Early start of growth hormone is associated with positive effects on auxology and metabolism in Prader-Willi-syndrome
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(2020) 15:283
RESEARCH
Open Access
Early start of growth hormone is associated with positive effects on auxology and metabolism in Prader-Willi-syndrome Lucy Magill1, Constanze Laemmer2, Joachim Woelfle3, Rolf Fimmers4 and Bettina Gohlke1*
Abstract Background: Prader-Willi-Syndrome (PWS) is characterized by hypothalamic-pituitary dysfunction. Recent research suggests starting growth hormone-treatment (GHT) as soon as possible. The aim of this study is to analyze possible differences in auxological parameters, carbohydrate and lipid metabolism between two groups of children with PWS that started GHT either during or after their first year of life. Study design: Retrospective longitudinal study of 62 children (31 males) with genetically confirmed PWS. Upon diagnosis all children were offered GHT, some started immediately, others commenced later. Cohort A (n = 21; 11 males) started GHT at 0.3–0.99 yrs. (mean 0.72 yrs) and Cohort B (n = 41; 20 males) commenced GHT at 1.02–2.54 yrs. (mean 1.42 yrs) of age. Fasting morning blood samples and auxological parameters were obtained before the start of therapy and semi-annually thereafter. Differences between the two cohorts were estimated with a linear mixed-effect model. Results: Mean length/height-SDSPWS differed significantly between the groups [1 yr: A: 0.37 (±0.83) vs B: 0.05 (±0.56); 5 yrs.: A: 0.81 (±0.67) vs B: 0.54 (±0.64); p = 0.012]. No significant differences were found in BMI, lean body mass or body fat. Low-density cholesterol was significantly lower in A than in B [LDL: 1 yr: A: 79 (±20) mg/dl vs B: 90 (±19) mg/dl; 5 yrs.: A: 91(±18) mg/dl vs 104 (±26) mg/dl; p = 0.024]. We found significant differences in the glucose homeostasis between the groups [fasting insulin: p = 0.012; HOMA-IR: p = 0.006; HbA1c: p < 0.001; blood glucose: p = 0.022]. Conclusions: An early start of GHT during the first year of life seems to have a favorable effect on height-SDS and metabolic parameters. Keywords: Prader-Willi-syndrome, Growth hormone therapy, Carbohydrate and lipid metabolism, Insulin-like growth factor-I
Background Prader-Willi-Syndrome (PWS), first described by Prader, Willi, and Labhart in 1956 [1], is a rare multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction [2]. The population prevalence of this disorder has been estimated between 1/10,000–1/30,000 * Correspondence: [email protected] 1 Department of Pediatric Endocrinology and Diabetology, Children’s Hospital, University of Bonn, Venusberg-Campus, Building 30, 53127 Bonn, Germany Full list of author information is available at the end of the article
[3]. Characteristic features in children with PWS are severe neonatal hypotonia and feeding difficulties with failure to thrive, an early onset of hyperphagia with food seeking behavior and, accordingly, with a progressive development of severe obesity, an abnormal body composition and a short stature, a delayed overall development with cognitive deficiency and behavioral abnormalities [3–6]. Several endocrine problems such as
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