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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Abnormal pericyte recruitment as a cause for pulmonary hypertension in Adams-Oliver syndrome
American Journal of Medical Genetics, Volume 129 A, No. 3, Year 2004
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Description
Adams-Oliver syndrome (AOS) consists of congenital scalp defects with variable limb defects of unknown pathogenesis. We report on two children with AOS plus additional features including intrauterine growth retardation (IUGR), cutis marmorata telangiectatica congenita (CMTC), pulmonary hypertension (PH), intracranial densities shown in one case to be sites of active bleeding and osteopenia. Autopsy in one case revealed defective vascular smooth muscle cell/pericyte coverage of the vasculature associated with two blood vessel abnormalities. Pericyte absence correlated with vessel dilatation while hyperproliferation of pericytes correlated with vessel stenosis. These findings suggest a unifying pathogenic mechanism for the abnormalities seen in AOS. These and previously reported cases establish that a subset of AOS patients is at high risk for PH. © 2004 Wiley-Liss, Inc.
Authors & Co-Authors
Patel, Millan S.
Canada, Toronto
Hospital for Sick Children University of Toronto
United States, Houston
Baylor College of Medicine
Taylor, Glenn P.
Canada, Vancouver
The University of British Columbia
Bharya, Simi
Canada, Vancouver
The University of British Columbia
Al-Sannaa, Nouriya Abbas
Canada, Toronto
Hospital for Sick Children University of Toronto
Saudi Arabia, Dhahran
Johns Hopkins Aramco Healthcare
Adatia, Ian
Canada, Toronto
Hospital for Sick Children University of Toronto
Chitayat, David A.
Canada, Toronto
Hospital for Sick Children University of Toronto
Lewis, Suzanne
Canada, Vancouver
The University of British Columbia
Human, D. G.
Canada, Vancouver
The University of British Columbia
Statistics
Citations: 78
Authors: 8
Affiliations: 4
Identifiers
Doi:
10.1002/ajmg.a.30221
ISSN:
15524825
Research Areas
Health System And Policy
Maternal And Child Health
Noncommunicable Diseases