..., instructions or products referred to in the content or advertisements. 2024 Prader-Willi syndrome Growth hormone Resting energy expenditure Energy intake Prader-Willi syndrome (PWS) is a rare genetic disorder, caused by the lack of expression of imprinted genes in the PWS region...

...Lionne N. Grootjen; Gwenaelle Diene; Catherine Molinas; Véronique Beauloye; T. Martin Huisman; Jenny A. Visser; Patric J.D. Delhanty; Gerthe F. Kerkhof; Maithe Tauber; Anita C.S. Hokken-Koelega Introduction: Prader-Willi syndrome (PWS) is characterized by a switch from failure to thrive...

...Alicia F. Juriaans; Gerthe F. Kerkhof; Mark Garrelfs; Demi Trueba-Timmermans; Anita C.S. Hokken-Koelega Introduction: Schaaf-Yang syndrome (SYS) is a rare neurodevelopmental disorder caused by truncating mutations of the MAGEL2 gene, located in the Prader-Willi syndrome (PWS) region. PWS and SYS...

...Urs Eiholzer; Anika Stephan; Christiane Fritz; Claudia Katschnig; Cees Noordam; on behalf of the Clinical and Scientific Advisory Board of the International Prader-Willi Syndrome Organisation (IPWSO) Background: Prader-Labhart-Willi syndrome (PWS) is a rare genetic disorder characterized...

...Maja Zimmermann; Constanze Laemmer; Joachim Woelfle; Rolf Fimmers; Bettina Gohlke Objective: The aim of this study was to consider sleep apnea in Prader-Willi syndrome (PWS) children depending on age at growth hormone (GH) therapy onset. Study Design: We analyzed longitudinally cardiorespiratory...

...Nienke E. Bakker; Elbrich P.C. Siemensma; Marjon van Rijn; Dederieke A.M. Festen; Anita C.S. Hokken-Koelega Background/Aims: Growth hormone (GH) treatment is beneficial for children with Prader-Willi syndrome (PWS), but data about health-related quality of life (HRQOL) and effects of GH treatment...

...N.E. Bakker; E.P.C. Siemensma; C. Koopman; A.C.S. Hokken-Koelega Background/Aims: Dietary management is a difficult but key aspect of care in children with Prader-Willi syndrome (PWS). We therefore investigated the effect of growth hormone (GH) treatment on reported energy intake in children...

... Prader-Willi syndrome (PWS) infant boys and have not been reported in PWS infant girls. Objectives: To measure gonadotropins and gonadal hormones in PWS male and female infants and assess gender-specific patterns of hormone secretion. Methods: Hormone levels in 14 (9 male, 5 female) PWS infants ages 1-3...

...Girolamo Di Giorgio; Graziano Grugni; Danilo Fintini; Sarah Bocchini; Sabrina Spera; Marina Cuttini; Marco Cappa; Antonino Crinò Background: In Prader-Willi syndrome (PWS) a reduced growth hormone (GH) response to several stimulators has been documented in many studies, but none have focused...

...I.C. van Nieuwpoort; M. Sinnema; J.A. Castelijns; J.W.R. Twisk; L.M.G. Curfs; M.L. Drent Background: In adults with Prader-Willi syndrome (PWS), limited information is available about pituitary function, more specifically the prevalence of growth hormone deficiency (GHD). The aim of this study...

...A. Colmenares; G. Pinto; P. Taupin; A. Giuseppe; T. Odent; C. Trivin; K. Laborde; J.C. Souberbielle; M. Polak Background/Aims: Prader-Willi syndrome (PWS) is a complex genetic disorder whose many manifestations include obesity and short stature. Diabetes, osteoporosis, and scoliosis are common. We...

..., Kleinwuchs, Kryptorchidismus und Oligophrenie nach myotoniertigem Zustand im Neugeborenenalter. Schweiz Med Wochenschr 1956;6:1260–1261. 2. Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY, Greenberg F: Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics 1993;91:398–402...

...Talia Eldar-Geva; Harry J. Hirsch; Ron Rabinowitz; Fortu Benarroch; Orit Rubinstein; Varda Gross-Tsur Background/Aims: To investigate the etiology of hypogonadism in women with Prader-Willi Syndrome (PWS). Methods: Ten women aged 23 ± 5.5 years with PWS and 10 age- and BMI-matched controls were...

..., hyperglycemia and a low serum testosterone level must be emphasized. The patient fulfills the clinical criteria of typical Prader-Willi syndrome. Cytogenetic and fluorescence in situ hybridization analysis showed a karyotype 47, XXY, del(15)(qll;ql3). To our knowledge this is the first report...

...A.C. Lindgren; A. Lindberg Background: Abnormal body composition, with low muscle mass and increased fat mass, as well as short adult stature are common features in Prader-Willi syndrome (PWS), as in growth hormone (GH) deficiency. Methods: We followed a cohort of 22 genetically verified patients...

... function. Of the 1,135 patients in KIGS with Prader-Willi syndrome, GH secretion was tested in 424 (37%). While 26% of those tested had normal GH levels, nearly three quarters of the patients had low GH levels. IGF-I levels are also low in nearly all children with Prader-Willi syndrome. These data...

...A. Crinò; G. Di Giorgio; M. Manco; G. Grugni; A. Maggioni In Prader-Willi syndrome (PWS) growth hormone therapy (GHT) improves height, body composition, agility and muscular strength. In such patients it is necessary to consider the potential diabetogenic effect of GHT, since they tend to develop...

...Charlotte Höybye Background: In Prader-Willi syndrome (PWS) obesity and partial growth hormone (GH) deficiency are frequently observed. The risks of cardiovascular diseases and early death are increased. We examined inflammatory markers in adult PWS, before and during 12 months of GH treatment...

... for the pathogenesis of obesity in this syndrome. 6 07 2005 7 11 2005 1 2 2006 Prader-Willi syndrome (PWS) is a genetic disorder due to the absence or lack of expression of the paternally inherited genetic material located in region 15q11,q13. The clinical features include neonatal hypotony...

... from any ideas, methods, instructions or products referred to in the content or advertisements. Ghrelin Growth hormone secretagogue receptor Fetal pancreas Anorexia nervosa Obesity Prader-Willi syndrome Growth hormone secretagogues (GHS) are artificial compounds with potent growth...