Tumor necrosis factor receptor-associated factor 3 (TRAF3) is an anti-inflammatory molecule that negatively regulates the non-canonical nuclear factor-κB pathway. Although TRAF3 haploinsufficiency (TRAF3 HI) can influence innate and adaptive immune cells, its effect on inflammatory bowel disease (IBD) development remains unclear. Here, we report the first case of severe early-onset IBD with a novel TRAF3 variant leading to HI, successfully treated with ustekinumab. A 6-year-old girl with a recurrent parotitis, otitis media, tonsilitis, and atopic dermatitis developed IBD involving the stomach, small intestine, and colon. At diagnosis, the immunoglobulin (Ig)G and IgA levels were relatively high, and lymphocyte subsets showed increased counts of plasmablasts, class-switch recombination B cells, and circulating T-follicular helper cells. Treatment with azathioprine and infliximab failed to maintain remission marked by several relapses accompanied by erythema nodosum and arthritis; however, ustekinumab, an anti-interleukin (IL)-12/23p40 antibody, led to long-term clinical remission, normalizing the Ig level and reducing abnormal lymphocyte counts. Whole-exome sequencing revealed a novel heterozygous mutation in TRAF3 [p.(Pro487Leufs*8)], resulting in TRAF3 under-expression. Our case may highlight the contribution of TRAF3 HI to the development of IBD and provide insights into IBD pathophysiology, suggesting the involvement of the IL-12/23-T-follicular helper cell pathway affected by genetic mutations.

Purpose: This pilot study was conducted to evaluate half-value layer (HVL) measurements obtained using a semi conductor dosimeter for intraoral radiography. Materials and Methods: This study included 8 aluminum plates, 4 of which were low-purity (less than 99.9%) and 4 high-purity (greater than 99.9%). Intraoral radiography was performed using an intraoral X-ray unit in accordance with the dental protocol at the authors’ affiliated hospital: tube voltage, 60 kVp and 70 kVp; tube current, 7 mA;and exposure time, 0.10 s. The accuracy of HVL measurements for intraoral radiography was assessed using a semiconductor dosimeter. A simple regression analysis was performed to compare the aluminum plate thickness and HVL in relation to the tube voltage (60 kVp and 70 kVp) and aluminum purity (low and high). Results: For the low-purity aluminum plates, the HVL at 60 kVp (Y) and 70 kVp (Y) was significantly correlated with the thickness of the aluminum plate (X), with Y = 1.708 + 0.415X (r = 0.999, P<0.05) and Y = 1.980 + 0.484X (r = 0.999, P<0.05), respectively. Similarly, for the high-purity aluminum plates, the HVL at 60 kVp (Y) and 70 kVp (Y) was significantly correlated with the plate thickness (X), with Y = 1.696 + 0.454X (r = 0.999, P<0.05) and Y = 1.968 + 0.515X (r = 0.998, P<0.05), respectively. Conclusion This pilot study examined the relationship between aluminum plate thickness and HVL measurements using a semiconductor dosimeter for intraoral radiography. Semiconductor dosimeters may prove useful in HVL measurement for purposes such as quality assurance in dental X-ray imaging.

Purpose: The purpose of this study was to evaluate the cone-beam computed tomographic (CBCT) imaging and histopathological characteristics of osteoradionecrosis (ORN) and medication-related osteonecrosis of the jaw (MRONJ). Materials and Methods: Ten surgical specimens from segmental mandibulectomy (3 ORN and 7 MRONJ) were analyzed using CBCT. The CBCT parameters were as follows: high-resolution mode (tube voltage, 90.0 kV; tube current, 4.00 mA; rotation time, 16.8 s; field of view, 56 mm×56 mm; thickness, 0.099 mm). Histopathological characteristics were evaluated using histological slides of the surgical specimens. The Pearson chi-square test was used to compare ORN and MRONJ in terms of CBCT findings (internal texture, sequestrum, periosteal reaction and cortical perforation) and histopathological characteristics (necrotic bone, inflammatory cells, reactive bone formation, bacteria, Actinomyces, and osteoclasts). A P value less than 0.05 was considered to indicate statistical significance. Results: MRONJ showed periosteal reaction on CBCT more frequently than ORN (7 of 7 [100%] vs. 0 of 3 [0%], P<0.05). Regarding histopathological characteristics, MRONJ showed osteoclasts more frequently than ORN (6 of 7 [85.7%] vs. 0 of 3 [0%], P<0.05). Conclusion This study evaluated the CBCT imaging and histopathological characteristics of ORN and MRONJ, and the findings suggest that CBCT could be useful for the evaluation of ORN and MRONJ.

