International Journal of Oral and Maxillofacial Surgery
Mucopolysaccharidosis: MPS VI and associated delayed tooth eruption
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Cited by (33)
Imaging features of mucopolysaccharidoses in the head and neck
2020, International Journal of Pediatric OtorhinolaryngologyMucopolysaccharidoses
2020, Emery and Rimoin’s Principles and Practice of Medical Genetics and Genomics: Metabolic DisordersA novel compound heterozygote mutation in the ARSB gene in a patient with Maroteaux-Lamy syndrome and its Insilico evaluation
2018, Meta GeneCitation Excerpt :Common clinical phenotypes include coarse face, macrocephaly, short stature, corneal clouding, recurrent otitis media, recurrent sinusitis, enlarged tongue, abnormal teeth, sleep apnea, recurrent pulmonary infections, valvular disease, hepatosplenomegaly, umbilical and inguinal hernias, dysostosis multiplex and cervical spinal cord compression. These patients do not have cognitive impairment (Miller and Partridge, 1983; Smith et al., 1995; Vougioukas et al., 2001; Choy et al., 2015). Patients with fast progressing form of the disease represent skeletal abnormalities, joint stiffness and deformities, short stature with reduced growth rate, cardiovascular symptoms, and recurrent upper airway obstructions and infections during the first few years of life (Vairo et al., 2015; Harmatz and Shediac, 2017).
Diagnostic Imaging: Head and Neck
2017, Diagnostic Imaging: Head and NeckChallenges managing individuals with hereditary defects of the teeth
2016, Seminars in OrthodonticsCitation Excerpt :It is essential to ensure that the eruptive pathway is clear and free of obstructions. Other conditions with cyst development and failure of tooth eruption, such as the mucopolysaccharidoses (OMIM #309900) with severe growth inhibition and medical issues, might be best managed by no treatment depending on the individual’s medical status.35 Physical blockage of tooth eruption by excessive soft tissue or supernumerary teeth will require the orthodontist to work with the oral surgeon, periodontist, or primary care dentist to remove the obstruction.
Mucopolysaccharidosis: Radiographic findings in a series of 16 cases
2015, Oral Surgery, Oral Medicine, Oral Pathology and Oral RadiologyCitation Excerpt :Although the exfoliation of deciduous teeth is essential for permanent teeth proper eruption, there are no studies that specify a relationship between the delay in deciduous teeth exfoliation and MPS. Some have reported delayed eruption of the permanent dentition associated with MPS.14,16,19 Teeth impaction was observed in 71.4% of MPS VI patients, 50% of MPS IV patients, and in the MPS I subject.