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Saturday, October 13, 2007

Adrenoleukodystrophy, X-linked type

General:
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

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