This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

Methyl salicylate is widely used in various topical products, including sports creams, ointments, patches, and oral hygiene products. These products are mainly used for localized treatment of musculoskeletal pain. Given their intended topical application, their ingestion can result in salicylic acid poisoning due to their high concentrations of methyl salicylate. Symptoms of salicylic acid poisoning may include dizziness, vomiting, hallucinations, seizures, and, in severe cases, unconsciousness, respiratory failure, and circulatory disorders. We report a case of a 71-year-old male who ingested Mensolatum Lotion to commit suicide and died.

The purpose of this study was to observe the surface morphology and roughness of micro-hybrid and nano-filled resin composites and compare wear resistance by conducting a toothbrushing wear test with toothpastes with different abrasive ingredients. Two types of resin composites containing micro-hybrid fillers (Z100 Restorative, Filtek Z250) and one type of resin composite containing nanofillers (Filtek Z350 XT) were used. For the toothbrushing wear test, 90 resin composite samples with a diameter of 10 mm and a thickness of 1 mm were prepared. A force of 2 N and 100,000 cycles of brushing were performed using a pin-on-disk wear tester. The toothpastes used in the test were classified into 4 groups according to the abrasive ingredients (hydroxyapatite, calcium carbonate, sodium bicarbonate, and zeolite-M). After the toothbrushing wear test, the surface morphology of the samples was observed using an optical microscope and a scanning electron microscope (SEM), and the surface roughness was measured using atomic force microscopy (AFM). Relatively large filler particles (micro size) protruded from the surface of the micro-hybrid resin composite groups, and small crater-shaped defects were observed. The surface roughness values of the groups that performed the wear test with toothpaste containing zeolite-M were significantly higher than the other groups (P<0.05). The surface roughness value was significantly (P<0.05) highest in the group where the nanofilled resin composite was wear-tested with toothpaste containing zeolite-M. However, regardless of the type of toothpaste, the surface roughness showed low values of less than 0.1 μm. The surface appeared uniform and smooth compared to the surface of micro-hybrid resin composites. Finally, the nano-filled resin composite showed relatively higher wear resistance than the micro-hybrid resin composite. This means that wear resistance during brushing may indicate the durability of the material in the clinic.

Objectives: . Thyroid cancer is the most common endocrine tumor, with rapidly increasing incidence worldwide. However, its transcriptomic characteristics associated with immunological signatures, driver fusions, and recurrence markers remain unclear. We aimed to investigate the transcriptomic characteristics of advanced papillary thyroid cancer. Methods: . This study included 282 papillary thyroid cancer tumor samples and 155 normal samples from Chungnam National University Hospital and Seoul National University Hospital. Transcriptomic quantification was determined by high-throughput RNA sequencing. We investigated the associations of clinical parameters and molecular signatures using RNA sequencing. We validated predictive biomarkers using the Cancer Genome Atlas database. Results: . Through a comparison of differentially expressed genes, gene sets, and pathways in papillary thyroid cancer compared to normal tumor-adjacent tissue, we found increased immune signaling associated with cytokines or T cells and decreased thyroid hormone synthetic pathways. In addition, patients with recurrence presented increased CD8+ T-cell and Th1-cell signatures. Interestingly, we found differentially overexpressed genes related to immune-escape signaling such as CTLA4, IDO1, LAG3, and PDCD1 in advanced papillary thyroid cancer with a low thyroid differentiation score. Fusion analysis showed that the PI3K and mitogen-activated protein kinase (MAPK) signaling pathways were regulated differently according to the RET fusion partner genes (CCDC6 or NCOA4). Finally, we identified HOXD9 as a novel molecular biomarker that predicts the recurrence of thyroid cancer in addition to known risk factors (tumor size, lymph node metastasis, and extrathyroidal extension). Conclusion . We identified a high association with immune-escape signaling in the immune-hot group with aggressive clinical characteristics among Korean thyroid cancer patients. Moreover, RET fusion differentially regulated PI3K and MAPK signaling depending on the partner gene of RET, and HOXD9 was found to be a recurrence marker for advanced papillary thyroid cancer.

