Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy
Objectives 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. P...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Panwar, Neha [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2022 |
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| Schlagwörter: |
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| Anmerkung: |
© Indian Academy of Pediatrics 2022 |
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| Übergeordnetes Werk: |
Enthalten in: Indian Pediatrics - Springer-Verlag, 2010, 59(2022), 6 vom: 26. Apr., Seite 451-454 |
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| Übergeordnetes Werk: |
volume:59 ; year:2022 ; number:6 ; day:26 ; month:04 ; pages:451-454 |
| Links: |
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| DOI / URN: |
10.1007/s13312-022-2533-2 |
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SPR047503491 |
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| 520 | |a Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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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10.1007/s13312-022-2533-2 doi (DE-627)SPR047503491 (SPR)s13312-022-2533-2-e DE-627 ger DE-627 rakwb eng Panwar, Neha verfasserin aut Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Academy of Pediatrics 2022 Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. Desferrioxamine (dpeaa)DE-He213 Iron (dpeaa)DE-He213 Hemosiderosis (dpeaa)DE-He213 Pulmonary function tests (dpeaa)DE-He213 Gomber, Sunil aut Dewan, Pooja aut Kumar, Raj aut Enthalten in Indian Pediatrics Springer-Verlag, 2010 59(2022), 6 vom: 26. Apr., Seite 451-454 (DE-627)SPR031274943 nnns volume:59 year:2022 number:6 day:26 month:04 pages:451-454 https://dx.doi.org/10.1007/s13312-022-2533-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_70 GBV_ILN_72 GBV_ILN_110 GBV_ILN_131 GBV_ILN_160 GBV_ILN_376 GBV_ILN_607 AR 59 2022 6 26 04 451-454 |
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10.1007/s13312-022-2533-2 doi (DE-627)SPR047503491 (SPR)s13312-022-2533-2-e DE-627 ger DE-627 rakwb eng Panwar, Neha verfasserin aut Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Academy of Pediatrics 2022 Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. Desferrioxamine (dpeaa)DE-He213 Iron (dpeaa)DE-He213 Hemosiderosis (dpeaa)DE-He213 Pulmonary function tests (dpeaa)DE-He213 Gomber, Sunil aut Dewan, Pooja aut Kumar, Raj aut Enthalten in Indian Pediatrics Springer-Verlag, 2010 59(2022), 6 vom: 26. Apr., Seite 451-454 (DE-627)SPR031274943 nnns volume:59 year:2022 number:6 day:26 month:04 pages:451-454 https://dx.doi.org/10.1007/s13312-022-2533-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_70 GBV_ILN_72 GBV_ILN_110 GBV_ILN_131 GBV_ILN_160 GBV_ILN_376 GBV_ILN_607 AR 59 2022 6 26 04 451-454 |
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10.1007/s13312-022-2533-2 doi (DE-627)SPR047503491 (SPR)s13312-022-2533-2-e DE-627 ger DE-627 rakwb eng Panwar, Neha verfasserin aut Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Academy of Pediatrics 2022 Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. Desferrioxamine (dpeaa)DE-He213 Iron (dpeaa)DE-He213 Hemosiderosis (dpeaa)DE-He213 Pulmonary function tests (dpeaa)DE-He213 Gomber, Sunil aut Dewan, Pooja aut Kumar, Raj aut Enthalten in Indian Pediatrics Springer-Verlag, 2010 59(2022), 6 vom: 26. Apr., Seite 451-454 (DE-627)SPR031274943 nnns volume:59 year:2022 number:6 day:26 month:04 pages:451-454 https://dx.doi.org/10.1007/s13312-022-2533-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_70 GBV_ILN_72 GBV_ILN_110 GBV_ILN_131 GBV_ILN_160 GBV_ILN_376 GBV_ILN_607 AR 59 2022 6 26 04 451-454 |
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10.1007/s13312-022-2533-2 doi (DE-627)SPR047503491 (SPR)s13312-022-2533-2-e DE-627 ger DE-627 rakwb eng Panwar, Neha verfasserin aut Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Academy of Pediatrics 2022 Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. Desferrioxamine (dpeaa)DE-He213 Iron (dpeaa)DE-He213 Hemosiderosis (dpeaa)DE-He213 Pulmonary function tests (dpeaa)DE-He213 Gomber, Sunil aut Dewan, Pooja aut Kumar, Raj aut Enthalten in Indian Pediatrics Springer-Verlag, 2010 59(2022), 6 vom: 26. Apr., Seite 451-454 (DE-627)SPR031274943 nnns volume:59 year:2022 number:6 day:26 month:04 pages:451-454 https://dx.doi.org/10.1007/s13312-022-2533-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_70 GBV_ILN_72 GBV_ILN_110 GBV_ILN_131 GBV_ILN_160 GBV_ILN_376 GBV_ILN_607 AR 59 2022 6 26 04 451-454 |
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10.1007/s13312-022-2533-2 doi (DE-627)SPR047503491 (SPR)s13312-022-2533-2-e DE-627 ger DE-627 rakwb eng Panwar, Neha verfasserin aut Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Indian Academy of Pediatrics 2022 Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. Desferrioxamine (dpeaa)DE-He213 Iron (dpeaa)DE-He213 Hemosiderosis (dpeaa)DE-He213 Pulmonary function tests (dpeaa)DE-He213 Gomber, Sunil aut Dewan, Pooja aut Kumar, Raj aut Enthalten in Indian Pediatrics Springer-Verlag, 2010 59(2022), 6 vom: 26. Apr., Seite 451-454 (DE-627)SPR031274943 nnns volume:59 year:2022 number:6 day:26 month:04 pages:451-454 https://dx.doi.org/10.1007/s13312-022-2533-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_70 GBV_ILN_72 GBV_ILN_110 GBV_ILN_131 GBV_ILN_160 GBV_ILN_376 GBV_ILN_607 AR 59 2022 6 26 04 451-454 |
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Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy |
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Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy |
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Panwar, Neha |
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Indian Pediatrics |
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Panwar, Neha Gomber, Sunil Dewan, Pooja Kumar, Raj |
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10.1007/s13312-022-2533-2 |
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pulmonary dysfunction in transfusion-dependent thalassemia and response to intensive chelation therapy |
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Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy |
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Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. © Indian Academy of Pediatrics 2022 |
| abstractGer |
Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. © Indian Academy of Pediatrics 2022 |
| abstract_unstemmed |
Objectives 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) & 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 (ΔFEV1/FVC) (P=0.009), ΔDLCO (P=0.006) and ΔSF (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. © Indian Academy of Pediatrics 2022 |
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Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy |
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