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Associations with Organ Involvement and Autoantibodies in Systemic Sclerosis: Results from the Canadian Scleroderma Research Group (CSRG) 被引量:1
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作者 Vikram Tangri Carly Hewson +3 位作者 Murray Baron A. Bonner Marvin Fritzler Janet E. Pope 《Open Journal of Rheumatology and Autoimmune Diseases》 2013年第2期113-118,共6页
Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients h... Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram. 展开更多
关键词 scleroderma SSc systemic Sclerosis Antibodies Anticentromere TOPOISOMERASE I RNA Polymerase III ORGAN INVOLVEMENT scleroderma Renal Crisis PULMONARY Fibrosis ILD ANA PULMONARY Hypertension
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Predictors of a Cerebrovascular Accident in a Population of Systemic Sclerosis Patients Followed at a Large Academic Center with a Dedicated Scleroderma Center
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作者 Christopher J. Inserra Chris T. Derk 《Open Journal of Rheumatology and Autoimmune Diseases》 2020年第2期45-56,共12页
Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior... Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior studies have showed an increased risk of cardiovascular disease among patients with chronic inflammatory disorders, with chronic inflammation leading to atherosclerosis believed to be the culprit. Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by diffuse fibrosis of the skin and internal organs. Previous studies have suggested a possible link between systemic sclerosis and macrovascular complications such as stroke. Methods: This is a retrospective chart review of patients treated within the University of Pennsylvania Health System from October 2015 to April 2019 with a diagnosis of SSc. Using ICD10 codes, we identified a cohort of SSc patients who suffered a stroke. Information regarding demographics and stroke risk factors were gathered from the charts of patients with a diagnosis of both SSc plus stroke and compared to a control group of randomly selected patients with SSc who never suffered a stroke. Continuous variables were conveyed using a mean plus a standard deviation. A two-sample t-test was used to compare the two groups of patients. Qualitative variables were compared using a two-tailed Fisher’s exact test. Results: Based on a large cohort of SSc patients (n = 2080) followed between October 2015 and April 2019, we identified 36 SSc patients who developed a subsequent stroke (1.7% of cohort). When looking at risk factors for stroke in SSc patients, we identified hypertension and atrial fibrillation to be associated with the diagnosis of stroke in such patients. Specifically, 28 of the 36 patients with both SSc and stroke also had a diagnosis of hypertension while in the control group, only 17 of 36 patients had hypertension. Atrial fibrillation was seen in 9 of 36 patients with both SSc and CVA while it was seen in only 2 of 36 patients in the control group. Conclusions: This case control study demonstrated that the presence of hypertension and atrial fibrillation had a statistically significant association with the diagnosis of CVA in patients with SSc. 展开更多
关键词 systemic SCLEROSIS scleroderma Stroke CEREBROVASCULAR Accident ATRIAL FIBRILLATION Hypertension
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Systemic Scleroderma at University Teaching Hospital (UTH) of Cocody (Abidjan—Cote d’Ivoire): A 19 Cases Report
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作者 Mariam Gbané Cesar Gael Houndénou +6 位作者 Baly Ouattara Guy Leopold Kengni Mohamed Diomande Kouassi Jean Mermoz Djaha Abidou Kawalé Coulibaly Yaya Coulibaly Edmond Eti 《Open Journal of Rheumatology and Autoimmune Diseases》 2019年第4期134-143,共10页
Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a per... Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients’ files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases;a Raynaud’s phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine. 展开更多
关键词 systemic scleroderma Sclerodactyly ABIDJAN
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Iatrogenic scleroderma renal crisis:A case report and mini literature review for renal crisis
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作者 Gülsah Boz Samet Karahan Nurdan Yüksek 《Discussion of Clinical Cases》 2021年第3期4-8,共5页
Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more f... Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step. 展开更多
关键词 scleroderma systemic sclerosis scleroderma renal crisis systemic sclerosis-associated thrombotic microangiopathy
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B cell depletion in scleroderma lung disease: A promising new treatment?
