How is Silver-Russell syndrome (SRS) treated?

Updated: Feb 11, 2019
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Answer

Significant effort must be undertaken to optimize caloric intake during evaluation of growth. Careful monitoring of linear growth after optimizing caloric intake is critical for early diagnosis of catch-up growth failure by 2 years of age and early intervention with recombinant human growth hormone (rhGH) for maximizing final adult height. Without treatment, mean adult height may be 151.2 cm (59.5 in) in males and 139.9 cm (55.1 in) in females. [16]

GH was approved in the United States in 2001 by the US Food and Drug Administration for use in children born SGA who have not yet manifested adequate catch-up growth by age 2 years. GH is given via daily subcutaneous injections. The recommended starting dose is 0.24 mg/kg/wk, but the dose can be titrated up to 0.48 mg/kg/wk, according to the response. A study by Smeets et al demonstrated that in SGA children who underwent GH treatment, patients with Silver-Russell syndrome achieved height gains similar to those in subjects without Silver-Russell syndrome. Height gain tended to be greater in Silver-Russell syndrome patients with mUPD7 than in those with 11p15 defects. Greater height gain was also seen in children with idiopathic Silver-Russell syndrome. [17]

Development

An early intervention program, including physical therapy, is beneficial. Special education courses are needed when the child is older.


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