Abstract

Background: Beta-thalassemia major (β-TM), is a type of chronic, microcytic anaemia that is characterized by impaired biosynthesis of the β-globin chain leading to accumulation of unpaired α-globin chain. Due to the severe anaemia caused in Thallassemia, patient must undergo repeated blood transfusions for survival. Repeated blood transfusions lead to built-up of iron overload which is responsible for tissue deposition of excess iron, ultimately resulting in iron toxicity. There are evidences of aminoaciduria, hypercalciuria, phosphaturia, magnesiuria, hyperuricosuria, low urine osmolality, and excess urinary secretion of markers of tubular damage such as N-acetyl-D-glucosaminidase in patients with beta-thalassemia major. In the present study, authors tried to investigate the proportion of patients diagnosed with β-Thalassemia Major manifesting renal dysfunction and electrolyte imbalance.
 Material and Methods: It was a hospital based case-control study involving 100 patients coming to Department of Pediatric Medicine, and belonging to the age group 1 to 18 years. Equal number of healthy age and sex matched children were taken as controls.
 Results: When cases and controls were compared with respect to albuminuria, blood pH, serum bicarbonate, phosphorus and blood urea, higher number of cases had deranged values as compared to the healthy controls [p<0.05].
 Conclusion: Renal damage and tubular dysfunctions leading to eletrolyte imbalances exist in children with β-thalassemia major.
 Keywords: kidney, Thalassemia, Electrolytes, children.

Highlights

  • Background: Beta-thalassemia major (β-TM), is a type of chronic, microcytic anaemia that is characterized by impaired biosynthesis of the β-globin chain leading to accumulation of unpaired α-globin chain

  • When cases and controls were compared with respect to albuminuria, blood pH, serum bicarbonate, phosphorus and blood urea, higher number of cases had deranged values as compared to the healthy controls [p

  • Renal damage and tubular dysfunctions leading to eletrolyte imbalances exist in children with β-thalassemia major

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Summary

Introduction

Beta-thalassemia major (β-TM), is a type of chronic, inherited, microcytic anaemia that is characterized by impaired biosynthesis of the β-globin chain leading to accumulation of unpaired α-globin chain. Beta thalassemia major is the most prevalent type of thalassemia It produces severe anaemia in its homozygous state.[1] Due to the severe anaemia caused in Thallassemia, patient must undergo repeated blood transfusions for survival. Beta-thalassemia major (β-TM), is a type of chronic, microcytic anaemia that is characterized by impaired biosynthesis of the β-globin chain leading to accumulation of unpaired α-globin chain. Conclusion: Renal damage and tubular dysfunctions leading to eletrolyte imbalances exist in children with β-thalassemia major.

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