Objective:To analyze the clinical features of pachydermoperiostosis (PDP) and improve its diagnostic level.Methods:A retrospective analysis was conducted on the clinical data of four patients with PDP treated at Sun Yat-sen Memorial Hospital, Sun Yat-sen University from 2015 to 2023, including clinical manifestations, laboratory tests, imaging examinations, and genetic testing results.Results:All four patients were male with an average onset age of 15 years old (ranging from 9 to 18 years old). One patient′s uncle had PDP, and another patient′s parents were consanguineous, though neither parent showed signs of PDP. All four patients exhibited clubbing, skin thickening, and acne; three had frontal bossing and deepened nasolabial folds; two showed scalp sulci changes on head MRI, and all had periosteal thickening of the phalanges visible on X-ray. One patient accompanied with hypokalemic nephropathy, and another had gastric ulcer. One patient underwent whole exome sequencing test which revealed a homozygous mutation, SLCO2A1 gene c.1406C>T, leading to a protein change p.Pro469Leu. Computational tools REVEL, SIFT, and Polyphen2 predicted this variant as deleterious.Conclusion:In addition to skin thickening, frontal bossing, scalp sulci changes, clubbing, and periosteal proliferation, patients with PDP may also present with hypokalemic nephropathy and gastric ulcer. The SLCO2A1 gene c.1406C>T mutation may be pathogenic.

Objective:To explore the clinical characteristics of patients with primary ureteral sarcomatoid carcinoma, thereby enhancing the understanding of the clinical context surrounding this disease.Methods:We report a case of primary ureteral sarcomatoid carcinoma in an elderly patient diagnosed through postoperative pathology at the Fourth Medical Center of the General Hospital of the People's Liberation Army.Additionally, a literature review was conducted to analyze articles related to primary ureteral sarcomatoid carcinoma published both domestically and internationally up to July 2024.This review summarizes and analyzes information regarding the clinical manifestations, diagnosis, treatment, and prognosis associated with this condition.Results:A total of 20 articles were reviewed, encompassing 24 cases of primary ureteral sarcomatoid carcinoma.Among the patients, 17 were older than 60 years, and when combined with the cases presented in this review, a total of 18 cases involved patients over the age of 60.Of these, 66.7%(12/18)were male, with a median age at diagnosis of 65 years.The reported cases often exhibited no clinical manifestations in the early stages; however, as the disease progressed, common urinary symptoms emerged, including frequency, urgency, pain, or painless hematuria, occurring throughout the ureteral course.The lesions were predominantly located in the left ureter(17 cases), which frequently exhibited dilation with effusion.Pathologic examination revealed the presence of microscopic spindle cells.Surgical treatment was administered to 20 patients, of whom 9 did not experience recurrence or metastasis during the follow-up period, while 7 cases resulted in mortality.Conclusions:Primary ureteral sarcomatoid carcinoma is a rare malignant tumor of the ureter.Its clinical manifestations are generally regarded as non-specific, making pathological examination the gold standard for diagnosis.Immunohistochemical and imaging studies can assist in the diagnostic process.The current preferred treatment modality is surgical resection, while first-line therapies that combine chemotherapy with immunotherapy and targeted therapy have been shown to enhance tumor control.

Objective:To explore the current status of clinical trial registration for TCM treatment of heart failure.Methods:The clinical trials of TCM in the treatment of heart failure registered by China Clinical Trial Registry (ChiCTR) from the establishment of the database to June 30, 2024 were retrieved. Excel 2019 was used to input data and register time, registration area and institution, registration center and sample size, funding source, intervention measures, outcome indicators, random methods and blind methods were used for frequency analysis.Results:A total of 71 clinical trials of TCM in the treatment of heart failure in China were included, involving 13 provincial-level administrative regions. There were 39 clinical trial institutions participating in the registration, with Shanghai (16 items), Beijing (15 items), and Tianjin (12 items) ranking at the top in terms of registration numbers; the funding mainly relied on local finances, followed by national finances and hospital grants; in the registered projects, interventional studies accounted for the majority, mainly grouped through random number table methods, and only a small number of trials used blinding methods, with single-center participation being the norm; in terms of intervention measures, mainly Chinese patent medicines were used, while TCM decoctions were used less frequently. Outcome indicators were mainly efficacy indicators such as biochemical results and echocardiography, lacking monitoring of safety indicators.Conclusions:The number of clinical trial registrations for TCM in the treatment of heart failure in China is relatively small, but it shows a trend of increasing year by year. In terms of intervention measures, the treatment methods are also continuously enriching. However, there are still issues with clinical trials of TCM for the prevention and treatment of heart failure, such as insufficient awareness of registration among researchers, unbalanced regional and institutional distribution, and the need for improvement in the use of randomization and blinding methods.