Computed tomography (CT) and magnetic resonance imaging (MRI) can be useful for the evaluation of palatal lesions, and strain elastography (performed together with intraoral ultrasonography) is a relatively new sonographic imaging modality. This report describes 2 clinical cases in which strain elastography was used to assess palatal tumors in conjunction with intraoral ultrasonography, CT, and MRI. In the first case, diagnosed as a myoepithelioma, the strain was determined to be 0.000% (strain of normal tissue, 0.556%). In the second case, diagnosed as an adenoid cystic carcinoma, the determined strain was 0.000% (strain of normal tissue, 1.077%). Therefore, we conclude that intraoral strain elastography can be useful for evaluating palatal lesions.

PURPOSE: The purpose of this study was to evaluate the morphology of the mandibular cortex in cases of medication-related osteonecrosis of the jaw (MRONJ) in patients with osteoporosis or bone metastases using a computer programme.MATERIALS AND METHODS: Fifty-four patients with MRONJ (35 with osteoporosis and 19 with bone metastases) were examined using panoramic radiography. The morphology of the mandibular cortex was evaluated using a computer programme that scanned the mandibular inferior cortex and automatically assessed the mandibular cortical index (MCI) according to the thickness and roughness of the mandibular cortex, as follows: normal (class 1), mildly to moderately eroded (class 2), or severely eroded (class 3). The MCI classifications of MRONJ patients with osteoporosis or bone metastases were evaluated with the Pearson chi-square test. In these analyses, a 5% significance level was used.RESULTS: The MCI of MRONJ patients with osteoporosis (class 1: 6, class 2: 15, class 3: 14) tended to be higher than that of patients with bone metastases (class 1: 14, class 2: 5, class 3: 0) (P=0.000).CONCLUSION: The use of a computer programme to assess mandibular cortex morphology may be an effective technique for the objective and quantitative evaluation of the MCI in MRONJ patients with osteoporosis or bone metastases.
Bisphosphonate-Associated Osteonecrosis of the Jaw ; Classification ; Cytochrome P-450 CYP1A1 ; Evaluation Studies as Topic ; Humans ; Image Processing, Computer-Assisted ; Jaw ; Neoplasm Metastasis ; Osteonecrosis ; Osteoporosis ; Radiography, Panoramic

PURPOSE: To present characteristic findings of Tc-99m hydroxymethylene diphosphonate (HMDP) scintigraphy, computed tomography (CT), and magnetic resonance (MR) imaging for osteonecrosis in the mandible, especially osteoradionecrosis (ORN) and medication-related osteonecrosis of the jaw (MRONJ). MATERIALS AND METHODS: Thirteen patients with MRONJ and 7 patients with ORN in the mandible underwent Tc-99m HMDP scintigraphy, CT, and MR imaging (T1-weighted images [T1WI], T2-weighted images [T2WI], short inversion time inversion recovery images [STIR]), diffusion-weighted images [DWI], and apparent diffusion coefficient [ADC] mapping). The associations of scintigraphy, CT, and MR imaging findings with MRONJ and ORN were analyzed using the chi-square test with the Pearson exact test. RESULTS: Thirteen patients with MRONJ and 7 patients with ORN in the mandible showed low signal intensity on T1WI and ADC mapping, high signal intensity on STIR and DWI, and increased uptake on scintigraphy. Periosteal bone proliferation on CT was observed in 69.2% of patients with MRONJ (9 of 13) versus 14.3% of patients with ORN (1 of 7) (P=0.019). CONCLUSION: This study presented characteristic imaging findings of MRONJ and ORN on scintigraphy, CT, and MR imaging. Our results suggest that CT can be effective for detecting MRONJ and ORN.
Diffusion ; Humans ; Jaw ; Magnetic Resonance Imaging ; Mandible ; Osteonecrosis ; Osteoradionecrosis ; Radionuclide Imaging ; Tomography, X-Ray Computed

PURPOSE: The aim of this study was to evaluate the quantitative strain elastography of tongue carcinoma using intraoral ultrasonography. MATERIALS AND METHODS: Two patients with squamous cell carcinoma (SCC) who underwent quantitative strain elastography for the diagnosis of tongue lesions using intraoral ultrasonography were included in this prospective study. Strain elastography was performed using a linear 14 MHz transducer (Aplio 300; Canon Medical Systems, Otawara, Japan). Manual light compression and decompression of the tongue by the transducer was performed to achieve optimal and consistent color coding. The variation in tissue strain over time caused by the compression exerted using the probe was displayed as a strain graph. The integrated strain elastography software allowed the operator to place circular regions of interest (ROIs) of various diameters within the elastography window, and automatically displayed quantitative strain (%) for each ROI. Quantitative indices of the strain (%) were measured for normal tissues and lesions in the tongue. RESULTS: The average strain of normal tissue and tongue SCC in a 50-year-old man was 1.468% and 0.000%, respectively. The average strain of normal tissue and tongue SCC in a 59-year-old man was 1.007% and 0.000%, respectively. CONCLUSION: We investigated the quantitative strain elastography of tongue carcinoma using intraoral ultrasonography. Strain elastography using intraoral ultrasonography is a promising technique for characterizing and differentiating normal tissues and SCC in the tongue.
Carcinoma, Squamous Cell ; Clinical Coding ; Decompression ; Diagnosis ; Elasticity Imaging Techniques ; Humans ; Middle Aged ; Prospective Studies ; Tongue Neoplasms ; Tongue ; Transducers ; Ultrasonography

