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Physical performance in patients with thalassemia before and after transfusion
Author(s) -
Villa Maria Pia,
Rotili Pier Luigi,
Santamaria Francesca,
Vania Andrea,
Bonci Enea,
Tancredi Giancarlo,
Ronchetti Roberto
Publication year - 1996
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/(sici)1099-0496(199606)21:6<367::aid-ppul4>3.0.co;2-i
Subject(s) - medicine , cardiorespiratory fitness , oxygen pulse , airway obstruction , vo2 max , vital capacity , pulmonary function testing , anesthesia , respiratory minute volume , cardiology , airway , ventilation (architecture) , respiratory system , heart rate , lung , diffusing capacity , lung function , mechanical engineering , blood pressure , engineering
Patients with thalassemia who are on chronic transfusion programs have chronic ventilatory and cardiocirculatory abnormalities. We studied flow‐volume curves, blood gas exchange, and cardiorespiratory responses to exercise in 12 patients with thalassemia major (TM) before and 24 hours after transfusions. Cardiorespiratory fitness was assessed with an exercise tolerance test on a cycle‐ergometer. Ten healthy controls underwent the same protocol twice, first at baseline and then 24 hours later, without having had transfusions. We identified two subgroups of patients with a questionnaire: 1) those with no history of airway disease; and 2) those with a history of airway obstruction. Patients with no history of airway disease had normal baseline expiratory flows and no posttransfusion changes; those with a history of airway obstruction had lower pretransfusion expiratory flows rates and significantly decreased posttransfusion forced expiratory volume in 1 second (FEV 1 ) and forced expiratory flow at 25–75% of forced vital capacity (FEV 25–75% ). As a group, TM patients had significantly lower pretransfusion cardiorespiratory function than controls; TM patients' maximum workload was 33% lower, maximum ventilation was 38% lower, maximum oxygen uptake was 25.7% lower, oxygen pulse was 28.6% lower, dyspnea index was 10.6% lower, and ventilatory equivalent for oxygen was 27.1% lower than in control subjects. Although cardiorespiratory responses to exercise improved in both subgroups after transfusion, patients with a history of airways obstruction had a significant posttransfusion increase in their dyspnea index ( P = 0.05) and further increased their already abnormally high values of P ET CO 2 (43 mmHg). These results suggest that the transfusion worsened relative hypoventilation at the maximum workload only in the subgroup with a history of airway obstruction. Pediatr Pulmonol. 1996; 21:367–372. © 1996 Wiley‐Liss, Inc.