Document Detail


Malignant hypertension in a child with phakomatosis pigmentovascularis type II b.
MedLine Citation:
PMID:  18671691     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. CONCLUSION: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.
Authors:
Y Kanaheswari; A H Hamzaini; S W Wong; A Zulfiqar
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-07-29
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  97     ISSN:  1651-2227     ISO Abbreviation:  Acta Paediatr.     Publication Date:  2008 Nov 
Date Detail:
Created Date:  2008-10-30     Completed Date:  2008-12-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  1589-91     Citation Subset:  IM    
Affiliation:
Department of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia. kanahes@mail.hukm.ukm.my
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Female
Humans
Hypertension, Malignant / etiology*
Infant
Infant, Newborn
Nevus of Ota / complications
Renal Artery Obstruction / complications*
Sturge-Weber Syndrome / complications*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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