Document Type : Case report
Authors
Clinical Research Development Unit of Akbar Hospital, Faculty of Medicine, Mashhad University of medical Sciences, Mashhad, Iran.
Abstract
Mucopolysaccharidosis is a group of progressive metabolic disease (lysosomal storage) characterized by the deposition of glycosaminoglycans in the body, affecting various organs including the skeletal system, (dysostosis multiplex), viscera (hepatomegaly and splenomegaly), neurological complications, eyes (corneal opacity and optic atrophy), and cardiac (thickening and valvular heart disease). The disease can be classified into different types based on the specific enzymatic deficiency. In some types of this disease, treatment is administered weekly through enzyme replacement therapy. Due to the potential for severe reactions and even anaphylaxis during infusion, these medications are prescribed under medical supervision. This article presents a report of an 8-year-old girl diagnosed with Mucopolysaccharidosis type 1 was treated weekly with 2 vials of the enzyme Aldurazyme involving drug allergy to this medication along with a desensitization protocol. The protocol includes more premedication with prednisolone and the use of lower dilutions of the drug, lower infusion rate and gradually increasing the concentration and speed of the drug in addition to the routine administration of the enzyme.
Keywords
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