Difficult intubation management in a child with I-cell disease
SJA-Saudi Journal of Anaesthesia. 2010; 4 (2): 105-107
em Inglês
| IMEMR
| ID: emr-129146
ABSTRACT
I-cell disease [mucolipidosis II] is a rare metabolic disorder resulting from the deficiency of a specific lysosomal enzyme, N-acetylglucosamine-1-phosphotransferease. Developmental delay and growth failure are common presentations of I-cell disease. Psychomotor deterioration is rapid and progressive. Some physical signs such as hip dislocations, inguinal hernia, hepatomegaly, joint limitation, and skin changes may be present at birth. Coarse facial features and skeletal abnormalities become more conspicuous with time. The life expectancy of children with this condition is poor, with death usually occurring around the fifth year. A case report of the anesthetic management of gingivectomy with multiple dental extractions in a 5-year-old Omani female with I-cell disease is presented. The problems faced and their management during anesthesia are described
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Índice:
IMEMR (Mediterrâneo Oriental)
Assunto principal:
Extração Dentária
/
Criança
/
Transferases (Outros Grupos de Fosfato Substituídos)
/
Gerenciamento Clínico
/
Gengivectomia
/
Intubação
/
Anestesia Dentária
Tipo de estudo:
Relato de Casos
Limite:
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Saudi J. Anaesth.
Ano de publicação:
2010
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