Two types: X-linked, and Neonatal(autosomal recessive)
Airway:
Hypotonia/airway obstruction
Respiratory:
Airway obstruction
Gastrointestinal:
Liver function abnl
GERD
Endocrine:
Hyperpigmentation due to ACTH oversecretion
Neurological:
Motor/mental retardation
Hypotonia
Seizures
Spastic paraplegia
Musculoskeletal:
Hypotonia
Considerations:
Premedication/hypotonia bad combination
Perioperative aspiration
Possible excess K+ release with succinylcholine
Positioning nightmare
Post-anesthetic airway obstruction
Liver induction from anticonvulsants
Perioperative stress-dose steroids
Avoid dopaminergic blocker drugs
Ondansetron is your friend!
Links:
Adrenomyeloneuropathy
Metachromatic leukodystrophy
Krabbe disease
Canavan disease
Pelizaeus-Merzbacher disease
Alexander disease
Articles:
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