{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T23:11:10Z","timestamp":1774566670216,"version":"3.50.1"},"reference-count":11,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2005,12,12]],"date-time":"2005-12-12T00:00:00Z","timestamp":1134345600000},"content-version":"vor","delay-in-days":6555,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[1988,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Meconium ileus (MEC), failure to thrive (FTT), and a combination of FTT and pulmonary symptoms (COMB) are the most frequent symptoms of cystic fibrosis (CF) at the time of diagnosis. The purpose of this study was to compare to normal controls (NC) the pulmonary function of CF infants at the time of diagnosis, when grouped by these symptoms. The measurements of pulmonary function included oxygen saturation (Sa<jats:sub>O<\/jats:sub><jats:sub>2<\/jats:sub>), functional residual capacity (FRC), mixing index (MI), total respiratory system compliance (Crs), and maximal flow at FRC (VmaxFRC).<\/jats:p><jats:p>Compared to NC (n = 33), the MEC group (n = 5) had a higher MI (54 vs. 42%) and no difference in Sa<jats:sub>O<\/jats:sub><jats:sub>2<\/jats:sub>, Crs or VmaxFRC. There were no significant differences between FTT (n = 8) and NC groups although there was a tendency for Crs to be lower in the FTT group (5.1 vs. 6.8 ml\/cm H<jats:sub>2<\/jats:sub>O). When compared to all other groups, the COMB group (n=11) had significantly lower Sa<jats:sub>O<\/jats:sub><jats:sub>2<\/jats:sub>, MI, Crs, and VmaxFRC.<\/jats:p><jats:p>The normal lung function in the MEC group is consistent with the normal anatomy reported in CF infants dying secondary to meconium ileus. Longitudinal evaluation of the infants in this study, following initiation of care as patients with a diagnosis of CF, may allow us to determine whether symptoms at diagnosis remain an important determinant of lung function in infancy.<\/jats:p>","DOI":"10.1002\/ppul.1950050105","type":"journal-article","created":{"date-parts":[[2007,3,6]],"date-time":"2007-03-06T13:08:39Z","timestamp":1173186519000},"page":"15-18","source":"Crossref","is-referenced-by-count":45,"title":["Infants with cystic fibrosis: Pulmonary function at diagnosis"],"prefix":"10.1002","volume":"5","author":[{"given":"Robert S.","family":"Tepper","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Hiatt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard","family":"Eigen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Scott","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jay","family":"Grosfeld","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mervin","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,12,12]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"335","article-title":"Report of 130 patients diagnosed under 3 months of age over a 20 year period","volume":"46","author":"Schwachman H","year":"1970","journal-title":"Pediatrics."},{"key":"e_1_2_1_3_2","first-page":"270","article-title":"The incidence and prescntation of cystic fibrosis in Victoria 1955\u20101978.","volume":"16","author":"Allan JL","year":"1980","journal-title":"Aust Paediatr J."},{"key":"e_1_2_1_4_2","first-page":"85","volume-title":"Cystic Fibrosis","author":"Rosenstein B","year":"1984"},{"key":"e_1_2_1_5_2","first-page":"53","article-title":"The pathogenesis of fibrocystic disease of the pancreas. A study of 36 cases with special reference to the pulmonary lesions","volume":"4","author":"Zuelzer WW","year":"1949","journal-title":"Pediatrics."},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442171"},{"key":"e_1_2_1_7_2","first-page":"195","article-title":"The bronchial mucous glands\u2014Their hypertrophy and changes in intracellular mucus","volume":"86","author":"Reid L","year":"1967","journal-title":"Bibl Pediatr."},{"key":"e_1_2_1_8_2","first-page":"303","article-title":"Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants","volume":"106","author":"Sturgess J","year":"1982","journal-title":"Am J Pathol."},{"key":"e_1_2_1_9_2","first-page":"513","article-title":"Physiologic growth and development of the lungs during the first year of life.","volume":"134","author":"Tepper RS","year":"1986","journal-title":"Am Rev Respir Dis."},{"key":"e_1_2_1_10_2","first-page":"461","article-title":"Non\u2010invasive determiantion of total respiratory system compliance in infants by the weighted\u2010spirometer method.","volume":"130","author":"Tepper RS","year":"1984","journal-title":"Am Rev Respir Dis."},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.44.235.393"},{"key":"e_1_2_1_12_2","first-page":"1075","article-title":"Total respiratory system compliance in asymptomatic infants with cystic fibrosis.","volume":"135","author":"Tepper RS","year":"1987","journal-title":"Am Rev Respir Dis."}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.1950050105","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.1950050105","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T00:01:30Z","timestamp":1695772890000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.1950050105"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1988,1]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1988,1]]}},"alternative-id":["10.1002\/ppul.1950050105"],"URL":"https:\/\/doi.org\/10.1002\/ppul.1950050105","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[1988,1]]}}}