Literature DB >> 26469095

PHACE syndrome--clinical features, aetiology and management.

Pieta R Winter1, Tinte Itinteang1, Phillip Leadbitter1,2,3, Swee T Tan1,2.   

Abstract

UNLABELLED: PHACE syndrome comprises a spectrum of anomalies including posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye anomalies. PHACE should be considered in any patient with a large facial segmental infantile haemangioma (IH), and multidisciplinary management is crucial. Low-dose propranolol is effectively for the treatment of IH associated with PHACE syndrome. Recent evidence suggests IH is comprised of mesoderm-derived haemogenic endothelium.
CONCLUSION: The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome. ©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

Entities:  

Keywords:  Aetiology; Infantile haemangioma; PHACE syndrome; Renin-angiotensin system; β-blockers

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Year:  2015        PMID: 26469095     DOI: 10.1111/apa.13242

Source DB:  PubMed          Journal:  Acta Paediatr        ISSN: 0803-5253            Impact factor:   2.299


  2 in total

1.  PHACES Syndrome with Intestinal Hemangioma Causing Recurrent Intussusceptions: A Case Report and Literature Review of Associated Intestinal Hemangioma.

Authors:  Buthaina Al-Musalhi; Zainab Al-Balushi
Journal:  Oman Med J       Date:  2020-11-30

2.  Infantile Hemangioma and Cardiac Defects: a Puzzling Association. A Single-center Experience.

Authors:  Andrea Bassi; Andrea Azzarelli; Angelina Vaccaro; Carlo Mazzatenta
Journal:  Dermatol Pract Concept       Date:  2022-07-01
  2 in total

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