Manifestations of Hurler syndrome

Abstract

Hurler Syndrome is a rare illness, resulting from genetic deficiency of lysosomal enzymes responsible for hydrolysis of mucopolysaccharides, which accumulates this macro-molecule in cells of connective tissue, causing clinical characteristics of this syndrome. The individuals present visual deficiency (opacification), splenohepatomegaly, mental retardation, heart disease, gingival alterations, macroglossia, dental eruption retardation, impacted teeth. The most usual characteristics are the impaction of permanent teeth and retardation of eruption. The gingival alterations observed by the authors were fibrosis and the gingival hyperplasia, because of the hygienic conditions and habits as buccal breathing. Regarding the histological aspects, characteristics as intracellular accumulations of mucopolysaccharide in various cells of the organism were observed, affecting liver, spleen, heart, cartilage, bones, as well as the fibroblasts which present aspect of “clear” cells also called gargoyle cells. This study relates the necessity of continuing researches on this subject, providing to the dentist a deeper vision on the illness.