Oral findings in the Morquio syndrome (mucopolysaccharidosis IV). 1975

L S Levin, and R J Jorgenson, and C F Salinas

The Morquio syndrome is characterized by a specific pattern of platyspondylia, corneal opacities, keratosulfate excretion in the urine, and dental abnormalities. Oral examinations were performed on twelve patients with the condition. The maxillary anterior teeth were widely spaced and flared. The posterior teeth were tapered and had pointed cusp tips. The enamel was of normal hardness, and in some patients the surface was pitted. In roentgenograms, the enamel was less than one fourth its normal thickness but was of normal radiodensity. The prevalence of caries may have been reduced. The hard palates were broad and flat. The dental abnormalities in the Morquio syndrome are of a type that is unique among the group of genetic mucopolysaccharidoses. Our findings therefore support the conjecture that the biochemical defect in this condition is different from that which occurs in the other mucopolysaccharidoses.

UI MeSH Term Description Entries
D008297 Male Males
D009059 Mouth Diseases Diseases involving the MOUTH. Disease, Mouth,Diseases, Mouth,Mouth Disease
D009085 Mucopolysaccharidosis IV Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme. Eccentro-Osteochondrodysplasia,Morquio's Disease,Eccentroosteochondrodysplasia,GALNS Deficiency,Galactosamine-6-Sulfatase Deficiency,MPS IV A,MPS IV B,MPS IVA,MPS IVB,Morquio A Disease,Morquio B Syndrome,Morquio Disease,Morquio Syndrome,Morquio Syndrome A,Morquio Syndrome B,Morquio Syndrome, Type B,Morquio's Disease Type B,Morquio's Syndrome,Morquio-B Disease,Mucopolysaccharidosis 4,Mucopolysaccharidosis 4B,Mucopolysaccharidosis Type IV,Mucopolysaccharidosis Type IV A,Mucopolysaccharidosis Type IV B,Mucopolysaccharidosis Type IVA,Mucopolysaccharidosis Type IVB,Deficiencies, GALNS,Deficiencies, Galactosamine-6-Sulfatase,Deficiency, GALNS,Deficiency, Galactosamine-6-Sulfatase,Disease, Morquio,Disease, Morquio A,Disease, Morquio's,Disease, Morquio-B,Eccentro Osteochondrodysplasia,Eccentro-Osteochondrodysplasias,Eccentroosteochondrodysplasias,GALNS Deficiencies,Galactosamine 6 Sulfatase Deficiency,Galactosamine-6-Sulfatase Deficiencies,IV, Mucopolysaccharidosis Type,IVs, Mucopolysaccharidosis Type,Morquio B Disease,Morquio Syndromes,Morquios Disease,Morquios Syndrome,Mucopolysaccharidosis 4Bs,Mucopolysaccharidosis Type IVBs,Mucopolysaccharidosis Type IVs,Syndrome A, Morquio,Syndrome, Morquio,Syndrome, Morquio B,Syndrome, Morquio's,Syndromes, Morquio,Type IV, Mucopolysaccharidosis,Type IVs, Mucopolysaccharidosis
D010159 Palate The structure that forms the roof of the mouth. It consists of the anterior hard palate (PALATE, HARD) and the posterior soft palate (PALATE, SOFT). Incisive Papilla,Incisive Papillas,Palates,Papilla, Incisive,Papillas, Incisive
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003743 Dental Enamel A hard thin translucent layer of calcified substance which envelops and protects the dentin of the crown of the tooth. It is the hardest substance in the body and is almost entirely composed of calcium salts. Under the microscope, it is composed of thin rods (enamel prisms) held together by cementing substance, and surrounded by an enamel sheath. (From Jablonski, Dictionary of Dentistry, 1992, p286) Enamel,Enamel Cuticle,Dental Enamels,Enamel, Dental,Enamels, Dental,Cuticle, Enamel,Cuticles, Enamel,Enamel Cuticles,Enamels
D003744 Dental Enamel Hypoplasia An acquired or hereditary condition due to deficiency in the formation of tooth enamel (AMELOGENESIS). It is usually characterized by defective, thin, or malformed DENTAL ENAMEL. Risk factors for enamel hypoplasia include gene mutations, nutritional deficiencies, diseases, and environmental factors. Enamel Hypoplasia,Enamel Agenesis,Enamel Hypoplasia, Dental,Hypoplasia, Dental Enamel,Hypoplastic Enamel,Agenesis, Enamel,Enamel Ageneses,Enamel Hypoplasias,Enamel, Hypoplastic,Hypoplasia, Enamel
D004245 DMF Index "Decayed, missing and filled teeth," a routinely used statistical concept in dentistry. Decayed, Missing, and Filled Teeth,DMF Indexes,DMF Indexe,DMF Indices,Index, DMF,Indexe, DMF,Indexes, DMF,Indices, DMF
D005260 Female Females

Related Publications

L S Levin, and R J Jorgenson, and C F Salinas
January 1979, Revista chilena de pediatria,
L S Levin, and R J Jorgenson, and C F Salinas
June 2007, The Journal of the Association of Physicians of India,
L S Levin, and R J Jorgenson, and C F Salinas
August 1975, Der Radiologe,
L S Levin, and R J Jorgenson, and C F Salinas
June 1993, Ophthalmic paediatrics and genetics,
L S Levin, and R J Jorgenson, and C F Salinas
March 1992, Der Radiologe,
L S Levin, and R J Jorgenson, and C F Salinas
May 2023, Pediatric radiology,
L S Levin, and R J Jorgenson, and C F Salinas
January 1998, Ryoikibetsu shokogun shirizu,
L S Levin, and R J Jorgenson, and C F Salinas
October 1975, The Johns Hopkins medical journal,
L S Levin, and R J Jorgenson, and C F Salinas
February 2022, Radiology case reports,
L S Levin, and R J Jorgenson, and C F Salinas
January 1981, Brain & development,
Copied contents to your clipboard!