AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high...AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high and low visual acuities and contrast sensitivity functions.These tests were conducted under photopic and then mesopic conditions.Twenty-one Sjögren’s syndrome patients and 21 aged-matched healthy volunteers completed all the measurements in this study.RESULTS:Sjogren’s syndrome patients have greater impairment in contrast sensitivity than standardized visual acuity.This reduction was significant under the mesopic condition.Also,Sjogren’s syndrome patients treated with pilocarpine suffer more than patients without pilocarpine treatment under low light conditions.CONCLUSION:Sjogren’s syndrome patients shows greater impairment in different visual resolution tasks due to dry eye symptoms.展开更多
AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a majo...AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a major form of aqueous deficient DED and meibomian gland dysfunction(MGD), a common cause of evaporative DED.METHODS: A total 135 eyes of 135 subjects included in this study: patients with DED associated with coGVHD(n=30), patients with SS(n=35), patients with MGD(n=35), and normal controls(n=35). All participants completed the Ocular Surface Disease Index(OSDI) questionnaire, ocular surface examination [Schirmer test, tear film breakup time(TFBUT), and ocular surface staining], and meibomian gland assessment [meiboscore(gland dropout detected on meibography using infrared camera of the Keratograph 5 M), meibum expressibility score(MES), meibum quality score(MQS), lid margin abnormality]. In addition, correlations of meibomian gland characteristics with ocular surface parameters as well as disease severity score were investigated in coGVHD group.RESULTS: The coGVHD group showed significantly higher meiboscore, MES, and MQS than the other 3 groups(all P<0.05). In the coGVHD group, parameters of meibomian gland showed a significant correlation each other and those of ocular surface. The correlation between meibomian gland parameters and severity score of co GVHD was also established(meiboscore, r=0.62; MES, r=0.47; MQS, r=0.47; lid margin abnormality score, r=0.55; all P<0.05).CONCLUSION: Patients with DED associated with co GVHD show poorer gland morphology and worse glandfunction than other types of DED. In addition, meibomian gland damage is not only associated with ocular surface damage but also disease severity of coGVHD.展开更多
BACKGROUND Primary Sjogren's syndrome(pSS)is an autoimmune disease,and renal involvement has been considered to be one of the systemic complications of pSS.Patients who have sjogren's syndrome with renal disea...BACKGROUND Primary Sjogren's syndrome(pSS)is an autoimmune disease,and renal involvement has been considered to be one of the systemic complications of pSS.Patients who have sjogren's syndrome with renal disease as the first manifestation and no exocrine gland involvement or autoantibodies can be missed clinically.CASE SUMMARY We here in report an unusual case of a primary Sjogren's syndrome in a 43-yearold female who had minimal lesion nephropathy as the initial presentation,and the patient was negative for serum anti-SSA and anti-SSB antibodies and did not have signs of exocrine gland involvement.The patient’s Sjogren's syndrome was confirmed by a minor salivary gland biopsy(MSGB)and a filter paper test.the patient’s oedema subsided,and the patient’s urinary protein resolved,showing that the treatment was effective.CONCLUSION MSGB should be considered if pSS is suspected in patients who do not have the typical pSS symptoms or who are positive for the specific autoantibodies.展开更多
BACKGROUND Subclavian steal syndrome(SSS)caused by Sjogren's syndrome is rare,especially for elderly patients with risk factors for atherosclerosis.The current report presents the uncommon etiology and treatment o...BACKGROUND Subclavian steal syndrome(SSS)caused by Sjogren's syndrome is rare,especially for elderly patients with risk factors for atherosclerosis.The current report presents the uncommon etiology and treatment of SSS,aiming to improve doctor’s clinical experience.CASE SUMMARY A 69-year-old man was diagnosed with hypertension and acute cerebral infarction presenting with left upper limb weakness and pain even gradually aggravating to left limb hemiplegia 30 years ago.He was managed with antihypertensive and antithrombotic therapy;however,his condition was recurrent,and he never had any further examination.It was found that the difference of the bilateral upper arm systolic pressure was over 20 mmHg,and Doppler examination showed that the blood flow of the left vertebral artery was reversed,suggesting SSS.Further tests revealed a benign lymphoepithelial lesion in salivary gland tissue,confirming the Sjogren's syndrome.CONCLUSION The patient was found to have hypertension when he was 33 years old,and the blood pressure of both sides was asymmetric,which was ignored.The patient's symptoms of dizziness and upper limb weakness were misdiagnosed as general cerebral infarction.It is necessary to test the aorta computed tomography angiography to prove secondary hypertension factors such as Sjogren's syndrome.展开更多
Objective:To systematically evaluate the efficacy and safety of traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of Sjogren's Syndrome(SS).Methods:Two researchers searched the...Objective:To systematically evaluate the efficacy and safety of traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of Sjogren's Syndrome(SS).Methods:Two researchers searched the databases of Chinese National Knowledge Infrastructure(CNKI),Wanfang Database,Weipu Database,Chinese Biomedical Literature Database,PubMed Database,Web of science Database and The Cochrane Library Database(the retrieval period is from the establishment of the database to October 2020)and screened the literatures independently.The data were extracted for bias risk assessment,and then meta-analysis was performed using Revman 5.3 software.Results:A total of 19 articles involving 1185 patients were included,including 617 cases in the treatment group and 568 cases in the control group.Combined data analysis showed that the total effective rate of traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of Sjogren's syndrome was significantly higher than that of hydroxychloroquine sulfate alone[R R=1.34,95%CI(1.24,1.44),P<0.00001].The improvement of salivary gland[SMD=0.66,95%CI(0.47,0.85),P<0.00001],tear secretion function[MD=2.56,95%CI(1.29,3.83),P<0.0001]and reducing CRP[MD=-3.53,95%CI(-3.93,-3.14),P<0.00001],ESR[MD=-5.80,95%CI(-8.48,-3.13),P<0.00001]were better than those of the control group.However,there was no significant difference in the reduction of IgG and the incidence of adverse reactions between the two groups.Conclusion:Traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of SS is superior to the control group in total effective rate,improvement of salivary gland and tear secretion function,and reduction of CRP and ESR,and we didn’t see the significantly increase on the adverse event.However,due to the small sample size and low quality of the included studies,the conclusion needs to be verified by larger sample and higher quality randomized controlled trials.展开更多
