Abstract | OBJECTIVE: To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks). METHODS: This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO. RESULTS: 50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01). CONCLUSIONS: Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.
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Authors | Neha Panwar, Sunil Gomber, Pooja Dewan, Raj Kumar |
Journal | Indian pediatrics
(Indian Pediatr)
Vol. 59
Issue 6
Pg. 451-454
(06 15 2022)
ISSN: 0974-7559 [Electronic] India |
PMID | 35481485
(Publication Type: Journal Article)
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Chemical References |
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Topics |
- Chelation Therapy
- Child
- Ferritins
- Humans
- Respiratory Function Tests
- Thalassemia
(complications, therapy)
- beta-Thalassemia
(complications, therapy)
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