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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 214-238 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 140 (1983), S. 109-111 
    ISSN: 1432-1076
    Keywords: Cerebral gigantism ; Somatomedin ; Growth hormone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In five cases of Sotos Syndrome serum somatomedin activities were measured. In two of these cases elevated levels and an increased secretion of growth hormone was observed. In one case (index case) a suspected hypothalamic tumor mass could be excluded, but hydrocephalus with increased intracranial pressure was present. The pathogenesis of gigantism in this syndrome is discussed.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1988), S. 220-227 
    ISSN: 1432-1076
    Keywords: Noonan syndrome ; Growth ; Ullrich-Turner syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have analysed growth and the major clinical manifestations of 144 patients (89 males, 55 females) with Noonan syndrome from two West German centres. Size at birth was normal in both sexes. In both males and females, the mean height followed along the 3rd per centile until puberty, but decreased transiently due to an approximately 2 year delay in onset of puberty. Final height approaches the lower limits of normal at the end of the 2nd decade of life. The mean adult height was found to be (n=20) 162.5 cm in males and (n=13) 152.7 cm in females, respectively. Smoothed means and standard deviations for height were derived. These data may be used for the statistical evaluation of height of Noonan syndrome patients. Except for mental retardation and microcephaly, which are more frequent in males, the relative frequencies of minor anomalies and malformations were found to be similar in both sexes. The characteristic non-cyanotic heart defects in the Noonan Syndrome do not appear to have a major influence on growth. The auxological data were compared with those in the Ullrich-Turner syndrome.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: GRF ; Growth hormone deficiency ; Somatomedin C
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Growth hormone (GH) responses to GRF (1 μg/kg BW i.v.) were investigated. Comparison between GRF(1–40) and GRF(1–29)NH2 in 11 young adult volunteers gave identical results. One hundred and thirty-one children and adolescents (45 with idiopathic GHD) were tested with GRF (1–29)NH2. The maximal GH levels (max GH) in response to GRF during the 120 min test period were found suitable to characterize the response. In cases without GHD no correlation to age, sex and pubertal development was observed. A maximal GH level of above 10 ng/ml was found to be normal. In 3 out of 86 children without GHD (one with Turner syndrome; two with simple obesity) max GH fell short of 10 ng/ml, while 11 of 45 cases with GHD exceeded this margin. In GHD, max GH was inversely correlated with age. There was no difference in max GH between groups with or without perinatal pathology as a presumed cause of GHD. GH levels to GRF were positively correlated with maximal GH level during sleep in GHD, but not correlated with responses seen to insulin or arginine. The value of GRF testing for the confirmation of GHD is discussed in the light of other GH stimulatory tests and basal somatomedin C measurements. It is suggested that the combination of testing with GRF and the determination of a basal SmC level offers a safe and convenient way to diagnose GHD in clinically suspected cases, though in some cases further diagnostic tests may be needed.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1989), S. 431-434 
    ISSN: 1432-1076
    Keywords: Chlamydia trachomatis ; Impetigo contagiosa ; IgG-sub-class-deficiency ; Pneumothorax
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An unusual case ofChlamydia trachomatis (C. trachomatis) pneumonia, complicated by the development of a pneumothorax, is reported in an IgG2/IgG4 deficient infant delivered by caesarean section.C. trachomatis was isolated initially from a throat smear and subsequently from pleural effusions. Serological examinations using the complement fixation test were negative in sera of both mother and child. However, using immunofluorescence the presence of an acute or recent infection was confirmed by IgM-antibodies in the serum of the infant and IgA-antibodies in the serum of the mother. At the age of 7 months the girl suffered from impetigo contagiosa which was partially resistant to antibiotic treatment. IgG-subclass deficiency was diagnosed after the onset of this disease and the girl was then treated by immunoglobulin transfusion.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 452-456 
    ISSN: 1432-1076
    Keywords: Marfan syndrome ; Cardiovascular manifestation ; Height and weight per centiles
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty-eight children and adolescents (mean age 10.5 years, range 1.25–18 years) with clinical evidence of Marfan syndrome were studies. Height and weight percentiles were established. Cardiac dimensions and morphology were studied by M-mode and 2D-echocardiography. At diagnosis left atrial and left ventricular end-diastolic diameter and left ventricular posterior wall thickness were within normal limits except in a few adolescent patients. Interventricular septum was thickened in about 20% and aortic diameter increased in 56% of the patients. An additional 13% of patients developed aortic dilation during the study period. At diagnosis regression analysis revealed a significant (P〈0.05) correlation of the aortic diameter, septal thickness and the posterior left ventricular wall thickness and body surface area. Follow up studies of 19 patients allowed documentation of the development of aortic root dilation.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 916-921 
    ISSN: 1432-1076
