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Glycogen storage disease type II (GSD-II), also called Pompe disease, and formerly known as GSD-IIa or Limb–girdle muscular dystrophy 2V, is an autosomal recessive metabolic disorder [1] which damages muscle and nerve cells throughout the body.
Avalglucosidase alfa is composed of the human GAA enzyme that is conjugated with a couple of bis-mannose-6-phosphate (bis-M6P) tetra-mannose glycans. [12] The bis-MGP of avalglucosidase alpha binds to the cation-independent mannose-6-phosphate receptor which is located on the skeletal muscles. [12]
Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal.This tumor is rare, with several names used in the past. [3] [4] Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), [1] [2] and ceruminous mucoepidermoid carcinoma.
Keratoacanthoma is commonly found on sun-exposed skin, often face, forearms and hands. [2] [3] It is rarely found at a mucocutaneous junction or on mucous membranes. [2] Keratoacanthoma may be difficult to distinguish visually from a skin cancer. [4] Under the microscope, keratoacanthoma very closely resembles squamous cell carcinoma. In order ...
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