Objective:To observe the relationship between the distribution of TCM Syndromes of primary Sjogren's syndrome(pSS)and the changes of erythrocyte sedimentation rate(ESR),C-reactive protein(CRP),tumor necrosis fact...Objective:To observe the relationship between the distribution of TCM Syndromes of primary Sjogren's syndrome(pSS)and the changes of erythrocyte sedimentation rate(ESR),C-reactive protein(CRP),tumor necrosis factor-α(TNF-α),interleukin-6(IL-6),immunoglobulin IgA,IgG and IgM.Methods:157 diagnosed PSS patients were divided into dampness heat obstructing collaterals syndrome,dryness evil invading lung syndrome,Qi blood stasis syndrome,liver kidney yin deficiency syndrome and Qi Yin deficiency syndrome according to TCM syndrome differentiation standard.The age,course of disease,general health score in international universal quality of life scale,self rating Anxiety scale(SAS),self rating Depression Scale(SDS)and erythrocyte sedimentation rate were observed and detected(ESR,C-reactive protein(CRP),tumor necrosis factor-α(TNF-α),interleukin-6(IL-6),immunoglobulin IgA,IgG and IgM and other immune inflammatory indexes were detected.The relationship between different TCM Syndromes of PSS and molecular level changes of immune inflammatory indexes was analyzed and compared,as well as the impact on quality of life and mental emotion.Results:The overall health score of patients with Qi Yin deficiency syndrome was significantly lower than that of patients with dampness heat obstructing collaterals and dampness evil invading lung syndrome(P<0.01),while the SAS and SDS scores of patients with Qi Yin deficiency syndrome were significantly higher than those of patients with dampness heat invading lung syndrome and dampness heat obstructing collaterals syndrome(P<0.05,P<0.01);ESR,CRP,RF,TNF-α,IL-6,IgA,IgG and IgM related laboratory indexes of patients with dampness heat obstructing collaterals syndrome were significantly higher than those of patients with dry evil invading lung syndrome,liver kidney yin deficiency syndrome and liver kidney yin deficiency syndrome Qi Yin deficiency syndrome and Qi blood stasis syndrome(P<0.05,P<0.01).Correlation test found that the above five syndrome types were positively correlated with hs-CRP,IL-6,TNF-ɑindicators(P<0.05).Conclusion:The overall health,SAS,SDS and related hs-CRP,IL-6,TNF-ɑand some others indexes of SS patients are associated with TCM syndromes.展开更多
Liu Chunying is a famous old Chinese medicine doctor for more than 30 years.He has rich experience in the understanding and treatment of Sjogren's syndrome(SS).Liu Shi systematically discusses the etiology and pat...Liu Chunying is a famous old Chinese medicine doctor for more than 30 years.He has rich experience in the understanding and treatment of Sjogren's syndrome(SS).Liu Shi systematically discusses the etiology and pathogenesis of SS from the angle of liver depression.In the treatment,it is emphasized that"Muyu Da Zhi"is the first,with the addition and subtraction of the disease,to provide a new idea for the treatment of Sjogren's syndrome from liver depression,and to attach a test case.展开更多
Brain-derived neurotrophic factor(BDNF)is a widely studied neurotrophic factor,which plays an important role in the growth,development,dif-ferentiation,injury,repair,survival and apoptosis of nerve cells.More and more...Brain-derived neurotrophic factor(BDNF)is a widely studied neurotrophic factor,which plays an important role in the growth,development,dif-ferentiation,injury,repair,survival and apoptosis of nerve cells.More and more studies have found that there is a high prevalence of depressive disorders in patients with autoimmune diseases.Sjogren's syndrome is a chronic autoimmune exocrine disease characterized by lympho-cytic infiltration and exocrine gland destruction.Depres-sive disorders are common in patients with Sjogren's syndrome.The quality of life of patients with Sjogren's syndrome with depression was generally lower than that of patients with Sjogren's syndrome without depres-sion.In this article,we reviewed the research progress of BDNF and depression in Sjogren's syndrome at home and abroad.展开更多
AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely...AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time(TBUT) and corneal staining score(CSS) were detected in 34 eyes of 34 dry eye patients with SS(SSDE group) and 34 dry eye subjects without SS(NSSDE group) using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS:The differences in age, sex, intraocular pressure(IOP) and central corneal thickness(CCT) were not significant between the two groups(P〉0.05). The tear film parameters had significant differences between the SSDE group and NSSDE group(all P〈0.05). Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA(P=0.01, 0.02, and 0.02, respectively) compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT(rho=-0.38, P=0.03); HC-time was negatively correlated with CSS(rho=-0.43, P=0.02). In the NSSDE group, HC-time was again negatively correlated with CSS(rho=-0.39, P=0.02).CONCLUSION:There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less "stiffness", as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.展开更多
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
Objective: To study on in immunological characteristics of T lymphocyte in peripheral blood from patients with Sjogren's syndrome (SS), provide the theory basis for the further research of Traditional Chinese medi...Objective: To study on in immunological characteristics of T lymphocyte in peripheral blood from patients with Sjogren's syndrome (SS), provide the theory basis for the further research of Traditional Chinese medicine (TCM) targeted therapy.Methods:T lymphocyte subsets in peripheral blood from SS, RA and normal control groups were tested with various monocbnal antibodies and multicolor flow cytometry.T lymphocyte subsets were tested in peripheral blood from SS patients of different TCM Syndromes. The relationship between T Lymphocyte subsets and disease activity was measured by erythrocyte sedimentation rate (SR)and serum IgG. Results: The percentage of CD4^+T cells and the ration of CD4^+/CD8^+T cells in peripheral bbod from SS patients decreased significantly, and the percentage of CD8^+T cells increased than that in control groups (P<0.05). Compared with patients in inactive period, the percentage of CD4^+T cells in peripheral bLood from SS patients in the active stage increased (P>0.05), the percentage of CD8^+T cells decreased (P>0.05), and the ration of CD4^+/ CD8^+T cells increased (P<0.05).Compared with control groups, the percentage of CD69 expressed on T cells in peripheral blood from SS patients decreased, and the percentage of CD95's expression increased (P<0.05).The percentage of CD69CD95's expression also increased (P>0.05). There were no differences among CD4^+T cells, CD8^+T ceLs and CD4^+/ CD8^+ in peripheral blood from SS patients of different TCM Syndromes (P>0.05). CD69 and CD95's expression on SS patients of different TCM Syndromes was no significantLy different (P>0.05).T cell receptor Vβ(TCR Vβ) subfamilies gene in peripheral blood from SS patients and control groups were expressed in all. Compared witji control groups, Vβ 5.3,Vβ 13.1,Vβ 12,Vβ 7.2 had significant differences (P<0.05).Comparison of different TCM Syndromes in SS group: Vβ 13.1 and Vβ 14 were significantly different (P<0.05).Conclusion:There was dysimmunity on T lymphocyte subsets in peripheral blood of SS patients, which was related to disease activity;apoptosis after activation of autoreactive T cell may be induced by Chinese medicine;TCR Vβ subfamilies of different TCM syndromes were different, which noted that different autoreactive T cells of abnormal activation tended to result in different expression of TCM Different syndromes.展开更多
Introduction:Multicentric reticulohistiocytosis(MRH)is a rare disease that is known to affect the skin and joints,primarily.It is considered a rare form of non-Langerhans cell histiocytosis(Group C)that can cause dest...Introduction:Multicentric reticulohistiocytosis(MRH)is a rare disease that is known to affect the skin and joints,primarily.It is considered a rare form of non-Langerhans cell histiocytosis(Group C)that can cause destructive inflammatory arthritis involving both the small and large joints.Cutaneous eruptions of periungual,“coral beads”and nodules appearing over the distal fingers are considered pathognomonic clues for identifying this disorder.Histology evaluation of the cutaneous papules typically shows infiltrative histiocytes and multinucleated giant cells.Although no well-established therapies exist to date,a variety of immunosuppressants have been used with varying degrees of success.Case Description:A 53-year-old Caucasian female patient with a family history of rheumatoid arthritis and a personal history of Sjogren's syndrome presented to the rheumatology clinic complaining of pain in her bilateral hands and fingers.There were several small,papulo-nodular lesions ranging from 1 to 2mm in size noted at the base of her nails.A 4mm punch biopsy of one of the papules from the neck showed dermal infiltration of eosinophilic mononucleated and multinucleated giant cells with“ground glass”appearing cytoplasm consistent with MRH.X-ray of her hands showed periarticular demineralization and erosions surrounding several bilateral proximal and distal interphalangeal joints,and thus tofacitinib in addition to methotrexate,hydroxychloroquine,and dexamethasone(dosed weekly)was started to help control any further articular damage.Conclusion:Our aim is to further support the relation of MRH with autoimmune diseases,including Sjogren's syndrome.Autoimmune diseases have been reported in association with MRH,although a clear association has yet to be made.展开更多
