Mehrnaz Nazarirad; Nasrin Moazzen; Mojtaba Lotfi
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, ...
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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.
Nasrin moazzen; sepideh bagheri; Nosrat Ghaemi; Sara Nikpour; Mojtaba Lotfi; Hamid Ahanchian
Abstract
Abstract: Background: type 1 diabetes is one of the most chronic diseases of childhood. The therapeutic recommendation for this condition is quite complex and needs changes in lifestyle. Recently the role of gut microbiota has received attention as a possible mechanism in the pathogenesis and control ...
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Abstract: Background: type 1 diabetes is one of the most chronic diseases of childhood. The therapeutic recommendation for this condition is quite complex and needs changes in lifestyle. Recently the role of gut microbiota has received attention as a possible mechanism in the pathogenesis and control of the disease.Methods: One hundred thirty Children aged 6-18 years old with newly diagnosed diabetes type one participate in the trial. They are randomly assigned to the intervention or control group. The patients will receive a six month course of probiotics or placebo besides their conventional treatment with insulin and they will be followed for at least one year. Participants are followed at 3 month intervals for one year. At each visit anthropometrical data, glycemic control and total dose of insulin required to keep the optimal glycemic control is evaluated and registered.Discussion: There is evidence that alterations in gut microbiota is seen in children with diabetes. In this study we aim to evaluate the effect of probiotic administration on the duration of honeymoon period in pediatrics with type one diabetes and their glycemic control and insulin requirements.