Low birthweight dwarfism with asymmetry (silver's syndrome): Treatment with human growth hormone

Abstract

Six children with low birthweight dwarfism and asymmetry (Silver's syndrome) are described, and 2 with all the features of the syndrome except that either birthweight or stature were just within the lower limits of normal. In addition to the usual features, 4 had bilateral pseudo-epiphyses at the base of the second metacarpals, and 1 a true supernumerary epiphysis, signs not previously noted in this disorder. Two of these children were treated with human growth hormone (approximately 24 I.U./week) and responded with significant increases in rate of height growth. One child was treated for one year, the other for three; the latter reached the 3rd centile. It is proposed that the term Silver's syndrome be restricted to children of short stature (without microcephaly or other special features) who have low birthweight for the length of gestation, and measurable asymmetry of arms, legs, body, or head, with incurved 5th fingers, usually with a deformity visible on x-ray. The term Russell dwarf should be restricted to similar children but without asymmetry. The facial appearances are similar in the two syndromes. The arms, measured from shoulder to wrist, are short relative to the legs when compared with healthy children of either the same age or size. It seems that some children may exist who have somewhat low birth-weights (i. e. above 2 kg.) and are somewhat small (i. e. above the 3rd centile) but have the other features of this syndrome; they may be examples of mild degrees of intrauterine growth pathology.