BACKGROUND Irritable bowel syndrome(IBS)is one of the most frequent and debilitating conditions leading to gastroenterological referrals.However,recommended treatments remain limited,yielding only limited therapeutic ...BACKGROUND Irritable bowel syndrome(IBS)is one of the most frequent and debilitating conditions leading to gastroenterological referrals.However,recommended treatments remain limited,yielding only limited therapeutic gains.Chitin-glucan(CG)is a novel dietary prebiotic classically used in humans at a dosage of 1.5-3.0 g/d and is considered a safe food ingredient by the European Food Safety Authority.To provide an alternative approach to managing patients with IBS,we performed preclinical molecular,cellular,and animal studies to evaluate the role of chitin-glucan in the main pathophysiological mechanisms involved in IBS.AIM To evaluate the roles of CG in visceral analgesia,intestinal inflammation,barrier function,and to develop computational molecular models.METHODS Visceral pain was recorded through colorectal distension(CRD)in a model of long-lasting colon hypersensitivity induced by an intra-rectal administration of TNBS[15 milligrams(mg)/kilogram(kg)]in 33 Sprague-Dawley rats.Intracolonic pressure was regularly assessed during the 9 wk-experiment(weeks 0,3,5,and 7)in animals receiving CG(n=14)at a human equivalent dose(HED)of 1.5 g/d or 3.0 g/d and compared to negative control(tap water,n=11)and positive control(phloroglucinol at 1.5 g/d HED,n=8)groups.The anti-inflammatory effect of CG was evaluated using clinical and histological scores in 30 C57bl6 male mice with colitis induced by dextran sodium sulfate(DSS)administered in their drinking water during 14 d.HT-29 cells under basal conditions and after stimulation with lipopolysaccharide(LPS)were treated with CG to evaluate changes in pathways related to analgesia μ-opioid receptor(MOR),cannabinoid receptor 2(CB2),peroxisome proliferator-activated receptor alpha,inflammation[interleukin(IL)-10,IL-1b,and IL-8]and barrier function[mucin 2-5AC,claudin-2,zonula occludens(ZO)-1,ZO-2]using the real-time PCR method.Molecular modelling of CG,LPS,lipoteichoic acid(LTA),and phospholipomannan(PLM)was developed,and the ability of CG to chelate microbial pathogenic lipids was evaluated by docking and molecular dynamics simulations.Data were expressed as the mean±SEM.RESULTS Daily CG orally-administered to rats or mice was well tolerated without including diarrhea,visceral hypersensitivity,or inflammation,as evaluated at histological and molecular levels.In a model of CRD,CG at a dosage of 3 g/d HED significantly decreased visceral pain perception by 14%after 2 wk of administration(P<0.01)and reduced inflammation intensity by 50%,resulting in complete regeneration of the colonic mucosa in mice with DSS-induced colitis.To better reproduce the characteristics of visceral pain in patients with IBS,we then measured the therapeutic impact of CG in rats with TNBS-induced inflammation to long-lasting visceral hypersensitivity.CG at a dosage of 1.5 g/d HED decreased visceral pain perception by 20%five weeks after colitis induction(P<0.01).When the CG dosage was increased to 3.0 g/d HED,this analgesic effect surpassed that of the spasmolytic agent phloroglucinol,manifesting more rapidly within 3 wk and leading to a 50%inhibition of pain perception(P<0.0001).The underlying molecular mechanisms contributing to these analgesic and anti-inflammatory effects of CG involved,at least in part,a significant induction of MOR,CB2 receptor,and IL-10,as well as a significant decrease in pro-inflammatory cytokines IL-1b and IL-8.CG also significantly upregulated barrier-related genes including muc5AC,claudin-2,and ZO-2.Molecular modelling of CG revealed a new property of the molecule as a chelator of microbial pathogenic lipids,sequestering gram-negative LPS and gram-positive LTA bacterial toxins,as well as PLM in fungi at the lowesr energy conformations.CONCLUSION CG decreased visceral perception and intestinal inflammation through master gene regulation and direct binding of microbial products,suggesting that CG may constitute a new therapeutic strategy for patients with IBS or IBSlike symptoms.展开更多
BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relatio...BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relationship between body composition,metabolic syndrome and insulin resistance in T1DM to guide clinical treatment and intervention.AIM To assess body composition(BC)in T1DM patients and evaluate the relationship between BC,metabolic syndrome(MS),and insulin resistance in these indi-viduals.METHODS A total of 101 subjects with T1DM,aged 10 years or older,and with a disease duration of over 1 year were included.Bioelectrical impedance analysis using the Tsinghua-Tongfang BC Analyzer BCA-1B was employed to measure various BC parameters.Clinical and laboratory data were collected,and insulin resistance was calculated using the estimated glucose disposal rate(eGDR).RESULTS MS was diagnosed in 16/101 patients(15.84%),overweight in 16/101 patients(15.84%),obesity in 4/101(3.96%),hypertension in 34/101(33.66%%)and dyslip-idemia in 16/101 patients(15.84%).Visceral fat index(VFI)and trunk fat mass were significantly and negatively correlated with eGDR(both P<0.001).Female patients exhibited higher body fat percentage and visceral fat ratio compared to male patients.Binary logistic regression analysis revealed that significant factors for MS included eGDR[P=0.017,odds ratio(OR)=0.109],VFI(P=0.030,OR=3.529),and a family history of diabetes(P=0.004,OR=0.228).Significant factors for hypertension included eGDR(P<0.001,OR=0.488)and skeletal muscle mass(P=0.003,OR=1.111).Significant factors for dyslipidemia included trunk fat mass(P=0.033,OR=1.202)and eGDR(P=0.037,OR=0.708).CONCLUSION Visceral fat was found to be a superior predictor of MS compared to conventional measures such as body mass index and waist-to-hip ratio in Chinese individuals with T1DM.BC analysis,specifically identifying visceral fat(trunk fat),may play an important role in identifying the increased risk of MS in non-obese patients with T1DM.展开更多