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作者 Dimitrios Daoussis Andrew P Andonopoulos 《World Journal of Rheumatology》 2013年第2期9-11,共3页
Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomiz... Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomized controlled study from our research group it was shown that treatment with 2 courses of RTX leads to a significant improvement of lung function at 1 year compared to baseline. All relevant studies have so far reported clinical and/or histologic improvement of skin fibrosis something that adds further evidence in favor of a disease modifying role of RTX in SSc. It is more than obvious that novel therapies for SSc-associated lung disease are needed. A large scale, randomized, controlled study assessing the efficacy of RTX in SSc associated interstitial lung disease is warranted. If RTX turns out to be effective it would be a major therapeutic advance in SSc since it can be administered on a long term basis due to its acceptable safety profile. 展开更多
关键词 RITUXIMAB scleroderma systemic SCLEROSIS INTERSTITIAL lung disease TREATMENT
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Journey of a patient with scleroderma from renal failure up to kidney transplantation
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作者 Fedaey Abbas Mohsen El Kossi +2 位作者 Ihab Sakr Shaheen Ajay Sharma Ahmed Halawa 《World Journal of Transplantation》 2021年第9期372-387,共16页
The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an as... The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an associated disorder that may involve 2%-15%of SS patients.Patients presented with earlier,rapidly progressing,diffuse cutaneous SS disease,mostly in the first 3-5 years after non-Raynaud clinical manifestations,are more vulnerable to develop SRC.SRC comprises a collection of acute,mostly symptomatic rise in blood pressure,elevation in serum creatinine concentrations,oliguria and thrombotic microangiopathy in almost 50%of cases.The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis.In a scleroderma patient maintained on regular dialysis;every effort should be exerted to declare any possible evidence of renal recovery.A given period of almost two years has been suggested prior to proceeding in a kidney transplant(KTx).Of note,SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease(ESRD).KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients.Compared to other primary renal diseases,SS-related ESRD was considered for a long period of poor patient and allograft survivals.Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death.Recurrence of SRC after transplantation has been observed in some patients.However,an excellent post-transplant patient and graft outcome have been recently reported.Consequently,the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx. 展开更多
关键词 systemic sclerosis scleroderma renal crisis Risk factors Renal failure HEMODIALYSIS Kidney transplant
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Pembrolizumab induced scleroderma
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作者 Reem Alkilany Stanley Ballou 《Discussion of Clinical Cases》 2019年第4期6-9,共4页
