Two types: X-linked, and Neonatal(autosomal recessive)
Airway:
High arched palate
Gastrointestinal:
Liver function abnl
GERD
Endocrine:
Hyperpigmentation due to ACTH oversecretion
Neurological:
Mental retardation
Motor delay
Seizures
Musculoskeletal:
Motor delay
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:
Zellweger syndrome
Refsum disease
Articles:
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