Abstract

Background:Blood transfusion is a traditional treatment for β-thalassemia (β-thal) that improves the patients’ anemia and lifespan, but it may lead to iron overload in parenchymal tissue organs and endocrine glands that cause their dysfunctions as the iron regulatory system can’t excrete excess iron from the bloodstream.Objective:To evaluate the prevalence of iron-related complications (short stature, growth retardation, and growth hormone deficiency) in β-thalassemia major (βTM) patients.Methods:We performed an electronic search in PubMed, Scopus, and Web of Sciences to evaluate the prevalence of growth hormone impairment in β-thalassemia major (βTM) patients worldwide. Qualities of eligible studies were assessed by the Joanna Briggs Institute checklist for the prevalence study. We used Comprehensive Meta-Analysis (Version 2) to calculate the event rate with 95% CIs, using a random-effects model for all analyses.Findings:Seventy–four studies were included from five continents between 1978 and 2019; 70.27% (Asia), 16.21% (Europe), 6.75% (Africa), 2.70% (America), 1.35% (Oceania), and 2.70% (Multicenter). The overall mean age of the participants was about 14 years. The pooled prevalence of short stature (ST) was 48.9% (95% CI 35.3–62.6) and in male was higher than female (61.9%, 95% CI 53.4–69.7 vs. 50.9%, CI 41.8–59.9). The pooled prevalence of growth retardation (GR) was 41.1% and in male was higher than in female (51.6%, 95% CI 17.8–84 vs. 33.1%, CI 9.4–70.2). The pooled prevalence of growth hormone deficiency (GHD) was 26.6% (95% CI 16–40.8).Conclusion:Our study revealed that near half of thalassemia patients suffer from growth impairments. However, regular evaluation of serum ferritin levels, close monitoring in a proper institute, suitable and acceptable treatment methods besides regular chelation therapy could significantly reduce the patients’ complications.

Highlights

  • Arab-Zozani et al.Annals of Global HealthThalassemia is the most prevalent inherited disease worldwide [1]

  • Our study revealed that near half of thalassemia patients suffer from growth impairments

  • We found that 41.1% of β-thalassemia major (βTM) patients all around the world are growth retarded

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Summary

Introduction

Thalassemia is the most prevalent inherited disease worldwide [1]. If the body is unable to produce sufficient amounts of these chains, an imbalance of hemoglobin chains will result in ineffective erythropoiesis and chronic hemolysis. This anemia starts in early childhood and continues throughout the whole life. If this chain deficiency presents in ɑ-chain of Hb, this type of thalassemia is called ɑ-thalassemia, but β-thal is the reduced synthesis of hemoglobin β-chain [2]. Blood transfusion is a traditional treatment for β-thalassemia (β-thal) that improves the patients’ anemia and lifespan, but it may lead to iron overload in parenchymal tissue organs and endocrine glands that cause their dysfunctions as the iron regulatory system can’t excrete excess iron from the bloodstream

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