TY - JOUR
T1 - Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia
AU - Arteta, Manuel
AU - Campbell, Andrew
AU - Nouraie, Mehdi
AU - Rana, Sohail
AU - Onyekwere, Onyinye C.
AU - Ensing, Gregory
AU - Sable, Craig
AU - Dham, Niti
AU - Darbari, Deepika
AU - Luchtman-Jones, Lori
AU - Kato, Gregory J.
AU - Gladwin, Mark T.
AU - Castro, Oswaldo L.
AU - Minniti, Caterina P.
AU - Gordeuk, Victor R.
PY - 2014/4
Y1 - 2014/4
N2 - Obstructive and restrictive pulmonary changes develop in children with sickle cell disease, but reports conflict as to the type of change that predominates. We prospectively performed spirometry, plethysmography, and lung diffusing capacity in 146 children aged 7 to 20 years with hemoglobin SS or Sβ-thalassemia. Nineteen percent of the patients had obstructive physiology as defined according to guidelines of the American Thoracic Society. In addition, 9% had restrictive physiology and 11% had abnormal but not categorized physiology. Increasing age, patient-reported or family-reported history of asthma or wheezing, and higher lactate dehydrogenase concentration were independent predictors of obstruction as reflected in lower forced expiratory volume in the first second/forced vital capacity. In conclusion, abnormal pulmonary function, most often obstructive, is common in children with hemoglobin SS and Sβ-thalassemia. Full pulmonary function testing should be performed in children with hemoglobin SS or Sβ-thalassemia, especially with history of asthma or wheezing and accentuated elevations in hemolytic markers.
AB - Obstructive and restrictive pulmonary changes develop in children with sickle cell disease, but reports conflict as to the type of change that predominates. We prospectively performed spirometry, plethysmography, and lung diffusing capacity in 146 children aged 7 to 20 years with hemoglobin SS or Sβ-thalassemia. Nineteen percent of the patients had obstructive physiology as defined according to guidelines of the American Thoracic Society. In addition, 9% had restrictive physiology and 11% had abnormal but not categorized physiology. Increasing age, patient-reported or family-reported history of asthma or wheezing, and higher lactate dehydrogenase concentration were independent predictors of obstruction as reflected in lower forced expiratory volume in the first second/forced vital capacity. In conclusion, abnormal pulmonary function, most often obstructive, is common in children with hemoglobin SS and Sβ-thalassemia. Full pulmonary function testing should be performed in children with hemoglobin SS or Sβ-thalassemia, especially with history of asthma or wheezing and accentuated elevations in hemolytic markers.
KW - Sickle cell anemia
KW - lactate dehydrogenase
KW - obstructive pulmonary function
KW - pulmonary function tests
KW - reticulocyte count
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U2 - 10.1097/MPH.0000000000000011
DO - 10.1097/MPH.0000000000000011
M3 - Article
C2 - 24309610
AN - SCOPUS:84897541108
SN - 1077-4114
VL - 36
SP - 185
EP - 189
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 3
ER -