Abstract
Extract: The growth of 39 children with Silver-Russell syndrome has been followed for 1—13 years. Pregnancy and labor were normal; none of the 61 sibs had the syndrome. Height at referral (mean age 4.6 years) averaged 3.6 SD below the mean and remained at this level during subsequent growth. Bone age averaged 69% of normal at referral but caught up by puberty, which occurred at the normal time. Nineteen cases were treated with human growth hormone without lasting effect. There is no clear-cut distinction between the Silver and Russell syndromes; the name should be Silver-Russell. It is likely that some 10% of cases have birth weights in the −1.5 to −2.0 SD range.
Speculation: A detailed longitudinal study of the growth and development of children with the syndromes of Silver and Russell reveals a characteristic growth curve, very little affected by administration of growth hormone; a normal, not advanced, puberty; and a poor outcome.
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Tanner, J., Lejarraga, H. & Cameron, N. The Natural History of the Silver-Russell Syndrome: A Longitudinal Study of Thirty-nine Cases. Pediatr Res 9, 611–623 (1975). https://doi.org/10.1203/00006450-197508000-00001
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DOI: https://doi.org/10.1203/00006450-197508000-00001
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