期刊文献+
共找到213篇文章
< 1 2 11 >
每页显示 20 50 100
Ziwan-Taoren herb pair can exert an therapeutical effect in primary Sjogren’s syndrome through inhibiting the TLR/NF-κB pathway
1
作者 Kuok-Tong Lei Yun-Xia Wu +5 位作者 Yun Lu Zi-Shan Wang Thi-Huong Nguyen Qiu-Ying Cai Wen Zhu Yue Wang 《Traditional Medicine Research》 2024年第8期11-22,共12页
Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still un... Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway. 展开更多
关键词 traditional Chinese medicine primary sjogren’s syndrome NF-κB RELA
下载PDF
Renal Vein Thrombosis Suggestive of Extramembranous Glomerulonephritis Associated with Sjögren’s Syndrome (Case Report)
2
作者 Mariam El Galiou Mina Agrou +4 位作者 Rihab Dkhissi Loubna Benamar Naima Ouzeddoun Lamyae Rouass Tarik Bouattar 《Open Journal of Internal Medicine》 2024年第2期181-187,共7页
Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the cas... Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis. 展开更多
关键词 sgren’s syndrome Extramembranous Glomerulonephritis Nephrotic syndrome Anti-PLA2R Antibodies
下载PDF
EVALUATION OF INTERNATIONAL CLASSIFICATION CRITERIA (2002) FOR PRIMARY SJGREN'S SYNDROME IN CHINESE PATIENTS 被引量:4
3
作者 Yan Zhao Jian Kang +4 位作者 Wen-jie Zheng Wei Zhou Xiao-ping Guo Yan Gao Yi Dong 《Chinese Medical Sciences Journal》 CAS CSCD 2005年第3期190-193,共4页
Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjogren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients.... Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjogren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Mothoda Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease (GVHD), and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies (including anti-SSA/SSB antibodies) test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched (47.8 ± 10.9 years vs. 46.2±13.6 years, P 〉 0.05). The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB antibodies were diagnosed as pSS by lower lip biopsy. Coadwion The international classification criteria (2002) for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice. 展开更多
关键词 primary sjogrens syndrome CRITERIA CLAssIFICATION
下载PDF
Iguratimod in treatment of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia:A case report 被引量:1
4
作者 Juan Zhang Xin Wang +4 位作者 Jing-Jing Tian Rong Zhu Rui-Xue Duo Yi-Chen Huang Hai-Li Shen 《World Journal of Clinical Cases》 SCIE 2022年第4期1286-1290,共5页
BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.Ho... BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease. 展开更多
关键词 Autoimmune hemolytic anemia IGURATIMOD primary sgren’s syndrome Case report
下载PDF
Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjogren's syndrome 被引量:1
5
作者 Li-na Gu Min Zhang +1 位作者 Hui Zhu Jing-yao Liu 《Neural Regeneration Research》 SCIE CAS CSCD 2016年第10期1633-1637,共5页
Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogr... Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogreffs syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren's-syndrome-related antigen A an- tibodies, anti-Sjogren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjogren's syndrome and in 60% (6/10) of patients without primary Sj6gren's syndrome. More brain abnormalities were observed in patients without primary Sj6gren's syndrome than in those with primary Sj6gren's syndrome. Segments lesions (〉 3 centrum) were noted in 50% (5/10) of patients without primary Sj6gren's syndrome and in 67% (4/6) of patients with primary Sjogren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities. 展开更多
