The average half-life of avalglucosidase alfa was 1.6 hours, measured in patients with late stage Pompe disease. There is little information availible on the metabolism of the avalglucosidase alfa. The protein portion of the drug however does break down into small peptides via catabolic pathways. The clearance of the drug is 0.9 L/hour in patients that exhibited late-stage Pompe disease. Blackbox warnings
Avalglucosidase alfa has a blackbox warning for hypersensitivity, infusion-related reactions, and cardiorespiratory failure. Society and culture
Legal status
In July 2021, the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) adopted a positive opinion, recommending the granting of a marketing authorization for the medicinal product Nexviadyme, intended for the treatment of glycogen storage disease type II (Pompe disease). The applicant for this medicinal product is Genzyme Europe BV. In August 2021, Genzyme Europe BV requested a re-examination. Avalglucosidase alfa was approved for medical use in the European Union in June 2022.The U.S. Food and Drug Administration (FDA) granted the application for avalglucosidase alfa fast track, priority review, breakthrough therapy, and orphan drug designations. The FDA granted the approval of Nexviazyme to Genzyme Corporation. Names
Avalglucosidase alfa is the international nonproprietary name (INN). References
External links
"Avalglucosidase alfa". Drug Information Portal. U.S. National Library of Medicine.
This is seen as a PR interval greater than 200 ms in length on the surface ECG. It is usually an incidental finding on a routine ECG.First-degree heart block does not require any particular investigations except for electrolyte and drug screens, especially if an overdose is suspected. Treatment
The management includes identifying and correcting electrolyte imbalances and withholding any offending medications. This condition does not require admission unless there is an associated myocardial infarction. Even though it usually does not progress to higher forms of heart block, it may require outpatient follow-up and monitoring of the ECG, especially if there is a comorbid bundle branch block. If there is a need for treatment of an unrelated condition, care should be taken not to introduce any medication that may slow AV conduction. If this is not feasible, clinicians should be very cautious when introducing any drug that may slow conduction; and regular monitoring of the ECG is indicated. Prognosis
Isolated first-degree heart block has no direct clinical consequences. There are no symptoms or signs associated with it. It was originally thought of as having a benign prognosis. In the Framingham Heart Study, however, the presence of a prolonged PR interval or first degree AV block doubled the risk of developing atrial fibrillation, tripled the risk of requiring an artificial pacemaker, and was associated with a small increase in mortality.