{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:33:41Z","timestamp":1790037221363,"version":"4.0.1"},"reference-count":38,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,1,25]],"date-time":"2018-01-25T00:00:00Z","timestamp":1516838400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["NIH 1R01HL079937"],"award-info":[{"award-number":["NIH 1R01HL079937"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["UL1 RR024989 (CWRU CRU)"],"award-info":[{"award-number":["UL1 RR024989 (CWRU CRU)"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV<jats:sub>1<\/jats:sub>% predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi\u2010center cohort of children with SCA, we tested the hypotheses that: (1) FEV<jats:sub>1<\/jats:sub>% predicted declines over time; and (2) SCA\u2010specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbS\u03b2<jats:sup>0<\/jats:sup> thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study\u2010certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV<jats:sub>1<\/jats:sub>, FVC, and FEV<jats:sub>1<\/jats:sub>\/FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4\u201019.3 years), had a minimum of three spirometry measurements, over an average of 4.4 years (range 1.1\u20106.5 years) from baseline to endpoint. In a multivariable model, FEV<jats:sub>1<\/jats:sub>% predicted declines by 0.3% for every additional year of age (95% CI \u22120.56 to \u22120.05, <jats:italic>P<\/jats:italic>\u2009=\u2009.020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV<jats:sub>1<\/jats:sub>% predicted. In a large, rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV<jats:sub>1<\/jats:sub>% predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline.<\/jats:p>","DOI":"10.1002\/ajh.25003","type":"journal-article","created":{"date-parts":[[2017,12,11]],"date-time":"2017-12-11T08:06:52Z","timestamp":1512979612000},"page":"408-415","source":"Crossref","is-referenced-by-count":25,"title":["Age is a predictor of a small decrease in lung function in children with sickle cell anemia"],"prefix":"10.1002","volume":"93","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6691-816X","authenticated-orcid":false,"given":"Shaina M.","family":"Willen","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology Vanderbilt\u2010Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center Nashville Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn","family":"Cohen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonary and Allergy Boston University School of Medicine Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Rodeghier","sequence":"additional","affiliation":[{"name":"Rodeghier Consultants Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fenella","family":"Kirkham","sequence":"additional","affiliation":[{"name":"Developmental Neurosciences Unit UCL Great Ormond Street, Institute of Child Health London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan S.","family":"Redline","sequence":"additional","affiliation":[{"name":"Department of Medicine and Neurology, Division of Sleep and Circadian Disorders, Brigham and Women's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol","family":"Rosen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonary, Allergy\/Immunology and Sleep University Hospitals\u2010Cleveland Medical Center, Rainbow Babies and Children's Hospitals Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Kirkby","sequence":"additional","affiliation":[{"name":"Respiratory, Critical Care and Anaesthesia UCL Great Ormond Street, Institute of Child Health, University College London London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0574-1604","authenticated-orcid":false,"given":"Michael R.","family":"DeBaun","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology Vanderbilt\u2010Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center Nashville Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,1,25]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000189089.45003.52"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201510-706OC"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000011"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20883"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200601-125OC"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-009-9141-y"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.313.7059.711"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm\/140.2.379"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00008504"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199204303261804"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00166412"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-05-644435"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2013.10.003"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200708-1219OC"},{"key":"e_1_2_6_16_1","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1002\/ppul.23367","article-title":". Longitudinal assessment of lung function in children with sickle cell disease","volume":"723","author":"Lunt A","year":"2016","journal-title":". Pediatr Pulmonol."},{"issue":"2","key":"e_1_2_6_17_1","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1378\/chest.06-1999","article-title":"Sources of long\u2010term variability in measurements of lung function: implications for interpretation and clinical trial design","volume":"132","author":"Jensen RL","year":"2007","journal-title":"Chest."},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1243"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00034805"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20950"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00035005"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00161011"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.48.8.803"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22538"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00035205"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.08-1235"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-0804"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23819"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.11.034"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/0197-2456(87)90016-X"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24598"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/sim.3504"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20601"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.resp.2012.08.017"},{"issue":"6","key":"e_1_2_6_36_1","first-page":"859","article-title":"Within\u2010subject variability and per cent change for significance of spirometry in normal subjects and in patients with cystic fibrosis","volume":"122","author":"Nickerson BG","year":"1980","journal-title":"Am Rev Respir Dis."},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/ije\/dyh299"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201606-1119LE"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201703-254ED"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.25003","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.25003","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T16:53:05Z","timestamp":1695574385000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.25003"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,1,25]]},"references-count":38,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,3]]}},"alternative-id":["10.1002\/ajh.25003"],"URL":"https:\/\/doi.org\/10.1002\/ajh.25003","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,1,25]]}}}