BACKGROUND Cardiovascular disease is a major complication of diabetes mellitus(DM).Type-2 DM(T2DM)is associated with an increased risk of cardiovascular events and mortality,while serum biomarkers may facilitate the p...BACKGROUND Cardiovascular disease is a major complication of diabetes mellitus(DM).Type-2 DM(T2DM)is associated with an increased risk of cardiovascular events and mortality,while serum biomarkers may facilitate the prediction of these outcomes.Early differential diagnosis of T2DM complicated with acute coronary syndrome(ACS)plays an important role in controlling disease progression and improving safety.AIM To investigate the correlation of serum bilirubin andγ-glutamyltranspeptidase(γ-GGT)with major adverse cardiovascular events(MACEs)in T2DM patients with ACS.METHODS The clinical data of inpatients from January 2022 to December 2022 were analyzed retrospectively.According to different conditions,they were divided into the T2DM complicated with ACS group(T2DM+ACS,n=96),simple T2DM group(T2DM,n=85),and simple ACS group(ACS,n=90).The clinical data and laboratory indices were compared among the three groups,and the correlations of serum total bilirubin(TBIL)levels and serumγ-GGT levels with other indices were discussed.T2DM+ACS patients received a 90-day follow-up after discharge and were divided into event(n=15)and nonevent(n=81)groups according to the occurrence of MACEs;Univariate and multivariate analyses were further used to screen the independent influencing factors of MACEs in patients.RESULTS The T2DM+ACS group showed higherγ-GGT,total cholesterol,low-density lipoprotein cholesterol(LDL-C)and glycosylated hemoglobin(HbA1c)and lower TBIL and high-density lipoprotein cholesterol levels than the T2DM and ACS groups(P<0.05).Based on univariate analysis,the event and nonevent groups were significantly different in age(t=3.3612,P=0.0011),TBIL level(t=3.0742,P=0.0028),γ-GGT level(t=2.6887,P=0.0085),LDL-C level(t=2.0816,P=0.0401),HbA1c level(t=2.7862,P=0.0065)and left ventricular ejection fraction(LEVF)levels(t=3.2047,P=0.0018).Multivariate logistic regression analysis further identified that TBIL level and LEVF level were protective factor for MACEs,and age andγ-GGT level were risk factors(P<0.05).CONCLUSION Serum TBIL levels are decreased andγ-GGT levels are increased in T2DM+ACS patients,and the two indices are significantly negatively correlated.TBIL andγ-GGT are independent influencing factors for MACEs in such patients.展开更多
BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effe...BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome,individual variations in the optimal patient position have been noted.In this report,we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient.CASE SUMMARY Case 1:A 90-year-old man with nausea and vomiting.Following diagnosis of SMA syndrome by computed tomography(CT),ultrasonography(US)revealed the SMA-Ao distance in the supine position(4 mm),which slightly improved in the lateral position(5.7–7.0 mm)without the passage of duodenal contents.However,in the sitting position,the SMA-Ao distance was increased to 15 mm accompanied by improved content passage.Additionally,US indicated enhanced passage upon abdominal massage on the right side.By day 2,the patient could eat comfortably with the optimal position and massage.Case 2:An 87-year-old woman with vomiting.After the diagnosis of SMA syndrome and aspiration pneumonia by CT,dynamic US confirmed the optimal position(SMA-Ao distance was improved to 7 mm in forward-bent position,whereas it remained at 5 mm in the supine position).By day 7 when her pneumonia recovered,she could eat with the optimal position.CONCLUSION The optimal position for SMA syndrome varies among individuals.Dynamic US appears to be a valuable tool in improving patient outcomes.展开更多
Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole ...Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole origin of a cascade of pathophysiological mechanisms directly affecting the kidneys in this context.In the absence of established heart disease,cirrhotic cardiomyopathy may occur more frequently in those with liver cirrhosis and kidney disease.It is a specific form of cardiac dysfunction characterized by blunted contractile responsiveness to stress stimuli and altered diastolic relaxation with electrophysiological abnormalities.Despite the clinical description of these potential cardiac-related complications of the liver,the role of the heart has traditionally been an overlooked aspect of circulatory dysfunction in HRS.Yet from a physiological sense,temporality(prior onset)of cardiorenal interactions in HRS and positive effects stemming from portosystemic shunting demonstrated an important role of the heart in the development and progression of kidney dysfunction in cirrhotic patients.In this review,we discuss current concepts surrounding how the heart may influence the development and progression of HRS,and the role of systemic inflammation and endothelial dysfunction causing circulatory dysfunction within this setting.The temporality of heart and kidney dysfunction in HRS will be discussed.For a subgroup of patients who receive portosystemic shunting,the dynamics of cardiorenal interactions following treatment is reviewed.Continued research to determine the unknowns in this topic is anticipated,hopefully to further clarify the intricacies surrounding the liver-heart-kidney connection and improve strategies for management.展开更多
Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-cons...Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-consanguineous marriage, admitted to the neonatology and neonatal intensive care unit at Day 1 of life for a poly-malformative syndrome. Clinically, the newborn presented with bilateral anophthalmia, cleft palate, dysmorphic facies with a rounded forehead, hypertelorism, micrognathia, low-set ears and a short neck, syndactility and bilateral cryptorchidism. Trans fontanellar ultrasound revealed tri ventricular hydrocephalus. Cerebral MRI angiography showed malformative tri-ventricular hydrocephalus, hypoplasia of the brainstem and cerebellum, and poly-microgyria. Transthoracic and renal ultrasonography were unremarkable, and the chest X-ray was normal. The authors discuss the malformative clinical and para-clinical aspects of this syndrome, multidisciplinary management and the importance of prenatal diagnosis.展开更多
Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of...Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of Lynch syndrome showing excellent response to immunotherapy. A 59-year-old male with a history of rectal cancer 30 years ago came to the hospital due to a fever and further found a large necrotic colon mass. Biopsy was positive for colorectal cancer;however, due to the size of the tumor, the patient was deemed not a surgical candidate and offered hospice with palliative chemotherapy. Based on further workup, the patient was diagnosed with Lynch syndrome, with colon cancer determined to be responsive to Immunotherapy. He was started on JEMPERLI (Dosterlimab-gxly), and after three cycles of therapy, imaging and PET scan were repeated, showing decreased activity and extent of the tumor—a tremendous success.展开更多
Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,in...Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,including improving glucose and lipid metabolism,enhancing insulin sensitivity,reducing oxidative stress and inflammation,promoting ceramides degradation,and stimulating adipose tissue vascularity.Based on those,it can serve as a positive regulator in many metabolic syndromes,such as type 2 diabetes(T2D),cardiovascular diseases,non-alcoholic fatty liver disease(NAFLD),sarcopenia,neurodegenerative diseases,and certain cancers.Therefore,a promising therapeutic approach for treating various metabolic diseases may involve elevating ADP levels or activating ADP receptors.The modulation of ADP genes,multimerization,and secretion covers the main processes of ADP generation,providing a comprehensive orientation for the development of more appropriate therapeutic strategies.In order to have a deeper understanding of ADP,this paper will provide an all-encompassing review of ADP.展开更多
文摘AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high and low visual acuities and contrast sensitivity functions.These tests were conducted under photopic and then mesopic conditions.Twenty-one Sjögren’s syndrome patients and 21 aged-matched healthy volunteers completed all the measurements in this study.RESULTS:Sjogren’s syndrome patients have greater impairment in contrast sensitivity than standardized visual acuity.This reduction was significant under the mesopic condition.Also,Sjogren’s syndrome patients treated with pilocarpine suffer more than patients without pilocarpine treatment under low light conditions.CONCLUSION:Sjogren’s syndrome patients shows greater impairment in different visual resolution tasks due to dry eye symptoms.
