PMID- 12883307
OWN - NLM
STAT- MEDLINE
DCOM- 20040203
LR  - 20190817
IS  - 0277-2116 (Print)
IS  - 0277-2116 (Linking)
VI  - 37
IP  - 2
DP  - 2003 Aug
TI  - Plasma markers of platelet activation in cystic fibrosis liver and lung disease.
PG  - 187-91
AB  - BACKGROUND: Transforming growth factor beta 1 (TGFbeta1) is a major fibrogenic 
      cytokine, the expression of which is increased in the livers of children with 
      cystic fibrosis liver disease (CFLD) and in the bronchoalveolar lavage fluid of 
      patients with cystic fibrosis pulmonary disease (CFPD). The purpose of our study 
      was to investigate the usefulness of plasma TGFbeta1 as a noninvasive marker of 
      CFLD and CFPD, or both and to investigate the contribution of platelet-derived 
      TGFbeta1 to plasma TGFbeta1 by correlating the latter with platelet factor 4 
      (PF4). METHODS: Three groups of patients with cystic fibrosis were studied: 1) 
      those with CFLD, 2) those with CF and no liver disease (CFNLD), and 3) those with 
      CFPD. Controls were healthy adolescents and adults. Plasma TGFbeta1 was assayed 
      using the R and D Quantikine quantitative sandwich enzyme immunoassay technique 
      and PF4 (American Bioproducts ELISA kit). RESULTS: Plasma TGFbeta1 was markedly 
      increased in CFPD (15 +/- 3 ng/mL) versus healthy adults (1 +/- 0 ng/mL; P < 
      0.004. The PF4 values followed a similar pattern: 105 +/- 8 ng/mL in CFPD versus 
      12 +/- 4 ng/mL (P < 0.0001). Plasma TGFbeta1 values in CFLD did not differ from 
      CFNLD patients of comparable age nor from values for healthy adolescents. Plasma 
      TGFbeta1 values were strongly correlated with values for PF4: r = 0.543, P < 
      0.0001. CONCLUSIONS: Plasma TGFbeta1 is not a useful marker of CFLD. The 
      increased plasma TGFbeta1 and PF4 in CFPD patients are of interest both as 
      possible noninvasive markers of pulmonary fibrosis and because the increased 
      values suggest that platelet activation may play a pathogenetic role in CFPD.
FAU - Schwarz, Kathleen B
AU  - Schwarz KB
AD  - Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, 
      Maryland, USA. kschwarz@jhmi.edu
FAU - Rosensweig, Jeffrey
AU  - Rosensweig J
FAU - Sharma, Savitri
AU  - Sharma S
FAU - Jones, Lawrence
AU  - Jones L
FAU - Durant, Michael
AU  - Durant M
FAU - Potter, Carol
AU  - Potter C
FAU - Narkewicz, Michael R
AU  - Narkewicz MR
LA  - eng
GR  - 5M01 RR 00052/RR/NCRR NIH HHS/United States
GR  - M01 RR00069/RR/NCRR NIH HHS/United States
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - J Pediatr Gastroenterol Nutr
JT  - Journal of pediatric gastroenterology and nutrition
JID - 8211545
RN  - 0 (Biomarkers)
RN  - 0 (TGFB1 protein, human)
RN  - 0 (Transforming Growth Factor beta)
RN  - 0 (Transforming Growth Factor beta1)
RN  - 37270-94-3 (Platelet Factor 4)
SB  - IM
EIN - J Pediatr Gastroenterol Nutr. 2003 Sep;37(3):402
MH  - Adult
MH  - Biomarkers/blood
MH  - Blood Platelets/metabolism
MH  - Bronchoalveolar Lavage Fluid/chemistry
MH  - Case-Control Studies
MH  - Cystic Fibrosis/*blood/complications
MH  - Female
MH  - Humans
MH  - Liver Diseases/*blood/etiology
MH  - Lung Diseases/*blood/etiology
MH  - Male
MH  - *Platelet Activation
MH  - Platelet Factor 4/analysis/metabolism
MH  - Transforming Growth Factor beta/*blood
MH  - Transforming Growth Factor beta1
EDAT- 2003/07/29 05:00
MHDA- 2004/02/05 05:00
CRDT- 2003/07/29 05:00
PHST- 2003/07/29 05:00 [pubmed]
PHST- 2004/02/05 05:00 [medline]
PHST- 2003/07/29 05:00 [entrez]
AID - 10.1097/00005176-200308000-00019 [doi]
PST - ppublish
SO  - J Pediatr Gastroenterol Nutr. 2003 Aug;37(2):187-91. doi: 
      10.1097/00005176-200308000-00019.