GROWTH-RETARDATION IN HOMOZYGOUS SICKLE-CELL DISEASE - ROLE OF CALORIE INTAKE AND POSSIBLE GENDER-RELATED DIFFERENCES

To evaluate the possible role of inadequate food intake in the pathoge nesis of the growth retardation of patients with sickle cell anemia, w e determined the daily intake of calories and macronutrients and measu red several anthropometric indices in 20 patients with sickle cell ane mia aged 17-35 years and in 15 of their normal siblings of similar age . Compared to the control groups, the male patients, but not the femal es, had a significantly lower mean weight, body mass index, midarm cir cumference, and triceps and subscapular skinfold thicknesses. Also, wh ile the male patients consumed significantly less total calories, prot eins, carbohydrates, and fats per day than their control group, no dif ference was noted between the daily intake of calories or macronutrien ts in the female patients and their control group. However, when the i ntake of calories and macronutrients was corrected for body weight, th ere was no statistically significant difference between the intake of nutrients in the male patients and their control subjects or between t he female patients and their control group. These results suggest a se x-related difference in the somatic growth of adolescent and adult pat ients with sickle cell anemia and also suggest that, although an inade quate food intake may be partly responsible for the impaired somatic g rowth in sickle cell anemia, other factors are also probably important . (C) 1993 Wiley-Liss, Inc.