关键词 nerve regeneration neuromyelitis optica primary sjogrens syndrome neuromyelitis optica spectrum disorder xerostomiaxerophthalmia neurological involvements magnetic resonance imaging anti-aquaporin 4 neural regeneration
下载PDF
Management of primary ciliary dyskinesia/Kartagener's syndrome in infertile male patients and current progress in defining the underlying genetic mechanism 被引量:16
6
作者 Yan-Wei Sha Lu Ding Ping Li 《Asian Journal of Andrology》 SCIE CAS CSCD 2014年第1期101-106,共6页
Kartagener's syndrome (KS) is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia (PCD). As it is accompanied by many complications, PCD/KS severely ... Kartagener's syndrome (KS) is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia (PCD). As it is accompanied by many complications, PCD/KS severely affects the patient's quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility. 展开更多
关键词 assisted reproductive technology clinical diagnosis and treatment genetic mechanism Kartagener's syndrome malefertifity primary ciliary dyskinesia
下载PDF
Role of ranulas in early diagnosis of Sjögren’s syndrome: A case report 被引量:1
7
作者 Na Chen Da-Shun Zeng Yu-Tong Su 《World Journal of Clinical Cases》 SCIE 2021年第20期5701-5708,共8页
BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult... BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult patient with SS,who developed a unilateral simple ranula and was diagnosed primary SS 3 years later.We also provide a review of cases of SS and ranulas from 1980 to 2020.CASE SUMMARY A 22-year-old girl was found to have a left painless floor-of-mouth lesion 3 years ago,without obvious trauma or inducement.The diagnosis of a unilateral(left)simple ranula was made,and the ranula was surgically treated.Within 3 years after the ranula surgery,she developed acute lymphadenectasis in unilateral parotid twice without inducement,and ultrasonic examination revealed diffuse lesions in bilateral parotids and submandibular glands,which strongly suggested SS.Serologic tests and the unstimulated whole saliva flow rate confirmed the SS diagnosis.CONCLUSION Our study underlines that ranulas are early clinical signs of SS.As early diagnosis and early intervention of SS are important to obtain better outcomes,our findings underline the need for histopathological test after sublingual adenectomy and imaging detection of exocrine glands for the patients with ranulas. 展开更多
关键词 sgren’s syndrome Ranulas Early diagnosis PAROTITIs Case report
下载PDF
Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome:A case report 被引量:1
8
作者 Hiroki Teragawa Chikage Oshita +4 位作者 Yuichi Orita Kunihiro Hashimoto Hirofumi Nakayama Yuto Yamazaki Hironobu Sasano 《World Journal of Clinical Cases》 SCIE 2021年第5期1119-1126,共8页
BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative disea... BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative diseases of AI.However,the coexistence of PA and SCS has been reportedly observed.Recently,we encountered a case of AI,in which PA and SCS coexisted,confirmed by histopathological examinations after a laparoscopic adrenalectomy.We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARY A 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg.A subsequent computed tomography scan revealed right ureterolithiasis,which was the cause of right abdominal pain,and right AI measuring 22 mm×25 mm.After the disappearance of right abdominal pain,subsequent endocrinological examinations were performed.Aldosterone-related evaluations,including adrenal venous sampling,revealed the presence of bilateral PA.In addition,several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma.A laparoscopic right adrenalectomy was then performed.The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma,while CYP11B2 immunoreactivity was absent in this adenoma.However,in the adjacent nonneoplastic adrenal,multiple CYP11B2-positive adrenocortical micronodules were detected,showing the presence of aldosterone-producing adrenocortical micronodules.CONCLUSION Careful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA. 展开更多
关键词 primary aldosteronism subclinical Cushing’s syndrome Adrenal incidentaloma Micronodular hyperplasia Case report
下载PDF
Peripheral Neuropathies Revealing Gougerot-Sjögren’s Syndrome: Description of 3 Cases
9
作者 Ndiaga Matar Gaye Alassane Mamadou Diop +6 位作者 Khalifa Ababacar Mbaye Serigne Abdou Aziz Fall Mamadou Ka Momo Banda Ndiaye Maouly Fall Moustapha Ndiaye Amadou Gallo Diop 《Neuroscience & Medicine》 CAS 2022年第3期120-125,共6页
Introduction: Sj&#246;gren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of periphe... Introduction: Sj&#246;gren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of peripheral neuropathy revealing Gougerot-Sj&#246;gren’s syndrome, collected in the Neurology Department of the Fann University Hospital in Dakar (Senegal). Observations: The first patient, aged 48 years, presented with a length-dependent sensitivomotor polyneuropathy associated with retrobulbar optic neuritis, with dry eyes and dry mouth noticed by the patient for several years. The second patient, aged 28 years, was admitted to the hospital with chronic generalized paresthesia in the context of xerostomia and xerophthalmia. The results of the clinical examination and the electroeneuromyogram were in favour of pure sensory neuronopathy. The third patient was 32 years old female, with a history of thyroidectomy and acute inflammatory demyelinating polyneuropathy (AIDP), who was seen for acute ascending flaccid tetraplegia with facial diplegia, preceded by diffuse paresthesia. The diagnosis of recurrence of acute demyelinating polyradiculonueropathy was retained in view of the rapidly increasing character of the deficit, the hyperproteinorachy at the lumbar puncture, and the signs of demyelination at the ENMG. The diagnosis of Gougerot-Sj&#246;gren’s syndrome in our three patients was established on the basis of the 2016 ACR/EULAR criteria. Indeed, the anti-SSA antibodies (Ro) were positive in our 3 patients with a biopsy of the salivary glands which showed stage 3 in the first patient and stage 4 in the two others. Corticosteroid therapy and immunosuppressive treatment resulted in a favourable clinical evolution on the neurological and general levels. Conclusion: Gougerot-Sj&#246;gren’s syndrome is an autoimmune exocrinopathy that may present with peripheral neuropathy, which may precede the diagnosis of Sj&#246;gren’s syndrome, be concomitant or occur during the course of the disease. 展开更多
