Asymmetry, Short Stature, and Variations in Sexual Development

Silver, Henry K.

doi:10.1001/archpedi.1964.02080060497011

Cited by 109 publications

(33 citation statements)

References 38 publications

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“…This aspect of treatment seems to merit further investigation. An abnormal timing or pattern of puberty was originally I 2 J r 5 6 7 B 9 10 1 1 11 I > 14 IS i d 11 ra 19 reported by Silver et al (35,37) as one of the features of the syndrome. However, increased knowledge of the great variation to Fig.…”

Section: Effect O F H U M a N G R O W T H H O R M O N E O N H E I G Hmentioning

confidence: 90%

“…An occasional familial incidence has been suggested by two reports in which sibs were affected (8,16). Rimoin (30) described the syndrome in a pair of identical twins: Silver (35) in one twin only in two pairs, one like-and one opposite-sexed. Pregnancy is apparently normal in the majority of cases, as is length of gestation and labor.…”

Section: Etiology a N D T R E A T M E N Tmentioning

confidence: 98%

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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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Section: Effect O F H U M a N G R O W T H H O R M O N E O N H E I G Hmentioning

confidence: 90%

Section: Etiology a N D T R E A T M E N Tmentioning

confidence: 98%

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

1975

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“…Russell [80] published five similar cases in 1954 emphasizing the craniofacial abnormalities in these children with severe intra-uterine growth retardation. Meanwhile it has been accepted that this is a uniform malformation syndrome with a severe reduction in weight and length at birth, short stature in later life, typical craniofacial abnormalities with a relatively large, prominent forehead, a small, triangular face, asymmetry of head and limbs and other less constant abnormalities [3,59,81,91,107]. The term Silver-Russell syndrome (SRS) is used to describe these patients.…”

Section: Introductionmentioning

confidence: 99%

Growth and symptoms in Silver-Russell syndrome: Review on the basis of 386 patients

et al. 1995

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“…While more than 100 cases of the syndrome have been reported, no autopsy findings have been described (Silver, 1964). We report here such an instance.…”

Section: Introductionmentioning

confidence: 70%