The Russell-Silver syndrome without asymmetry

Gareis, Frank J.; Smith, David W.; Summitt, Robert L.

doi:10.1016/s0022-3476(71)80390-6

Cited by 39 publications

(9 citation statements)

References 7 publications

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“…Considerable skeletal growth retardation undoubtedly occurs in many small-for-dates babies (Usher, 1970), so that it is likely that large fontanelle size in these infants is a further manifestation of intrauterine growth retardation. The very large anterior fontanelle of the Russell-Silver syndrome (Gareis, Smith, and Summitt, 1971), where there is marked intrauterine growth retardation, seems to support this suggestion of a delay in osseous maturation of the skull bones in small-for-dates infants.…”

Section: Resultsmentioning

confidence: 71%

Anterior fontanelle size in the neonate.

Davies¹,

Ansari²,

Cooke³

1975

Archives of Disease in Childhood

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Section: Resultsmentioning

confidence: 71%

Anterior fontanelle size in the neonate.

Davies¹,

Ansari²,

Cooke³

1975

Archives of Disease in Childhood

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“…Although it is clear that the face, and particularly the mandible, is smaller than normal, the head circumference has been variously described as within normal limits (17,31) or small (29,37,38 Age, years about half of the patients fell within normal limits. nine boys and skinfold in these patients (47).…”

Section: Head Size Intelligence a N D Educationmentioning

confidence: 99%

The Natural History of the Silver-Russell Syndrome: A Longitudinal Study of Thirty-nine Cases

1975

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ExtractThe 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 S D 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 Silrer-Russell. I t is likely that some 10% of cases have birth weights in the -1.5 to 2 . 0 S D range. SpeculationA detailed longitudinal study of the growth and development of children with the syndromes of Silver and Russell rereals a characteristic growth curve, very little affected by administration of growth hormone; a normal, not advanced, puberty; and a poor outcome.Among children brought to the physician because of short stature there are a number who have a low birth weight for length of gestation. Some of these have recognizable syndromes, such as Turner's, congenital rubella. and de Lange's. Others. often equally recognizable, are short, thin (in the sense of lacking subcutaneous fat), and apparently healthy. They have a characteristic appearance; the face is triangular and small. the ears appear to be set low, the corners of the mouth are frequently turned downwards, and the mandible is small in relation to the maxilla. The fifth fingers are usually short and curved inwards. Although the relation of trunk to limbs is normal, in some of these patients the limbs, trunk. or face show a degree of asymmetry which exceeds the usual limits.These children were first recognized by Silver et al. (37) in 1953, who described two cases, both with characteristic asymmetry. Independently, Russell (33) in 1954 described five similar children of whom only two had asymmetry. In the subsequent literature some authors have separated the cases with asymmetry (Silver's syndrome) from those without it (Russell's syndrome, Russell's intrauterine dwarfism). whereas others have combined them. For reasons discussed below we have placed symmetrical and asymmetrical cases together under the name Silver-Russell syndrome.We have studied longitudinally the growth of 39 children with this.syndrome, measuring them at regular intervals for periods between I and 13 years. Nineteen have been treated for 1 or more years with human growth hormone. In addition, we have reviewed 66 cases reported in the literature up to the end of 1972. (This number excludes those reported by Tanner and H a m (44), which form part of the present series.) SUBJECTS A N D METHODSAmong all children referred to the Growth Disorder Clinic of The Hospital for Sick Children between 1960 and 1974 were 22 boys and 17 girls who satisfied the following three criteria: ( I ) stature 2 S D or more below the 50th percentile for height on the British standards (49); (2) birthweight adjusted for sex, length of ges...

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“…Approximately 150 patients have been reported as having SRS. Most have been sporadic, although a few familial cases have been reported (Silver & Gruskay 1957, Gray & Evans 1959, Silver 1959, Warkany et al 1961, Rimoin 1969, Callahan 1970, Ganner & Schwingschakl 1970, Fuleihan et al 1971, Gareis et al 1971, Taussig et al 1973.…”

mentioning

confidence: 99%