NDLI: Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: An imbalance in biosynthesis of thromboxane A2 and prostacyclin.

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Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: An imbalance in biosynthesis of thromboxane A2 and prostacyclin.

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Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: An imbalance in biosynthesis of thromboxane A2 and prostacyclin.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Fukushima, Hiroyuki Kosaki, Kenjiro Sato, Reiko Yagihashi, Tatsuhiko Gatayama, Ryohei Kodo, Kazuki Hayashi, Takuya Nakazawa, Maki Tsuchihashi, Takatoshi Maeda, Jun Kojima, Yoshifumi Yamagishi, Hiroyuki Takahashi, Takao
Description	Country affiliation: Japan Author Affiliation: Fukushima H ( Department of Pediatrics, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan. fuku-h@sc.itc.keio.ac.jp)
Abstract	Patients with Down syndrome (DS) and a left-to-right shunt often develop early severe pulmonary hypertension (PH) and pulmonary vascular obstructive disease (PVOD); the pathophysiological mechanisms underlying the development of these complications are yet to be determined. To investigate the mechanisms, we evaluated the biosynthesis of thromboxane (TX) A(2) and prostacyclin (PGI(2)) in four groups of infants, cross-classified as shown below, by measuring the urinary excretion levels of 11-dehydro-TXB(2) and 2,3-dinor-6-keto-PGF(1alpha): DS infants with a left-to-right shunt and PH (D-PH, n = 18), DS infants without congenital heart defect (D-C, n = 8), non-DS infants with a left-to-right shunt and PH (ND-PH, n = 12), and non-DS infants without congenital heart defect (ND-C, n = 22). The urinary excretion ratios of 11-dehydro-TXB(2) to 2,3-dinor-6-keto-PGF(1alpha) in the D-PH, D-C, ND-PH, and ND-C groups were 7.69, 4.71, 2.10, and 2.27, respectively. The ratio of 11-dehydro-TXB(2) to 2,3-dinor-6-keto-PGF(1alpha) was higher in the presence of DS (P < 0.001), independently of the presence of PH (P = 0.297). The predominant biosynthesis of TXA(2) over PGI(2), leading to vasoconstriction, was observed in DS infants, irrespective of the presence/absence of PH. This imbalance in the biosynthesis of vasoactive eicosanoids may account for the rapid progression of PVOD in DS infants with a left-to-right shunt.
File Format	HTM / HTML
ISSN	15524825
Issue Number	8
Volume Number	152A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2010-08-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics Down Syndrome Metabolism Epoprostenol Biosynthesis Heart Defects, Congenital Hypertension, Pulmonary Lung Diseases, Obstructive Pulmonary Heart Disease Thromboxane A2 6-ketoprostaglandin F1 Alpha Analogs & Derivatives Urine Child, Preschool Cross-sectional Studies Complications Female Etiology Humans Infant Male Prognosis Radioimmunoassay Thromboxane B2 Journal Article
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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