"Today, the U.S. Food and Drug Administration approved Kanuma (sebelipase alfa) as the first treatment for patients with a rare disease known as lysosomal acid lipase (LAL) deficiency.
Patients with LAL deficiency (also known as Wolman disea"...
(Generic versions may still be available.)
It is suggested that the management principles (consistent with the clinical status of the patient when first seen) outlined below be instituted and that simultaneous contact with a Regional Poison Control Center be established. In this way both updated information and individualization regarding required therapy may be provided.
1. When potential oral overdose is established and seizure has not occurred:
- If patient is alert and seen soon after ingestion, induction of emesis may be of value. Gastric lavage has been demonstrated to be of no value in influencing outcome in patients who present more than 1 hour after ingestion.
- Administer a cathartic. Sorbitol Solution is reported to be of value.
- Administer repeated doses of activated charcoal and monitor theophylline serum levels.
- Prophylactic administration of phenobarbital has been shown to increase the seizure threshold in laboratory animals, and administration of this drug may be of value.
2. If patient presents with a seizure:
- Establish an airway.
- Administer oxygen.
- Treat the seizure with intravenous diazepam 0.1 to 0.3 mg/kg up to 10 mg. If seizures cannot be controlled, the use of general anesthesia should be considered.
- Monitor vital signs, maintain blood pressure and provide adequate hydration.
3. If postseizure coma is present:
- Maintain airway and oxygenation.
- Follow above recommendations to prevent absorption of drug, but intubation and lavage will have to be performed instead of inducing emesis and the cathartic and charcoal will need to be introduced via a large-bore gastric lavage tube.
- Continue to provide full supportive care and adequate hydration until the drug is metabolized. In general, drug metabolism is sufficiently rapid so as not to warrant dialysis. If repeated oral activated charcoal is ineffective (as noted by stable or rising serum levels), charcoal hemoperfusion may be indicated.
Slo-Phyllin (theophylline, anhydrous) Tablets and Syrup are contraindicated in individuals who have shown hypersensitivity to any of the components of this product or to xanthine derivatives. It is also contraindicated in patients with active peptic ulcer disease, and in individuals with underlying seizure disorders (unless receiving appropriate anticonvulsant medication).This monograph has been modified to include the generic and brand name in many instances.
Last reviewed on RxList: 12/8/2004
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