Systemic sclerosis(SSc)is a rare connective tissue disorder.It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue,with skin hardening and thickening(known as scle... Systemic sclerosis(SSc)is a rare connective tissue disorder.It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue,with skin hardening and thickening(known as scleroderma)being the hallmark of the disease.It tends to affect females more than males and has a higher prevalence in African American population with earlier onset and more severe disease.While scleroderma can be a manifestation of conditions other than SSC,the presence of skin thickening of the fingers,extending proximally to metacarpophalangeal joints is sufficient to classify a patient as having SSc.SSc treatment is challenging given the heterogeneity of the disease,multiple organ involvement,different subtypes and poorly understood etiology and pathogenesis.Yet,systemic immunosuppressive therapy is often the treatment of choice.Here we present a 60-year-old white female who developed skin thickening of her fingers extending to the forearms and of her proximal thighs after being treated with pembrolizumab for metastatic non-small cell lung cancer.It was difficult to determine internal organ involvement given her history of metastatic lung cancer,but scleroderma specific autoantibodies were negative.Her symptoms improved after treatment with methotrexate and stopping pembrolizumab.This is one of the first case reports of scleroderma secondary to pembrolizumab. 展开更多
关键词 Pembrolizumab Immune check point inhibitor scleroderma systemic sclerosis Sclerodactyly Scleredema diabeticorum
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Juvenile Systemic Sclerosis: About 9 Cases
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作者 Kaoutar Danaoui Houda Nassih +4 位作者 Khadija Oujennane Rabiy El Qadiry Aicha Bourrahouat Said Amal Imane Ait Sab 《Open Journal of Pediatrics》 2024年第2期320-326,共7页
Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutane... Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, Hospital Center in Marrakech, Morocco. 展开更多
关键词 scleroderma systemic Sclerosis CHILDREN PEDIATRIC
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Anti-RNA Polymerase III Antibodies in Systemic Sclerosis
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作者 Andrew Kelly Chris T. Derk 《Open Journal of Rheumatology and Autoimmune Diseases》 2015年第3期81-86,共6页
Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attem... Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attempt to review the current data on anti-RNA polymerase II as it relates to Systemic Sclerosis. 展开更多
关键词 AUTOANTIBODIES systemic SCLEROSIS scleroderma Anti-RNA POLYMERASE III Cancer
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Lower Extremity Ulcers in Patients with Systemic Sclerosis
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作者 Marta B. Bean Chris T. Derk 《Open Journal of Rheumatology and Autoimmune Diseases》 2022年第1期35-45,共11页
Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and s... Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and substantial cost of wound care led us to undertake this study to describe and identify risk factors. Methods: After Institutional Review Board approval, we identified 30 patients with SSc and lower extremity ulcers over a 10-year period at a single center with an SSc clinic, which were included in a descriptive analysis. Results: Median age of onset of lower extremity ulcers was 59.5 years (range 20 - 84). Ninety percent of patients were female, 60% were Caucasian, 63% had limited SSc, 13% diffuse SSc and 23% an overlap syndrome. Immunomodulators or steroids were prescribed in 53%;hypercoagulable state identified in 16%. Ulcers were attributed