PURPOSE: To evaluate the usefulness of shear wave elastography in the diagnosis of oral and maxillofacial diseases. MATERIALS AND METHODS: Ten patients with oral and maxillofacial diseases and 28 volunteers drawn from our student doctors were examined by shear wave elastography with a 14-MHz linear transducer using an Aplio 300 apparatus (Canon Medical Systems, Otawara, Japan). A statistical analysis of the shear elastic modulus (kPa) of healthy tissue (the sublingual gland, submandibular gland, anterior belly of the digastric muscle, and geniohyoid muscle) in the 28 volunteers was performed using 1-way repeated measures analysis of variance with the Tukey honest significant difference test. The maximum shear elastic modulus (kPa) of 8 patients with squamous cell carcinoma (SCC) and 2 patients with benign lesions was evaluated with the Mann-Whitney U test. The analysis used a 5% significance level. RESULTS: The mean shear elastic modulus of the sublingual gland (9.4±3.7 kPa) was lower than that of the geniohyoid muscle (19.2±9.2 kPa, P=.000) and the anterior belly of the digastric muscle (15.3±6.1 kPa, P=.004). The maximum shear elastic modulus of the SCCs (109.6±14.4 kPa) was higher than that of the benign lesions (46.4±26.8 kPa, P=.044). CONCLUSION: Our results demonstrated the usefulness of shear wave elastography in the diagnosis of oral and maxillofacial diseases. Shear wave elastography has the potential to be an effective technique for the objective and quantitative diagnosis of oral and maxillofacial diseases.
Carcinoma, Squamous Cell ; Diagnosis ; Elastic Modulus ; Elasticity Imaging Techniques ; Humans ; Neck ; Sublingual Gland ; Submandibular Gland ; Transducers ; Ultrasonography ; Volunteers

Central giant cell lesions are rare, benign, osteolytic, pseudocystic, solitary, localized lesions that are common in the skeletal structure, but less so in the maxillofacial region. Furthermore, to perform panoramic radiography and cone-beam computed tomography, it is necessary to prepare patients properly and to position their heads carefully. However, this can be difficult in pediatric patients, who may be anxious. In this report, we describe the case of a central giant cell lesion of the mandible in a 2-year-old girl that was evaluated with multidetector computed tomography.
Child ; Child, Preschool ; Cone-Beam Computed Tomography ; Female* ; Giant Cells* ; Granuloma, Giant Cell ; Head ; Humans ; Mandible* ; Multidetector Computed Tomography ; Radiography, Panoramic

PURPOSE: Sialolithiasis is one of the most prevalent large obstructive disorders of the submandibular glands. The aim of this study was to investigate submandibular sialolithiasis with computed tomography (CT) and scintigraphy, with a particular focus on the relationship between CT values of the submandibular glands and their excretion rate. MATERIALS AND METHODS: Fifteen patients with submandibular sialolithiasis who underwent CT and salivary gland scintigraphy were included in this study. The relationship between the CT values of submandibular glands with and without sialoliths and salivary gland excretion measured using salivary gland scintigraphy was statistically analyzed. Dynamic images were recorded on the computer at 1 frame per 20 seconds. The salivary gland excretion fraction was defined as A (before stimulation test [counts/frame]) / B(after stimulation test [counts/frame]) using time-activity curves. RESULTS: The CT values in the submandibular glands with and without sialoliths was 9.9±44.9 Hounsfield units (HU) and 34.2±21.8 HU, respectively (P=.233). Regarding the salivary gland excretion fraction using scintigraphy, the A/B value in the submandibular glands with sialoliths (1.09±0.23) was significantly lower than in the submandibular glands without sialoliths (1.99±0.57, P=.000). CONCLUSION: Assessments of the CT values and the salivary gland excretion fraction using scintigraphy in the submandibular glands seem to be useful tools evaluating submandibular sialolithiasis.
Gamma Cameras ; Humans ; Multidetector Computed Tomography ; Radionuclide Imaging* ; Salivary Duct Calculi ; Salivary Gland Calculi* ; Salivary Glands* ; Submandibular Gland*