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Early start of growth hormone is associated with positive effects on auxology and metabolism in Prader-Willi-syndrome

Magill L, Laemmer C, Wölfle J, Fimmers R, Gohlke B (2020)

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Publication Type: Journal article

Publication year: 2020

Journal

Book Volume: 15

Article Number: 283

Journal Issue: 1

DOI: 10.1186/s13023-020-01527-0

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.

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APA:

Magill, L., Laemmer, C., Wölfle, J., Fimmers, R., & Gohlke, B. (2020). Early start of growth hormone is associated with positive effects on auxology and metabolism in Prader-Willi-syndrome. Orphanet Journal of Rare Diseases, 15(1). https://doi.org/10.1186/s13023-020-01527-0

MLA:

Magill, Lucy, et al. "Early start of growth hormone is associated with positive effects on auxology and metabolism in Prader-Willi-syndrome." Orphanet Journal of Rare Diseases 15.1 (2020).

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