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著者: Michael B Ranke, Anders Lindberg, Martin Carlsson, Cecilia Camacho-Hübner, Raoul Rooman
雑誌名: Horm Res Paediatr. 2019;91(1):46-55. doi: 10.1159/000498859. Epub 2019 Apr 2.
Abstract/Text
BACKGROUND/AIMS: There is little information how rhGH treatment affects height in NS. This study aims to analyze data from the NS patients assembled in KIGS over 25 years.
PATIENTS/METHODS: Of 613 (389 m/224 f) NS patients documented, 476 (302 m/174 f) were treated for 1 year, 237 (160 m/77 f) of which served to develop a 1st year height velocity (HV) prediction algorithm. One-hundred and forty (74 m/66 f) had reached near adult height (NAH). Factors affecting NAH on rhGH were determined.
RESULTS: At the start of rhGH, the NAH groups were (median, m, f) 11.0 and 10.3 years, with a height SDS of -3.2 and -3.8 SDS (Prader), respectively. The total gain after 6.3 and 5.6 years on rhGH (0.27 and 0.30 mg/kg/week) was 1.2 and 1.3 SDS. Age at the start of rhGH (negative), height at the start of rhGH, rhGH dose, number of rhGH injections/wk and birth weight (all positive) explained 36% of the variability of 1st year HV. Height at the start of rhGH, 1st year growth on rhGH, birth weight, and gender explained 74% of the variability of NAH. Causes for rhGH treatment discontinuation and adverse events were also analyzed.
CONCLUSION: rhGH treatment increases NAH in NS. Prediction algorithms may optimize treatment in the future.
© 2019 S. Karger AG, Basel.
PMID 30939478 Horm Res Paediatr. 2019;91(1):46-55. doi: 10.1159/000498859. Epub 2019 Apr 2.
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