Objective:To explore the clinical characteristics of patients with primary ureteral sarcomatoid carcinoma, thereby enhancing the understanding of the clinical context surrounding this disease.Methods:We report a case of primary ureteral sarcomatoid carcinoma in an elderly patient diagnosed through postoperative pathology at the Fourth Medical Center of the General Hospital of the People's Liberation Army.Additionally, a literature review was conducted to analyze articles related to primary ureteral sarcomatoid carcinoma published both domestically and internationally up to July 2024.This review summarizes and analyzes information regarding the clinical manifestations, diagnosis, treatment, and prognosis associated with this condition.Results:A total of 20 articles were reviewed, encompassing 24 cases of primary ureteral sarcomatoid carcinoma.Among the patients, 17 were older than 60 years, and when combined with the cases presented in this review, a total of 18 cases involved patients over the age of 60.Of these, 66.7%(12/18)were male, with a median age at diagnosis of 65 years.The reported cases often exhibited no clinical manifestations in the early stages; however, as the disease progressed, common urinary symptoms emerged, including frequency, urgency, pain, or painless hematuria, occurring throughout the ureteral course.The lesions were predominantly located in the left ureter(17 cases), which frequently exhibited dilation with effusion.Pathologic examination revealed the presence of microscopic spindle cells.Surgical treatment was administered to 20 patients, of whom 9 did not experience recurrence or metastasis during the follow-up period, while 7 cases resulted in mortality.Conclusions:Primary ureteral sarcomatoid carcinoma is a rare malignant tumor of the ureter.Its clinical manifestations are generally regarded as non-specific, making pathological examination the gold standard for diagnosis.Immunohistochemical and imaging studies can assist in the diagnostic process.The current preferred treatment modality is surgical resection, while first-line therapies that combine chemotherapy with immunotherapy and targeted therapy have been shown to enhance tumor control.

Objective:To analyze the clinical features of pachydermoperiostosis (PDP) and improve its diagnostic level.Methods:A retrospective analysis was conducted on the clinical data of four patients with PDP treated at Sun Yat-sen Memorial Hospital, Sun Yat-sen University from 2015 to 2023, including clinical manifestations, laboratory tests, imaging examinations, and genetic testing results.Results:All four patients were male with an average onset age of 15 years old (ranging from 9 to 18 years old). One patient′s uncle had PDP, and another patient′s parents were consanguineous, though neither parent showed signs of PDP. All four patients exhibited clubbing, skin thickening, and acne; three had frontal bossing and deepened nasolabial folds; two showed scalp sulci changes on head MRI, and all had periosteal thickening of the phalanges visible on X-ray. One patient accompanied with hypokalemic nephropathy, and another had gastric ulcer. One patient underwent whole exome sequencing test which revealed a homozygous mutation, SLCO2A1 gene c.1406C>T, leading to a protein change p.Pro469Leu. Computational tools REVEL, SIFT, and Polyphen2 predicted this variant as deleterious.Conclusion:In addition to skin thickening, frontal bossing, scalp sulci changes, clubbing, and periosteal proliferation, patients with PDP may also present with hypokalemic nephropathy and gastric ulcer. The SLCO2A1 gene c.1406C>T mutation may be pathogenic.