基金Supported by the Chonnam National University Hospital Biomedical Research Institute(CRI 18093-1)Basic Science Research Program through the National Research Foundation of Korea(NRF)funded by the Ministry of Science,ICT&Future Planning(No.2017R1A2B4003367)
文摘AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a major form of aqueous deficient DED and meibomian gland dysfunction(MGD), a common cause of evaporative DED.METHODS: A total 135 eyes of 135 subjects included in this study: patients with DED associated with coGVHD(n=30), patients with SS(n=35), patients with MGD(n=35), and normal controls(n=35). All participants completed the Ocular Surface Disease Index(OSDI) questionnaire, ocular surface examination [Schirmer test, tear film breakup time(TFBUT), and ocular surface staining], and meibomian gland assessment [meiboscore(gland dropout detected on meibography using infrared camera of the Keratograph 5 M), meibum expressibility score(MES), meibum quality score(MQS), lid margin abnormality]. In addition, correlations of meibomian gland characteristics with ocular surface parameters as well as disease severity score were investigated in coGVHD group.RESULTS: The coGVHD group showed significantly higher meiboscore, MES, and MQS than the other 3 groups(all P<0.05). In the coGVHD group, parameters of meibomian gland showed a significant correlation each other and those of ocular surface. The correlation between meibomian gland parameters and severity score of co GVHD was also established(meiboscore, r=0.62; MES, r=0.47; MQS, r=0.47; lid margin abnormality score, r=0.55; all P<0.05).CONCLUSION: Patients with DED associated with co GVHD show poorer gland morphology and worse glandfunction than other types of DED. In addition, meibomian gland damage is not only associated with ocular surface damage but also disease severity of coGVHD.
文摘BACKGROUND Primary Sjogren's syndrome(pSS)is an autoimmune disease,and renal involvement has been considered to be one of the systemic complications of pSS.Patients who have sjogren's syndrome with renal disease as the first manifestation and no exocrine gland involvement or autoantibodies can be missed clinically.CASE SUMMARY We here in report an unusual case of a primary Sjogren's syndrome in a 43-yearold female who had minimal lesion nephropathy as the initial presentation,and the patient was negative for serum anti-SSA and anti-SSB antibodies and did not have signs of exocrine gland involvement.The patient’s Sjogren's syndrome was confirmed by a minor salivary gland biopsy(MSGB)and a filter paper test.the patient’s oedema subsided,and the patient’s urinary protein resolved,showing that the treatment was effective.CONCLUSION MSGB should be considered if pSS is suspected in patients who do not have the typical pSS symptoms or who are positive for the specific autoantibodies.
基金Supported by General Funding of Health Commission of Jiangsu Province,No.H2019076.
文摘BACKGROUND Subclavian steal syndrome(SSS)caused by Sjogren's syndrome is rare,especially for elderly patients with risk factors for atherosclerosis.The current report presents the uncommon etiology and treatment of SSS,aiming to improve doctor’s clinical experience.CASE SUMMARY A 69-year-old man was diagnosed with hypertension and acute cerebral infarction presenting with left upper limb weakness and pain even gradually aggravating to left limb hemiplegia 30 years ago.He was managed with antihypertensive and antithrombotic therapy;however,his condition was recurrent,and he never had any further examination.It was found that the difference of the bilateral upper arm systolic pressure was over 20 mmHg,and Doppler examination showed that the blood flow of the left vertebral artery was reversed,suggesting SSS.Further tests revealed a benign lymphoepithelial lesion in salivary gland tissue,confirming the Sjogren's syndrome.CONCLUSION The patient was found to have hypertension when he was 33 years old,and the blood pressure of both sides was asymmetric,which was ignored.The patient's symptoms of dizziness and upper limb weakness were misdiagnosed as general cerebral infarction.It is necessary to test the aorta computed tomography angiography to prove secondary hypertension factors such as Sjogren's syndrome.
基金Fund Project:General program of National Natural Science Foundation of China(No.81673954)。
文摘Objective:To systematically evaluate the efficacy and safety of traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of Sjogren's Syndrome(SS).Methods:Two researchers searched the databases of Chinese National Knowledge Infrastructure(CNKI),Wanfang Database,Weipu Database,Chinese Biomedical Literature Database,PubMed Database,Web of science Database and The Cochrane Library Database(the retrieval period is from the establishment of the database to October 2020)and screened the literatures independently.The data were extracted for bias risk assessment,and then meta-analysis was performed using Revman 5.3 software.Results:A total of 19 articles involving 1185 patients were included,including 617 cases in the treatment group and 568 cases in the control group.Combined data analysis showed that the total effective rate of traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of Sjogren's syndrome was significantly higher than that of hydroxychloroquine sulfate alone[R R=1.34,95%CI(1.24,1.44),P<0.00001].The improvement of salivary gland[SMD=0.66,95%CI(0.47,0.85),P<0.00001],tear secretion function[MD=2.56,95%CI(1.29,3.83),P<0.0001]and reducing CRP[MD=-3.53,95%CI(-3.93,-3.14),P<0.00001],ESR[MD=-5.80,95%CI(-8.48,-3.13),P<0.00001]were better than those of the control group.However,there was no significant difference in the reduction of IgG and the incidence of adverse reactions between the two groups.Conclusion:Traditional Chinese medicine combined with hydroxychloroquine sulfate in the treatment of SS is superior to the control group in total effective rate,improvement of salivary gland and tear secretion function,and reduction of CRP and ESR,and we didn’t see the significantly increase on the adverse event.However,due to the small sample size and low quality of the included studies,the conclusion needs to be verified by larger sample and higher quality randomized controlled trials.