关键词 sgren’s syndrome Peripheral Neuropathy salivary Gland Biopsy senegal
下载PDF
Sjögren’s Syndrome Revealed by Obstructive Renal Failure: A Case Report and Review of the Literature
10
作者 Mamadou Badou Sanogo Aboubacar Sidiki Fofana +6 位作者 Atabième Kodio Sidy Toure Magara Samake Seydou Sy Alkaya Toure Hamadoun Yattara Saharé Fongoro 《Open Journal of Nephrology》 CAS 2022年第4期375-381,共7页
Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between ... Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between 0.1% and 0.6%. This observation illustrates an incidental finding of a case of SS in a young female patient in a context of obstructive renal failure (ARF) due to uterine fibroids. Observation: This was a 31-year-old woman hospitalized for anuric AKI (Acute Kidney Injury) with a creatinine level of 1247 μmol/l. Her history included sickle cell disease A/C and an unoperated uterine fibroid diagnosed 3 years ago. Approximately 2 months before her admission, her symptomatology was made of dizziness, physical asthenia, vomiting, poly-arthralgia, morning rash, pollakiuria and oral dryness. Abdominal examination showed a painless transverse mass in the pelvis. Biological examination showed a CRP (C-reactive protein) level of 488 mg/l. The cytobacteriological examination of the urine was normal and the proteinuria was 1.35 g/24 hours. The CT scan showed kidneys measuring 110 mm on the right and 113 mm on the left associated with bilateral pyelo-caliceal dilatation on a large polymyomatous uterus of interstitial and submucosal type. Immunologically, the anti-nuclear factor, the rheumatoid factor and the anti-SSA antibodies were positive. The resumption of the interrogation within the framework of the research of the subjective dry syndrome to find a notion of intermittent xerophthalmia 4 months ago. The Schirmer test was positive in the left eye. The initial management consisted of a polymyomectomy after 3 sessions of hemodialysis. Background treatment combining prednisone 5 mg/day and methotrexate 20 mg/week was started in parallel with the use of artificial tears. The evolution after twelve (12) months of treatment was favorable with a complete disappearance of the signs dry syndrome and full recovery of renal function. Conclusion: SS can have an insidious evolution and remain stable for many years, hence its fortuitous discovery in this case of obstructive ARF on uterine fibroid. In this context we insist on the interest of the immunological assessment in a patient in period of genital activity with a significant proteinuria and non-specific extrarenal signs. 展开更多
关键词 Obstructive Renal Failure FIBROID sgren’s syndrome
下载PDF
Gastrointestinal microbiome and primary Sjogren’s syndrome: a review of the literature and conclusions
11
作者 Yu-Feng Yao Mei-Ying Wang Xiao-Yan Dou 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2022年第11期1864-1872,共9页
The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjo... The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications. 展开更多
关键词 gastrointestinal microbiome primary sjogren’s syndrome dry eye dysbiosis autoimmunity clinical therapy
下载PDF
Primary thymic mucosa-associated lymphoid tissue lymphoma with multiple thin walled lung cysts: case report and literature review 被引量:5
12
作者 Lung-Yun Kang Szu-Pei Ho Yi-Pin Chou 《Chinese Journal of Cancer Research》 SCIE CAS CSCD 2013年第3期354-357,共4页
Mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus is rare. We reported a case of a 37-year-old Chinese female with Sjtgren's syndrome and hyperglobulinemia. She suffered from chronic cough for 3 weeks... Mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus is rare. We reported a case of a 37-year-old Chinese female with Sjtgren's syndrome and hyperglobulinemia. She suffered from chronic cough for 3 weeks. Chest computed tomography (CT) demonstrated a multiloculated cystic mass in mediastinum prevascular space and multiple lung cysts. Laboratory exam of autoimmune markers showed positive of antinuclear antibody (ANA), Sjtgren's syndrome A (SSA), Sjtgren's syndrome B (SSB), and rheumatoid factors (RF). Thymectomy with lymph node dissection was performed. The pathology report revealed thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Under immunohistochemical stains, CD20 and Bcl-2 were positive. No evidence of recurrence of disease was found. 展开更多
关键词 Mucosa-associated lymphoid tissue (MALT) lymphoma THYMUs sj6grens syndrome hyperglobulinemia
下载PDF
Concomitant extrahepatic autoimmune diseases do not compromise the long-term outcomes of primary biliary cholangitis 被引量:2
13
作者 Sha Chen Meng-Qi Li +5 位作者 Wei-Jia Duan Bu-Er Li Shu-Xiang Li Ting-Ting Lv Lin Ma Ji-Dong Jia 《Hepatobiliary & Pancreatic Diseases International》 SCIE CAS CSCD 2022年第6期577-582,共6页
Background:Primary biliary cholangitis(PBC)patients often have concomitant extrahepatic autoimmune(EHA)diseases including Sjögren’s syndrome(SS),systemic sclerosis(SSc),rheumatoid arthritis(RA),and autoimmune th... Background:Primary biliary cholangitis(PBC)patients often have concomitant extrahepatic autoimmune(EHA)diseases including Sjögren’s syndrome(SS),systemic sclerosis(SSc),rheumatoid arthritis(RA),and autoimmune thyroid disease.The present study aimed to describe the prevalence of EHA diseases in PBC and explore the impact of EHA diseases on the long-term outcomes of PBC in Chinese patients.Methods:Medical records of PBC patients diagnosed in our institute were retrospectively reviewed.Pa-tients were followed up by a standardized telephone interview.The endpoints were defined as liver-related death and/or liver transplantation.Results:Totally 247 of the 985(25.1%)PBC patients enrolled in the study had at least one concomi-tant EHA disease.Sjögren’s syndrome(n=140,14.2%)was the most frequent one,followed by rheuma-toid arthritis(RA)(n=56,5.7%)and Hashimoto’s thyroiditis(n=45,4.6%).Patients with EHA dis-eases were more common in females(P<0.001)and in those with a family history of autoimmune disease(P=0.017).Overall,no differences were found between PBC patients with and without EHA dis-eases in terms of biochemical response rates to ursodeoxycholic acid,the incidence of hepatic events,or transplant-free survival.RA and EHA≥2 were protective factors for hepatic events in univariate Cox analysis,but the results became insignificant in multivariate analysis.Conclusions:Concomitant EHA diseases were common in PBC patients but did not compromise the long-term outcomes of PBC. 展开更多
关键词 primary biliary cholangitis Extrahepatic autoimmune diseases sgren’s syndrome Rheumatoid arthritis Prognosis
下载PDF
Clinical spectrum of primary ciliary dyskinesia in childhood 被引量:3
14
