Pulmonary function in thalassaemia major and its correlation with body iron stores

Br J Haematol. 2011 Oct;155(1):102-5. doi: 10.1111/j.1365-2141.2011.08808.x. Epub 2011 Aug 2.

Abstract

This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for airways obstruction, there were indicators of small airways disease and air trapping. PFTs did not correlate with somatic iron burden, blood counts or haemolysis. Restrictive lung disease was associated with inflammation. We conclude that TM patients have pulmonary abnormalities consistent with small airways obstruction. Restrictive disease and impaired diffusion are less common.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Female
  • Humans
  • Iron / metabolism*
  • Iron Overload / etiology
  • Iron Overload / metabolism
  • Iron Overload / physiopathology
  • Liver / metabolism
  • Lung / physiopathology
  • Lung Diseases / etiology*
  • Lung Diseases / metabolism
  • Lung Diseases / physiopathology
  • Male
  • Middle Aged
  • Myocardium / metabolism
  • Pancreas / metabolism
  • Pulmonary Disease, Chronic Obstructive / etiology
  • Pulmonary Disease, Chronic Obstructive / metabolism
  • Pulmonary Disease, Chronic Obstructive / physiopathology
  • Respiratory Function Tests / methods
  • Respiratory Mechanics / physiology
  • Young Adult
  • beta-Thalassemia / complications*
  • beta-Thalassemia / metabolism
  • beta-Thalassemia / physiopathology

Substances

  • Iron