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Ahn  Sung Soo  Ha  Jang Woo  Park  Yong-Beom  Lee  Sang-Won 《Clinical rheumatology》2022,41(11):3429-3437
Clinical Rheumatology - Birmingham vasculitis activity score (BVAS) version 3 (BVAS 3.0) and BVAS/granulomatosis with polyangiitis (BVAS/GPA) are used as indicators of disease activity in...  相似文献   
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Stickler syndrome is a genetic disorder of connective tissue. One of the major symptoms associated with this disorder is an oro-facial malformation, which may cause a submucous cleft or a complete cleft of the hard palate. A 32-year-old man diagnosed with Stickler syndrome and a submucosal cleft palate (SMCP) visited our hospital with a chief complaint of excessive daytime sleepiness. The patient was diagnosed with severe obstructive sleep apnea (OSA), and administration of a polysomnography test revealed an apnea-hypopnea index (AHI) of 30.9 events/hour (h). Auto-titrating continuous positive airway pressure was initiated to control the OSA symptoms and subsequently the patient showed some improvement. However, due to continuous velopharyngeal insufficiency symptoms, intravelar veloplasty was performed. Three months after surgery, the AHI had decreased to 12.4 events/h. Recent studies have described a greater risk for OSA in individuals with cleft palate, than in the general population. The present case demonstrates surgical success in a patient with OSA and SMCP, suggesting that palatal surgery may be considered an optional surgical treatment for OSA patients with SMCP.  相似文献   
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International Journal of Clinical Oncology - Previous studies have shown a relationship between the occurrence and recurrence of prostate cancer; however, this relationship remains controversial....  相似文献   
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Background: Early-onset or familial gastric cancer (GC) is known to have clinicopathologic profiles different fromthose of sporadic GC. We aimed to compare DNA damage response marker expression between early-onset or familialGC and sporadic GC. Methods: GC samples were obtained from patients who underwent gastrectomy for GC at SeoulNational University Hospital. Immunohistochemical analyses of various DNA damage response markers, includingBRCA1, BRCA2, MRE11, RAD51C, and γH2AX, were performed using 54 early-onset GC, 59 familial GC, and 337sporadic GC tissue microarray samples. Correlations between marker expression and clinicopathologic features wereevaluated by univariate and multivariate analyses, and overall survival was analyzed. Results: The rate of γH2AXpositivity was significantly higher (p < 0.001) in early-onset or familial GC than in sporadic GC. In contrast, the rates ofMRE11 negativity and RAD51C negativity were significantly higher in sporadic GC than in early-onset or familial GC.BRCA1 negativity was associated with decreased overall survival in sporadic GC (p = 0.002), and MRE11 negativitywas associated with decreased overall survival in sporadic GC (p = 0.012). Conclusion: Our results show significantdifferences in DNA damage response marker expression between early-onset or familial GC and sporadic GC.  相似文献   
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Introduction

The American Joint Committee on Cancer (AJCC) tumor, node, metastasis classification system (TNM) staging manual has been updated and provides more specified stage grouping for prostate cancer (PCa). We aimed to validate the updated AJCC stage groups for PCa using a radical prostatectomy (RP) cohort.

Patients and Methods

We analyzed the data of 3032 patients previously treated with RP for localized PCa. We stratified patients into stage groups according to the 8th edition of the AJCC manual and compared biochemical recurrence (BCR)-free survival using Kaplan-Meier analyses.

Results

There were 217 patients in stage group I, 33 in IIA, 1101 in IIB, 535 in IIC, 129 in IIIA, 781 in IIIB, and 236 in IIIC. There were no significant differences in BCR-free survival between stage groups IIC and IIIA (P = .875). Subsequently, the low–Gleason score (GS) IIIA subgroup (GS ≤ 3 + 4, P = .025) showed superior BCR-free survival than the IIC group, and the high-GS IIIA subgroups (GS ≥ 4 + 3, P = .004) showed a poorer BCR-free survival than the IIC group. Furthermore, there were no significant differences between groups I and IIA (P = 330) and between groups IIA and IIB (P = .942). Our new staging system provided a better ability to discriminate the prognosis of each group. However, our study has several limitations, such as retrospective design, relatively short follow-up period, and need for further validation.

Conclusion

The current AJCC prognostic groups show some contradictory results, particularly concerning prognosis of the IIC and IIIA groups. We suggest that GS be given more weight than serum prostate-specific antigen level in stage group stratification.  相似文献   
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PurposeTo determine the prognostic effect of upper tract urothelial carcinoma (UTUC) with variant histology (VH) after radical nephroureterectomy (RNU).Patients and MethodsThe data of 1173 patients who received RNU for UTUC without neoadjuvant chemotherapy in 11 institutions between 2002 and 2016 were retrospectively reviewed. A matched propensity score analysis was performed. Clinicopathologic variables, recurrence-free survival (RFS), cancer-specific survival (CSS), and overall survival (OS) were compared between patients with pure UTUC and patients with UTUC and VH. Univariate and multivariate Cox proportional regression models were used to determine the independent variables associated with oncologic outcomes.ResultsUTUC with VH was observed in 93 patients (7.9%). After propensity score matching, UTUC with VH showed no difference in clinicopathologic features compared to pure UTUC; however, it was associated with shorter RFS, CSS, and OS (log rank, P = .011, P = .002, P = .006, respectively). Additionally, the multivariate analysis revealed that VH was independently associated with a poor RFS [hazard ratio (HR) = 1.92; 95% confidence interval (CI), 1.27-2.89; P = .002], CSS (HR = 4.47; 95% CI, 1.99-10.1; P = .001), and OS (HR = 3.00; 95% CI, 1.55-5.78; P = .001). However, the Kaplan-Meier method revealed that differences in RFS, CSS, and OS were not significant in patients who received adjuvant chemotherapy (log rank, P = .562, P = .060, P = .153, respectively).ConclusionUTUC with VH was independently associated with poor oncologic outcomes in patients with UTUC after RNU. Although patients with UTUC and VH had a poor prognosis compared to patients with pure UTUC, adjuvant chemotherapy would be helpful in improving the survival rates of these patients.  相似文献   
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