to venous stasis (27%), SSc (20%), trauma (20%), arterial disease (17%), and multifactorial/unknown (17%). In patients with ulcers attributed to SSc, age at onset was lower (45.5 vs 59.5 years). Biopsies generally did not contribute to management. Multidisciplinary treatment was routine;20% required amputation, 10% endovascular intervention, 20% frequent surgical debridement, 10% hyperbaric oxygen, 26% local treatment and antibiotics and 13% received immunosuppression for wound treatment. Conclusion: Lower extremity ulcers are a serious clinical problem in patients with SSc. The clinical exam, venous dopplers, ankle-brachial indices and assessment of vascular risk factors helped define causality. In younger patients, ulcers were more frequently attributed to SSc and these patients were more likely to be on immunosuppressants/DMARDS, possibly indicating severe phenotype of SSc. 展开更多
关键词 Lower Extremity Ulcers systemic Sclerosis scleroderma Peripheral Vascular Disease Venous Stasis
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CLINICOPATHOLOGICAL ANALYSIS OF 5785 CASES WITH RESPIRATORY SYSTEM TUMORS
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作者 王新允 王爱香 +5 位作者 马莹 刘婷 孙翠云 李艳 赵敏 吴兴业 《Chinese Journal of Cancer Research》 SCIE CAS CSCD 2006年第1期70-73,共4页
Objective; To study the characteristics and tendency of incidence of patients with respiratory system tumors during the past 23 y in Tianjin. Methods: All data in our research was obtained from the surgical pathology... Objective; To study the characteristics and tendency of incidence of patients with respiratory system tumors during the past 23 y in Tianjin. Methods: All data in our research was obtained from the surgical pathology files of Department of Pathology of the general and the Second Hospitals of Tianjin Medical University between 1981 and 2003. All data was analyzed by Spss 11.5 statistics program. The comparisons were made by u-test, P〈0.05 was considered as significant. Results: 1. The detection rate of malignant tumors is significantly higher than that of benign tumors (U=52.68, p=0.000) in respiratory system. 2. The common sites of benign tumors are nose and pharynx, but the common sites of malignant tumors are lung and larynx. 3. The incidence of benign tumors generally peaks between the ages of 40 and 50, but the incidence of malignant tumor generally peaks between the ages of 50 and 60. 4. The commonest histological type of malignant tumors is squamous cell carcinoma, but the commonest histological type of benign tumors is papilloma. 5. The detection rate of malignant lung tumors steadily increased between 1981 and 1999 and increased sharply from 1999 to 2003, but the detection rate of malignant Nasopharyngeal tumors steadily decreased from 1981 to 2003. Between 1981 and 1997, the detection rate of malignant laryngeal tumors steadily increased, followed by a decrease between 1997 and 2003. Conclusion: The detection rate of malignant respiratory system tumors especially lung cancer is gradually increasing. Therefore early prevention and treatment are critical to patients' prognosis. 展开更多
关键词 Respiratory system tumors Analysis of pathological data Characteristics of incidence
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Anatomical Pathology Laboratory Activity in Meknes, Morocco
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作者 Hajar El Hilali Fatiha El Hilali +1 位作者 Bakary Traore Fatiha Laziri 《Open Journal of Pathology》 2019年第3期50-57,共8页