Sorafenib is an inhibitor of receptor tyrosine kinases and the rat sarcoma/mitogen-activated protein kinase (RAS/MAPK) pathway that is approved for the treatment of patients with metastatic hepatocellular carcinoma (HCC) and renal cell carcinoma. Sorafenib is known to have various cutaneous adverse effects, including hand-foot reaction, facial and scalp eruption, xerosis, and alopecia1.A 56-year-old man presented with non-painful, nonpruritic psoriasiform lesions that has been present for approximately 1 month (Fig. 1A∼E). Six months prior to presentation, he had been prescribed sorafenib at a daily dosage of 600∼800 mg after diagnosis of HCC with distant metastasis to the lung. A punch biopsy showed psoriasiform dermatitis (Fig. 1F, G). The skin lesions improved gradually after discontinuing sorafenib. However, at 1 month after discontinuation of sorafenib, considering the dose-dependent adverse effect of the medication, the patient resumed sorafenib at 400 mg daily after an oncology consultation. The lesions recurred beginning at 1 week after restarting sorafenib. A clinical diagnosis of sorafenib-associated psoriasiform drug eruption was made. The sorafenib treatment was maintained at 400 mg daily in conjunction with concurrent phototherapy and topical and intralesional corticosteroids for thick erythematous plaques, and intermittent systemic corticosteroid treatment when the cutaneous eruptions flared up.After approximately 2 years of sorafenib treatment, the patient presented with new crusting lesions without any other systemic adverse reactions. Multiple papules and plaques with central hyperkeratotic and crusted papules were present (Fig. 2A∼D). Punch biopsy showed a ‘perforating phenomenon’ (PP) (Fig. 2E∼G). The brownish hyperkeratotic crusts occurred consistently in prolonged psoriasiform plaques and resolved over time (Fig. 2A∼D). The psoriasiform eruptions and delayed-onset PP persisted with continuing sorafenib use (Fig. 2H). Along with a dose-decrease of sorafenib at 400mg daily, he was treated with systemic and topical corticosteroids, intralesional triamcinolone injection and narrowband ultraviolet B therapy. However, the patient showed recurrent cutaneous lesions aggravation upon tapering the dosage of corticosteroid.The psoriasiform lesions improved and then worsened with sorafenib dose change, and the PP featured hyperkeratotic crusts within multiple, long-lasting psoriasiform plaques. This phenomenon might have occurred to eliminate connective tissue or inflammatory material2 and differs from the appearance of transepidermal elimination in previously reported sorafenib-associated acquired perforating dermatosis cases3,4. Transepidermal elimination is a similar process to wound healing2, and considering that our patient had no history of diabetes, renal insufficiency, and trauma, our case might have exhibited the perforating and resolving phenomenon in response to the abnormal psoriasiform drug eruption.The RAS/MAPK cascade that is inhibited by sorafenib could be activated paradoxically; due to its role in antiangiogenesis, this activation results in epidermal disruption. The reduction and suppression of the hepatocyte growth factor-enhanced expression of matrix metalloproteinase induced by sorefenib could influence homeostasis of dermal elastic fibres, resulting in their disruption5. A few cases of psoriasiform drug eruption and PP after administration of sorafenib and other various tyrosine kinase inhibitors have been reported.The PP could represent a manifestation of the resolution of inflammation whereby the psoriasiform hyperplasia and the proliferated dermal tissue might be eliminated via a trans-epidermal route.

Objective: To evaluate the prognostic implications of preoperative magnetic resonance imaging (MRI) features of hepatocellular carcinoma (HCC) with a focus on those with targetoid appearance based on the Liver Imaging Reporting and Data System (LI-RADS), as well as known microvascular invasion (MVI) features. Materials and Methods: This retrospective study included 242 patients (190 male; mean age, 57.1 years) who underwent surgical resection of a single HCC (≤ 5 cm) as well as preoperative gadoxetic acid-enhanced MRI between January 2012 and March 2015. LI-RADS category was assigned, and the LR-M category was further classified into two groups according to rim arterial-phase hyperenhancement (APHE). The imaging features associated with MVI were also assessed. The overall survival (OS), recurrence-free survival (RFS), and their associated factors were evaluated. Results: Among the 242 HCCs, 190 (78.5%), 25 (10.3%), and 27 (11.2%) were classified as LR-4/5, LR-M with rim APHE, and LR-M without rim APHE, respectively. LR-M with rim APHE (vs. LR-4/5; hazard ratio [HR] for OS, 5.48 [p = 0.002]; HR for RFS, 2.09 [p = 0.042]) and tumor size (per cm increase; HR for OS, 6.04 [p = 0.009]; HR for RFS, 1.77 [p = 0.014]) but not MVI imaging features (p > 0.05) were independent factors associated with OS and RFS. Compared to the 5-year OS and RFS rates in the LR-4/5 group (93.9% and 66.8%, respectively), the LR-M with rim APHE group had significantly lower rates (68.0% and 45.8%, respectively, both p < 0.05), while the LR-M without rim APHE group did not significantly differ in the survival rates (91.3% and 80.2%, respectively, both p > 0.05). Conclusion Further classification of LR-M according to the presence of rim APHE may help predict the postoperative prognosis of patients with a single HCC.

Background: and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). Methods: The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. Results: This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. Conclusions The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.