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Background/Aims@#This study applied the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with systemic sclerosis (SSc) and investigated the frequency of overlap syndrome of SSc and AAV (SSc-AAV-OS). @*Methods@#Among the 232 patients diagnosed with SSc, 105 with signs suggestive of small- or medium-vessel vasculitis, which were defined as the present of interstitial lung disease (ILD), peripheral neuropathy, or suspected renal vasculitis, were included in this study and analyzed. @*Results@#Among the 105 SSc patients, the detection rate of ANCA was 19.0%. When the 2022 ACR/EULAR criteria were applied, the frequency of SSc-AAV-OS was 20.0%, which was much higher than 1.7% reported with previous criteria for AAV. ANCA positivity contributed to the reclassification of SSc-AAV-OS more than ANCA negativity in SSc patients with signs suggestive of small- or medium-vessel vasculitis. @*Conclusions@#The frequency of SSc-AAV-OS in SSc patients with signs suggestive of small- or medium-vessel vasculitis at diagnosis was 20.0%. Therefore, we suggest that physicians should perform ANCA tests in SSc patients exhibiting signs suggestive of small- or medium-vessel vasculitis and apply the new criteria for AAV.
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Objective@#The objective of this prospective, observational multicenter study (NCT03264703) was to compare the effectiveness of single conventional disease-modifying anti-rheumatic drug (cDMARD) plus anti-tumor necrosis factor (TNF) therapy versus multiple cDMARD treatments in patients with moderate-to-severe rheumatoid arthritis (RA) following cDMARD failure in the real-world setting in South Korea. @*Methods@#At the treating physicians’ discretion, patients received single cDMARD plus anti-TNF therapy or multiple cDMARDs. Changes from baseline in disease activity score 28-joint count with erythrocyte sedimentation rate (DAS28-ESR), corticosteroid use, and Korean Health Assessment Questionnaire (KHAQ-20) scores were evaluated at 3, 6, and 12 months. @*Results@#Of 207 enrollees, the final analysis included 45 of 73 cDMARD plus anti-TNF and 91 of 134 multiple-cDMARD recipients. There were no significant between-group differences (BGDs) in ANCOVA-adjusted changes from baseline in DAS28-ESR at 3, 6 (primary endpoint), and 12 months (BGDs −0.18, −0.38, and −0.03, respectively). More cDMARD plus anti-TNF than multiple-cDMARD recipients achieved a >50% reduction from baseline in corticosteroid dosage at 12 months (35.7% vs 14.6%; p=0.007). Changes from baseline in KHAQ-20 scores at 3, 6, and 12 months were significantly better with cDMARD plus antiTNF therapy than with multiple cDMARDs (BGD −0.18, −0.19, and −0.19 points, respectively; all p≤0.024). @*Conclusion@#In the real-world setting, relative to multiple cDMARDs, single cDMARD plus anti-TNF therapy significantly improved quality-of-life scores and reduced corticosteroid use, with no significant BGD in disease activity, in RA patients in whom previous cDMARD therapy had failed.
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Adenomatoid odontogenic tumor (AOT) is a rare, asymptomatic, slow-growing benign tumor that can be divided into three variants: follicular, extrafollicular, and peripheral. By treating AOT using an enucleation and curettage approach, recurrence can be avoided. We report a case of a 24-year-old female who presented with a lump in the right mandibular premolar area along with diastema between displaced teeth #43 and #44 and was diagnosed with extrafollicular AOT. The patient was managed with enucleation-curettage surgery without additional bone graft procedure along with routine follow-up. A successful outcome without recurrence was achieved, and diastema closure with repositioning of the displaced teeth did not require orthodontic treatment. AOT should be managed via enucleation and curettage to obtain successful outcomes without recurrence. Spontaneous bone regeneration following enucleation can be achieved without guided bone regeneration. Also, diastema closure and repositioning of displaced teeth can occur without orthodontic interventions through physiologic drift.
