The short-term effects of growth hormone therapy on height velocity and cardiac ventricular wall thickness in children with Noonan's syndrome

The Journal of Clinical Endocrinology and Metabolism
A M CotterillM O Savage

Abstract

Noonan's syndrome (NS) is associated with short stature and cardiac defects. Small studies reported linear growth increases with recombinant human GH (rhGH) therapy, but also raised concerns related to the anabolic effects of rhGH and the possible progression of ventricular hypertrophy. We report a multicenter study examining the efficacy and safety of rhGH (4 IU/m2.day, sc) in children with NS. Entry criteria were: NS confirmed by single observer, height SD score less than -2(UK Height Standards 1990), prepubertal, and normal maximal left ventricular (LV) wall thickness less than 1 cm by 2-dimensional echocardiography. Thirty subjects were recruited (19 males and 11 females), aged 8.9 +/- 0.5 yr (range, 4.8-13.7 yr). Growth was monitored for 12 months before and at 3-month intervals during therapy. Measurements of maximal LV wall thickness were taken at 0 and 12 months. Serum insulin-like growth factor I(IGF-I), IGF-II, and IGF-binding protein-3 levels were determined at 0, 3, 6, 9, and 12 months. Ten subjects with NS (4 females and 6 males), aged 8.8 +/- 0.7 yr (range, 6.3-11.8 yr), were monitored over the same period as a comparison group. In the treatment group, 27 subjects completed 12 months of therapy. Height SD score in...Continue Reading

Citations

Apr 24, 2001·Archives of Disease in Childhood·J M Kirk Group tU
Jul 22, 2010·Molecular Syndromology·M TartagliaB D Gelb
Dec 21, 2006·Reviews in Endocrine & Metabolic Disorders·Jacqueline A Noonan
Oct 9, 2015·Italian Journal of Pediatrics·Niki ZavrasMauro Bozzola
Feb 13, 2007·Journal of Plastic, Reconstructive & Aesthetic Surgery : JPRAS·J G Qian, X J Wang
Jun 22, 2021·Frontiers in Endocrinology·Fernando RodríguezFernando Cassorla

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