作者 Andrew Fretzayas Maria Moustaki 《World Journal of Clinical Pediatrics》 2016年第1期57-62,共6页
Although the triad of bronchiectasis, sinusitis and situs inversus was first described by Kartagener in 1933, the clinical spectrum of primary ciliary dyskinesia is still under investigation. Heterotaxy defects as wel... Although the triad of bronchiectasis, sinusitis and situs inversus was first described by Kartagener in 1933, the clinical spectrum of primary ciliary dyskinesia is still under investigation. Heterotaxy defects as well as upper and lower respiratory tract symptoms are the main manifestations in childhood. It is now recognized that situs inversus is encountered in only half of patients. The first lower respiratory symptoms may be present from infancy as neonatal respiratory distress. The most common lower airway manifestations are chronic wet cough, recurrent pneumonia and therapy resistant wheezing. Patients are at risk of developing bronchiectasis which may even be the presenting finding due to delayed diagnosis. Upper respiratory tract infections such as nasal congestion, nasal drainage and recurrent sinusitis as well as otologic manifestations such as otitis media or otorrhea with conductive hearing loss are also often encountered. It seems that the type of ciliary ultrastructure defects and the involved mutated genes are associated to some extent to the clinical profile. The disease, even in nowadays, is not recognized at an early age and the primary care clinician should have knowledge of its clinical spectrum in order to select appropriately the children who need further investigation for the diagnosis of this disorder. 展开更多
关键词 primary CILIARY DYsKINEsIA Kartagener’s syndrome Immotile CILIA HETEROTAXY Respiratory TRACT
下载PDF
组织蛋白酶S在原发性干燥综合征中的临床意义 被引量:5
15
作者 李霞 张丽丽 +1 位作者 费允云 赵岩 《中华临床免疫和变态反应杂志》 2016年第1期46-51,84,共7页
目的探讨组织蛋白酶(Cathepsin,Cat)S在原发性干燥综合征(primary Sjgren’s syndrome,p SS)患者中的临床意义。方法通过酶联免疫吸附法检测p SS患者及健康对照组血清总Cat S和pro-Cat水平,并分析比较其在p SS患者血清中的变化及其与... 目的探讨组织蛋白酶(Cathepsin,Cat)S在原发性干燥综合征(primary Sjgren’s syndrome,p SS)患者中的临床意义。方法通过酶联免疫吸附法检测p SS患者及健康对照组血清总Cat S和pro-Cat水平,并分析比较其在p SS患者血清中的变化及其与欧洲抗风湿联盟p SS疾病活动度(EULAR Sjgren’s syndrome disease activity index,ESSDAI)评分和Ig G水平之间的相关性;免疫组化检测唇腺组织Cat S的表达。采用受试者工作特征曲线(receiver operating characteristic curve,ROC曲线)、t检验、Spearman相关性分析进行统计学分析。结果 p SS组(n=37)血清总Cat S、pro-Cat S和active-Cat S表达水平高于健康对照组(n=33),差异有统计学意义[总Cat S(18.70±2.53)ngml vs.(15.10±3.16)ngml;pro-Cat S(14.14±3.56)ngml vs.(12.20±2.92)ngml;active-Cat S(4.56±2.49)ngml vs.(2.90±1.95)ngml,P<0.05];而高免疫球蛋白血症(Ig G>25 gL)组与非高免疫球蛋白组比较,三者表达水平差异无统计学意义(P>0.05)。p SS患者血清总Cat S、proCat S及active-Cat S水平与ESSDAI评分及血清Ig G水平无明确相关性(P>0.05)。以2002年p SS国际分类诊断标准作为参照,ROC分析发现,血清总Cat S、pro-Cat S和active-Cat S水平曲线下面积分别为0.811(P<0.01)、0.655(P<0.01)和0.701(P<0.01);最佳阈值分别为15.77、12.62、4.06 ngml;敏感性和特异性分别为0.919和0.697、0.676和0.667、0.622和0.758。唇腺组织免疫组化发现p SS患者唇腺腺上皮细胞、导管上皮细胞及淋巴细胞均表达Cat S升高,而对照者未见明显表达。结论血清总Cat S、pro-Cat S、active-Cat S升高,唇腺组织表达Cat S升高,可能通过增强自身抗原呈递、降解细胞外基质、促进淋巴细胞浸润等作用,在p SS患者发病中发挥重要作用,这也为p SS患者提供了一种新的血清学标志物及潜在的治疗靶点。 展开更多
关键词 原发性干燥综合征 组织蛋白酶s 生物标志物 唇腺活检
下载PDF
ETS1基因多态性与原发性干燥综合征的相关性 被引量:3
16
作者 孙菲 李萍 +7 位作者 徐涓娟 陈华 吴子燕 栾海霞 张文 田新平 李永哲 张奉春 《中华临床免疫和变态反应杂志》 2013年第2期103-108,共6页
