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Hemoglobinopatias e sua relação com doenças da cavidade oral: revisão narrativa
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As hemoglobinopatias estão entre as doenças hereditárias mais comuns em todo o mundo. Consistem em alterações genéticas que afetam a estrutura ou a produção normal da hemoglobina. As hemoglobinopatias podem ser divididas em dois grupos principais: as que provocam alterações quantitativas, isto é, envolvem um desequilíbrio no número de cadeias de globina, e aquelas cujas alterações provocam distúrbios qualitativos, que resultam em alterações estruturais na molécula de hemoglobina. Alterações quantitativas da hemoglobina resultam em talassemias, enquanto os defeitos qualitativos estão associados por exemplo a anemia falciforme. Enquanto algumas formas leves de talassemia podem passar despercebidas e causar apenas anemia leve e problemas de deficiência de ferro, existem outras formas mais graves. As principais manifestações orais da talassemia são má oclusão, protrusão maxilar, alto índice de cárie e gengivite severa. A anemia falciforme resulta de glóbulos vermelhos com forma anormal e função alterada, sendo a principal característica clínica a anemia hemolítica. As complicações agudas são frequentes e variáveis e incluem síndrome torácica, acidente vascular cerebral, infeções e dor óssea. Outras complicações crónicas que podem ocorrer são necrose óssea, nefropatia e distúrbios cardíacos, pulmonares e cutâneos. As lesões orais também são muito comuns e incluem necrose asséptica da polpa, danos na mucosa devido a anemia, atrasos na erupção dentária, dor óssea e osteomielite da maxila e neuropatias orais. A higiene oral neste tipo de pacientes requer cuidados específicos e um acompanhamento regular.
Hemoglobinopathies are among the most common hereditary diseases worldwide. They consist of genetic alterations that affect the normal structure or production of hemoglobin. Hemoglobinopathies can be divided into two main groups: those in which the genetic alterations cause quantitative changes, involving an imbalance in the number of globin chains, and those whose genetic change causes qualitative disorders, which result in changes in hemoglobin structure. Quantitative changes in hemoglobin result in thalassemia, while qualitative defects are associated, for example, with sickle cell anemia. While some mild forms of thalassemia can go undetected and cause only mild anemia and iron deficiency problems, there are other more serious forms. The main oral manifestations of thalassemia are malocclusion, maxillary protrusion, high caries rate and severe gingivitis. Sickle cell anemia results from abnormally shaped red blood cells with altered function, the main clinical feature being hemolytic anemia. Acute complications are frequent and variable and include chest syndrome, stroke, infections, and bone pain. Other chronic complications that may occur are bone necrosis, nephropathy, and heart, lung, and skin disorders. Oral lesions are also very common and include aseptic pulp necrosis, mucosal damage due to anemia, delayed tooth eruption, bone pain and osteomyelitis of the jaw, and oral neuropathies. In patients having this type of disease, oral hygiene requires specific care and regular monitoring.
Hemoglobinopathies are among the most common hereditary diseases worldwide. They consist of genetic alterations that affect the normal structure or production of hemoglobin. Hemoglobinopathies can be divided into two main groups: those in which the genetic alterations cause quantitative changes, involving an imbalance in the number of globin chains, and those whose genetic change causes qualitative disorders, which result in changes in hemoglobin structure. Quantitative changes in hemoglobin result in thalassemia, while qualitative defects are associated, for example, with sickle cell anemia. While some mild forms of thalassemia can go undetected and cause only mild anemia and iron deficiency problems, there are other more serious forms. The main oral manifestations of thalassemia are malocclusion, maxillary protrusion, high caries rate and severe gingivitis. Sickle cell anemia results from abnormally shaped red blood cells with altered function, the main clinical feature being hemolytic anemia. Acute complications are frequent and variable and include chest syndrome, stroke, infections, and bone pain. Other chronic complications that may occur are bone necrosis, nephropathy, and heart, lung, and skin disorders. Oral lesions are also very common and include aseptic pulp necrosis, mucosal damage due to anemia, delayed tooth eruption, bone pain and osteomyelitis of the jaw, and oral neuropathies. In patients having this type of disease, oral hygiene requires specific care and regular monitoring.
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Keywords
Hemoglobinopatias Talassemia Doenças orais Cáries Hemoglobinopathies Thalassemia Oral diseases Caries