Objective:To analyze the clinical characteristics of psoriatic arthritis (PsA) and to raise clinicians′ awareness.Methods:We retrospectively analyzed the clinical characteristics of hospitalized patients with the diagnosis of PsA in our hospital. The PsA were classified according to the Classification of Psoriatic Arthritis (CASPAR) criteria. We compared the clinical characteristics and risk factors between the oligoarthritis type and symmetrical polyarthritis subtypes. Comparing the clinical features of PsA patients with hyperuricemia to those with normal uric acid levels. The t-test was utilized to assess the differences in normally distributed continuous variables between the two groups, while the Mann-Whitney U test was employed for the comparison of skewed distributed continuous variables across the groups. Results:162 PsA patients were included, the average age at PsA onset was (38±15)years old and the average of PsA duration was (7.3±6.6) years, 72.8%(118/162) patients were male. Eighty-nine patients (54.9%) presented with symmetric polyarthritis, and 49 patients (30.2%) with oligoarthritis. The median duration of PsA in patients with oligoarthritis was significantly shorter than patients with symmetric polyarthritis [4.0(0.8, 8.5) years vs. 7.0(2.0 10.0) years, Z=-2.83, P=0.005]. Mean serum uric acid levels [(391±126)μmol/L vs. (334±130)μmol/L, t=2.00, P=0.016] and the proportion of patients with concomitant hyperuricemia [(44.9%(22/49) vs. 23.6%(21/89), χ2=6.68, P=0.010] in patients with oligoarthritis was significantly higher than that of patients with symmetric polyarthritis. Multivariate logistic regression analysis showed that patients with hyperuricemia had a significantly increased risk of presenting with oligoarthritis subtype of PsA [ OR(95% CI)=2.938(1.252, 6.890), P=0.013]. Compared with patients with normal uric acid, PsA patients with hyperuricemia were older in age [(51±13)years vs. (48±16)years, t=-3.30, P=0.001], and had a higher proportion of males [86.0%(37/45) vs. 55.6%(45/81), χ2=11.66, P=0.001] longer median duration of psoriasis (11.0(6.0, 11.5)years vs. 8.5(8.0, 18.0)years), higher proportion of oligoarthritis [51.2%(22/43) vs. 28.4%(23/81), χ2=6.30, P=0.012] and higher proportion of hypertension [20.9%(9/43) vs. 7.4%(6/81), χ2=4.83, P=0.028] and cardiovascular disease [9.3%(4/43) vs. 1.2%(1/81), χ2=2.87, P=0.049] all were with statistically significant differences (all P<0.05). Conclusion:Oligoarthritis subtype of PsA patients have a shorter disease duration, higher serum uric acid level, and a higher proportion of hyperuricemia compared to symmetrical polyarthritis subtype of PsA patients; PsA patients with hyperuricemia are more likely to have oligoarthritis, with a higher risk of concomitant hypertension and cardiovascular disease.

Objective:To investigate the epidemiology of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with maintenance hemodialysis (MHD) in Jiangsu province during SARS-CoV-2 pandemic in China from December 7, 2022 to January 27, 2023, and to analyze the influencing factors of all-cause death.Methods:It was a multi-center cross-sectional investigation. Structured questionnaire was used to collect patient information by medical staff of each hemodialysis center (room) as investigators. Part of the demography data and laboratory examination data came from the Jiangsu Province Hemodialysis Data Information System. MHD patients from hemodialysis centers (rooms) at all levels of medical institutions and independent hemodialysis institutions in Jiangsu province during the outbreak of SARS-CoV-2 infection were included, and the clinical characteristics and all-cause mortality of confirmed and suspected cases of SARS-CoV-2 infection were analyzed.Results:Questionnaire surveys and data analysis on 57 278 patients in 407 hemodialysis centers (rooms) were completed, accounting for 90.41% of the total number of MHD patients (63 357 cases) in Jiangsu province during the same period. There were 24 038 cases (41.97%) of SARS-CoV-2 infection and 14 805 cases (25.85%) of suspected infection, which were widely distributed in all dialysis centers in Jiangsu province. After clinical classification of 38 843 confirmed and suspected SARS-CoV-2 infection cases, 3 662 cases were severe and critical cases, accounting for 9.43% of the infected and suspected cases. Among the patients who had completed the questionnaires, there were 1 812 all-cause deaths, with an all-cause mortality rate of 3.16%. Multivariate logistic regression analysis showed that elderly (taking ≤50 years as a reference, 51-59 years: OR=1.583, 95% CI 1.279-1.933, P=0.001; 60-69 years: OR=3.972, 95% CI 3.271-4.858, P<0.001; 70-79 years: OR=7.236, 95% CI 5.917-8.698, P<0.001; ≥80 years: OR=11.738, 95% CI 9.459-14.663, P<0.001), male ( OR=1.371, 95% CI 1.229-1.529, P<0.001), and co-infection with hepatitis B virus (HBV) (positive serum HBV surface antigen, OR=0.629, 95% CI 0.484-0.817, P<0.001) were independent influencing factors for all cause mortality. Receiver-operating characteristic curve analysis showed that the area under the curve for male, age and current HBV infection prediction of all-cause death was 0.529 ( P<0.001), 0.724 ( P<0.001) and 0.514 ( P=0.042), respectively, and the cut-off value for age prediction of all-cause death was 65.5 years old. Compared with patients without HBV infection, MHD patients with HBV infection significantly reduced the proportion of severe and critically ill patients, all-cause hospitalizations and all cause deaths when infected with SARS-CoV-2 (4.99% vs. 6.41%, χ2=6.136, P=0.013; 8.90% vs. 11.44%, χ2=11.662, P<0.001; 2.01% vs. 3.37%, χ2=10.713, P=0.001, respectively). Conclusion:The MHD patients in Jiangsu province are susceptible to SARS-CoV-2. Elderly age and male gender are independent risk factors for death in MHD patients during the epidemic, while the HBV infection may be a protective factor for death of MHD patients infected with SARS-CoV-2.

