PMID- 10228112
OWN - NLM
STAT- MEDLINE
DCOM- 19990614
LR  - 20111117
IS  - 1073-449X (Print)
IS  - 1073-449X (Linking)
VI  - 159
IP  - 5 Pt 1
DP  - 1999 May
TI  - HLA class II polymorphism in cystic fibrosis. A possible modifier of pulmonary 
      phenotype.
PG  - 1464-8
AB  - Evolution of lung damage is highly variable in cystic fibrosis (CF) even in 
      patients with the same cystic fibrosis transmembrane conductance regulator (CFTR) 
      mutations. The analysis of genetic factors other than CFTR may help our 
      understanding of genotype-phenotype relationships in CF. As human leukocyte 
      antigen (HLA) class II polymorphism has been associated with a number of diseases 
      including autoimmune and inflammatory diseases, asthma, and allergy, we 
      investigated the possibility that HLA polymorphism contributes to CF-associated 
      pulmonary inflammation. Among the 98 adult CF patients tested, the genotypic 
      frequencies of DR4 and DR7 alleles (serologic group DR53) and DR7/ DQA*0201 
      haplotype were higher than in 39 selected control subjects without atopy (p </= 
      10(-)6, relative risk [RR] = 22, and p </= 5.10(-)4, RR = 27, respectively) and 
      in a random population. No significant difference of these allelic distributions 
      was found according to the CFTR genotype. In the CF patients, the DR7 allele was 
      significantly associated with an increase in total IgE and with chronic 
      Pseudomonas aeruginosa colonization (100% of DR7 versus 83% of non-DR7 patients 
      being colonized, p < 0.05). Our results suggest that genetic factors known to 
      modulate the immune response might contribute to chronic infection with 
      Pseudomonas, increased total IgE, and pulmonary outcome in CF.
FAU - Aron, Y
AU  - Aron Y
AD  - Laboratoire de Physiologie Respiratoire, Service de Pneumologie, Laboratoire de 
      Biochimie et Genetique Moleculaire, Universitaire Cochin Port-Royal, Cedex, 
      France.
FAU - Polla, B S
AU  - Polla BS
FAU - Bienvenu, T
AU  - Bienvenu T
FAU - Dall'ava, J
AU  - Dall'ava J
FAU - Dusser, D
AU  - Dusser D
FAU - Hubert, D
AU  - Hubert D
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Respir Crit Care Med
JT  - American journal of respiratory and critical care medicine
JID - 9421642
RN  - 0 (CFTR protein, human)
RN  - 0 (HLA-DQ Antigens)
RN  - 0 (HLA-DQ alpha-Chains)
RN  - 0 (HLA-DQ beta-Chains)
RN  - 0 (HLA-DQA1 antigen)
RN  - 0 (HLA-DQB1 antigen)
RN  - 0 (HLA-DR Antigens)
RN  - 0 (HLA-DRB1 Chains)
RN  - 0 (Histocompatibility Antigens Class II)
RN  - 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
RN  - 37341-29-0 (Immunoglobulin E)
SB  - IM
MH  - Adult
MH  - Alleles
MH  - Colony Count, Microbial
MH  - Cystic Fibrosis/*genetics/*immunology/microbiology
MH  - Cystic Fibrosis Transmembrane Conductance Regulator/genetics
MH  - Female
MH  - Gene Frequency
MH  - HLA-DQ Antigens/genetics
MH  - HLA-DQ alpha-Chains
MH  - HLA-DQ beta-Chains
MH  - HLA-DR Antigens/genetics
MH  - HLA-DRB1 Chains
MH  - Histocompatibility Antigens Class II/*genetics
MH  - Humans
MH  - Immunoglobulin E/analysis
MH  - Lung/physiopathology
MH  - Male
MH  - Mutation/genetics
MH  - Phenotype
MH  - Polymorphism, Genetic/*genetics
MH  - Pseudomonas aeruginosa/isolation & purification
EDAT- 1999/05/06 00:00
MHDA- 1999/05/06 00:01
CRDT- 1999/05/06 00:00
PHST- 1999/05/06 00:00 [pubmed]
PHST- 1999/05/06 00:01 [medline]
PHST- 1999/05/06 00:00 [entrez]
AID - 10.1164/ajrccm.159.5.9807046 [doi]
PST - ppublish
SO  - Am J Respir Crit Care Med. 1999 May;159(5 Pt 1):1464-8. doi: 
      10.1164/ajrccm.159.5.9807046.