PubMed · 4375364
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G Sterzik. 1974. [Embryology].. https://pubmed.ncbi.nlm.nih.gov/4375364/
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Marshall-Smith syndrome is characterized by accelerated osseous maturation, craniofacial anomalies, failure to thrive, psychomotor delay, hypotonia, pulmonary dysfunction, and limited life expectancy. We describe a 7-year-old girl who, in addition to meeting these criteria for Marshall-Smith syndrome, had multiple fractures and skeletal anomalies. The purpose of this report is to draw attention to Marshall-Smith syndrome as one of the skeletal dysplasias characterized by osseous fragility.
Growth retardation is a common problem in children with chronic renal failure (CRF). Few published data are available on whether the normalization of height in these children during growth hormone (GH) treatment is accompanied by proportional growth of the other parts of the body. In this study, body proportions before and during GH therapy were assessed in children with severe growth retardation due to CRF. Various body segments, such as sitting height, arm span, tibia, hand and foot length, biacromial and biiliacal diameter were measured in 15 children participating in a double-blind placebo-controlled cross-over trial and in 22 children participating in a double-blind dose-response trial. Twelve children continued GH therapy after having participated in one of the two former trials and received GH therapy for 4 years. All results were adjusted for age and sex, and expressed as SD scores using reference values for healthy Dutch children. To assess body proportions, the various body segments were related to height and expressed as shape values (SV). At baseline all body segments SD scores were significantly lower than zero, indicating that the stunted growth of children with CRF included all body segments. Since height was not significantly more or less affected than the other body segments, all children had normal SV, indicating normal body proportions. The placebo-controlled study showed a significant increase of the SD scores of height and several body segments during 6 months of GH [28 IU/m(2) per week (or 1.3 mg/m(2) per day)] versus placebo. The dose-response study demonstrated that height SDS as well as all other body segments SD scores increased significantly during 2 years of GH therapy with 28 IU/m(2) per week, compared with treatment with 14 IU/m(2) per week. Also during 4 years of GH therapy with 28 IU/m(2) per week, body segment SD scores increased to the same extent as height SDS, showing that GH did not significantly change SV i.e., body proportions. Both before and during GH therapy, children on dialysis had normal body proportions, comparable with children on conservative renal treatment. In conclusion, children with severe growth retardation due to CRF maintain normal body proportions in spite of their chronic disease. GH therapy with 28 IU/m(2) per week induces and maintains catch-up growth of height and all body segments without signs of disproportionate growth. Thus GH therapy does not negatively influence body proportions in children with severe growth retardation secondary to CRF.
The radiological atlas of Greulich and Pyle published in 1959 has been validated for the modern measurement of skeletal development in a population of white school-age children from Rotterdam. This is a useful study result. Although contemporary children grow to become much taller than in previous generations, this increased growth potential is apparently realised within the same time span.