Cancer continues to be a major health problem in the world. During 2018, there were 9.6 million deaths and 18.1 million new cases registered across the globe. It is estimated that one in 5 men and one in 6 women will ... Cancer continues to be a major health problem in the world. During 2018, there were 9.6 million deaths and 18.1 million new cases registered across the globe. It is estimated that one in 5 men and one in 6 women will develop cancer during their lifetime. This disease kills one in 8 men and one in 11 women. Developing countries have seen an increase in cancer occurrence and change in type of cancers due to change in social and economic conditions. The objective of this research was to further evaluate 1659 specimens for anatomical pathology testing. The data was collected from the records of the pathological anatomy laboratory at the Mohamed V Hospital in Meknes, Morocco. It is one-year study period (January-December 2013). Results of the anatomy pathology exam showed cancer positive for 9.6% of specimens;inconclusive for 1%;and negative for 66.2%. For all specimens, the most analyzed organs are breast with 29.7%;skin with 21.3%;cervical with 2.6%. For cancer patients, the most affected organs are skin (21.3%) and breast (29.7%). For cancer patients, Neoplasm is present in 9.3% of patients. Patients with metastatic cancer represent 0.3%. Patients with non-neoplastic lesions represent 66.2%. 1% of patients represent uncertain results. Additional immunohistochemistry research is needed before conclusive recommendations are made. 展开更多
关键词 CANCER BREAST CANCER Morocco HEALTH system ANATOMICAL pathology Testing
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Focus on laboratory tests related to the pathological types of lupus nephritis to implement renal biopsy as early as possible:clinical and pathological analysis of 217 cases of single center lupus nephritis
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作者 Xiao-Yu Wang Hong-Dong Li +2 位作者 Bo-Wen Tian Ping Wei Shu-Mei Shi 《Clinical Research Communications》 2024年第3期43-47,共5页
Objective:To analyze the clinical and laboratory indices of patients with lupus nephritis(LN)of different pathological types and explore the related factors of LN pathological classification,it is helpful to grasp the... Objective:To analyze the clinical and laboratory indices of patients with lupus nephritis(LN)of different pathological types and explore the related factors of LN pathological classification,it is helpful to grasp the timing of renal biopsy.Methods:The clinical manifestations,laboratory parameters and renal pathological types of LN patients in recent 20 years were analyzed retrospectively by SPSS 26.0 software.Results:In this study,the first three pathological types were V,IV,V+IV;latent nephritis was common in type II and V;nephritic syndrome was common in type V;nephrotic syndrome was common in type V+IV;chronic renal insufficiency group was mostly type IV;pathological types were correlated with serum creatinine,C3,albumin and erythrocyte sedimentation rate(r=0.315,P<0.001),and serum creatinine was moderately correlated(r=0.315,P<0.001);AI,CI and SLEDAI scores were significantly different among LN patients of different pathological types.Conclusion:LN is closely related to clinical pathology,clinical manifestations,comprehensive analysis of laboratory indicators and SLEDAI score to make a preliminary prediction of LN pathological type,help to initially assess the severity of pathology,improve the timing of renal biopsy implementation,optimize the timing of treatment. 展开更多