基金National key research and development plan of ministry of science and technology for Chinese medicine modernization research key special project(No.2018YFC1705204)Construction project of famous Chinese medicine doctor Liu Jian studio in Anhui province(No.[2018]11)+1 种基金Anhui province’s 12th batch of“115”innovation team(No.[2019]1)National training project for key talents of TCM clinical characteristic technical inheritance(No.[2019]36)。
文摘Objective:To observe the relationship between the distribution of TCM Syndromes of primary Sjogren's syndrome(pSS)and the changes of erythrocyte sedimentation rate(ESR),C-reactive protein(CRP),tumor necrosis factor-α(TNF-α),interleukin-6(IL-6),immunoglobulin IgA,IgG and IgM.Methods:157 diagnosed PSS patients were divided into dampness heat obstructing collaterals syndrome,dryness evil invading lung syndrome,Qi blood stasis syndrome,liver kidney yin deficiency syndrome and Qi Yin deficiency syndrome according to TCM syndrome differentiation standard.The age,course of disease,general health score in international universal quality of life scale,self rating Anxiety scale(SAS),self rating Depression Scale(SDS)and erythrocyte sedimentation rate were observed and detected(ESR,C-reactive protein(CRP),tumor necrosis factor-α(TNF-α),interleukin-6(IL-6),immunoglobulin IgA,IgG and IgM and other immune inflammatory indexes were detected.The relationship between different TCM Syndromes of PSS and molecular level changes of immune inflammatory indexes was analyzed and compared,as well as the impact on quality of life and mental emotion.Results:The overall health score of patients with Qi Yin deficiency syndrome was significantly lower than that of patients with dampness heat obstructing collaterals and dampness evil invading lung syndrome(P<0.01),while the SAS and SDS scores of patients with Qi Yin deficiency syndrome were significantly higher than those of patients with dampness heat invading lung syndrome and dampness heat obstructing collaterals syndrome(P<0.05,P<0.01);ESR,CRP,RF,TNF-α,IL-6,IgA,IgG and IgM related laboratory indexes of patients with dampness heat obstructing collaterals syndrome were significantly higher than those of patients with dry evil invading lung syndrome,liver kidney yin deficiency syndrome and liver kidney yin deficiency syndrome Qi Yin deficiency syndrome and Qi blood stasis syndrome(P<0.05,P<0.01).Correlation test found that the above five syndrome types were positively correlated with hs-CRP,IL-6,TNF-ɑindicators(P<0.05).Conclusion:The overall health,SAS,SDS and related hs-CRP,IL-6,TNF-ɑand some others indexes of SS patients are associated with TCM syndromes.
文摘Liu Chunying is a famous old Chinese medicine doctor for more than 30 years.He has rich experience in the understanding and treatment of Sjogren's syndrome(SS).Liu Shi systematically discusses the etiology and pathogenesis of SS from the angle of liver depression.In the treatment,it is emphasized that"Muyu Da Zhi"is the first,with the addition and subtraction of the disease,to provide a new idea for the treatment of Sjogren's syndrome from liver depression,and to attach a test case.
基金Yunnan Provincial Department of Science and Tech-nology Project(202001AY070001-280).
文摘Brain-derived neurotrophic factor(BDNF)is a widely studied neurotrophic factor,which plays an important role in the growth,development,dif-ferentiation,injury,repair,survival and apoptosis of nerve cells.More and more studies have found that there is a high prevalence of depressive disorders in patients with autoimmune diseases.Sjogren's syndrome is a chronic autoimmune exocrine disease characterized by lympho-cytic infiltration and exocrine gland destruction.Depres-sive disorders are common in patients with Sjogren's syndrome.The quality of life of patients with Sjogren's syndrome with depression was generally lower than that of patients with Sjogren's syndrome without depres-sion.In this article,we reviewed the research progress of BDNF and depression in Sjogren's syndrome at home and abroad.
基金Supported by the National Natural Science Foundation of China(No.81070755)
文摘AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time(TBUT) and corneal staining score(CSS) were detected in 34 eyes of 34 dry eye patients with SS(SSDE group) and 34 dry eye subjects without SS(NSSDE group) using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS:The differences in age, sex, intraocular pressure(IOP) and central corneal thickness(CCT) were not significant between the two groups(P〉0.05). The tear film parameters had significant differences between the SSDE group and NSSDE group(all P〈0.05). Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA(P=0.01, 0.02, and 0.02, respectively) compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT(rho=-0.38, P=0.03); HC-time was negatively correlated with CSS(rho=-0.43, P=0.02). In the NSSDE group, HC-time was again negatively correlated with CSS(rho=-0.39, P=0.02).CONCLUSION:There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less "stiffness", as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.
文摘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.
文摘Objective: To study on in immunological characteristics of T lymphocyte in peripheral blood from patients with Sjogren's syndrome (SS), provide the theory basis for the further research of Traditional Chinese medicine (TCM) targeted therapy.Methods:T lymphocyte subsets in peripheral blood from SS, RA and normal control groups were tested with various monocbnal antibodies and multicolor flow cytometry.T lymphocyte subsets were tested in peripheral blood from SS patients of different TCM Syndromes. The relationship between T Lymphocyte subsets and disease activity was measured by erythrocyte sedimentation rate (SR)and serum IgG. Results: The percentage of CD4^+T cells and the ration of CD4^+/CD8^+T cells in peripheral bbod from SS patients decreased significantly, and the percentage of CD8^+T cells increased than that in control groups (P<0.05). Compared with patients in inactive period, the percentage of CD4^+T cells in peripheral bLood from SS patients in the active stage increased (P>0.05), the percentage of CD8^+T cells decreased (P>0.05), and the ration of CD4^+/ CD8^+T cells increased (P<0.05).Compared with control groups, the percentage of CD69 expressed on T cells in peripheral blood from SS patients decreased, and the percentage of CD95's expression increased (P<0.05).The percentage of CD69CD95's expression also increased (P>0.05). There were no differences among CD4^+T cells, CD8^+T ceLs and CD4^+/ CD8^+ in peripheral blood from SS patients of different TCM Syndromes (P>0.05). CD69 and CD95's expression on SS patients of different TCM Syndromes was no significantLy different (P>0.05).T cell receptor Vβ(TCR Vβ) subfamilies gene in peripheral blood from SS patients and control groups were expressed in all. Compared witji control groups, Vβ 5.3,Vβ 13.1,Vβ 12,Vβ 7.2 had significant differences (P<0.05).Comparison of different TCM Syndromes in SS group: Vβ 13.1 and Vβ 14 were significantly different (P<0.05).Conclusion:There was dysimmunity on T lymphocyte subsets in peripheral blood of SS patients, which was related to disease activity;apoptosis after activation of autoreactive T cell may be induced by Chinese medicine;TCR Vβ subfamilies of different TCM syndromes were different, which noted that different autoreactive T cells of abnormal activation tended to result in different expression of TCM Different syndromes.
文摘Introduction:Multicentric reticulohistiocytosis(MRH)is a rare disease that is known to affect the skin and joints,primarily.It is considered a rare form of non-Langerhans cell histiocytosis(Group C)that can cause destructive inflammatory arthritis involving both the small and large joints.Cutaneous eruptions of periungual,“coral beads”and nodules appearing over the distal fingers are considered pathognomonic clues for identifying this disorder.Histology evaluation of the cutaneous papules typically shows infiltrative histiocytes and multinucleated giant cells.Although no well-established therapies exist to date,a variety of immunosuppressants have been used with varying degrees of success.Case Description:A 53-year-old Caucasian female patient with a family history of rheumatoid arthritis and a personal history of Sjogren's syndrome presented to the rheumatology clinic complaining of pain in her bilateral hands and fingers.There were several small,papulo-nodular lesions ranging from 1 to 2mm in size noted at the base of her nails.A 4mm punch biopsy of one of the papules from the neck showed dermal infiltration of eosinophilic mononucleated and multinucleated giant cells with“ground glass”appearing cytoplasm consistent with MRH.X-ray of her hands showed periarticular demineralization and erosions surrounding several bilateral proximal and distal interphalangeal joints,and thus tofacitinib in addition to methotrexate,hydroxychloroquine,and dexamethasone(dosed weekly)was started to help control any further articular damage.Conclusion:Our aim is to further support the relation of MRH with autoimmune diseases,including Sjogren's syndrome.Autoimmune diseases have been reported in association with MRH,although a clear association has yet to be made.
