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Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis

¹ Neonatology and Paediatric Intensive Care Medicine, Dept of General Paediatrics, and ² Dept of Pathology, Heinrich-Heine-University, Düsseldorf, Germany, ³ Dept of Pathology, Glasgow Royal Infirmary, and ⁴ Dept of Physiology and Pharmacology, University of Strathclyde, Glasgow, UK.

CORRESPONDENCE: T. Hoehn, Neonatology and Paediatric Intensive Care Medicine, Dept of General Paediatrics, Heinrich-Heine-University, Moorenstr. 5, D-40225 Düsseldorf, Germany. Fax: 49 2118119786. E-mail: thomas.hoehn{at}uni-duesseldorf.de

Keywords: Endothelial cells, endothelial nitric oxide synthase, inducible nitric oxide synthase, inhaled nitric oxide, newborn, nitrotyrosine

Received: July 15, 2005
Accepted November 5, 2005

Abnormal growth and development of lymphatic pulmonary structures leads to severe hypoxia in congenital pulmonary lymphangiectasis (CPL).

This case study aims to determine the cellular source and topographical distribution of the nitric oxide synthases in CPL. It studies the post mortem tissue of a term newborn with the clinical course and histological findings of CPL and three controls without pulmonary pathology.

It was found that endothelial cells of pulmonary arteries and lymphatic structures stained significantly more for endothelial nitric oxide synthase protein in the CPL patient compared to the controls.

The authors conclude that synthesis of endothelial nitric oxide synthase is upregulated in vascular and lymphatic endothelial cells in congenital pulmonary lymphangiectasis.