Sukla Samaddar
Department of Pediatrics, Kalawati Saran Children’s Hospital & Lady Hardinge Medical College, New Delhi, India
Title: Assessment of pubertal status in adolescents with transfusion dependent thalassemia: A Cross sectional study
Biography
Biography: Sukla Samaddar
Abstract
Children with transfusion dependent thalassemia (TDT) suffer from various endocrinological manifestations due to iron deposition: particularly hypogonadism. We performed this study to assess pubertal status in children with TDT in recent, at a tertiary care hospital in Northern India. Fifty-eight patients aged 17 to 19 years [Males = 33(56.9%)] with TDT who were on regular follow-up and chelation therapy were included. After a detailed clinical assessment, the past records of these patients were reviewed. Sixteen (27.6%) patients in the cohort had pubertal arrest or failure and were started on hormonal replacement therapy (HRT). A further 8 (13.8%) patients had delayed onset but normal progression of puberty, while the remaining 34 (58.6%) had normal onset and progression of puberty. The HRT group had a significantly higher annual transfusion rate (ATR) [Mean (SD) = 146.4 (17.2) vs. 135.6 (11.3) ml/kg (p=0.007)] and serum ferritin levels [mean (SD) = 5084.9 (1640.5) vs. 2752.4 (1082.0) ng/ ml (p=0.0001)] compared to the non-HRT group. Ten (62.5%) patients in the HRT group had elevated hepatic transaminases (> 2 times the upper limit of normal) compared to 10 patients (23.8%) in the other group (p=0.006). HRT group had higher prevalence of hypoparathyroidism than the non-HRT group [4 (25%) vs. 1(2.3%); p= 0.006]. Other co-morbidities such as hypothyroidism, Type 1 diabetes mellitus, seropositivity for hepatotrophic virus (hepatitis B and C) and severe magnetic resonance imaging T2 * liver and cardiac scores did not show correlation with the pubertal status.
Conclusion: Higher ATR and serum ferritin levels, presence of liver dysfunction significantly correlate with pubertal failure.