compound; 7 received Ox (0.0625 mg/kg/day po) and 6 low dose T (5 mg i.m. every 14 days). Chronological age was comparable in these groups (10.7 ±  2.7 vs 10.7  ± 3.6 years). After 6 months of combination therapy with Ox, T4, T3 and TSH decreased. As T4 and T3 showed a parallel decrease the T3/T4 ratio remained elevated. TBG declined after 6 and 12 months (P 〈 0.05), while IGF-I showed a further increment (P 〈 0.05). There was no correlation between the changes in T4 and IGF-I, TSH and TBG, respectively. In the T-treated group only IGF-I increased (P 〈 0.05) to the same extent as in the Ox-treated patients, whereas the thyroid parameters did not change. Conclusion The observed changes in thyroid hormone and TBG levels in the Ox group were not mediated by GH or IGF-I. The Ox-induced TBG decrease might be linked to altered pancreatic functions regulating carbo-hydrate metabolism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050563
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  • 8
    Electronic Resource
    Electronic Resource
    A new X-linked dysplasia gigantism syndrome: Identical with the Simpson dysplasia syndrome? (1984)
    Springer
    Human genetics 〈Berlin〉 67 (1984), S. 409-413 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Thirteen male newborns of a family spanning five generations revealed a syndrome consisting in elevated birth weight and length, a disproportionately large head with coarse, distinctive facies, short neck, slight obesity, and broad, short hands and feet. The affected who reached adulthood attained heights of about 2 m; their unusual facial and general appearance and the clumsiness of all their motions, remarkable during infancy and childhood, had become somewhat less conspicuous. In all but one affected individual, intellectual development was normal. In two index cases neither clinical nor laboratory evaluations revealed a basic defect. X-linked recessive inheritance is most probable.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00291401
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  • 9
    Electronic Resource
    Electronic Resource
    Fatal arthrogryposis with respiratory insufficiency: A possible case of muscle phosphorylase b-kinase deficiency (1994)
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 153-155 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00735424
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  • 10
    Electronic Resource
    Electronic Resource
    Polarographic studies of saponin-skinned muscle fibres in patients with mitochondrial myopathies (1994)
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 307-310 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00711815
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