关键词 systemic lupus erythematosus lupus nephritis clinical features pathological types
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Novel indications for autologous fat grafting in reconstruction:scleroderma
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作者 Abed Rahman Kawakibi Alexander N.Khouri +1 位作者 Paul S.Cederna Amy L.Strong 《Plastic and Aesthetic Research》 2023年第1期680-688,共9页
Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapi... Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapies are limited and insufficiently treat the cutaneous manifestations of scleroderma.Autologous fat transfer(AFT)is a surgical technique that has been utilized for many decades for facial rejuvenation.The adipose stem cells(ASCs)present in fat grafts have also shown significant promise for their anti-inflammatory and regenerative properties.Recently,AFT has been repurposed to treat the skin manifestations of systemic sclerosis and localized scleroderma.Studies suggest that AFT in scleroderma patients improves mouth and hand functions,Raynaud’s symptoms,and digital ulcerations.AFT is a safe procedure with rare postoperative complications,making it a promising intervention for the treatment of scleroderma.Further studies are required to better characterize the influence of fat grafts on the recipient site and to establish standards for fat transfer in fibrotic skin diseases. 展开更多
关键词 scleroderma systemic sclerosis localized scleroderma AFT
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系统性硬化症合并干燥综合征的临床特征分析及危险因素研究
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作者 邹松炎 张日伊 +1 位作者 李孝东 穆银玉 《中国全科医学》 CAS 北大核心 2025年第6期737-741,750,共6页
背景系统性硬化症(SSc)是异质性疾病,常同时合并干燥综合征(SS),SSc患者部分症状与SS相似,临床诊治过程中容易漏诊SS。目的探讨SSc合并SS患者的临床和实验室特点及重叠发病的危险因素。方法回顾性纳入2019—2023年在宁波市医疗中心李惠... 背景系统性硬化症(SSc)是异质性疾病,常同时合并干燥综合征(SS),SSc患者部分症状与SS相似,临床诊治过程中容易漏诊SS。目的探讨SSc合并SS患者的临床和实验室特点及重叠发病的危险因素。方法回顾性纳入2019—2023年在宁波市医疗中心李惠利医院住院治疗的SSc患者为研究对象,收集患者基线资料和实验室检查结果。依据是否合并SS将患者分为SSc组(n=91)和SSc合并SS组(n=36)。采用多因素Logistic回归分析探究SSc合并SS的危险因素。结果SSc合并SS组患者女性比例、病程、血液受累、局限性皮肤型SSc(lcSSc)、自身免疫性肝病比例高于SSc组,肺部受累比例、环磷酰胺使用比例低于SSc组(P<0.05)。SSc合并SS组患者血小板分布宽度、补体C4、抗硬皮病70抗体检出率低于SSc组,碱性磷酸酶、谷氨酰转肽酶、免疫球蛋白M(IgM)、抗着丝点蛋白B抗体(抗CENP-B抗体)、抗干燥综合征A/Ro52抗体(抗SSA/Ro52抗体)、抗干燥综合征A/Ro60抗体(抗SSA/Ro60抗体)、抗干燥综合征B抗体(抗SSB抗体)、抗线粒体M2抗体(AMA-M2)检出率高于SSc组(P<0.05)。多因素Logistic回归分析结果显示,IgM升高(OR=3.796,95%CI=1.021~14.115)、抗SSA/Ro52抗体阳性(OR=15.099,95%CI=1.750~130.264)、抗CENP-B抗体阳性(OR=11.681,95%CI=1.662~82.097)是SSc合并SS的独立危险因素(P<0.05)。结论SSc合并SS患者同时具备两者的特点,当SSc患者IgM偏高,抗SSA/Ro52抗体、抗CENP-B抗体阳性并出现相应临床症状时,应进行唇腺活检等系统全面的检查,以防漏诊。 展开更多
关键词 硬皮病 系统性 系统性硬化症 干燥综合征 临床特点 实验室特点 危险因素
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数智化全自动标本转移系统在手术病理标本前处理流程中的应用 被引量:1
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作者 张颖 雷梦君 +4 位作者 玄力娟 孙碧海 司超增 陈静 牛云 《中国护理管理》 CSCD 北大核心 2024年第6期924-928,共5页
目的:探讨在数智化管理背景下,由手术室护士主导的数智化全自动标本转移系统在手术病理标本前处理流程中的应用效果,为提高手术病理标本质量提供参考。方法:构建数智化全自动标本转移系统,并将其应用于手术病理标本前处理流程中,比较系... 目的:探讨在数智化管理背景下,由手术室护士主导的数智化全自动标本转移系统在手术病理标本前处理流程中的应用效果,为提高手术病理标本质量提供参考。方法:构建数智化全自动标本转移系统,并将其应用于手术病理标本前处理流程中,比较系统应用前(2023年4月—7月)和系统应用后(2023年8月—11月)的病理标本送检效率、送检质量、手术室护士心理压力及医护人员满意度。结果:应用数智化全自动标本转移系统后,病理标本前处理流程时长较应用前平均减少3.66 min,一次性接收合格率由87.07%提高至96.42%,病理送检质量满意率较高,护士工作压力显著缓解,医务人员满意度明显提高,以上差异均具有统计学意义(P<0.05)。结论:数智化全自动标本转移系统应用于手术标本前处理流程中可有效提高手术标本前处理效率并缓解护士工作压力,保障病理标本送检质量,为医疗诊断提供可靠的依据,有效减少医务人员职业暴露,提升医护人员满意度。 展开更多
