Metabolic Health and Long-term Safety of Growth Hormone Treatment in Silver-Russell Syndrome.

Metabolic Health and Long-term Safety of Growth Hormone Treatment in Silver-Russell Syndrome.

J Clin Endocrinol Metab. 2016 Dec 21;:jc20163388

Authors: Smeets CC, Renes JS, van der Steen M, Hokken-Koelega AC

Abstract
CONTEXT: Children with Silver-Russell syndrome (SRS) are born small for gestational age (SGA) and remain short. Growth hormone (GH)-treatment improves height in short SGA children, including those with SRS. Data on metabolic health and long-term safety of GH-treatment in SRS are lacking.
OBJECTIVE: To investigate metabolic health in SRS patients during and until two years after discontinuation of GH-treatment.
DESIGN: Metabolic health was assessed longitudinally at GH-start, GH-stop, and six months and two years thereafter.
PATIENTS: 29 SRS patients versus 171 non-SRS subjects born SGA.
MAIN OUTCOME MEASURES: Lean Body Mass (LBM) and Fat Mass percentage (FM%) measured by DXA, Insulin sensitivity (Si) and β-cell function (DI) by frequently sampled intravenous glucose tolerance tests, and blood pressure and serum lipids.
RESULTS: At GH-start (mean (SD) age 5.4 (2.1) years in SRS and 6.7 (2.0) in non-SRS (p=0.003)), blood pressure, serum lipids, glucose and insulin levels were similar and within normal ranges in SRS and non-SRS. LBM SDS and FM% SDS were lower than average in both groups. During treatment, LBM SDS remained stable while FM% SDS increased in both groups. During the two years after GH-stop, LBM decreased and FM% increased, while Si and DI improved. At two years after GH-stop (mean age 18 years), all parameters were similar and within normal ranges in SRS and non-SRS. None of the SRS patients developed metabolic syndrome, DM2, or adverse events.
CONCLUSION: GH-treated SRS patients have a similar metabolic health and safety profile as non-SRS subjects born SGA, both during and until 2 years after GH-stop.

PMID: 28001454 [PubMed - as supplied by publisher]

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