scholarly journals Multiple Sulfatase Deficiency (MSD): Review of the Literature and Case Reports of Two Siblings with Dental Caries and Trauma

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Lorna Hirst ◽  
Gehan Abou-Ameira ◽  
Mari-Liis Uudelepp

Multiple sulfatase deficiency (MSD) (MIM # 272200) is an extraordinarily rare inborn error of metabolism (IEM). The phenotypic spectrum is largely heterogeneous and attributed to the combined effects of deficiencies in the nine sulfatases currently known to be related to human diseases. Systemic sequelae of MSD are vast and multisystemic, primarily encompassing developmental delay and neurological, cardiopulmonary, dermatological, gastroenterological, and skeletal manifestations. The dental phenotype is scarcely described in the literature due to a paucity of cases. Dental treatment under local and general anaesthesia mandates an integrated multidisciplinary approach to safeguard systemic health and optimise outcomes. This paper presents two siblings with multiple sulfatase deficiency who presented to the Paediatric Dental Department at Great Ormond Street Hospital, requiring comprehensive care under general anaesthesia for dental caries and trauma.

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Lorna Hirst ◽  
Gehan Abou-Ameira ◽  
Simon Critchlow

Raine Syndrome (RS) also referred to as lethal osteosclerotic bone dysplasia describes an exceptionally rare autosomal recessive disorder with an estimated prevalence of <1 in 1,000,000. Endocrinological manifestations such as hypophosphataemic rickets depict a recent finding within the phenotypic spectrum of nonlethal RS. The dental sequelae of hypophosphataemic rickets are significant. Spontaneous recurrent abscesses on noncarious teeth result in significant odontogenic pain and multiple dental interventions. The dental presentations of nonlethal RS are less widely described within the literature. Amelogenesis Imperfecta (AI), however, was recently postulated as a key characteristic. This article presents the dental manifestations and extensive restorative and oral surgical intervention of three siblings with hypophosphataemic rickets secondary to Raine Syndrome treated at Great Ormond Street Hospital for Children, a tertiary referral hospital.


Author(s):  
Marie-Laure Munoz-Sanchez ◽  
Natacha Linas ◽  
Nicolas Decerle ◽  
Valérie Collado ◽  
Denise Faulks ◽  
...  

Evidence-based evaluations of dental treatment are needed to support the development of special care dentistry services. This retrospective study was designed to collect and analyse X-ray images of permanent teeth restored with stainless steel crowns (SSC) in patients treated under general anaesthesia. Between 2013 and 2019, 360 permanent molars were crowned with SSCs in 198 adult patients. One calibrated investigator used an original validated tool to evaluate four radiographic criteria for molars restored with SSCs: i) marginal adaptation; ii) interdental proximal contact; iii) the presence of glass ionomer cement overflow; and iv) the loss of alveolar bone. Overall, no defect or a minor defect was reported for the majority of SSCs for the criteria “Marginal adaptation” (62.5%, n = 320), “Proximal contact” (82.2%, n = 236) and “Cement overflow” (95.8%, n = 337). Alveolar bone resorption was reported in 8.3% of cases, n = 14, after a mean period of 8.9 ± 14.3 months. It was shown that the restoration of permanent teeth using SSCs placed under general anaesthesia presents a low risk of periodontal morbidity in the medium term when assessed radiographically.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hosam Alraqiq ◽  
Ahmid Eddali ◽  
Reema Boufis

Abstract Background In many developing countries, the prevalence of dental caries has increased due to lifestyle changes, lack of preventive services, and inadequate access to dental care. In Arab countries, the increased prevalence of caries has correlated with economic growth over the past decades, resulting in greater access to unhealthy foods and higher consumption of sugar, particularly among children. However, few studies have assessed caries prevalence among pediatric populations in Arab countries. The objective of this study was to assess the prevalence of dental caries and factors associated with caries among children in Tripoli, Libya. Methods This cross-sectional study included a convenience sample of 1934 children in first grade (age 6–7 years, n = 1000) and seventh grade (age 11–12 years, n = 934). Four health centers in Tripoli were selected for screening based on location and participation in school-entry health examinations. Data were collected through self-administered parent surveys and visual dental screenings by trained examiners from September 24 to October 15, 2019. The survey comprised questions about socioeconomic characteristics and oral health behaviors, including toothbrushing, sugar consumption, and dental care history. During screenings, untreated decay, missing teeth, and filled teeth (DMFT or dmft) were recorded. Prevalence of tooth decay was calculated as the proportion of children with high DMFT/dmft scores. Binary logistic and negative binomial regression analyses (with significance at p ≤ 0.05) were used to assess factors associated with caries. Results Among 1000 first-grade children, 78.0% had decay in their primary teeth, with a mean dmft of 3.7. Among 934 seventh-grade children, 48.2% had caries in their permanent teeth, with a mean DMFT of 1.7. The most significant factors associated with caries prevalence were socioeconomic, such as screening site (first grade, p = 0.02; seventh grade, p < 0.001) and maternal employment (seventh grade, p = 0.02), and behavioral, such as toothbrushing duration (seventh grade, p = 0.01), past dental treatment (both grades, p < 0.001), and past emergency visit (both grades, p < 0.001). Conclusions Caries prevalence was associated with several behavioral and socioeconomic factors, including screening site, maternal employment, toothbrushing duration, past dental treatment, and past emergency visit. Efforts should be made to address these factors to minimize barriers and improve oral health behavior and care utilization. These findings can be used to evaluate current public health initiatives and inform future planning.


Dental Update ◽  
2021 ◽  
Vol 48 (4) ◽  
pp. 266-270
Author(s):  
Niraj Halai ◽  
Shash Bhakta

The term double tooth has often replaced the clinical diagnosis of gemination or fusion. If teeth have been extracted or exfoliated, the use of the neutral term ‘double tooth’ avoids the need to arbitrarily decide if it was gemination or fusion in origin. This case reports on a 16-year-old male who presented with an UL2 ‘double tooth’ and his combined restorative, surgical and orthodontic intervention to achieve his ideal result. CPD/Clinical Relevance: A multidisciplinary approach should be adopted when treating patients with a double tooth and an appropriate protocol as cited can be used.


Dental Update ◽  
2021 ◽  
Vol 48 (4) ◽  
pp. 302-306
Author(s):  
Jessica Hamilton ◽  
Mary Gittins ◽  
Andrew Geddis-Regan ◽  
Graham Walton

As the overweight and obese population increases, one must be mindful of the implications on the delivery of dental care to this group. Appropriate facilities must be available, which may warrant structural and equipment adaptations to clinical and non-clinical areas. The complexity of dental treatment planning and delivery may be compounded by medical comorbidities, and careful consideration must be given to the suitability and safety of conscious sedation and general anaesthesia in order to facilitate treatment. This article aims to discuss how safe provision and equitable access to dental care can be achieved for the bariatric population. CPD/Clinical Relevance: This article aims to discuss the challenges posed by the increase in the overweight and obese population and considerations to be taken for provision of safe and equitable bariatric dental care.


Author(s):  
Sara Cooke ◽  
Sara Warraich ◽  
Jeroen Poisson ◽  
Simon Blackburn ◽  
Abhimanu Lall

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