Oral manifestations in children with mucopolysaccharidosis

Ponciano, Sara; Areias, Cristina

doi:10.5935/medicalexpress.2017.05.04

Cited by 4 publications

(13 citation statements)

References 26 publications

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“…The researchers observed in 92% of cases with people with MPS frontal bite and limitations in the opening of the mouth. In a half of the cases, there was a gap between the central incisors in the study group, known as diastema ( Figure 2) [40]. Furthermore, we can observe the following: delayed eruption of teeth, and adenoids often become enlarged and can contribute to block the airway.…”

Section: Alpha-mannosidosismentioning

confidence: 92%

“…Katarzyna Z. Machut, Agata Żółtowska quantitative disturbances in enamel structure, which appear as enamel hypoplasia, as well as abnormalities in the anatomical structure of the teeth (misshapen teeth), peg-shaped or hypoplastic [4,40]. Patients who suffer from MPS type VI (Marretoux-Lamy syndrome) are predisposed to developing union cysts (DC, dentigerous cysts) [47].…”

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confidence: 99%

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Manifestations of lysosomal storage diseases in the stomatognathic system – a literature review

Machut¹,

Zółtowska²

2019

jos

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The stomatognathic system comprises the mouth, teeth, jaws, pharynx, and related structures associated with mastication, swallowing, and speech. Lysosomal storage diseases (LSDs) are metabolic disorders, which cause an increase of systemic complication and are even life-threatening. They may also lead to a change in the socioeconomic conditions of people suffering from these diseases, and their families. LSDs are inherited in an autosomal recessive pattern. Many syndromes of lysosomal diseases represent anomalies of the craniofacial region and in the oral cavity. For the vast majority, treatment is not required, but for some of them it is necessary to reconstruct the morphology or the proper function. The appearance of the face can have a negative impact on the quality of the patient's life. Breathing, chewing, speaking, smiling, and mimic movements are the consequences of facial deformities. Irregularities of the sensory organs can sometimes be observed-especially of hearing, smell, and sight. The most frequent manifestations of lysosomal diseases in the oral cavity are malocclusion, macroglossia, tooth eruption, and development disorders as well as periodontal disorders, for instance hypertrophy of the gingiva. The population of people suffering from lysosomal diseases often have specific oral health needs compared to the population of generally healthy people.

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Section: Alpha-mannosidosismentioning

confidence: 92%

mentioning

confidence: 99%

Manifestations of lysosomal storage diseases in the stomatognathic system – a literature review

Machut¹,

Zółtowska²

2019

jos

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“…Patients with Maroteaux Lamy syndrome (MPS VI) have a predilection for thickening of the dental follicle (>90% of patients in a sample size of 17 18 ) around any unerupted teeth and potential transformation into a dentigerous cyst. 3,12,28 Clinically, dentigerous cysts have the propensity to delay or even preclude normal eruption and may occasionally cause displacement of adjacent teeth or become secondarily infected. 29 MPS VI, caused by a deficiency of enzyme aryl sulfatase-B, results in an inability to degrade dermatan sulphate, a compound which can be found histologically in the dental follicles of patients with MPS VI.…”

Section: Maroteaux Lamy Syndrome (Mps Vi)mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Clinically, dentigerous cysts have the propensity to delay or even preclude normal eruption and may occasionally cause displacement of adjacent teeth or become secondarily infected 29 . MPS VI, caused by a deficiency of enzyme aryl sulfatase‐B, results in an inability to degrade dermatan sulphate, a compound which can be found histologically in the dental follicles of patients with MPS VI 28 . Additionally, patients with MPS VI may present with hypoplastic condyles (>90%), 18 ectopic teeth (>90%), anterior open bite (>85%), taurodontism (>50%), generalised bony rarefactions, expansion of marrow spaces and osteosclerosis 17,18 …”

Section: Introductionmentioning

confidence: 99%

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Inborn errors of metabolism and their impact in paediatric dentistry

Hirst

Chakrapani

Mubeen

2022

J of Inher Metab Disea

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The management of paediatric patients with inborn errors of metabolism (IEM) presents an unparalleled challenge for paediatric dentists owing to the multiplex of interrelated dental manifestations and metabolic management necessitating modifications to dental care. Inborn errors of metabolism describe a largely heterogenous group of genetic disorders namely attributable to a single gene defect essential for a specific metabolic pathway. Approximately 400 disorders have been described with an overall incidence of 1 in 5000 live births worldwide. Clinical presentation is classically inconspicuous and insidious in the neonatal period with pathophysiology attributable to accumulation of toxic by‐products which interfere with normal function, or insufficient synthesis of essential compounds. This paper aims to discuss the primary oral and maxillofacial manifestations across the scope of inborn errors of metabolism, whilst also considering how metabolic treatment has the propensity to complicate dental management.

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Orofacial abnormalities in mucopolysaccharidosis and mucolipidosis typeIIandIII: A systematic review

et al. 2022

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Mucopolysaccharidoses (MPSs) and mucolipidosis II and III (ML II and III) often manifest with orofacial (progressive) abnormalities, which may have a major impact on quality of life. However, because these patients have multiple somatic health issues, orofacial problems are easily overlooked in clinical practice and available literature on this topic solely consists of case reports, small case series, and small cohort studies. The aim of this systematic review was to gain more insight in the nature and extent of orofacial abnormalities in MPS, ML II, and III. A systematic review of all previously published articles addressing orofacial abnormalities in MPS, ML II, and III was performed. Both clinical studies and case reports were included. Outcome was the described orofacial abnormalities, subdivided into abnormalities of the face, maxilla, mandible, soft tissues, teeth, and occlusion. The search resulted in 57 articles, describing orofacial features in 340 patients. Orofacial abnormalities were present in all subtypes of MPS, ML II, and III, and consisted of thickened lips, a hypoplastic midface, a high‐arched palate, hypoplastic condyles, coronoid hyperplasia, macroglossia, gingival hyperplasia, thick dental follicles, dentigerous cysts, misshapen teeth, enamel defects, and open bite. Orofacial abnormalities are present in all subtypes of MPS, ML II, and III. As orofacial abnormalities may cause complaints, evaluation of orofacial health should be part of routine clinical care.

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Oral manifestations in children with mucopolysaccharidosis

Cited by 4 publications

References 26 publications

Manifestations of lysosomal storage diseases in the stomatognathic system – a literature review

Manifestations of lysosomal storage diseases in the stomatognathic system – a literature review

Inborn errors of metabolism and their impact in paediatric dentistry

Orofacial abnormalities in mucopolysaccharidosis and mucolipidosis typeIIandIII: A systematic review

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