Background: Research has shown that some of the endocrine disorders in patients with sickle cell disease include delayed growth and pubertal development. No study has been done in Kenya to investigate whether this applies for the local children with sickle cell anaemia.
Objective: To describe growth and pubertal development in children and adolescents with Sickle cell disease.
Methodology: This was a cross-sectional descriptive study involving 142 children with confirmed sickle cell disease who were being followed up at Kenyatta National Hospitals Paediatric Hematology clinic. Demographic information including age, gender, tribe and socio-economic data were obtained by standard questionnaire while the clinical information including height, weight and BMI were obtained by actual measurements and plotted on the CDC charts for age and gender. Pubertal status in girls was determined by assessing breast tanner stage while in boys it was assessed by measuring the testicular volume. Presence of pubic and axillary hair was assessed in both.
Results: The median age of patients was 7.0 years (IQR 5.5, 9.5 years). Of these patients 86 (60.6%) were male and 56 (39.4%) were female with a male to female ratio of 43:28. Those with underweight were 16.3% male and 16.1% female (<−2 S.D.) while 11.1% male and 13.5% females were stunted (<−2 S.D.).33.9% female and 30.2% male had BMI of less than −2SD. None of the differences in the anthropometry parameters between male and females were statistically significant. Among boys aged > 9 years (n=29) 20 (69%) had testicular size tanner (< 3cc) while among girls aged >8 years (n=22) 18 (82.6%) had breast tanner <2 (pre-pubertal). Twenty girls (96.4%) and all the boys in their respective age groups had no axillary hair while 19 of the girls (85%) and 27 of the boys (93%) had no pubic hair.
Conclusion: A large proportion of these children were either underweight, stunted or had low BMI. There is need for longitudinal studies to determine whether this growth failure is due to the sickle cell condition or other environmental factors. Most of the children with sickle cell disease had not initiated puberty at their expected respective ages. Follow up needs be done on these patients to determine at what age they will go into puberty and what effects this condition has on their fertility rate.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology