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...

In Argentina, there is no information on ages of attainment of developmental milestones and very few data about environmental factors that influence them. A national survey on the psychomotor development of children under 6 years of age was carried out with the help of 129 paediatricians. Logistic regression was applied to a final sample of 3573 healthy, normal children in order to estimate selected centiles (25th, 50th, 75th and 90th), together with their respective confidence intervals, of the ages of attainment of 78 developmental items belonging to the following areas: personal-social (18 items), fine motor (19), language (18) and gross motor (23). The 50th centile obtained for each of the 43 comparable items was compared with those obtained in previously standardised tests: DDST, Denver II, Bayley and Chilean scales. Neither significant nor systematic differences were found between our results and those described in the tests used for comparison. Multiple logistic regressions showed that social class, maternal education and sex (female) were associated with earlier attainment of some selected developmental items, achieved at ages later than 1 year. Selected items achieved before the first year of life were not affected by any of the independent environmental variables studied. The information is useful in helping paediatricians in their daily practice for surveillance of development, as baseline information for epidemiological studies on development in our country and for cross-cultural analysis.

references presented here can also be used in other countries with similar ethnographics characteristics.

Information on prevalence and type of problems of psychomotor development (PPD) is necessary for implementation of specific care programmes at field level. With the purpose of obtaining this information, a screening test, the Prueba Nacional de Pesquisa (PRUNAPE) for PPD was implemented in three health centres in San Isidro, a city near Buenos Aires, attended by different socio-economic groups: centres A and B were located in the inner city, and C in a middle-class area. The test was administered by three previously trained paediatricians to 839 apparently healthy children aged 0-5 years. The failure rates were 24%, 19% and 16% in centres A, B and C respectively (20% in total). Out of the 170 children failing the test and referred to hospital for diagnosis and treatment, only 96 complied and went through a series of studies carried out by a previously prepared multidisciplinary team. With the exception of children who failed the Battelle test [classified as Global Developmental Delay (GDD)], final diagnoses were classified according to Diagnostic and Statistical Manual of Mental Disorders, 4th edition: GDD (60 children), pervasive developmental disorders (11), communication disorders (10), motor disorders (6, of whom 2 were with cerebral palsy), attention deficit disorders (5), attachment disorders (2), normal children (3). Co-morbidity was present in 22 affected children. Forty-three per cent of children failing the test did not attend hospital or did not complete studies because of major social and family problems, the family not living in the area, or the parents preferring to consult their own paediatrician. Health centres and children not selected in a randomised way, and a significant proportion of them not complying with the indication of hospital referral were major sources of bias, so that PPD prevalences, positive and negative predictive values should be interpreted with great caution. Further studies accounting for these sources of bias are needed to confirm the observed prevalence of PPD. Training of health personnel, at hospital and health centre level, priority settings, and operational research to evaluate effectiveness of treatments and care delivery systems at field level are necessary in Argentina for optimal use of limited healthcare resources.

No abstract

The name opsismodysplasia is proposed for a new chondrodysplasia, which was studied in three patients. Clinically, the condition is recognized at birth on the basis of shortness, short hands, and facial abnormalities with a short nose and a depressed bridge of nose. The most characteristic radiographic signs are: very retarded bone maturation; marked shortness of the bones of the hands and of the feet with concave metaphyses; and thin, lamellar vertebral bodies. The growth cartilage studied in one case showed a wide hypertrophic area containing thick connective tissue septa, irregular provisional calcification, and vascular invasion. Type I collagen was detected in the hypertrophic area by immunohistochemical and microchemical tests. The transmission of opsismodysplasia is probably autosomal recessive.

No abstract

Renal disease is the most important long-term complication of hemolytic-uremic syndrome (HUS). A comparative study of renal function was carried out in two groups of patients. Group 1 included 19 children followed for a median of 11 years, 1960-1980, with a low-sodium diet, antihypertensive drugs, and a restricted protein intake in the end stage of renal disease. Group 2 included 26 children treated for a median of 9 years, 1988-2002, on a low-sodium diet, early restriction of protein intake according to recommendations, and angiotensin converting enzyme inhibitors (ACEi). Long-term renal function was assessed by the inverse of the plasma creatinine concentration (1/[Cr]) over time. Linear regression lines were fitted to individual values of 1/[Cr] for each child. Regression coefficients of children in group 1 were all negative, ranging from -0.031 to -0.00043; 7 were significantly different from zero, indicating a linear fall in renal function over time. In contrast, children from group 2 had 11 negative slopes (only 1 significant) and 15 positive slopes, ranging from 0.17893 to -0.3899. Fisher's exact test showed that group 1 had significantly more children with negative slopes than group 2. This comparatively better long-term outcome of renal function in children under contemporary treatment was probably associated with early restriction of protein and use of ACEi.