Difficult intubation management in a child with I-cell disease
SJA-Saudi Journal of Anaesthesia. 2010; 4 (2): 105-107
in English
| 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
Search on Google
Index:
IMEMR (Eastern Mediterranean)
Main subject:
Tooth Extraction
/
Child
/
Transferases (Other Substituted Phosphate Groups)
/
Disease Management
/
Gingivectomy
/
Intubation
/
Anesthesia, Dental
Type of study:
Case report
Limits:
Female
/
Humans
Language:
English
Journal:
Saudi J. Anaesth.
Year:
2010
Similar
MEDLINE
...
LILACS
LIS