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Endocrine manifestations of PHACE syndrome

Authors
  • Bongsebandhu-phubhakdi, Chansuda
  • Tempark, Therdpong
  • Supornsilpchai, Vichit
Type
Published Article
Journal
Journal of Pediatric Endocrinology and Metabolism
Publisher
Walter de Gruyter GmbH
Publication Date
Jul 16, 2019
Volume
32
Issue
8
Pages
797–802
Identifiers
DOI: 10.1515/jpem-2019-0126
Source
De Gruyter
Keywords
License
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Abstract

PHACE syndrome is an uncommon disorder of posterior fossa anomalies, cervicofacial infantile hemangiomas, arterial anomalies, cardiac defects, eye anomalies, and midline/ventral defects. Endocrine abnormalities including hypopituitarism and ectopic thyroid were rarely described. In this article we review occurrence, onset, presenting symptoms, hormonal treatments and outcomes of all endocrine abnormalities in PHACE syndrome. Eleven of 20 (55%) had hypothalamic-pituitary dysfunction and 10 of 20 (50%) had thyroid dysgenesis. A thorough understanding of the endocrine manifestations is important for clinicians to early identify endocrine involvement in PHACE and develop plans for monitoring and treatment of its complications.

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