Objective:To improve the awareness of cholesterol crystal embolism syndrome (CCE) inrheumatologists.Methods:The clinical characteristics of 40 Chinese CCE patients admitted to our department (one case) were summarize and in the literature (thirty-nine cases) were reviewed.Results:Among these 40 patients, 87.5%(35/40) were male and the mean age was (68±6) years. All patients suffered from athero-sclerosis and 87.5%(35/40) of them had precipitating factors such as endovascular intervention, vascular surgery, anticoagulant, or thrombolytic therapy. The clinical manifestations included renal insufficiency (90.0%, 36/40), blue toe syndrome (82.5%, 33/40), ulceration or gangrene (25.0%, 10/40), and livedo reticularis (15%, 6/40). Acute phase reactant was tested in 25 cases, of whom 84.0%(21/25) showed elevated C-reactive protein (CRP) and 56.0%(14/25) showed elevated erythrocyte sedimentation rate (ESR).Conclusion:Rheumatologists should be alert that CCE is one of the differential diagnosis of systemic vasculitis, especially for patients with severe atherosclerosis.

Objective:To improve the differential diagnosis of systemic sclerosis (SSc) with hypertension and renal insufficiency.Methods:The clinical characteristics, diagnosis and treatment of a SSc patient with hypertension and renal insufficiency were reported and discussed.Results:A middle-aged female patient with a history of SSc for 5 years, headache and gross hematuria for 10 days was admitted. Abrupt increase in blood pressure and creatinine, glomerular hematuria, proteinuria, low complement C3 and C4, positive antinuclear antibody (ANA), anti-dsDNA antibody and perinuclear antineutrophil cytoplasmic antibody (pANCA) were presented. Renal pathology showed lupus nephritis (LN) (type Ⅳ). After glucocorticoid pulse therapy, followed by cyclophosphamide, belimumab, and symptomatic treatment, the symptoms were relievedand lupus disease activity were decreased.Conclusion:For SSc patients with increased blood pressure and creatinine, the presence of other diseases should be considered in addition to scleroderma renal crisis. Renal biopsy and pathological examination should be performed to confirm the diagnosis and avoid misdiagnosis.

Objective: To investigate the role of lncRNAs in the invasion and metastasis of salivary adenoid cystic carcinoma (SACC) cells. Methods: With SACC-LM as the experimental group and SACC83 as the control group, lncRNA chips were used to screen the differentially expressed lncRNAs. The differentially expressed lncRNAs were further verified by real-time quantitative RT-PCR (qRT-PCR). The invasion and migration abilities of the adenoid cystic carcinoma cell lines before and after transfection with lncRNA siRNAs were detected by invasion and migration experiments. The clinicopathological features and prognosis of patients with different expression of lncRNAs and SACC were analyzed. Results: The microarray showed that ADAMTS9-AS2 was highly expressed in the SACC-LM cells. Real-time quantitative RT-PCR further confirmed that ADAMTS9-AS2 was significantly upregulated in the SACC-LM cells. Invasion and migration experiments showed that the invasion and migration were significantly reduced after the expression level of ADAMTS9-AS2 was downregulated (P ＜ 0.001). Analysis of the clinicopathological data showed that ADAMTS9-AS2 was highly expressed in SACC. High expression of ADAMTS9-AS2 was associated with poor prognosis and a high tumor metastasis rate in SACC patients. Conclusion High expression of ADAMTS9-AS2 promotes the migration and invasion of SACC cells. ADAMTS9-AS2 is upregulated in the SACC tissues and is related to a high metastasis rate and poor prognosis.