关键词 数智化 全自动标本转移系统 手术病理标本前处理 抽真空处理 冷藏
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发生全身转移的成人胶质母细胞瘤临床及分子病理特征:个体数据汇总分析
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作者 曾令成 厉华 +4 位作者 陈如东 杨洪宽 陈坚 闵晓黎 于加省 《临床神经外科杂志》 2024年第5期488-494,共7页
目的评估成人胶质母细胞瘤(GBM)脑脊髓神经轴外全身转移的临床及分子病理特征。方法在“PubMed”“Embase”和“Web of Science”数据库中检索截至2023年12月31日报道的发生全身转移的成人GBM病例,收集汇总其临床特征,同时汇总分析报道... 目的评估成人胶质母细胞瘤(GBM)脑脊髓神经轴外全身转移的临床及分子病理特征。方法在“PubMed”“Embase”和“Web of Science”数据库中检索截至2023年12月31日报道的发生全身转移的成人GBM病例,收集汇总其临床特征,同时汇总分析报道的分子病理结果以评估转移发生的可能机制。结果通过数据库检索纳入1928年-2023年的113篇文献,共147例全身转移病例。男性多于女性,男女比例为2.3∶1;中位年龄45岁;转移灶几乎可见于全身各个部位,其中常见的转移部位为骨、肺及淋巴结;自GBM初次诊断至转移发生的中位间隔约10个月;自转移发生至患者死亡的中位间隔约4.5个月;中位总体生存约14.8个月。多因素分析显示年龄≤40岁、全切除以及未合并颅内复发与患者生存显著正相关。约39例患者(39/147,26.5%)报道了基因检测的结果。不同的研究所检测的基因项目存在区别,而且不同病例之间检测的基因结果呈现明显异质性。从报道的数量及阳性比例来看,异柠檬酸脱氢酶(IDH)野生型(阳性率27/30)、TERT启动子突变(阳性率11/13)、PTEN突变(阳性率10/11)、TP53突变(阳性率10/13)和RB1突变(阳性率8/9)为相对常见的基因突变。结论成人GBM脑脊髓神经轴外全身转移罕见,年轻患者相对多见,颅内病变的复发或进展仍为决定患者生存的关键因素。转移的发生应为多克隆基因突变进展的结果,其中,促增殖、促侵袭类基因改变例如癌基因或抑癌基因突变以及上皮间质转化类相关基因可能发挥了重要作用。 展开更多
关键词 胶质母细胞瘤 全身转移 临床特征 分子病理
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专业认证下基于项目教学法的植物病理学研究方法课程教学体系构建 被引量:1
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作者 李永刚 刘金鑫 +2 位作者 张雪 姜佰文 金羽 《黑龙江农业科学》 2024年第3期97-101,共5页
工程教育专业认证是国际通行的工程教育质量保证制度,能够为学生毕业后职业准备的范围和质量提供保证。植物病理学研究方法是东北农业大学植物保护专业认证的核心建设课程,也是具有较强应用性和实践性的实验课,是学生能够进入农业科研... 工程教育专业认证是国际通行的工程教育质量保证制度,能够为学生毕业后职业准备的范围和质量提供保证。植物病理学研究方法是东北农业大学植物保护专业认证的核心建设课程,也是具有较强应用性和实践性的实验课,是学生能够进入农业科研领域和解决农业生产实际问题的基础工具。本课程依据专业认证三大核心理念,采用项目教学法并将思政元素融入植物病理学研究方法的教学体系,力求实现“教书”“育人”“创新能力”和“职业能力”的协同一体化,进而促进学生全面发展。 展开更多
关键词 专业认证 项目教学法 教学体系 植物病理学研究方法 思政元素
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卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4鉴别附件良、恶性肿瘤
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作者 李健 刘芳欣 +5 位作者 戚建国 许廷兰 任永凤 王洲 陈飞 李姿灼 《中国医学影像技术》 CSCD 北大核心 2024年第6期893-897,共5页
目的观察卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4(RMI4)鉴别附件良、恶性肿瘤的价值。方法回顾性分析126例手术病理诊断为附件肿瘤患者,根据O-RADS US v2022将1~3类归为良性病变、4~5类归为恶性病变,... 目的观察卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4(RMI4)鉴别附件良、恶性肿瘤的价值。方法回顾性分析126例手术病理诊断为附件肿瘤患者,根据O-RADS US v2022将1~3类归为良性病变、4~5类归为恶性病变,以450为RMI4分类的临界值,基于二者进行联合分类。以病理结果为金标准,绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评估单一O-RADS US v2022、RMI4及其联合鉴别附件良、恶性肿瘤的效能。结果126例附件肿瘤中,良性94例、恶性32例。O-RADS US v2022鉴别附件良、恶性肿瘤的敏感度、特异度、准确率及AUC分别为78.13%、80.85%和80.16%、0.795,RMI4分别为71.88%、84.04%和80.95%、0.780;二者联合的特异度及准确率(93.62%、92.06%)均高于单一O-RADS US v2022(χ^(2)=7.322、5.967,P=0.007、0.015)或RMI4(χ^(2)=4.625、5.331,P=0.032、0.021),而敏感度及AUC(87.50%、0.906)差异均无统计学意义(P均>0.05)。结论O-RADS US v2022能有效鉴别附件良、恶性肿瘤,联合RMI4可提高鉴别特异度及准确率。 展开更多
关键词 卵巢肿瘤 病理学 临床 诊断 鉴别 卵巢-附件报告和数据系统 恶性风险指数4
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“双导师制”在病理专硕研究生实践创新能力培养中的探索
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作者 张文敏 陈丽红 +5 位作者 陈裕庆 陈刚 曲利娟 林洁 周子雄 黄爱民 《中国继续医学教育》 2024年第12期187-190,共4页
专业学位研究生教育是培养高层次应用型专门人才的主渠道,学校自从2015年开始招收病理专硕研究生以来,在病理专硕培养与住院医师规范化培训“双轨合一”过程中遇到培训模式单一、缺乏科研思维训练、基础病理与临床病理隔离等。针对以上... 专业学位研究生教育是培养高层次应用型专门人才的主渠道,学校自从2015年开始招收病理专硕研究生以来,在病理专硕培养与住院医师规范化培训“双轨合一”过程中遇到培训模式单一、缺乏科研思维训练、基础病理与临床病理隔离等。针对以上问题,本课题整合基础医学院病理学系和医院病理科的骨干力量,组建双导师团队,将“双导师制”培养模式贯穿于临床病理学研究生培养全过程,包括研究生录取、培养方案制定、病理规范化培训、科研训练、开题、毕业答辩等各个环节,充分发挥学校和医院的优势,取得一定成效。该培养模式不仅促进基础与临床的深度融合,而且对培养具有较高病理诊断水平、解决临床问题能力的高素质临床病理人才有积极的推动作用,有力提升了病理专硕研究生的实践创新能力。 展开更多
关键词 病理学 专业学位研究生 双导师制 规范化培训 能力 探索实践
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