Mucopolysaccharidosis: radiographic findings in a series of 16 cases

Sarmento, Dmitry José de Santana; Carvalho, Sérgio Henrique Gonçalves De; Melo, Saulo Leonardo Sousa; Fonseca, Fátima Roneiva Alves; Diniz, Denise Nóbrega; Bento, Patrícia Meira; Mesquita, Germana de Queiroz Tavares Borges; Melo, Daniela Pita de

doi:10.1016/j.oooo.2015.08.009

Cited by 17 publications

(23 citation statements)

References 23 publications

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“…Eventually, other genetic entities may present ST as dental anomalies: achondroplasia, 65 Ellisvan Creveld syndrome, 2,4 Kreiborg-Pakistani s y n d r o m e , A p e r t s y n d r o m e , 2 C r o u z o n syndrome, 2,66 mucopolysaccharidoses type IV (Morquio syndrome), 67 and type VI (Marateaux-Lamy syndrome), 67,68 Goldenhar syndrome, 69 Noonan syndrome, 70 neurofibromatosis type-1, 71 Ehlers-Danlos syndrome, Hallermann-Streiff syndrome, 2 Nicolaides-Baraitser syndrome, 72 Zimmermann-Laband syndrome, 2 dystrophic epidermolysis bullosa, 73 enamel-renal-gingival syndrome, 74 Fabry disease, 2,4 and non-syndromic cleft lip and palate, 4,75 among others.…”

Section: Other Entities That May Have St Among Their Clinical Findingsmentioning

confidence: 99%

Main genetic entities associated with supernumerary teeth

Cammarata‐Scalisi

Avendaño

Callea

2018

Arch Argent Pediatr

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Supernumerary teeth represent a common human dental anomaly, defined as presence of extra teeth-more than the normal number foreseen in primary or permanent dentition. The prevalence has been reported between 0.2 to 3%, and is more frequent in males than females. The etiology is heterogeneous, highly variable and most of the cases are idiopathic. However, the presence of multiple impacted or erupted supernumerary teeth is rare and associated with some genetic syndromes: cleidocranial displasia, familial adenomatous polyposis, trichorhinophalangeal syndrome type I, Rubinstein-Taybi syndrome, Nance-Horan syndrome, Opitz G/BBB syndrome, oculofaciocardiodental syndrome and Robinow syndrome (autosomal dominant). The supernumerary teeth should be considered in order to possibly diagnose these entities with the aim of offering an interdisciplinary management and treatment, as well as offer adequate family genetic counseling.

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Section: Other Entities That May Have St Among Their Clinical Findingsmentioning

confidence: 99%

Main genetic entities associated with supernumerary teeth

Cammarata‐Scalisi

Avendaño

Callea

2018

Arch Argent Pediatr

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“…In MPS IV, there is generalized loss of tooth structure because of enamel defects. Studies have reported the presence of multiple dentigerous cysts, macroglossia, expanded bone marrow spaces, root resorption, impacted teeth, symphysis demineralization, and TMJ impairment in MPS VI (73). Several types of MPS and other storage disorders have common skeletal characteristics that involve the skull, thorax, spine, pelvis, long bones, and hands, which are described as dysostosis multiplex.…”

Section: Mucopolysaccharidosesmentioning

confidence: 99%

Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach

et al. 2018

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Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. The management of neoplastic diseases such as lymphoma, leukemia, or Langerhans cell histiocytosis may be different if diagnosed early, and metastases to the maxillofacial bones may be the first manifestation of an otherwise occult neoplasm. Endocrinologic and metabolic disorders also may manifest with maxillofacial conditions. Earlier recognition of osteoporosis may alter treatment and prevent complications such as insufficiency fractures, and identification of acromegaly may lead to surgical treatment if there is an underlying growth hormone-producing adenoma. Bone dysplasias sometimes are associated with skull base foraminal narrowing and subsequent involvement of the cranial nerves. Inflammatory processes such as rheumatoid arthritis and sarcoidosis may affect the maxillofacial bones, skull base, and temporomandibular joints. Radiologists should be familiar with the maxillofacial computed tomographic and magnetic resonance imaging findings of common systemic disorders because these may be the first manifestations of an otherwise unrevealed systemic process with potential for serious complications. Online supplemental material is available for this article. RSNA, 2018.

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“…MPS are clinically characterized by short stature, mental retardation, multiple dysostoses, cardiovascular/digestive anomalies, ocular impairments, and skin thickening. Early detected mild forms of MPS have a good prognosis and relatively normal life; however, most severe cases exhibit an average survival age of 10 years [3].…”

Section: Introductionmentioning

confidence: 99%

Pediatric Dental Management of an Uncommon Case of Mucopolysaccharidosis Type IV A (Morquio A Syndrome): A Case Report of a Three-Year Follow-Up

Gómez-González

Rosales-Berber

Ávila-Rojas

et al. 2020

Case Reports in Dentistry

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Mucopolysaccharidosis type IV A or Morquio syndrome is an uncommon inherited metabolic condition caused by the deficient intralysosomal storage of glycosaminoglycans. Diagnosis is typically based on clinical examination, skeletal radiographs, and histochemical tests in blood cells or fibroblasts. It is characterized by evident skeletal deformities, poor joint mobility, severe growth deficit, occlusal anomalies, and enamel defects. The aim of the present clinical case report is to describe the general oral management provided to a 6-year-old female patient and its corresponding evolution for more than three years.

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Mucopolysaccharidosis: radiographic findings in a series of 16 cases

Cited by 17 publications

References 23 publications

Main genetic entities associated with supernumerary teeth

Main genetic entities associated with supernumerary teeth

Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach

Pediatric Dental Management of an Uncommon Case of Mucopolysaccharidosis Type IV A (Morquio A Syndrome): A Case Report of a Three-Year Follow-Up

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