A combination of skeletal deformations of the dorsal mandible and temporomandibular region detected in orthopantomograms of patients with neurofibromatosis type 1 indicates an associated ipsilateral plexiform neurofibroma

Friedrich, Reinhard E; Reul, Anika

doi:10.1016/j.jcms.2018.04.018

Cited by 10 publications

(20 citation statements)

References 62 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…PNF of the trigeminal nerve often leads to characteristic deformations of the jaw. The enlarged mandibular foramen is a radiologically conspicuous sign of a dorsal differentiation disorder through an adjacent PNF (36), as is the relatively frequently reported enlargement of the mandibular canal (37). However, the development of teeth in the orofacial PNF region is often disturbed in that the mesial migration of certain permanent teeth is incomplete or completely absent and the roots of the teeth are deformed due to the lack of bony space, but the visible structure of the tooth is not impaired (27).…”

Section: Discussionmentioning

confidence: 99%

“…NF1 is a disease with a plethora of signs and symptoms. For example, bony changes (36) are just as much a part of the spectrum of the syndrome as the often-plentiful accumulation of fat cells in body regions affected by diffuse in vivo 37: 548-558 (2023) neurofibromas (46). Basic research is of great importance for understanding the cellular and molecular basis of the disease and develop therapeutic strategies, for example in skeletal regeneration.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Characterization of Dental Pulp Stem Cell Populations in the Teeth of Patients With Neurofibromatosis Type 1 - Therapeutic Potential for Bone Tissue Engineering

Yan

Wang

Speth

et al. 2023

In Vivo

View full text Add to dashboard Cite

Background/Aim: Neurofibromas (NF) are the most common benign nerve sheath tumors in the tongue, gingiva, major salivary glands, and jaw bones. Nowadays, tissue engineering is a revolutionary technique for reconstructing tissues. To explore the feasibility of using stem cells derived from NF teeth to treat orofacial bone defects, the differences in cell biological properties between an NF teeth group and Normal teeth group. Patients and Methods: The intra-dental pulp tissues from each tooth were extracted. The cell survival rates, morphology, proliferation rates, cell activity, and differentiation abilities were contrastively analyzed between the NF teeth group and Normal teeth group. Results: Between the two groups, there were no differences in the primary generation (P0) cells (p>0.05), the cell yield, and the time required for the cells to grow out of the pulp tissue and attach to the culture plate. Furthermore, no differences were found at the first generation (passage) between the two groups in colony formation rate and cell survival rate. The proliferation capacity, cell growth curve, and surface marker expression of dental pulp cells was not altered in the third generation (p>0.05). Conclusion: Dental pulp stem cells from NF teeth were successfully obtained and were not different from normal dental pulp stem cells. Although, clinical research using tissue-engineered bone to repair bone defects is still in its infancy, it will eventually enter the clinic and become a routine means of bone defect reconstruction treatment as related disciplines and technologies develop.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Characterization of Dental Pulp Stem Cell Populations in the Teeth of Patients With Neurofibromatosis Type 1 - Therapeutic Potential for Bone Tissue Engineering

Yan

Wang

Speth

et al. 2023

In Vivo

View full text Add to dashboard Cite

show abstract

“…Neurofibromatosis is a complex group of syndromes which occurs due to the inactivation of the various tumour suppressor genes precipitating various complications in the body [ 1 , 2 ]. Classified into eight types, the most common type of neurofibromatosis that accounts for 90% of all cases is neurofibromatosis type 1 (NF1), which is underlain by germline mutations in the NF1 gene (locus 17q11.2) [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…The NF1 gene is ubiquitously expressed in all cells of the body, but the phenotypic expression differs from cell to cell and within a cell through different developmental stages of life [ 1 , 2 ]. Hence, apart from tumours involving the nervous system, NF1 is associated with cutaneous angiomas, subcuta neous leiomyomas, carcinoid tumours, and pheochromocytoma, as well as auditory, visionary, cognitive, sleep [ 3 ], musculoskeletal, endocrinal and cardiovascular complications [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

“…Due to its rarity, there has been a lack of studies reporting the oral complications of NF1, with findings of these works including an asymmetrical enlarged face with multiple cutaneous/disseminated and plexiform neurofibromas [ 1 , 2 , 8 – 10 , 13 , 14 ]. In addition to sphenoid and orbital bone dysplasia, mandibular bony changes such as deformities of the mandibular ramus and glenoid fossa (56%), condylar head (50%), increased dimensions of the coronoid notch, decreased jaw angle, and enlarged mandibular canals (25%) have been noted [ 1 , 2 , 8 – 10 , 13 , 14 ]. These changes are accompanied by facial plexiform neurofibromas along the trigeminal nerve that can cause cephalometric alterations.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Age-dependent oral manifestations of neurofibromatosis type 1: a case–control study

Thota

Veeravalli

Manchala

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

Introduction Most craniofacial manifestations of neurofibromatosis type 1 (NF1) are considered as a result of tumor compression. We sought to determine salivary changes, caries, and periodontal complications in NF1 patients without tumors in the oral cavity. Objective and methods Eleven NF1 patients without tumors in the oral cavity and 29 matched controls without NF1 were enrolled in this case–control study. Demographic information, medical history, and data of intraoral examinations, including the Decayed, Missing, and Filled Teeth (DMFT) scores and Russel’s periodontal index (PI), were recorded. The functional salivary analysis was performed for sialometry, salivary pH values, and amylase activity. Ingenuity Systems Pathway Analysis (IPA) was conducted to identify mutually activated pathways for NF1-associated oral complications. Results NF1 patients were associated with periodontitis (OR = 1.40, 95% CI = 1.06–1.73, P = 0.04), gingivitis (OR = 1.55, 95% CI = 1.09–2.01, P = 0.0002), and decreased salivary flow rates (OR = 1.40, 95% CI = 1.05–1.76, P = 0.005). Periodontal destruction, salivary changes, and dental caries in NF1 patients were age-dependent. Subgroup analyses based on age stratification suggested that salivary flow rates and salivary amylase activities were significantly low in NF1 patients aged over 20 years and that salivary pH values, PI and DMFT scores were significantly high among NF1- controls aged over 20. All oral complications were not significantly presented in NF1 patients aged below 20 years. IPA analyses suggested that cellular mechanisms underlying NF1-associated oral complications involved chronic inflammatory pathways and fibrosis signaling pathway. Conclusion NF1 patients without tumors in the oral cavity presented a comparatively high prevalence of age-dependent oral complications, including periodontal destruction and salivary gland dysfunction, which were associated with chronic inflammatory pathogenesis.

show abstract

Evaluation of the dimensions, morphology, and position of the mandibular condyles in individuals with neurofibromatosis 1: a case-control study

et al. 2021

View full text Add to dashboard Cite

A combination of skeletal deformations of the dorsal mandible and temporomandibular region detected in orthopantomograms of patients with neurofibromatosis type 1 indicates an associated ipsilateral plexiform neurofibroma

Cited by 10 publications

References 62 publications

Characterization of Dental Pulp Stem Cell Populations in the Teeth of Patients With Neurofibromatosis Type 1 - Therapeutic Potential for Bone Tissue Engineering

Characterization of Dental Pulp Stem Cell Populations in the Teeth of Patients With Neurofibromatosis Type 1 - Therapeutic Potential for Bone Tissue Engineering

Age-dependent oral manifestations of neurofibromatosis type 1: a case–control study

Evaluation of the dimensions, morphology, and position of the mandibular condyles in individuals with neurofibromatosis 1: a case-control study

Contact Info

Product

Resources

About