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Article: Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus

TitleClinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus
Authors
Issue Date1989
PublisherMosby, Inc. The Journal's web site is located at http://www.elsevier.com/locate/jpeds
Citation
Journal Of Pediatrics, 1989, v. 114 n. 5, p. 767-773 How to Cite?
AbstractWe set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 ± 610 gm) than patients with no meconium ileus (3169 ± 534 gm; p < 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p < 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 ± 4.67 vs 7.18 ± 5.19 years), but there was no difference in age of acquisition ofP. cepacia. In families in which the first child had meconium ileus, 29% of subsequent sibling with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ilus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.
Persistent Identifierhttp://hdl.handle.net/10722/44230
ISSN
2015 Impact Factor: 3.89
2015 SCImago Journal Rankings: 1.849
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorKerem, Een_HK
dc.contributor.authorCorey, Men_HK
dc.contributor.authorKerem, Ben_HK
dc.contributor.authorDurie, Pen_HK
dc.contributor.authorTsui, LCen_HK
dc.contributor.authorLevison, Hen_HK
dc.date.accessioned2007-09-12T03:49:27Z-
dc.date.available2007-09-12T03:49:27Z-
dc.date.issued1989en_HK
dc.identifier.citationJournal Of Pediatrics, 1989, v. 114 n. 5, p. 767-773en_HK
dc.identifier.issn0022-3476en_HK
dc.identifier.urihttp://hdl.handle.net/10722/44230-
dc.description.abstractWe set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 ± 610 gm) than patients with no meconium ileus (3169 ± 534 gm; p < 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p < 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 ± 4.67 vs 7.18 ± 5.19 years), but there was no difference in age of acquisition ofP. cepacia. In families in which the first child had meconium ileus, 29% of subsequent sibling with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ilus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.en_HK
dc.languageengen_HK
dc.publisherMosby, Inc. The Journal's web site is located at http://www.elsevier.com/locate/jpedsen_HK
dc.relation.ispartofJournal of Pediatricsen_HK
dc.subject.meshCystic Fibrosis - complications - geneticsen_HK
dc.subject.meshIntestinal Obstruction - complications - congenitalen_HK
dc.subject.meshMeconiumen_HK
dc.subject.meshAnthropometryen_HK
dc.subject.meshInfant, Newbornen_HK
dc.titleClinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileusen_HK
dc.typeArticleen_HK
dc.identifier.emailTsui, LC: tsuilc@hkucc.hku.hken_HK
dc.identifier.authorityTsui, LC=rp00058en_HK
dc.description.naturelink_to_subscribed_fulltexten_HK
dc.identifier.doi10.1016/S0022-3476(89)80134-9-
dc.identifier.pmid2715890en_HK
dc.identifier.scopuseid_2-s2.0-0024599433en_HK
dc.identifier.volume114en_HK
dc.identifier.issue5en_HK
dc.identifier.spage767en_HK
dc.identifier.epage773en_HK
dc.identifier.isiWOS:A1989U474700007-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridKerem, E=55396399400en_HK
dc.identifier.scopusauthoridCorey, M=7005819978en_HK
dc.identifier.scopusauthoridKerem, B=35376353800en_HK
dc.identifier.scopusauthoridDurie, P=7005360997en_HK
dc.identifier.scopusauthoridTsui, LC=7102754167en_HK
dc.identifier.scopusauthoridLevison, H=7103193312en_HK

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