基金Supported by the Service Public de Wallonie(SPW-EER,convention 8588,Belgium).
文摘BACKGROUND Irritable bowel syndrome(IBS)is one of the most frequent and debilitating conditions leading to gastroenterological referrals.However,recommended treatments remain limited,yielding only limited therapeutic gains.Chitin-glucan(CG)is a novel dietary prebiotic classically used in humans at a dosage of 1.5-3.0 g/d and is considered a safe food ingredient by the European Food Safety Authority.To provide an alternative approach to managing patients with IBS,we performed preclinical molecular,cellular,and animal studies to evaluate the role of chitin-glucan in the main pathophysiological mechanisms involved in IBS.AIM To evaluate the roles of CG in visceral analgesia,intestinal inflammation,barrier function,and to develop computational molecular models.METHODS Visceral pain was recorded through colorectal distension(CRD)in a model of long-lasting colon hypersensitivity induced by an intra-rectal administration of TNBS[15 milligrams(mg)/kilogram(kg)]in 33 Sprague-Dawley rats.Intracolonic pressure was regularly assessed during the 9 wk-experiment(weeks 0,3,5,and 7)in animals receiving CG(n=14)at a human equivalent dose(HED)of 1.5 g/d or 3.0 g/d and compared to negative control(tap water,n=11)and positive control(phloroglucinol at 1.5 g/d HED,n=8)groups.The anti-inflammatory effect of CG was evaluated using clinical and histological scores in 30 C57bl6 male mice with colitis induced by dextran sodium sulfate(DSS)administered in their drinking water during 14 d.HT-29 cells under basal conditions and after stimulation with lipopolysaccharide(LPS)were treated with CG to evaluate changes in pathways related to analgesia μ-opioid receptor(MOR),cannabinoid receptor 2(CB2),peroxisome proliferator-activated receptor alpha,inflammation[interleukin(IL)-10,IL-1b,and IL-8]and barrier function[mucin 2-5AC,claudin-2,zonula occludens(ZO)-1,ZO-2]using the real-time PCR method.Molecular modelling of CG,LPS,lipoteichoic acid(LTA),and phospholipomannan(PLM)was developed,and the ability of CG to chelate microbial pathogenic lipids was evaluated by docking and molecular dynamics simulations.Data were expressed as the mean±SEM.RESULTS Daily CG orally-administered to rats or mice was well tolerated without including diarrhea,visceral hypersensitivity,or inflammation,as evaluated at histological and molecular levels.In a model of CRD,CG at a dosage of 3 g/d HED significantly decreased visceral pain perception by 14%after 2 wk of administration(P<0.01)and reduced inflammation intensity by 50%,resulting in complete regeneration of the colonic mucosa in mice with DSS-induced colitis.To better reproduce the characteristics of visceral pain in patients with IBS,we then measured the therapeutic impact of CG in rats with TNBS-induced inflammation to long-lasting visceral hypersensitivity.CG at a dosage of 1.5 g/d HED decreased visceral pain perception by 20%five weeks after colitis induction(P<0.01).When the CG dosage was increased to 3.0 g/d HED,this analgesic effect surpassed that of the spasmolytic agent phloroglucinol,manifesting more rapidly within 3 wk and leading to a 50%inhibition of pain perception(P<0.0001).The underlying molecular mechanisms contributing to these analgesic and anti-inflammatory effects of CG involved,at least in part,a significant induction of MOR,CB2 receptor,and IL-10,as well as a significant decrease in pro-inflammatory cytokines IL-1b and IL-8.CG also significantly upregulated barrier-related genes including muc5AC,claudin-2,and ZO-2.Molecular modelling of CG revealed a new property of the molecule as a chelator of microbial pathogenic lipids,sequestering gram-negative LPS and gram-positive LTA bacterial toxins,as well as PLM in fungi at the lowesr energy conformations.CONCLUSION CG decreased visceral perception and intestinal inflammation through master gene regulation and direct binding of microbial products,suggesting that CG may constitute a new therapeutic strategy for patients with IBS or IBSlike symptoms.
基金Supported by the“SDF-sweet doctor cultivation”Project of Sinocare Diabetes Foundation,No.2022SD11 and No.2021SD09.
文摘BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relationship between body composition,metabolic syndrome and insulin resistance in T1DM to guide clinical treatment and intervention.AIM To assess body composition(BC)in T1DM patients and evaluate the relationship between BC,metabolic syndrome(MS),and insulin resistance in these indi-viduals.METHODS A total of 101 subjects with T1DM,aged 10 years or older,and with a disease duration of over 1 year were included.Bioelectrical impedance analysis using the Tsinghua-Tongfang BC Analyzer BCA-1B was employed to measure various BC parameters.Clinical and laboratory data were collected,and insulin resistance was calculated using the estimated glucose disposal rate(eGDR).RESULTS MS was diagnosed in 16/101 patients(15.84%),overweight in 16/101 patients(15.84%),obesity in 4/101(3.96%),hypertension in 34/101(33.66%%)and dyslip-idemia in 16/101 patients(15.84%).Visceral fat index(VFI)and trunk fat mass were significantly and negatively correlated with eGDR(both P<0.001).Female patients exhibited higher body fat percentage and visceral fat ratio compared to male patients.Binary logistic regression analysis revealed that significant factors for MS included eGDR[P=0.017,odds ratio(OR)=0.109],VFI(P=0.030,OR=3.529),and a family history of diabetes(P=0.004,OR=0.228).Significant factors for hypertension included eGDR(P<0.001,OR=0.488)and skeletal muscle mass(P=0.003,OR=1.111).Significant factors for dyslipidemia included trunk fat mass(P=0.033,OR=1.202)and eGDR(P=0.037,OR=0.708).CONCLUSION Visceral fat was found to be a superior predictor of MS compared to conventional measures such as body mass index and waist-to-hip ratio in Chinese individuals with T1DM.BC analysis,specifically identifying visceral fat(trunk fat),may play an important role in identifying the increased risk of MS in non-obese patients with T1DM.
基金Supported by Science and Technology Major Project of Changzhou Science and Technology Bureau,No.CE20205047Natural Science Foundation of Xinjiang Uygur Autonomo us Region,No.ZD202220Changzhou A major scientific research project of the Municipal Health Commission,No.2022D01F52.
