PULMONARY FUNCTION TEST IN MULTITRANSFUSED CHILDREN WITH THALASSEMIA

Abstract

Thalassemia is the most common monogenetic disorder worldwide and is a common cause of chronic haemolytic anaemia. It was first described by Thomas B Colley in 1925. The disease is transmitted by autosomal recessive manner. Thalassemia is a major health problem, placing an emotional, psychological and economic burden on millions of people around the World. Recent data indicates that about 3% of the World’s population is a carrier of beta thalassemia. Every year 3,00,000 to 5,00,000 children are born each year worldwide with the severe homozygous state of this disease¹.

Blood transfusion is the mainstay of care for individuals with thalassemia major and many with intermedia. Over the past three decades, regular blood transfusions and iron chelation have dramatically improved the quality of life and thalassemia is transformed from a rapidly fatal disease to a chronic disease and is compatible with prolonged life.
The most important complication in patients with transfusion-dependent thalassemia is progressive iron accumulation secondary to transfusion of the blood (secondary hemochromatosis) and iron accumulation-induced organ damage.² The heart, liver, and pancreas are the target organs most frequently involved in which extensive iron induced injury is regularly observed at autopsy, although abnormalities of lung mechanics have been reported by almost all studies of patients with thalassemia major. The fact that iron accumulation in the lungs is known in these patients, but how this accumulation affects the lungs is still a matter of debate².

Many studies on patients with thalassemia have demonstrated restrictive lung disease, obstructive lung disease and diffusion abnormalities^3-7. However, there is no consensus about the nature, restrictive or obstructive, of these defects. Indeed, substantial iron deposition in the lung has been observed on postmortem examination in some but not in other cases. In addition to abnormal lung mechanics, patients with thalassemia major regularly exhibit a reduced pulmonary diffusing capacity, which in most instances is only partially due to lower haemoglobin concentration⁸.

The diagnosis and management of patients with pulmonary diseases have improved with introduction of pulmonary function tests. Pulmonary function tests provide objective evidence of the nature and severity of lung diseases⁹

Thus present study was conducted to describe the predominant type of lung impairment in transfusion dependent thalassemic patients and comparison with age and sex matched normal healthy school going children and effect of age, duration of blood transfusion, duration of chelation therapy and ferritin level on pulmonary function, among age group 6-16yrs in our geographical area.