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Endocrine dysfunction in a patient with phace syndrome, including port-wine stain of the right periorbital area

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dc.contributor.author Merheb M.
dc.contributor.author Hourani R.
dc.contributor.author Zantout M.S.
dc.contributor.author Azar S.T.
dc.contributor.editor
dc.date 2010
dc.date.accessioned 2017-10-05T15:39:31Z
dc.date.available 2017-10-05T15:39:31Z
dc.date.issued 2010
dc.identifier 10.4158/EP09179.CR
dc.identifier.isbn
dc.identifier.issn
dc.identifier.uri http://hdl.handle.net/10938/16924
dc.description.abstract Objective: To report a case of PHACE syndrome - Posterior fossa brain abnormalities, Hemangioma (usually facial), Arterial anomalies, Coarctation of the aorta along with cardiac defects, and Eye abnormalities - in a 16-year-old female patient with a port-wine stain of the right periorbital area present since birth in conjunction with hypoplasia of the contralateral internal carotid artery. Methods: Thyroid-stimulating hormone, free thyroxine, and growth hormone (GH) levels were measured, and insulin-induced hypoglycemia and arginine infusion tests were done. Radiologic investigations included sagittal enhanced T1-weighted magnetic resonance imaging of the brain and the pituitary gland as well as computed tomography and magnetic resonance angiography of the head and neck. Results: The patient had a normal karyotype. Her height and weight were below the 5th percentile for her chronologic age, and she had amenorrhea. Laboratory investigations revealed both thyroid and GH deficiencies and confirmed the diagnosis of hypogonadotropic hypogonadism. The imaging studies showed a right intraorbital hemangioma as well as an enhancing mass in the right internal auditory canal at the cerebellopontine angle, consistent with a posterior fossa hemangioma. Initiation of both thyroid and GH replacement therapy improved her growth rate and yielded a good clinical outcome. Conclusion: In patients with facial or neck hemangiomas, PHACE syndrome should be suspected, and brain imaging and cardiac, ophthalmologic, and endocrinologic evaluations are recommended to screen for other potential PHACE abnormalities. © 2010 AACE.
dc.format.extent
dc.format.extent Pages: (255-259)
dc.language English
dc.publisher JACKSONVILLE
dc.relation.ispartof Publication Name: Endocrine Practice; Publication Year: 2010; Volume: 16; no. 2; Pages: (255-259);
dc.relation.ispartofseries
dc.relation.uri
dc.source Scopus
dc.subject.other
dc.title Endocrine dysfunction in a patient with phace syndrome, including port-wine stain of the right periorbital area
dc.type Article
dc.contributor.affiliation Merheb, M., Department of Internal Medicine, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Hourani, R., Department of Diagnostic Radiology, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Zantout, M.S., Department of Internal Medicine, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Azar, S.T., Department of Internal Medicine, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.authorAddress Azar, S. T.; Department of Internal Medicine, American University, Beirut Medical Center, 3 Dag Hammarskjold Plaza, New York, NY 10017, United States; email: sazar@aub.edu.lb
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.authorDivision
dc.contributor.authorEmail sazar@aub.edu.lb
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials Merheb, M
dc.contributor.authorInitials Hourani, R
dc.contributor.authorInitials Zantout, MS
dc.contributor.authorInitials Azar, ST
dc.contributor.authorOrcidID
dc.contributor.authorReprintAddress Azar, ST (reprint author), Amer Univ Beirut, Med Ctr, Div Endocrinol and Metab, Dept Internal Med, 3 Dag Hammarskjold Plaza,8th Floor, New York, NY 10017 USA.
