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CASE REPORT
Year : 2018  |  Volume : 12  |  Issue : 2  |  Page : 601-603

Anesthetic management for fracture head of radius in a child with glutaric aciduria type-1


Department of Anaesthesiology and Intensive Care, Acharya Shri Chander College of Medical Sciences and Hospital, Jammu, Jammu and Kashmir, India

Correspondence Address:
Dr. Gurleen Kaur
Department of Anaesthesiology and Intensive Care, Acharya Shri Chander College of Medical Sciences and Hospital, Sidhra, Jammu - 180 017, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aer.AER_34_18

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Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect leads to secondary damage to central nervous system due to the accumulation of glutaric acid. Approximately 90% people will develop the neurological disease during a finite period of brain development (3–36 months) following an acute encephalopathic crisis often precipitated by gastroenteritis, immunization, surgical intervention, and intercurrent febrile illness. GA-1 can also develop insidiously without clinically apparent crisis in 10%–20% of the patients. We present a 10-year-old male child with GA-1 who required anesthetic care for fracture (left) neck of radius. Strategies for anesthetic management should include prevention of hypoglycemia, dehydration, electrolyte imbalance, and sufficient analgesia to prevent surgical stress.


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