Jaw bones’ involvement and dental features of type I and type III Gaucher disease: a radiographic study of 42 paediatric patients

Mohamed, Yomna S.A.; Zayet, Mohamed Khalifa; Omar, Ola; El‐Beshlawy, Amal

doi:10.1007/s40368-019-00471-3

Cited by 5 publications

(22 citation statements)

References 25 publications

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“…Pseudocystic RL lesions were identified in only 4 patients according to our study findings. This finding was consistent with the result of the study by Mohamed et al [38], in which they evaluated Type I and Type III GD patients and found that pseudocystic lesions were less common in Type I. Contrary to our findings, Saranjam et al [25] and several other researchers [15,20,[42][43][44] reported that the most common radiographic finding in an affected mandible was pseudocystic or honeycomb-shaped RL lesions, mostly in the premolar-molar regions, and that increased osteopenia and trabecular structure loss were frequently observed in these RL areas.…”

Section: Discussionsupporting

confidence: 94%

“…The study conducted by Mohamed et al [38] on the development of teeth in GD, investigated dental age and chronological age, but no significant difference was found. Carter et al [26], in their study in which they presented 28 GD patients, reported that the chronological development of permanent teeth was affected in 5 of the 9 patients under the age of 20.…”

Section: Discussionmentioning

confidence: 96%

“…Some studies have reported the use of bisphosphonates as supportive therapy for ERT in osteopenia/osteonecrosis, as they promote the proliferation and differentiation of osteoblast-like cells [38,39]. Bisphosphonates are known to inhibit osteoclastic bone resorption.…”

Section: Discussionmentioning

confidence: 99%

“…The underlying pathology of many bone diseases is thought to be related to the accumulation of Gaucher cells. Gaucher cells lead to direct or indirect local bone defects such as cortical thinning, osteonecrosis, lytic lesions, and effacement of surrounding anatomical structures by infiltrating the bone marrow and even into the apical region of the teeth [11,38].…”

Section: Discussionmentioning

confidence: 99%

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Clinical and radiological evaluation of dentomaxillofacial involvement in Type I Gaucher disease

et al. 2021

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Objectives This study aimed to evaluate the oral findings and dentomaxillofacial involvement of patients diagnosed with Type I Gaucher Disease (GD), and to compare these findings with the control group. Methods A total of 32 patients, 5 males, and 11 females in both the study (range 19-60, mean age 34.56) and control group (range 18-49, mean age 34.63) were included in the study. The file records of all patients obtained in the first evaluation were examined, and the hematological, visceral, and bone examination findings were recorded. Subsequently, oral examinations were performed for oral findings and dentomaxillofacial involvement, and radiological examinations were performed in panoramic images and cone-beam computed tomography (CBCT) images. Results There was mandible involvement in 9 (56.3%), and involvement in both jaws in 4 (25.0%) of the patients with Type I GD. According to the CBCT findings, generalized rarefaction in 10 (62.5%) patients, enlarged marrow spaces in 12 (75.0%) patients, thinning of the lamina dura in 10 (62.5%) patients, and loss in the cortical borders of the mandibular canal in 7 (43.8%) patients were among the common radiographic findings. When the control and study group were compared, in panoramic images, a significant difference was found in terms of generalized rarefaction and enlarged marrow spaces, while significant differences were found in CBCT images regarding generalized rarefaction, enlarged marrow spaces, loss in the cortical margins of the mandibular canal and widening in the periodontal ligament space. Conclusions The roadmap for the diagnosis of Gaucher lesions in the jawbones includes a comprehensive medical history and a clinical and radiological examination. In conclusion, dentists should be familiar with the dentomaxillofacial findings of GD and be aware of possible oral and dental complications that may develop.

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Section: Discussionsupporting

confidence: 94%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical and radiological evaluation of dentomaxillofacial involvement in Type I Gaucher disease

et al. 2021

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“…In types 1 and 3 patients have painful crises of bones due to the concentration of Gaucher's cells at the bone marrow provoking localized bone lesions. The main oral manifestations are pseudo cystic radiolucent lesions, thinning of cortex, median rhomboid glossitis, xerostomia anodontia and dental anomalies [60][61][62] .…”

Section: Gaucher Diseasementioning

confidence: 99%

A dental approach to hereditary and congenial metabolic bone diseases

Rallatou¹,

Sakka²,

Zafeiris³

2023

JRPMS

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Bone growth requires a balance of minerals such as calcium, phosphorus, and factors of bone biomineralization. The difference between jaws' bones and the rest of the bones is the existence of teeth. Although the development of teeth has no similarities with bones, the biomineralization of both is happening at the same time. Consequently, the disturbances in the metabolism of minerals and peptides which participate in osteogenesis, have an impact on teeth, jaws, periodontal tissues, and oral mucosa. The hereditary and congenial metabolic bone diseases are being analysed and classified based on their genetic profile and oral manifestations. Regarding the categories, there are metabolic bone diseases caused by genetic mutations, like hereditary rickets, and others caused by chromosomal abnormalities, such as Down syndrome. In addition, many disorders are provoked by enzymatic dysfunction, disturbances of creation of polypeptide chain, while others have an unknown explanation. Many oral manifestations are related with problems in the dentition, oral mucosa, periodontal tissues, and jaws. Especially some syndromes present with cleft lip and palate. To conclude, there is a big variety of dental symptoms of these disorders, while the most frequent are the delayed eruption of teeth, oligodontia, a dysplastic enamel and dentine.

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Jaw involvement in Gaucher disease: a not-so-uncommon feature of a rare disease

2021

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Gaucher disease is an inborn error of metabolism resulting from the deficiency of the enzyme glucocerebrosidase and consequent accumulation of glucocerebroside within the lysosomes of macrophages. The clinical presentation is very diverse, depending on the age of onset and the severity of the disease, and results from the progressive infiltration of lipid-laden cells in various organs. Common manifestations of Gaucher disease include enlarged liver and/or spleen (hepatosplenomegaly), bone marrow disease (pancytopenia) and bone abnormalities, which are extremely variable and can affect multiple skeletal sites. While bone involvement of long bones and vertebrae is a well-recognised feature of Gaucher disease, jawbone involvement is less commonly noted. Here, we describe a case of a 63-year-old patient with type 1 Gaucher disease with a history of long-term use of bisphosphonates and who had presented with dental pain, with subsequent investigations confirming the radiological features of jaw involvement in Gaucher disease, including periodontal disease.

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Jaw bones’ involvement and dental features of type I and type III Gaucher disease: a radiographic study of 42 paediatric patients

Cited by 5 publications

References 25 publications

Clinical and radiological evaluation of dentomaxillofacial involvement in Type I Gaucher disease

Clinical and radiological evaluation of dentomaxillofacial involvement in Type I Gaucher disease

A dental approach to hereditary and congenial metabolic bone diseases

Jaw involvement in Gaucher disease: a not-so-uncommon feature of a rare disease

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