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Congenital insensitivity to pain with anhidrosis (CIPA) is an extremely rare disease characterized by insensitivity to pain, anhidrosis, and intellectual disability. CIPA is caused by a genetic mutation in the neurotrophic tyrosine receptor kinase 1 (NTRK1) gene on chromosome 1. The anhidrosis leads to cutaneous changes such as skin dryness, lichenification, and impetiginization. Moreover, patients with CIPA may experience repeated trauma and recalcitrant eczema due to excessive scratching of wounds on their skin, because they do not feel any pain. Severe whole-body eczema in a patient with CIPA may be overlooked, leading these patients to be frequently diagnosed with atopic dermatitis and common eczema.Indeed, in patients with treatment-resistant or atypically distributed eczema and underlying anhidrosis, CIPA should be considered as a potential causative disease. Increased awareness of CIPA among dermatologists is necessary to ensure that patients receive an appropriate diagnosis. Herein, we report a rare case of generalized xerotic eczema in a patient with CIPA.
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The fifth edition of the WHO classification (2022 WHO) and the International Consensus Classification (2022 ICC) of myeloid neoplasms have been recently published. We reviewed the changes in the diagnosis distribution in patients with MDS with excess blasts (MDS-EB) or AML using both classifications. Forty-seven patients previously diagnosed as having AML or MDS-EB with available mutation analysis data, including targeted next-generation and RNA-sequencing data, were included. We reclassified 15 (31.9%) and 27 (57.4%) patients based on the 2022 WHO and 2022 ICC, respectively. One patient was reclassified as having a translocation categorized as a rare recurring translocation in both classifications. Reclassification was mostly due to the addition of mutation-based diagnostic criteria (i.e., AML, myelodysplasia-related) or a new entity associated with TP53 mutation. In both classifications, MDS diagnosis required the confirmation of multi-hit TP53 alterations. Among 14 patients with TP53 mutations, 11 harbored multi-hit TP53 alterations, including four with TP53 mutations and loss of heterozygosity. Adverse prognosis was associated with multi-hit TP53 alterations (P=0.009) in patients with MDS-EB, emphasizing the importance of detecting the mutations at diagnosis. The implementation of these classifications may lead to the identification of different subtypes from previously heterogeneous diagnostic categories based on genetic characteristics.
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Autoimmune hemolytic anemia (AIHA) is a common complication after hematopoietic stem cell transplantation (HSCT). Although post-HSCT AIHA is associated with poor prognosis and is more resistant to treatment than primary AIHA, the standard treatment has not yet been determined. We report a case of post-HSCT AIHA, which occurred 9 years after unrelated donor bone marrow transplantation for acute biphenotypic leukemia. Steroid treatment was initially effective but prolonged steroid usage, due to recurrent relapses, caused avascular necrosis. Although rituximab was unsuccessful in preventing relapses, sirolimus was effective in allowing the discontinuation of steroid treatment and sustaining transfusion-free status. Sirolimus, previously reported to be effective in pediatric post-HSCT AIHA and AIHA after solid organ transplantation, may be effective in adult post-HSCT AIHA as well.
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Purpose@#This study was conducted to update the previous evidence-based nursing clinical practice guidelines for pressure injury. @*Methods@#The guideline was revised in 22 steps based on the international standards. @*Results@#The updated nursing practice guideline for pressure injury consisted of four domains and 494 recommendations. The numbers of recommendations in each domain was: 31 hospital policy, 64 assessment, 386 prevention and management, and 13 education. The recommended grade was 10.3% for A, 13.8% for B, and 75.9% for C. Of these, the major revision was done in 40 recommendations (8.1%). A total of 55 recommendations (11.0%) were newly added. 25 recommendations had minor revisions such as changes or additions for some words, and only one recommendation was deleted. @*Conclusion@#The revised nursing practice guidelines for pressure injury are expected to serve as an evidence-based practice guideline for pressure injury in Korea. This guideline will provide healthcare providers, patients, and caregivers with information to help prevent and manage pressure injuries, leading to improving patient outcomes.
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The treatment strategy for postmenopausal symptoms resulting from estrogen deficiency in breast cancer survivors receiving endocrine therapy should differ from that in normal women. Several nonhormonal pharmacological therapies can be used to treat vasomotor symptoms. Cognitive-behavioral therapy can help alleviate psychophysiological symptoms, including depression and sleep disorders.Topical vaginal estrogen and moisturizers may aid in treating genitourinary symptoms. Additionally, chronic conditions must be individually managed. Prevention of osteoporosis should always be included in the management, and physicians should be alert to possible cardiovascular risk and cognitive function changes.