目的探讨ETS1基因单核苷酸多态性(single nucleotide polymorphism,SNP)与汉族人群原发性干燥综合征(primary sjgren’s syndrome,pSS)遗传易感性是否相关。方法提取261例pSS患者和597例健康对照者的全基因组DNA。使用Sequenom MassAr... 目的探讨ETS1基因单核苷酸多态性(single nucleotide polymorphism,SNP)与汉族人群原发性干燥综合征(primary sjgren’s syndrome,pSS)遗传易感性是否相关。方法提取261例pSS患者和597例健康对照者的全基因组DNA。使用Sequenom MassArray方法对ETS1基因上2个SNP位点(rs6590330和rs1128334)进行基因分型。分型结果使用PLINK 1.07软件进行统计分析。结果 rs6590330位点GG、GA、AA基因型频率和G、A等位基因频率分布在pSS组和对照组间差异无统计学意义(均P>0.05)。加性模型、显性模型、隐性模型下的分析显示,两组间差异仍无统计学意义(均P>0.05)。将pSS患者按照性别及抗SSA和抗SSB抗体产生情况分组,分析仍未发现任何相关性。rs1128334分型成功率过低无法分析。结论 ETS1基因的SNP位点rs6590330与汉族人群pSS患者遗传易感性不相关。 展开更多
关键词 原发性干燥综合征 ETs1 单核苷酸多态性
下载PDF
原发性失眠患者中医证型与神经肽S受体基因rs324981多态性的相关性 被引量:4
17
作者 杨军 谢宇平 +3 位作者 苏晓艳 马薇 惠培林 周丽雅 《中医药导报》 2021年第9期129-132,共4页
目的:探讨原发性失眠(PI)患者中医证型与神经肽S受体(NPSR)基因rs324981多态性的相关性。方法:纳入154例PI患者,进行中医辨证分型,并匹配130名健康者为对照组。采用匹兹堡睡眠质量指数量表(PSQI)评估受试者睡眠质量。使用Mass ARRAY SN... 目的:探讨原发性失眠(PI)患者中医证型与神经肽S受体(NPSR)基因rs324981多态性的相关性。方法:纳入154例PI患者,进行中医辨证分型,并匹配130名健康者为对照组。采用匹兹堡睡眠质量指数量表(PSQI)评估受试者睡眠质量。使用Mass ARRAY SNP基因分型技术检测受试者NPSR基因rs324981多态性,并分析PI患者不同中医证型与NPSR基因rs324981多态性的相关性。结果:154例PI患者中医辨证为肝郁化火证47例(30.52%),痰热内扰证16例(10.39%),阴虚火旺证60例(38.96%),心脾两虚证24例(15.58%),心虚胆怯证7例(4.55%)。PI阴虚火旺证患者NPSR基因rs324981多态性基因型分布与对照组比较,差异有统计学意义[P=0.034,OR=2.10,95%CI(1.05, 4.19)]。PI虚证和实证与对照组NPSR基因rs324981多态性的基因型和等位基因分布比较,差异均无统计学意义(P>0.05)。结论:PI患者阴虚火旺证可能与NPSR基因rs324981多态性存在相关性。 展开更多
关键词 原发性失眠 中医辨证 神经肽s受体 基因多态性
下载PDF
原发性干燥综合征患者血清sHLA-G检测分析 被引量:1
18
作者 张雄鹰 郭欢 +4 位作者 程红兵 李建宏 王浩 王丽丽 贺志花 《长治医学院学报》 2013年第6期410-412,共3页
目的:探讨血清可溶性人类白细胞抗原-G(sHLA-G)含量与原发性干燥综合征(pSS)的关系。方法:用酶联免疫吸附测定(ELISA)法检测71例pSS患者和69例健康对照者血清中sHLA-G含量。结果:69例健康人血清sHLA-G的浓度最低为1.43U/mL,最高为32.76U... 目的:探讨血清可溶性人类白细胞抗原-G(sHLA-G)含量与原发性干燥综合征(pSS)的关系。方法:用酶联免疫吸附测定(ELISA)法检测71例pSS患者和69例健康对照者血清中sHLA-G含量。结果:69例健康人血清sHLA-G的浓度最低为1.43U/mL,最高为32.76U/mL,平均值范围(14.35±8.88)U/mL。71例pSS患者sHLA-G的浓度最低为1.05U/mL,最高为28.82U/mL,平均值范围(8.43±7.24)U/mL。pSS患者血清sHLA-G水平显著低于健康对照组(P<0.01)。结论:pSS患者血清sHLA-G水平显著降低,sHLA-G水平降低可能参与了pSS的病理过程。 展开更多
关键词 原发性干燥综合征 可溶性人类白细胞抗原-G 酶联免疫吸附试验
下载PDF
血清淀粉样蛋白酶P(SAP)在3种常见结缔组织病患者中的表达及其意义 被引量:1
19
作者 吴丽芬 范倩 +5 位作者 陈风华 孙文闻 刘永玉 姬海燕 魏蔚 巩路 《中国医学创新》 CAS 2014年第30期124-126,共3页
目的:研究血清淀粉样蛋白P(SAP)在3种常见结缔组织病患者血清中含量,探讨其在结缔组织病发生发展中的作用及意义。方法:用ELISA方法测定正常人、系统性红斑狼疮活动期和缓解期、类风湿关节炎活动期和缓解期、初治干燥综合征等风湿性疾... 目的:研究血清淀粉样蛋白P(SAP)在3种常见结缔组织病患者血清中含量,探讨其在结缔组织病发生发展中的作用及意义。方法:用ELISA方法测定正常人、系统性红斑狼疮活动期和缓解期、类风湿关节炎活动期和缓解期、初治干燥综合征等风湿性疾病血清中血清淀粉样蛋白P(SAP)含量。统计各型患者血清中血清淀粉样蛋白P(SAP)的表达水平及其意义。结果:SAP在系统性红斑狼疮患者活动期血清中高表达,且其表达水平显著高于缓解期和正常人,差异均有统计学意义(P<0.05)。结论:血清淀粉样蛋白P(SAP)在系统性红斑狼疮活动期高表达,可以作为系统性红斑狼疮患者活动期的诊断指标之一。 展开更多
关键词 血清淀粉样蛋白酶P(sAP) 系统性红斑狼疮 类风湿关节炎 干燥综合征 serum amyloid P component(sAP) systemic LUPUs erythematosus(sLE) RHEUMATOID arthritis(RA) primary sjogren’s syndrome(Pss)
下载PDF
化学发光法与免疫印迹法检测抗SSA和SSB抗体的临床对比研究 被引量:3
20
作者 吕志文 申爱华 +3 位作者 陈小三 柳乐 李庆春 李纪阳 《国际检验医学杂志》 CAS 2018年第15期1845-1848,1852,共5页
目的比较化学发光法(CLIA)与免疫印迹法(LIA)针对抗SSA和SSB抗体的临床检测性能。方法应用CLIA和LIA对255例系统性红斑狼疮(SLE)、34例干燥综合征(SjS)、40例类风湿关节炎(RA)、25例系统性硬化症(SSc)及60例体检对照(HI)样本开展抗SSA和... 目的比较化学发光法(CLIA)与免疫印迹法(LIA)针对抗SSA和SSB抗体的临床检测性能。方法应用CLIA和LIA对255例系统性红斑狼疮(SLE)、34例干燥综合征(SjS)、40例类风湿关节炎(RA)、25例系统性硬化症(SSc)及60例体检对照(HI)样本开展抗SSA和SSB抗体平行检测。比较两种方法抗SSA和SSB抗体的检测性能和符合率。并对两种方法检测结果的差异样本,采用第三种检测方法(ELISA)进行对比复测。结果两种方法检测抗SSA抗体时,总符合率为96.3%(Kappa=0.92,P<0.01)。而在抗SSB抗体则为95.8%(Kappa=0.85,P<0.01)。两种方法的差异样本(包括抗SSA和SSB抗体)合计33例,其中21例样本ELISA复测结果与CLIA检测结果相符合。结论 CLIA与LIA在检测抗SSA和SSB抗体时具有良好的符合率和一致性。由于具备全自动、定量检测和随机上样等显著特点,因此CLIA更适合临床抗SSA和SSB抗体检测。 展开更多
关键词 化学发光法 ssA抗体 ssB抗体 系统性红斑狼疮 干燥综合征 免疫印迹法
下载PDF
上一页 1 2 11 下一页 到第
使用帮助 返回顶部