Publications by authors named "Prader A"

Seroprevalence rates for immunoglobulin G (IgG) antibodies to Tahyna virus (TAHV) and Inkoo virus (INKV) were determined in sera of 1630 blood donors from North, East, and South Tyrol by immunofluorescence assays (IFAs) and confirmatory serum neutralization tests (SNTs). Ten sera (0.6%) reacted positive by TAHV IFA, five of which (0.

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It is well established that insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein-3 (IGFBP-3) and insulin are low in growth hormone deficiency, but due to their dependence on nutrition, they are elevated in healthy obese children. As the presence of growth hormone deficiency in Prader-Labhart-Willi syndrome (PWS) is still controversial, we studied insulin, IGF-I and IGFBP-3 levels in 19 children with PWS (age range 0.5-14.

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Unlabelled: Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m2/week) during 1 year. The children were divided into three groups: group 1: overweight and prepubertal (n=6, age 3.8-7.

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In this paper age-to-age correlations for the body mass index and for skinfolds are evaluated for a sample of normal children studied from birth to adulthood. While correlations over larger age spans are modest, they become appreciable from childhood to adolescence and from adolescence to adulthood. Correlations are consistently higher for boys compared to girls, and only for the former does the body mass index correlate better than skinfolds.

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Removal of a craniopharyngioma usually results in panhypopituitarism. Some children, however, grow normally or even excessively after extirpation of the tumor despite a proven lack of GH and have so far not been treated with hGH. We studied the effects of short (2-day) and long term (1-yr) administration of hGH on metabolism and growth in six patients receiving regular hormonal replacement therapy.

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Using biceps and triceps skinfolds and upper arm circumference, areas of fat and lean tissue at the arm can be approximately determined based on a cylindrical assumption. Based on the first Zürich growth study a longitudinal analysis of estimated fat and lean areas is undertaken. Area may often be a more meaningful parameter than width in development, due to the increasing width of the limbs stretching the layer of fat.

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Based on the Zürich longitudinal growth study, differences are analyzed between those children who later became light adults (later light) and those who later became heavy (later heavy) adults. This is of interest to discover how and when overweight develops, and which variables are most affected (weight, body mass index, circumferences and skinfolds are studied). A further question is whether maturation proceeds differently in these children.

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In this paper we investigate first the choice of an appropriate index of body mass. The traditional indices weight/height (W/H), W/H2 and W/H3 are compared, as well as an approach due to Cole (1986) making the index W/Hp age-dependent, i.e.

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We describe a "new" mild malformation of the phalanx, which we call the "angel-shaped phalanx" (ASP) because of its resemblance to the little angels used for the decoration of Christmas trees. A particular middle phalangeal type of ASPs is found in a distinct variety of multiple epiphyseal dysplasia with marked retardation of bone age and severe coxarthrosis in adult life, previously reported as "hereditary peripheral dysostosis" [Bachman, 1967: Proc R Soc Med 60:21-22; Giedion, 1969: Fortschr Rontgenstr 110:507-524]. However, these authors overlooked the unique configuration of the middle phalanges.

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Based on structural average curves of distance, velocity and acceleration, an analysis of the longitudinally assessed growth of weight, arm and calf circumferences and skinfolds (biceps, triceps, suprailiac, subscapular) was undertaken. The data come from the first Zürich longitudinal growth study and represent a normal sample. In addition to a graphic analysis, timing, intensity and duration of the mid-growth spurt (MS) and of the pubertal spurt (PS) are quantified via descriptive parameters of growth.

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In an earlier report, we found that X-linked congenital adrenal hypoplasia may be associated with gonadotrophin deficiency. This combination has since been confirmed by many others. At the last examination, our patients were 22.

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Thirty-seven patients presenting features of the Prader-Willi syndrome (PWS) have been examined using cytogenetic and molecular techniques. Clinical evaluation showed that 29 of these patients fulfilled diagnostic criteria for PWS. A deletion of the 15q11.

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In this paper the dynamics and intensity of the growth of bihumeral and biiliac width and of humerus and femur bicondylar diameter are studied and compared, and sex differences are established. The analysis is based on a newly introduced statistical tool, the structural average curve for distance, velocity and acceleration. It accounts for individual developmental tempo and allows pooling data for a sample of subjects.

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Growth of body, leg, trunk and arm length from birth to adulthood is studied in the subjects of the First Zürich Longitudinal Growth Study, using a recently developed technique, the 'structural average curve'. In this way truly longitudinal average curves are obtained for velocity, acceleration and distances, and various phases of growth are analysed not only graphically, but also by descriptive parameters in terms of timing, intensity and duration. These phases consist of the pubertal spurt (PS), the mid-growth spurt (MS) and growth in infancy.

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In a consecutive series of 15 male adolescents and young adults with congenital adrenal hyperplasia (CAH), the size, shape, firmness and echostructure of the testes were assessed. The latter was abnormal in 7 patients under long standing treatment with glucocorticoids (group I). In 8, 5 under and 3 off treatment for several years, ultrasonography (US) was normal (group II).

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Transdermal 17 beta-oestradiol administration (17 beta-E2), used mainly in menopausal women, allows a continuous 17 beta-E2 delivery through the skin into the systemic circulation, avoiding intestinal and hepatic passage. In order to explore whether transdermal 17 beta-E2 could be used for the induction of puberty, 17 beta-E2 patches with low dose delivery were administered in nine prepubertal girls with Turner syndrome (bone age greater than 10.5 years) for a mean period of 2.

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A new statistical method is presented for determining an average growth curve which is valid in the sense of representing both the average dynamic, or tempo, and the average intensity of the growth process studied. In this context, growth curve means either distance, velocity or acceleration curve. The principal idea is to shift individual curves continuously (and non-linearly) in age to an average developmental age scale.

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We evaluated the growth records of 15 boys and 14 girls who developed end-stage renal failure before or during puberty and who were regularly followed from the onset to the end of their pubertal growth spurt. Height data were smoothed by using the kernel estimation method. Mean values for age, height, and height velocity at defined points of the pubertal growth period were compared with those of normal children entering puberty both at an average and late age.

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Physical growth from birth to adulthood in healthy Swiss children born 1954-1956 is described. The data are based on the First Zurich Longitudinal Study in which 137 individuals of each sex have been followed from birth to adulthood between 1954 and 1976. Distance standards of 20 anthropometric measurements such as weight, height and head circumference are presented as mean values and standard deviations or as median values (for weight and skinfold thickness) with smoothed empirical centiles.

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Congenital lipoid adrenal hyperplasia results in deficient virilization of genetic male infants, indicating that gonadal testosterone biosynthesis is impaired in such patients. In order to better define the characteristics of the gonadal lesion, we studied a Japanese genetic male infant with congenital lipoid adrenal hyperplasia who underwent bilateral orchiectomy at 14 months of age. The basal serum testosterone was low before and after orchiectomy (6.

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