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Objectives@#Bone mineral density (BMD) is measured in the hip and posteroanterior spine; moreover, according to the 2019 International Society for Clinical Densitometry guidelines, unilateral hip can be used. This study aimed to determine whether there is a difference between the BMD of both the femurs in postmenopausal women. @*Methods@#A total of 343 postmenopausal women were enrolled in this study from January 1, 2010, to December 31, 2019 at a single tertiary hospital. By using the Hologic® Horizon W DXA System, the femur and spine BMD was measured; BMD was recorded in g/cm 2 .Following regions were analyzed in both the femurs: the femur neck, the trochanter area, and total femur. @*Results@#Mean age at imaging was 62 ± 9.7 years, and significant difference in the total BMD of both the femurs (P = 0.003) was observed. In secondary analysis, patients with osteoporosis showed significant contralateral BMD discrepancies in trochanter and total proximal femur BMD (P = 0.041 and P = 0.011, respectively). However, in women with normal BMD, no significant difference between the right and left femur BMD was observed. Furthermore, measurement of solely the unilateral hip can lead to a 16.9% of underdiagnosis in postmenopausal women. @*Conclusions@#In conclusion, it is necessary to check BMD in both hips, particularly in patients suspected of osteoporosis.
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Sirtuins (SIRTs) belong to the nicotinamide adenine dinucleotide (NAD+)-dependent class III histone deacetylase family. They are key regulators of cellular and physiological processes, such as cell survival, senescence, differentiation, DNA damage and stress response, cellular metabolism, and aging. SIRTs also influence carcinogenesis, making them potential targets for anticancer therapeutic strategies. In this study, we investigated the anticancer properties and underlying molecular mechanisms of a novel SIRT1 inhibitor, MHY2251, in human colorectal cancer (CRC) cells. MHY2251 reduced the viability of various human CRC cell lines, especially those with wild-type TP53. MHY2251 inhibited SIRT1 activity and SIRT1/2 protein expression, while promoting p53 acetylation, which is a target of SIRT1 in HCT116 cells. MHY2251 treatment triggered apoptosis in HCT116 cells. It increased the percentage of late apoptotic cells and the sub-G1 fraction (as detected by flow cytometric analysis) and induced DNA fragmentation. In addition, MHY2251 upregulated the expression of FasL and Fas, altered the ratio of Bax/Bcl-2, downregulated the levels of pro-caspase-8, -9, and -3 proteins, and induced subsequent poly(ADP-ribose) polymerase cleavage. The induction of apoptosis by MHY2251 was related to the activation of the caspase cascade, which was significantly attenuated by pre-treatment with ZVAD-FMK, a pan-caspase inhibitor. Furthermore, MHY2251 stimulated the phosphorylation of c-Jun N-terminal kinase (JNK), and MHY2251-triggered apoptosis was blocked by pre-treatment with SP600125, a JNK inhibitor. This finding indicated the specific involvement of JNK in MHY2251-induced apoptosis. MHY2251 shows considerable potential as a therapeutic agent for targeting human CRC via the inhibition of SIRT1 and activation of JNK/p53 pathway.
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Medulloblastoma is the most common embryonal tumor of the central nervous system in childhood.Combined multimodality approaches, including surgery, radiation, and chemotherapy, have improved the outcome of medulloblastoma. Advances in genomic research have shown that medulloblastoma is not a biologically or clinically discrete entity. Previously, the risk was divided according to histology, presence of metastasis, degree of resection, and age at diagnosis. Through the development of integrated genomics, new biology-based risk stratification methods have recently been proposed. It is also important to understand the genetic predisposition of patients with medulloblastoma. Therefore, treatment goal aimed to improve the survival rate with minimal additional adverse effects and reduced longterm sequelae. It is necessary to incorporate genetic findings into the standard of care, and clinical trials that reflect this need to be conducted.
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Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.
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Background@#Management of periprosthetic femoral fractures (PFFs) is reportedly challenging. Different patterns of PFFs would occur based on whether stem fixation was primarily cemented or cementless and whether these patterns would be associated with clinical outcomes, such as subsidence, fracture union, and complications, after stem revision. @*Methods@#A retrospective comparative study was performed, involving 52 PFF patients treated with tapered fluted modular stems (TFMSs). In the 52 patients with Vancouver B2 or B3, including 21 cemented stems and 31 cementless stems, fracture patterns and bone stock were analyzed. Clinical outcomes after revision surgery using the TFMSs were compared between the two groups. @*Results@#Transverse or short oblique type PFFs occurred around the cemented stem with loosening at the bone-cement interface. The Paprosky type III femoral deficiency and Vancouver type B3 fracture were observed more frequently in the cemented stem group. Otherwise, spiral fractures occurred more frequently in the cementless group (p 5 mm was observed more frequently in the cemented stem group (p < 0.001). The re-revision rates were higher in the cemented group than in the cementless group (p = 0.047). @*Conclusions@#In our study, it was found that the patterns of transverse or oblique PFFs were more frequently produced with cemented stems, while long spiral fractures were more frequent with cementless stems. Stem subsidence and reoperation related to complications were more common in patients with PFFs around cemented stems than those with PFFs around cementless stems.
