PMID: 9165451May 1, 1997Paper

Active collagen synthesis by pulmonary arteries in pulmonary hypertension complicated by congenital diaphragmatic hernia

Journal of Pediatric Surgery
T Yamataka, P Puri

Abstract

The high mortality rate for patients with congenital diaphragmatic hernia (CDH) has been attributed to pulmonary hypoplasia and persistent pulmonary hypertension (PPH). The factors that cause vasoconstriction and vascular remodeling in PPH are not fully understood. Immunohistochemistry was performed on lung tissue obtained from postmortem CDH patients with pulmonary hypoplasia and PPH (n = 21) using the following antibodies: alpha smooth muscle-actin (ASMA), transforming growth factor-beta (TGF-beta), isoform specific (TGF-beta 1, -beta 2, -beta 3), and M-57. Normal lung tissues from age-matched sudden infant death syndrome patients (SIDS, n = 8) were obtained as controls. TGF-beta 3 immunoreactivity was observed in the adventitia but not in the media of pulmonary muscular arteries in patients with CDH. TGF-beta 1, -beta 2 immunoreactivity was either absent or faintly expressed in pulmonary arteries in CDH patients. No TGF-beta staining was observed in the pulmonary vasculature of SIDS patients. Newly synthesized procollagen (M-57) was easily detected in the media and adventitia in a large number of pulmonary arteries in all patients with CDH and in the neointima in two patients with long standing PPH. No M-57 staining was seen...Continue Reading

References

Feb 1, 1992·Molecular Biology of the Cell·J E Murphy-UllrichM Höök
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Dec 1, 1989·The American Review of Respiratory Disease·C KuhnJ A McDonald

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Citations

Dec 18, 1998·Journal of Pediatric Surgery·A YamatakaT Miyano
Apr 24, 2001·Pharmacology & Therapeutics·B WeinbergerJ D Laskin
Apr 28, 2007·Seminars in Pediatric Surgery·Doug Miniati
Aug 1, 2014·Birth Defects Research. Part B, Developmental and Reproductive Toxicology·Alejandro HofmannPrem Puri
Apr 17, 2007·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Paul A KhanBruno Piedboeuf
Sep 11, 1998·Developmental Dynamics : an Official Publication of the American Association of Anatomists·M BergwerffR E Poelmann
Feb 18, 1999·Journal of Pediatric Surgery·A YamatakaT Miyano

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