Abstract
We have previously shown that severe growth retardation in sickle cell disease is frequently associated with primary gonadal hypofunction and/or hGH deficiency. The present study was undertaken to determine the prevalence of growth retardation, retarded skeletal maturation and delayed puberty in an ambulatory clinic population: single determinations of weight age, height age, skeletal age and pubertal development (when appropriate) were made in 105 boys and girls with homozygous sickle cell disease (age range 5-17 yr). Weight age was retarded >2 SD in 10% and height age was retarded >2 SD in 20% (compared to standards for North American Negro children of similar socioeconomic groups); skeletal age was retarded >2 SD in 30% (compared to standards of Gruelich and Pyle). Skeletal maturation was retarded in 72% of the girls (p<0.005) and 50% of the boys (p<0.01) with retarded height age; 56% (9/16) of the adolescents had delayed puberty which was associated with retarded bone age in 9/9,retarded height age in 7/9 and retarded weight age in 7/9. Of the 7/16 adolescents with normal pubertal development, 5/7 had normal height age and 6/7 had normal weight age and skeletal maturation.
We conclude that a significant proportion of children with sickle cell disease have retarded growth, skeletal maturation, and pubertal development.
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Castells, S., Kassner, E. & Muthukrishnan, N. 3 GROWTH, SKELETAL NATURATION AND PUBERTY IN SICKLE CELL DISEASE. Pediatr Res 12 (Suppl 4), 364 (1978). https://doi.org/10.1203/00006450-197804001-00008
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DOI: https://doi.org/10.1203/00006450-197804001-00008