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Objective@#This study investigated the clinical implications of serious infections in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) who received the first cycle of rituximab (RTX) during the first 6 months of follow-up. @*Methods@#The medical records of 36 AAV patients treated with RTX were reviewed. A weekly dose of 375 mg/m 2 RTX was administered for 4 weeks to all patients along with glucocorticoids. Serious infections were defined as those requiring hospitalization. All-cause mortality during the first 6 months of follow-up was counted. The follow-up duration was defined as the period from the first RTX infusion to 6 months after the first RTX infusion. @*Results@#The median age was 60.5 years, and 16 patients were male. Seven of 36 patients (19.4%) died and three AAV patients had five cases of serious infection such as enterocolitis, pulmonary aspergillosis, atypical pneumonia, cytomegalovirus pneumonia, and cellulitis. AAV patients with serious infections during the first 6 months of follow-up exhibited a significantly lower cumulative survival rate than those without serious infections (p<0.001). However, we found no independent predictor of serious infections using the Cox hazard model analysis. @*Conclusion@#Serious infection is an important predictor of all-cause mortality in Korean patients with AAV who received their first cycle of RTX but there were no significant variables to predict the occurrence of serious infections at the first RTX. Thus, in cases refractory to other induction therapies, RTX should be strongly considered, despite an increase in mortality rate.
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Background@#Oxidative stress is generally accepted as one of the principal pathogenesis of vitiligo, and keratinocyte-melanocyte interactions are also thought to play critical roles. It is well-known that antioxidant response and autophagy protect cells against oxidative damage, but the details and the compensatory relationship between the two mechanisms in the keratinocytes of vitiligo lesions remain unclear. @*Objective@#To evaluate the antioxidant response and autophagy status of patients with vitiligo and to explore the interactions between these two mechanisms. @*Methods@#Ten patients with clinicopathologically proven vitiligo and 10 normal controls were enrolled in our Department of Dermatology, Soonchunhyang University Hospital, Bucheon, Korea. Tissue samples of vitiligo lesions in the patient group and normal skin in the control group were immunohistochemically stained for nuclear factor erythroid 2-related factor 2 (Nrf2), Beclin-1, microtubule-associated protein light chain 3 (LC3)-II, and p62. The immunopositivity of epidermal keratinocytes was evaluated. @*Results@#Keratinocytes in vitiligo lesions had a significantly lower expression of Nrf2 (p=0.002) than that in the cells of normal controls. The levels of autophagy markers did not differ significantly between the two groups, but decreases in the Beclin-1 and LC3-II levels, and an increase in the p62 level in the patient group may indicate a small decrease in autophagy of patients with vitiligo. @*Conclusion@#Decreased antioxidant response and reduced autophagy may trigger melanocyte apoptosis in vitiligo lesions.
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Multiple miliary osteoma cutis (MMOC) is a rare variant of osteoma, characterized by multiple eruptive hard nodules on the face. A 70-year-old female presented with multiple solid skin-colored papules on both cheeks, unresponsive to conventional medical treatments. She reported receiving an injection of an unknown cosmetic filler substance into her face by an unlicensed medical practitioner 20 years ago. Facial computed tomography showed multiple small calcifications immediately adjacent to foreign material assumed to be the filler substance in the dermis. Histological examination revealed osteoclasts, osteocytes, and eosinophilic bony tissue in the dermis, suggestive of osteoma cutis. Although the pathogenesis remains unclear, inflammation caused by injected foreign material may induce metaplastic transformation of multipotent mesenchymal cells into osteoblasts, resulting in heterotopic ossification. Dermatologists should be aware that MMOC may occur following injection of foreign material by unlicensed practitioners. Performing a detailed history and clinical evaluation may aid in the diagnosis of such recalcitrant skin lesions.
