Growth hormone therapy and growth in children with Noonan's syndrome: results of 3 years' follow-up

The Journal of Clinical Endocrinology and Metabolism
C E MacfarlaneC J H Kelnar

Abstract

Growth data from the first 3 yr of a multicenter study examining the efficacy and safety of recombinant human GH [rhGH; 4 IU (1.3 mg)/m(2).day, sc] in children with Noonan's syndrome (NS) are reported for 23 subjects. Sixteen male and seven female patients (age, 9.3 +/- 2.6 yr at onset of GH therapy, mean +/- SD; range, 4.8-13.7) were each assessed at 1, 2, and 3 yr after starting treatment. Comparisons were made with a group of eight subjects (six males and two females, age, 9.0 +/- 4.1 yr; range, 4.1-14.8) with NS, not treated with rhGH, measured over the same period. All treated subjects underwent annual cardiac assessment. Height SD score increased from -2.7 +/- 0.4 at the start of GH therapy to -1.9 +/- 0.9 3 yr later (P < 0.001, two-tailed t test). This corresponded to an increase in height from 116.1 +/- 13.2 to 137.3 +/- 14.0 cm. Height velocity increased from 4.4 +/- 1.7 cm/yr in the year before treatment to 8.4 +/- 1.7 (P < 0.001), 6.2 +/- 1.7 (P < 0.001), and 5.8 +/- 1.8 (P = 0.01, two-tailed t test compared with baseline) during the first, second, and third years of GH treatment, respectively. Height acceleration was not significant during the second or third years when pubertal subjects were excluded. The compariso...Continue Reading

Citations

Jul 22, 2010·Molecular Syndromology·M TartagliaB D Gelb
Dec 21, 2006·Reviews in Endocrine & Metabolic Disorders·Jacqueline A Noonan
Jul 9, 2011·Advances in Pediatrics·Bradley S Miller
Oct 9, 2015·Italian Journal of Pediatrics·Niki ZavrasMauro Bozzola
Jun 16, 2005·The Journal of Clinical Endocrinology and Metabolism·Lize V FerreiraAlexander A L Jorge
Sep 12, 2003·Der Internist·J StrehlT Sauerbruch
Feb 23, 2021·International Journal of Endocrinology and Metabolism·Louise Jayne ApperleySenthil Senniappan
Jun 22, 2021·Frontiers in Endocrinology·Fernando RodríguezFernando Cassorla

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