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Volume 5, Issue 4, Pages 180-182 (December 2010)


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Johanson–Blizzard syndrome—A case study of oral and systemic manifestations

Keila Martha Amorim BarrosoaCorresponding Author Informationemail address, Debora Farias Batista Leiteb, Pollianna Muniz Alvesc, Paula Frassinetti Vasconcelos de Medeirosb, Gustavo Pina Godoyc

Received 14 August 2009; received in revised form 30 September 2009; accepted 1 October 2009. published online 07 December 2009.

Abstract 

Johanson–Blizzard syndrome is a hereditary disorder extremely rare. The characteristic features include aplastic alae nasi, midline ectodermal scalp defects, deafness, dental abnormalities and malabsorption related to pancreatic exocrine deficiency. This paper presents a case of an 18-year-old patient with Johanson–Blizzard syndrome and emphasizes the importance of knowledge of the potential anaesthetic concerns of this syndrome for providing appropriate treatment for these patients.

a Federal University of the Paraíba, João Pessoa, Paraíba, Brazil

b Federal University of Campina Grande, Campina Grande, Paraíba, Brazil

c State University of the Paraíba, Campina Grande, Paraíba, Brazil

Corresponding Author InformationCorresponding author.

PII: S1871-4048(09)00060-4

doi:10.1016/j.pedex.2009.10.002


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