{"id":{"repo_id":"brazil-ufpb","oai_identifier":"oai:repositorio.ufpb.br:123456789/1358"},"canonical_url":"https://search.dev.ndltd.org/etd/brazil-ufpb/oai:repositorio.ufpb.br:123456789/1358","repository":{"repo_id":"brazil-ufpb","name":"Brazil UFPB","base_url":"https://repositorio.ufpb.br/oai/request"},"display":{"title":"Síndrome de morquio : uma revisão bibliográfica","abstract":"Mucopolysaccharidosis are a group of rare disease prevalence, characterized in that there is deficiency in the production of enzymes involved in the metabolic degradation of glycosaminoglycans lysosomal level. The accumulation of these intracellular substances causes various clinical manifestation s. The increase in intracellular glycosaminoglycan, caused by deficiency of the enzyme N - acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions. acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions.","abstract_html":"Mucopolysaccharidosis are a group of rare disease prevalence, characterized in that there is deficiency in the production of enzymes involved in the metabolic degradation of glycosaminoglycans lysosomal level. The accumulation of these intracellular substances causes various clinical manifestation s. The increase in intracellular glycosaminoglycan, caused by deficiency of the enzyme N - acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions. acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions.","abstract_has_math":false,"creators":["Cruz, Joyce Veloso Alves da"],"institution":"Universidade Federal da Paraíba","degree_name":null,"degree_level":null,"degree_discipline":null,"degree_department":null,"school":null,"contributors":[],"advisors":[],"committee_chairs":[],"committee_members":[],"year":2016,"date_issued":"2016-10-04","date_published":"2016-10-04","updated_at":"2026-07-24T01:18:10Z","subjects":["Mucopolisacaridose","Síndrome de Morquio","Perda Auditiva"],"languages":["por"],"rights":[],"rights_urls":[],"identifier_entries":[]},"links":{"outbound_url":"https://repositorio.ufpb.br/jspui/handle/123456789/1358","outbound_label":"Repository record","outbound_source":"dc:identifier.uri"},"metadata_groups":[{"id":"people","label":"People","entries":[{"key":"dc:creator","label":"Author","values":["Cruz, Joyce Veloso Alves da"]}]},{"id":"academic_context","label":"Academic Context","entries":[{"key":"dc:date.accessioned","label":"Dc Date Accessioned","values":["2016-10-04T14:19:39Z"]},{"key":"dc:date.available","label":"Dc Date Available","values":["2016-10-04T14:19:39Z"]},{"key":"dc:date.issued","label":"Date","values":["2016-10-04"]},{"key":"dc:publisher","label":"Institution","values":["Universidade Federal da Paraíba"]},{"key":"dc:publisher.department","label":"Dc Publisher Department","values":["Farmácia"]},{"key":"dc:type","label":"Dc Type","values":["TCC"]}]},{"id":"subjects_keywords","label":"Subjects and Keywords","entries":[{"key":"dc:subject","label":"Dc Subject","values":["Mucopolisacaridose","Síndrome de Morquio","Perda Auditiva"]}]},{"id":"language_rights","label":"Language and Rights","entries":[{"key":"dc:language.iso","label":"Language (ISO)","values":["por"]}]},{"id":"identifiers","label":"Identifiers","entries":[{"key":"dc:identifier.uri","label":"Identifier URI","values":["https://repositorio.ufpb.br/jspui/handle/123456789/1358"]}]},{"id":"additional","label":"Additional Metadata","entries":[{"key":"dc:description.abstract","label":"Abstract","values":["Mucopolysaccharidosis are a group of rare disease prevalence, characterized in that there is deficiency in the production of enzymes involved in the metabolic degradation of glycosaminoglycans lysosomal level. The accumulation of these intracellular substances causes various clinical manifestation s. The increase in intracellular glycosaminoglycan, caused by deficiency of the enzyme N - acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions. acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions."]},{"key":"dc:title","label":"Title","values":["Síndrome de morquio : uma revisão bibliográfica"]}]}],"canonical_facts":{"dc:creator":["Cruz, Joyce Veloso Alves da"],"dc:date.accessioned":["2016-10-04T14:19:39Z"],"dc:date.available":["2016-10-04T14:19:39Z"],"dc:date.issued":["2016-10-04"],"dc:description.abstract":["Mucopolysaccharidosis are a group of rare disease prevalence, characterized in that there is deficiency in the production of enzymes involved in the metabolic degradation of glycosaminoglycans lysosomal level. The accumulation of these intracellular substances causes various clinical manifestation s. The increase in intracellular glycosaminoglycan, caused by deficiency of the enzyme N - acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions. acetyl galactosamine - 6 - sulfatase causing Morquio syndrome type A, and type B is caused by a deficiency of beta - galactosidase. This syndrome brings as a consequence ske letal disorders, muscle and heart, thus impairing the physical and motor carrier without occurring psychiatric disorders. Diagnosis is made through a queratosulfato the dosage taken by urinalysis. Treatment is palliative, i.e., sãotratados only the symptom s that arise through the years. Is currently testing the treatment of enzyme replacement by Vimizim drug (elosulfase alpha) .The aim of this study is to conduct a literature review in order to promote specific and detailed knowledge regarding the clinical course of mucopolysaccharidosis VI, taking into consideraçãoa importance of early diagnosis, and discusses the evolution of therapeutic management. For this work become feasible readings and analyzes were performed of scientific papers, theses and disserta tions."],"dc:identifier.uri":["https://repositorio.ufpb.br/jspui/handle/123456789/1358"],"dc:language.iso":["por"],"dc:publisher":["Universidade Federal da Paraíba"],"dc:publisher.department":["Farmácia"],"dc:subject":["Mucopolisacaridose","Síndrome de Morquio","Perda Auditiva"],"dc:title":["Síndrome de morquio : uma revisão bibliográfica"],"dc:type":["TCC"]},"updated_at":"2026-07-24T01:18:10Z"}