PMID- 15003957
OWN - NLM
STAT- MEDLINE
DCOM- 20040409
LR  - 20220310
IS  - 0002-9262 (Print)
IS  - 0002-9262 (Linking)
VI  - 159
IP  - 6
DP  - 2004 Mar 15
TI  - Association between initial disease presentation, lung disease outcomes, and 
      survival in patients with cystic fibrosis.
PG  - 537-46
AB  - This US study was conducted to determine whether mode of diagnosis and initial 
      disease presentation influence lung disease and survival in patients with cystic 
      fibrosis. The study population included 27,703 patients reported to the 1986-2000 
      Cystic Fibrosis Foundation Registry. Patients were segregated into four 
      diagnostic categories: meconium ileus (MI), prenatal/neonatal screening (SCREEN), 
      positive family history (FH), and symptoms other than meconium ileus (SYMPTOM). 
      When compared with patients in the SCREEN group, those in the MI or SYMPTOM group 
      were found to have significantly greater risks of shortened survival, Pseudomonas 
      aeruginosa acquisition, and forced expiratory volume in 1 second (FEV(1)) below 
      70% of predicted. In the SYMPTOM group, the greatest risks of shortened survival, 
      P. aeruginosa acquisition, and FEV(1) <70% occurred for patients presenting with 
      combined respiratory and gastrointestinal symptoms, followed by respiratory or 
      gastrointestinal symptoms alone; the best outcomes were in patients with other 
      presenting features. Additionally, patients with presumably "severe" genotypes 
      (DeltaF508 plus other class I, II, III mutations in both alleles) had greater 
      risks of shortened survival and P. aeruginosa acquisition compared with patients 
      with presumably "mild" genotypes (class IV or V mutations in one or both 
      alleles).
FAU - Lai, HuiChuan J
AU  - Lai HJ
AD  - Department of Nutritional Sciences, University of Wisconsin College of 
      Agriculture and Life Sciences, 1415 Linden Drive, Madison, WI 53706-1562, USA. 
      lai@nutrisci.wisc.edu
FAU - Cheng, Yu
AU  - Cheng Y
FAU - Cho, Hyungjun
AU  - Cho H
FAU - Kosorok, Michael R
AU  - Kosorok MR
FAU - Farrell, Philip M
AU  - Farrell PM
LA  - eng
GR  - DK 34108/DK/NIDDK NIH HHS/United States
GR  - K01 DK 02891/DK/NIDDK NIH HHS/United States
GR  - M01 RR 03186/RR/NCRR NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Am J Epidemiol
JT  - American journal of epidemiology
JID - 7910653
SB  - IM
MH  - Age of Onset
MH  - Child, Preschool
MH  - Cystic Fibrosis/diagnosis/genetics/*mortality
MH  - Female
MH  - Forced Expiratory Volume
MH  - Genetic Predisposition to Disease/epidemiology
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Lung Diseases/epidemiology/microbiology
MH  - Male
MH  - Neonatal Screening
MH  - Prognosis
MH  - Proportional Hazards Models
MH  - Pseudomonas Infections/epidemiology
MH  - Risk Factors
MH  - Sex Factors
MH  - Statistics, Nonparametric
MH  - Survival Rate
MH  - United States/epidemiology
EDAT- 2004/03/09 05:00
MHDA- 2004/04/10 05:00
CRDT- 2004/03/09 05:00
PHST- 2004/03/09 05:00 [pubmed]
PHST- 2004/04/10 05:00 [medline]
PHST- 2004/03/09 05:00 [entrez]
AID - 10.1093/aje/kwh083 [doi]
PST - ppublish
SO  - Am J Epidemiol. 2004 Mar 15;159(6):537-46. doi: 10.1093/aje/kwh083.