dc.contributor.authorResearcherID
dc.contributor.authorUniversity American University of Beirut Medical Center
dc.description.cited Burrows PE, 1998, RADIOLOGY, V207, P601; Coats DK, 1999, OPHTHALMOLOGY, V106, P1739, DOI 10.1016-S0161-6420(99)90350-0; Frieden IJ, 1996, ARCH DERMATOL, V132, P307, DOI 10.1001-archderm.132.3.307; Ghosh A, 2007, INDIAN PEDIATR, V44, P144; Goddard DS, 2006, PEDIATR DERMATOL, V23, P476, DOI 10.1111-j.1525-1470.2006.00287.x; Hangaard J, 1996, J CLIN ENDOCR METAB, V81, P2502, DOI 10.1210-jc.81.7.2502; Heyer GL, 2006, PEDIATR NEUROL, V35, P419, DOI 10.1016-j.pediatrneurol.2006.06.021; Huang SA, 2000, NEW ENGL J MED, V343, P185, DOI 10.1056-NEJM200007203430305; Judd CD, 2007, AM J NEURORADIOL, V28, P25; Lasky JB, 2004, J AAPOS, V8, P495, DOI 10.1016-j.jaapos.2004.06.014; Milosević Maja, 2005, Med Pregl, V58, P410, DOI 10.2298-MPNS0508410M; MULLIKEN JB, 1982, PLAST RECONSTR SURG, V69, P412, DOI 10.1097-00006534-198203000-00002; PASCUALCASTROVIEJO I, 1978, NEURORADIOLOGY, V16, P82; Poindexter G, 2007, PEDIATR NEUROL, V36, P402, DOI 10.1016-j.pediatrneurol.2007.01.017; Rossi A, 2006, AM J NEURORADIOL, V27, P1602; Torer B, 2007, EUR J PEDIATR, V166, P1293, DOI 10.1007-s00431-006-0391-x; Tortori-Donati P, 1999, NEURORADIOLOGY, V41, P369; Weon YC, 2005, AM J NEURORADIOL, V26, P2635
dc.description.citedCount
dc.description.citedTotWOSCount 1
dc.description.citedWOSCount 1
dc.format.extentCount 5
dc.identifier.articleNo
dc.identifier.coden EPNRA
dc.identifier.pubmedID
dc.identifier.scopusID 77953955573
dc.identifier.url
dc.publisher.address 245 RIVERSIDE AVENUE, STE 200, JACKSONVILLE, FL 32202 USA
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dc.relation.ispartofConferenceCode
dc.relation.ispartofConferenceDate
dc.relation.ispartofConferenceHosting
dc.relation.ispartofConferenceLoc
dc.relation.ispartofConferenceSponsor
dc.relation.ispartofConferenceTitle
dc.relation.ispartofFundingAgency
dc.relation.ispartOfISOAbbr Endocr. Pract.
dc.relation.ispartOfIssue 2
dc.relation.ispartOfPart
dc.relation.ispartofPubTitle Endocrine Practice
dc.relation.ispartofPubTitleAbbr Endocr. Pract.
dc.relation.ispartOfSpecialIssue
dc.relation.ispartOfSuppl
dc.relation.ispartOfVolume 16
dc.source.ID WOS:000277497400020
dc.type.publication Journal
dc.subject.otherAuthKeyword
dc.subject.otherChemCAS arginine, 1119-34-2, 15595-35-4, 7004-12-8, 74-79-3
dc.subject.otherChemCAS growth hormone, 36992-73-1, 37267-05-3, 66419-50-9, 9002-72-6
dc.subject.otherChemCAS insulin, 9004-10-8
dc.subject.otherChemCAS thyrotropin, 9002-71-5
dc.subject.otherChemCAS thyroxine, 7488-70-2
dc.subject.otherIndex arginine
dc.subject.otherIndex growth hormone
dc.subject.otherIndex insulin
dc.subject.otherIndex thyrotropin
dc.subject.otherIndex thyroxine
dc.subject.otherIndex adolescent
dc.subject.otherIndex amenorrhea
dc.subject.otherIndex aorta coarctation
dc.subject.otherIndex artery malformation
dc.subject.otherIndex article
dc.subject.otherIndex brain
dc.subject.otherIndex brain malformation
dc.subject.otherIndex carotid artery
dc.subject.otherIndex case report
dc.subject.otherIndex computer assisted tomography
dc.subject.otherIndex congenital heart malformation
dc.subject.otherIndex endocrine disease
dc.subject.otherIndex eye malformation
dc.subject.otherIndex female
dc.subject.otherIndex free thyroxine index
dc.subject.otherIndex growth hormone deficiency
dc.subject.otherIndex growth rate
dc.subject.otherIndex head
dc.subject.otherIndex height
dc.subject.otherIndex hemangioma
dc.subject.otherIndex hormone substitution
dc.subject.otherIndex human
dc.subject.otherIndex hypoglycemia
dc.subject.otherIndex hypogonadotropic hypogonadism
dc.subject.otherIndex hypophysis
dc.subject.otherIndex hypoplasia
dc.subject.otherIndex hypothyroidism
dc.subject.otherIndex internal auditory canal
dc.subject.otherIndex laboratory test
dc.subject.otherIndex magnetic resonance angiography
dc.subject.otherIndex neck
dc.subject.otherIndex nevus flammeus
dc.subject.otherIndex nuclear magnetic resonance imaging
dc.subject.otherIndex orbit
dc.subject.otherIndex outcome assessment
dc.subject.otherIndex pons angle
dc.subject.otherIndex posterior fossa
dc.subject.otherIndex posterior fossa brain disease
dc.subject.otherIndex weight
dc.subject.otherKeywordPlus INTERNAL CAROTID ARTERIES
dc.subject.otherKeywordPlus INFANTILE HEMANGIOMAS
dc.subject.otherKeywordPlus CAPILLARY HEMANGIOMA
dc.subject.otherKeywordPlus MALFORMATIONS
dc.subject.otherKeywordPlus ASSOCIATION
dc.subject.otherKeywordPlus HYPOTHYROIDISM
dc.subject.otherKeywordPlus AGENESIS
dc.subject.otherWOS Endocrinology and Metabolism


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