OBJECTIVE: To determine the prevalence of growth abnormality and endocrine dysfunction in a group of multi transfused thalassemic children and to correlate these with their body iron stores. METHODS: This cross sectional study included 35 Thalassemia Major patients, aged 13 to 24 years. Growth and puberty were assessed clinically and the laboratory values of various hormone levels were stratified with their age and sexual maturity. RESULTS: 57.14% patients were short, 60% had not attained puberty, and 87.5% of the girls had primary amenorrhea. 14.29% had low FSH and 2.86% low LH levels. 89.47% of the boys had low free testosterone and 43.75% of the girls had low estradiol levels. While 20% had high TSH levels, 40% had high PTH levels, of which 92.8% had low levels of Vitamin D. Low levels of IGF-1 were noted in 51.43%. CONCLUSIONS: In this study analysis, short stature and hypogonadism were frequent findings. These results support the need for vigilant clinical evaluation of growth and puberty, as well as appropriate hormonal evaluation in poly transfused thalassemic children in order to detect and treat endocrine dysfunction early. The authors also recommend aggressive and adequate chelation from early life so that permanent damage to the endocrine glands can be prevented.
OBJECTIVE: To determine the prevalence of growth abnormality and endocrine dysfunction in a group of multi transfused thalassemic children and to correlate these with their body iron stores. METHODS: This cross sectional study included 35 Thalassemia Major patients, aged 13 to 24 years. Growth and puberty were assessed clinically and the laboratory values of various hormone levels were stratified with their age and sexual maturity. RESULTS: 57.14% patients were short, 60% had not attained puberty, and 87.5% of the girls had primary amenorrhea. 14.29% had low FSH and 2.86% low LH levels. 89.47% of the boys had low free testosterone and 43.75% of the girls had low estradiol levels. While 20% had high TSH levels, 40% had high PTH levels, of which 92.8% had low levels of Vitamin D. Low levels of IGF-1 were noted in 51.43%. CONCLUSIONS: In this study analysis, short stature and hypogonadism were frequent findings. These results support the need for vigilant clinical evaluation of growth and puberty, as well as appropriate hormonal evaluation in poly transfused thalassemic children in order to detect and treat endocrine dysfunction early. The authors also recommend aggressive and adequate chelation from early life so that permanent damage to the endocrine glands can be prevented.
Authors: S De Virgiliis; P Cossu; C Toccafondi; G Sanna; F Frau; R Lobrano; G Cornacchia; A Nucaro; A Loi; F Bertolino; A Cao Journal: Am J Pediatr Hematol Oncol Date: 1983
Authors: Abdulqadir J Nashwan; Mohamed A Yassin; Mohamed Izham Mohamed Ibrahim; Hanan F Abdul Rahim; Mujahed Shraim Journal: Front Med (Lausanne) Date: 2022-03-21
Authors: Mohamad H Qari; Yasser Wali; Muneer H Albagshi; Mohammad Alshahrani; Azzah Alzahrani; Ibrahim A Alhijji; Abdulkareem Almomen; Abdullah Aljefri; Hussain H Al Saeed; Shaker Abdullah; Ahmad Al Rustumani; Khoutir Mahour; Shaker A Mousa Journal: Orphanet J Rare Dis Date: 2013-09-17 Impact factor: 4.123