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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises group of small vessel vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In 2022, the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) jointly proposed new classification criteria for AAV (the 2022 ACR/EULAR criteria). In this review, we briefly summarize the 2022 ACR/EULAR criteria for GPA, MPA, and EGPA, and introduce our clinical experience with applying them to patients who were previously diagnosed with AAV based on three criteria: firstly, the classification criteria for GPA and EGPA proposed by the ACR in 1990; secondly, the algorithm for the classification of AAV and polyarteritis nodosa proposed by the European Medicines Agency algorithm in 2007 (the 2007 EMA algorithm); and thirdly, the revised International Chapel Hill Consensus Conference nomenclature of vasculitides in 2012 (the 2012 CHCC definitions). We found that concordance rate was highest in patients with MPA (96.6%), followed by those with EGPA (86.3%) and GPA (73.8%). In addition, compared to previous criteria, we noted several issues of the undervalued or overvalued items in the 2022 ACR/EULAR criteria for classifying AAV and provided several suggestions. To increase the diagnostic accuracy and reduce the discordance rate among the new and previous criteria for AAV, we suggest that the previous criteria should be considered together with the 2022 ACR/EULAR criteria when applying the classification criteria for AAV to patients suspected of AAV.
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In recent years, the emergence of digital therapeutics as a novel approach to managing conditions has garnered significant attention. This approach involves using evidence-based therapeutic interventions that are facilitated by high-quality software programs to treat, manage, or prevent medical conditions. The incorporation of digital therapeutics into the Metaverse has increased the feasibility of their implementation and application in all areas of medical services. In urology, substantial digital therapeutics are being produced and researched, including mobile apps, bladder devices, pelvic floor muscle trainers, smart toilet systems, mixed reality-guided training and surgery, and training and telemedicine for urological consultations. The purpose of this review article is to provide a comprehensive overview of the current impact of the Metaverse on the field of digital therapeutics and identify its current trends, applications, and future perspectives in the field of urology.
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Purpose@#: This study aimed to identify and describe the leadership experience of advanced practice nurses (APN). @*Methods@#: Data were collected through five focus group interviews in 2022 with a total of 24 APNs in groups of 4-6 participants. All interviews were recorded, transcribed, and data were analyzed using qualitative content analysis. @*Results@#: Nine categories emerged from three main themes. First, “Roles of APN leadership” comprised a trusted clinical expert, a moderator for the entire team, a resource person for nurturing the next generation, and a change agent for improving clinical practice. Second, “Facilitators and barriers to APN leadership” included ambiguity of APN role, support system, and institutional backing. Third, “Strategies for strengthening APN leadership competencies” comprised systematic leadership education and speaking up for APNs. @*Conclusion@#: APNs are passionate about their expertise and practice, but lack the legal and organizational authority and support to provide successive leadership. Systematic education including leadership and organizational advocacy will enable APN to provide leadership that benefits patients, institutions, and the wider healthcare system.
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Objectives@#This study aimed to demonstrate the bone mineral density (BMD) and body composition in postmenopausal women with knee osteoarthritis (OA) who underwent surgical treatment, such as total knee arthroplasty, osteotomy, or meniscectomy. @*Methods@#A total of 254 women with OA aged 50 years who underwent surgical treatment were enrolled in this study. We evaluated obesity-related factors, muscle components, and BMD using dual-energy X-ray absorptiometry. @*Results@#No significant differences were noted in the BMD of the hip joint between the symptomatic side of the leg with knee OA and the contralateral side. However, when comparing the BMD of each component, the results indicated a significantly higher BMD in the obesity group based on body mass index (BMI). When defining sarcopenic obesity (SO) using various indicators of obesity (BMI, the estimated visceral adipose tissue area, android/gynoid ratio, and total body fat percentage), the prevalence of SO in the OA group who underwent surgical treatment ranged from 22.0% to 49.6%. @*Conclusions@#This study investigated obesity-related factors in patients with advanced knee OA who underwent surgery, revealing a high prevalence of overweight/obese individuals, the presence of SO, and a complex relationship between obesity, body composition, and bone density, highlighting the potential protective effects of weight-bearing on bone health while exploring the impact of sarcopenia on bone density differences in the context of OA. Depending on various definitions of obesity, diverse proportions of SO in patients with OA have been observed, and further detailed research is required to understand its impact on the condition.