文摘BACKGROUND Cardiovascular disease is a major complication of diabetes mellitus(DM).Type-2 DM(T2DM)is associated with an increased risk of cardiovascular events and mortality,while serum biomarkers may facilitate the prediction of these outcomes.Early differential diagnosis of T2DM complicated with acute coronary syndrome(ACS)plays an important role in controlling disease progression and improving safety.AIM To investigate the correlation of serum bilirubin andγ-glutamyltranspeptidase(γ-GGT)with major adverse cardiovascular events(MACEs)in T2DM patients with ACS.METHODS The clinical data of inpatients from January 2022 to December 2022 were analyzed retrospectively.According to different conditions,they were divided into the T2DM complicated with ACS group(T2DM+ACS,n=96),simple T2DM group(T2DM,n=85),and simple ACS group(ACS,n=90).The clinical data and laboratory indices were compared among the three groups,and the correlations of serum total bilirubin(TBIL)levels and serumγ-GGT levels with other indices were discussed.T2DM+ACS patients received a 90-day follow-up after discharge and were divided into event(n=15)and nonevent(n=81)groups according to the occurrence of MACEs;Univariate and multivariate analyses were further used to screen the independent influencing factors of MACEs in patients.RESULTS The T2DM+ACS group showed higherγ-GGT,total cholesterol,low-density lipoprotein cholesterol(LDL-C)and glycosylated hemoglobin(HbA1c)and lower TBIL and high-density lipoprotein cholesterol levels than the T2DM and ACS groups(P<0.05).Based on univariate analysis,the event and nonevent groups were significantly different in age(t=3.3612,P=0.0011),TBIL level(t=3.0742,P=0.0028),γ-GGT level(t=2.6887,P=0.0085),LDL-C level(t=2.0816,P=0.0401),HbA1c level(t=2.7862,P=0.0065)and left ventricular ejection fraction(LEVF)levels(t=3.2047,P=0.0018).Multivariate logistic regression analysis further identified that TBIL level and LEVF level were protective factor for MACEs,and age andγ-GGT level were risk factors(P<0.05).CONCLUSION Serum TBIL levels are decreased andγ-GGT levels are increased in T2DM+ACS patients,and the two indices are significantly negatively correlated.TBIL andγ-GGT are independent influencing factors for MACEs in such patients.
文摘BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome,individual variations in the optimal patient position have been noted.In this report,we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient.CASE SUMMARY Case 1:A 90-year-old man with nausea and vomiting.Following diagnosis of SMA syndrome by computed tomography(CT),ultrasonography(US)revealed the SMA-Ao distance in the supine position(4 mm),which slightly improved in the lateral position(5.7–7.0 mm)without the passage of duodenal contents.However,in the sitting position,the SMA-Ao distance was increased to 15 mm accompanied by improved content passage.Additionally,US indicated enhanced passage upon abdominal massage on the right side.By day 2,the patient could eat comfortably with the optimal position and massage.Case 2:An 87-year-old woman with vomiting.After the diagnosis of SMA syndrome and aspiration pneumonia by CT,dynamic US confirmed the optimal position(SMA-Ao distance was improved to 7 mm in forward-bent position,whereas it remained at 5 mm in the supine position).By day 7 when her pneumonia recovered,she could eat with the optimal position.CONCLUSION The optimal position for SMA syndrome varies among individuals.Dynamic US appears to be a valuable tool in improving patient outcomes.
文摘Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole origin of a cascade of pathophysiological mechanisms directly affecting the kidneys in this context.In the absence of established heart disease,cirrhotic cardiomyopathy may occur more frequently in those with liver cirrhosis and kidney disease.It is a specific form of cardiac dysfunction characterized by blunted contractile responsiveness to stress stimuli and altered diastolic relaxation with electrophysiological abnormalities.Despite the clinical description of these potential cardiac-related complications of the liver,the role of the heart has traditionally been an overlooked aspect of circulatory dysfunction in HRS.Yet from a physiological sense,temporality(prior onset)of cardiorenal interactions in HRS and positive effects stemming from portosystemic shunting demonstrated an important role of the heart in the development and progression of kidney dysfunction in cirrhotic patients.In this review,we discuss current concepts surrounding how the heart may influence the development and progression of HRS,and the role of systemic inflammation and endothelial dysfunction causing circulatory dysfunction within this setting.The temporality of heart and kidney dysfunction in HRS will be discussed.For a subgroup of patients who receive portosystemic shunting,the dynamics of cardiorenal interactions following treatment is reviewed.Continued research to determine the unknowns in this topic is anticipated,hopefully to further clarify the intricacies surrounding the liver-heart-kidney connection and improve strategies for management.
文摘Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-consanguineous marriage, admitted to the neonatology and neonatal intensive care unit at Day 1 of life for a poly-malformative syndrome. Clinically, the newborn presented with bilateral anophthalmia, cleft palate, dysmorphic facies with a rounded forehead, hypertelorism, micrognathia, low-set ears and a short neck, syndactility and bilateral cryptorchidism. Trans fontanellar ultrasound revealed tri ventricular hydrocephalus. Cerebral MRI angiography showed malformative tri-ventricular hydrocephalus, hypoplasia of the brainstem and cerebellum, and poly-microgyria. Transthoracic and renal ultrasonography were unremarkable, and the chest X-ray was normal. The authors discuss the malformative clinical and para-clinical aspects of this syndrome, multidisciplinary management and the importance of prenatal diagnosis.
文摘Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of Lynch syndrome showing excellent response to immunotherapy. A 59-year-old male with a history of rectal cancer 30 years ago came to the hospital due to a fever and further found a large necrotic colon mass. Biopsy was positive for colorectal cancer;however, due to the size of the tumor, the patient was deemed not a surgical candidate and offered hospice with palliative chemotherapy. Based on further workup, the patient was diagnosed with Lynch syndrome, with colon cancer determined to be responsive to Immunotherapy. He was started on JEMPERLI (Dosterlimab-gxly), and after three cycles of therapy, imaging and PET scan were repeated, showing decreased activity and extent of the tumor—a tremendous success.
基金supported by the grants from the CAMS Innovation Fund for Medical Sciences(CIFMS)(Grant No.:2021-I2M-1-026)the Beijing Natural Science Foundation of China(Grant Nos.:7212155 and 7162135).
文摘Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,including improving glucose and lipid metabolism,enhancing insulin sensitivity,reducing oxidative stress and inflammation,promoting ceramides degradation,and stimulating adipose tissue vascularity.Based on those,it can serve as a positive regulator in many metabolic syndromes,such as type 2 diabetes(T2D),cardiovascular diseases,non-alcoholic fatty liver disease(NAFLD),sarcopenia,neurodegenerative diseases,and certain cancers.Therefore,a promising therapeutic approach for treating various metabolic diseases may involve elevating ADP levels or activating ADP receptors.The modulation of ADP genes,multimerization,and secretion covers the main processes of ADP generation,providing a comprehensive orientation for the development of more appropriate therapeutic strategies.In order to have a deeper understanding of ADP,this paper will provide an all-encompassing review of ADP.