PMID- 11371423
OWN - NLM
STAT- MEDLINE
DCOM- 20010628
LR  - 20121115
IS  - 1073-449X (Print)
IS  - 1073-449X (Linking)
VI  - 163
IP  - 6
DP  - 2001 May
TI  - Altered arterial expression patterns of inducible and endothelial nitric oxide 
      synthase in pulmonary plexogenic arteriopathy caused by congenital heart disease.
PG  - 1493-9
AB  - Flow-associated pulmonary hypertension leads to pulmonary plexogenic arteriopathy 
      (PPA), a specific pulmonary vascular disease that includes vascular lesions 
      characterized by abnormal vasodilatation and endothelial cell proliferation. 
      Increased local production of NO has been suggested in this condition. Because 
      reported data on the expression of endothelial NO-synthase (ecNOS) have been 
      contradictory, we speculated that the expression of the inducible isoform of NOS 
      (iNOS) is enhanced in this form of pulmonary hypertension. We investigated 
      immunohistochemically the expression of ecNOS and iNOS in lung tissue of patients 
      with flow-associated pulmonary hypertension (n = 18) and compared the findings 
      with those in patients with increased pulmonary blood flow but normal pulmonary 
      artery pressure (n = 10), with congestive vasculopathy (n = 6) and control 
      subjects (n = 4). Immunoreactivity for ecNOS and iNOS was present both in normal 
      and diseased pulmonary arteries. Marked immunoreactivity to both isoforms was 
      present within the advanced lesions of PPA, including plexiform lesions. 
      Semiquantitative analysis of immunoreactivity, both for ecNOS and iNOS, showed no 
      correlation with the severity of morphologic vascular lesions (p = 0.29 and p = 
      0.23, respectively). In contrast to ecNOS, immunoreactivity for iNOS was 
      increased in patients with flow-associated pulmonary hypertension compared with 
      other patients (p = 0.02). The present study has demonstrated enhanced expression 
      of iNOS in patients at risk for advanced PPA, but not in patients with other 
      forms of pulmonary arteriopathy. Moreover, high expression of both ecNOS and iNOS 
      were present in advanced lesions of PPA. These data suggest differentiated roles 
      for different isoforms of NOS in the pathogenesis of this specific pulmonary 
      arteriopathy.
FAU - Berger, R M
AU  - Berger RM
AD  - Department of Pediatrics, Division of Pediatric Cardiology, Sophia Children's 
      Hospital/University Hospital Rotterdam, Dr. Molewaterplein 60, 3015 GJ Rotterdam, 
      The Netherlands. berger@alkg.azr.nl
FAU - Geiger, R
AU  - Geiger R
FAU - Hess, J
AU  - Hess J
FAU - Bogers, A J
AU  - Bogers AJ
FAU - Mooi, W J
AU  - Mooi WJ
LA  - eng
PT  - Comparative Study
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  - EC 1.14.13.39 (NOS2 protein, human)
RN  - EC 1.14.13.39 (Nitric Oxide Synthase)
RN  - EC 1.14.13.39 (Nitric Oxide Synthase Type II)
SB  - IM
MH  - Analysis of Variance
MH  - Biopsy
MH  - Blood Flow Velocity
MH  - Cardiac Catheterization
MH  - Case-Control Studies
MH  - Child
MH  - Child, Preschool
MH  - Endothelium, Vascular/*chemistry
MH  - Heart Defects, Congenital/*complications
MH  - Hemodynamics
MH  - Humans
MH  - Hypertension, Pulmonary/*enzymology/*etiology/pathology/physiopathology
MH  - Immunohistochemistry
MH  - Nitric Oxide Synthase/*analysis
MH  - Nitric Oxide Synthase Type II
MH  - Pulmonary Artery/*cytology
MH  - Pulmonary Circulation
MH  - Pulmonary Wedge Pressure
MH  - Risk Factors
EDAT- 2001/05/24 10:00
MHDA- 2001/06/29 10:01
CRDT- 2001/05/24 10:00
PHST- 2001/05/24 10:00 [pubmed]
PHST- 2001/06/29 10:01 [medline]
PHST- 2001/05/24 10:00 [entrez]
AID - 10.1164/ajrccm.163.6.9908137 [doi]
PST - ppublish
SO  - Am J Respir Crit Care Med. 2001 May;163(6):1493-9. doi: 
      10.1164/ajrccm.163.6.9908137.