    Keywords: Serpentine fibulae ; Growth retardation ; Polycystic kidneys ; Deafness ; New syndrome versus Melnick-Needles syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on the third patient with serpentine fibula-polycystic kidney syndrome. Main features in the three reported cases were growth retardation, abnormal face, hirsutism, short neck, bowed forearms and lower legs due to bowed radii and elongated serpentine fibulae, and metatarsus adductus. Two patients including our own were deaf. All were mentally normal, all were female and sporadic. In addition, we report on a girl with Melnick-Needles syndrome and illustrate the similarities and differences between these syndromes.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Turner syndrome ; Growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The spontaneous growth of 150 patients with Turner syndrome from three German centers-90 with 45,X0 constitution, 60 with other chromosomal abnormalities-has been analyzed. The mean adult height was found to be (n=14) 146.8 cm. It was observed that growth in these patients can be divided into four phases: (1) Intrauterine growth, which is retarded; (2) Height development, which is normal up to abone-age of about 2 years; (3) Between a bone-age of 2 and 11 years when stunting of growth is most marked; (4) After a bone-age of 11 years—the time at which puberty should normally start—the growth phase is prolonged, but total height gain is only little below normal levels. No difference in height could be observed between cases with X0 karyotype and other chromosomal variants. The data are compared with those in the literature.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 182-186 
    ISSN: 1432-1076
    Keywords: Key words Isolated premature thelarche ; Central precocious puberty ; Pelvic ultrasonography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We examined 55 girls with isolated premature thelarche between the ages of 0.3 and 7.4 years (group A), 20 children with central precocious puberty between 2.1 and 7.7 years of age and 101 age-matched controls. The children with precocious puberty were divided according to distribution of pubic hair into group B (Tanner stages PH1, B2–3; n = 11), representing an early stage of the disorder, and group C (stages PH2–3, B3–4; n = 9), representing an advanced stage. Uterine and ovarian volumes were measured sonographically, peak serum levels of luteinizing hormone and follicle-stimulating hormone were determined after intravenous administration of luteinizing hormone-releasing hormone. The mean uterine and ovarian volumes were significantly greater in children with precocious puberty than in controls (group B: uterine volume: 3.8 ± 2.0 ml vs 0.9 ± 0.3 ml for controls, P 〈 0.001; ovarian volume: 2.2 ± 1.3 ml vs 0.6 ± 0.2 ml for controls, P 〈 0.01; group C: uterine volume: 8.0 ± 4.4 ml vs 1.0 ± 0.3 ml for controls, P 〈 0.01; ovarian volume; 2.6 ± 1.3 ml vs 0.4 ± 0.1 ml, P 〈 0.01). No significant differences were found between children with premature thelarche and the control group. As a diagnostic method for the early detection of central precocious puberty, ultrasound measurement of uterine volume had a sensitivity and specificity of 100% (cut-off value, 1.8 ml), while ultrasound determination of ovarian volume had a sensitivity of 82% and a specificity of 95% (cut-off value, 1.2 ml). In contrast, as a diagnostic criterion the ratio of levels of luteinizing hormone to follicle-stimulating hormone as determined following stimulation with luteinizing hormone releasing hormone had a sensitivity of 33% and a specificity of 100% (cut-off value, 1.0). Conclusion: ultrasonographic measurement of uterine and ovarian volume offers a reliable means of distinguishing between isolated premature thelarche and early stages of central precocious puberty.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Keywords: Key words Magnetic resonance imaging  ;  Pituitary gland  ;  Ectopia of the posterior pituitary  ;  Growth hormone deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to validate an association between pituitary size and severity of growth hormone deficiency (GHD) we evaluated the magnetic resonance images (MRI) of 107 children with different causes of short stature. Ninety-one MRIs were evaluable (64 male, 27 female; age: 9.1 ± 3.9 years). The levels of insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3), and tests of GH stimulation and spontaneous secretion, led to the following sub-groups: severe isolated GHD (SIGHD) (GH 〈 7 ng/ml) (n = 21); partial, isolated GHD (GH 7–10 ng/ml) (n = 22); multiple pituitary hormone deficiency (MPHD) (n = 13); neurosecretory dysfunction (n = 10); non-classifiable diagnosis (NC) (n = 13); idiopathic short stature (n = 9); and intra-uterine growth retardation (n = 3). Pituitary height (PHT) was measured and hypoplasia was assumed when PHT was 〈−2 SDS. An ectopic posterior pituitary with missing stalk and a hypoplastic anterior pituitary was present in 12 (57%) SIGHD cases, 12 (92%) MPHD cases and 1 patient from the NC group. An isolated hypoplastic anterior pituitary was observed in 15%−33% of the other groups. PHT (mm; mean, SD) in MPHD (1.7 ± 0.5) was lower than in SIGHD (2.7 ± 1.0, P 〈 0.05), with PHT of both groups being lower than in all the other groups (3.8 ± 0.9, P 〈 0.0001). PHT SDS correlates with IGF-I SDS (r = 0.48, P 〈 0.0001), IGFBP-3 SDS (r = 0.46, P 〈 0.0001) and the highest peaks in tests of GH stimulation and GH spontaneous secretion (r = 0.36, P 〈 0.0001). In contrast to all the other groups, no correlation with age was observed in MPHD and SIGHD. Breech delivery was recorded in up to 26% of patients in all seven groups. Surprisingly, only 1 out of 23 patients with an ectopic posterior pituitary was born by breech delivery, suggesting that ectopia of the posterior lobe is not necessarily related to breech delivery. Conclusion PHT is significantly correlated with GH secretion in several types of short stature. Patients with␣ectopic posterior pituitary, missing stalk and hypoplastic␣anterior pituitary either suffer from SIGHD or MPHD, and this anatomical defect is not necessarily related to breech delivery.
    Type of Medium: Electronic Resource
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