Objective:To retrospectively analyze the current status of consultation, clinical characteristics, and treatment status of patients with IgG4-related disease (IgG4-RD) in order to provide assistance and a basis for early and standardized diagnosis and treatment.Methods:IgG4-RD cases admitted to our hospital from June 2015 to October 2023 were collected. The details of patients' basic information, initial symptoms, department visits, laboratory and imaging findings, histopathological examination results, and treatment plans were recorded. A statistical descriptive analysis was performed on the data.Results:A total of 105 patients with IgG4-RD were included, with a median age of 59.0 (18.0, 78.0) years. The main departments visited were clinical immunology and gastroenterology (83.8%, 88/105). The median diagnostic duration was eight months, with a maximum of 300 months, and 33.3% (35/105) of patients needed over one year for diagnosis. 92 cases underwent histopathological examinations and IgG4 staining, with a total positivity rate of 87.0% (80/92). Among these, sixteen cases underwent pathological examination after surgery, with a positivity rate of 100%; the remaining 76 cases out of 92 underwent liver biopsy, with a positivity rate of 76.1%. Out of these, there were 22 cases from the pancreas, 21 from the submaxillary gland, nine from the labial gland, and seven each from the duodenal papilla and liver, with positivity rates of 81.8%, 81.0%, 55.6%, 85.7%, and 85.7%, respectively. Eleven cases (10.5%) with normal serum IgG4 were diagnosed based on multi-organ involvement and pathological results. 94 cases (89.5%) had elevated IgG4, with a predominance of＞2.70 g/L. The median follow-up period for the 87 cases was 14 months. Two cases had poor response, twelve patients relapsed, five cases relapsed without combined drug treatment after surgery, five cases relapsed due to drug withdrawal, and two cases relapsed while tapering off steroids.Conclusions:As a multisystem disease, IgG4-RD still faces the difficulties of time-consuming diagnosis and inappropriate treatment. Therefore, it is necessary to rely on a multidisciplinary collaboration model to improve the awareness level and promote the early and standardized diagnosis and treatment of patients with IgG4-RD.

Objective:To retrospectively analyze the current status of consultation, clinical characteristics, and treatment status of patients with IgG4-related disease (IgG4-RD) in order to provide assistance and a basis for early and standardized diagnosis and treatment.Methods:IgG4-RD cases admitted to our hospital from June 2015 to October 2023 were collected. The details of patients' basic information, initial symptoms, department visits, laboratory and imaging findings, histopathological examination results, and treatment plans were recorded. A statistical descriptive analysis was performed on the data.Results:A total of 105 patients with IgG4-RD were included, with a median age of 59.0 (18.0, 78.0) years. The main departments visited were clinical immunology and gastroenterology (83.8%, 88/105). The median diagnostic duration was eight months, with a maximum of 300 months, and 33.3% (35/105) of patients needed over one year for diagnosis. 92 cases underwent histopathological examinations and IgG4 staining, with a total positivity rate of 87.0% (80/92). Among these, sixteen cases underwent pathological examination after surgery, with a positivity rate of 100%; the remaining 76 cases out of 92 underwent liver biopsy, with a positivity rate of 76.1%. Out of these, there were 22 cases from the pancreas, 21 from the submaxillary gland, nine from the labial gland, and seven each from the duodenal papilla and liver, with positivity rates of 81.8%, 81.0%, 55.6%, 85.7%, and 85.7%, respectively. Eleven cases (10.5%) with normal serum IgG4 were diagnosed based on multi-organ involvement and pathological results. 94 cases (89.5%) had elevated IgG4, with a predominance of＞2.70 g/L. The median follow-up period for the 87 cases was 14 months. Two cases had poor response, twelve patients relapsed, five cases relapsed without combined drug treatment after surgery, five cases relapsed due to drug withdrawal, and two cases relapsed while tapering off steroids.Conclusions:As a multisystem disease, IgG4-RD still faces the difficulties of time-consuming diagnosis and inappropriate treatment. Therefore, it is necessary to rely on a multidisciplinary collaboration model to improve the awareness level and promote the early and standardized diagnosis and treatment of patients with IgG4-RD.

To analyze the correlation between lipid profile and disease activity in patients with inflammatory bowel disease (IBD).A total of 307 Crohn′s disease (CD) patients, 232 ulcerative colitis (UC) patients and 165 healthy subjects from the same geographic region were included. Total cholesterol (TC), triglyceride (TG), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C) and lipoprotein(a)[Lp(a)] were retrieved from their medical records. Crohn disease activity index (CDAI) and Mayo scores were calculated as measurement of disease severity for CD and UC separately. Patients with CD and UC had lower TC, TG, HDL-C and LDL-C levels than those in control group ( P<0.05). Additionally, CDAI was negatively associated with TC, HDL-C and LDL-C levels ( r=-0.218, -0.210, -0.176, P<0.05), while TG level was not associated with CDAI. Mayo scores was not significantly associated with TC, HDL-C, LDL-C and TG. Patients with CD had higher Lp(a) levels than those in UC and control group ( P<0.05). Furthermore, patients with active CD had higher Lp (a) levels than those with inactive disease ( P<0.05).The Lp(a) levels in CD patients were positively associated with CDAI ( r=0.151, P<0.05), while Lp(a) level in UC group was nor assocriated with Mayo score. Patients with IBD have dyslipidemia and lipid profile is associated with disease activity in CD patients.

Protein neddylation is catalyzed by a three-enzyme cascade, namely an E1 NEDD8-activating enzyme (NAE), one of two E2 NEDD8 conjugation enzymes and one of several E3 NEDD8 ligases. The physiological substrates of neddylation are the family members of cullin, the scaffold component of cullin RING ligases (CRLs). Currently, a potent E1 inhibitor, MLN4924, also known as pevonedistat, is in several clinical trials for anti-cancer therapy. Here we report the discovery, through virtual screening and structural modifications, of a small molecule compound HA-1141 that directly binds to NAE in both

Background/Aims: Some inflammatory bowel disease (IBD) patients in remission suffer from irritable bowel syndrome (IBS)-like symptoms (IBD-IBS). The pathogenesis has not yet been elucidated. The study aim is to evaluate relationships among quality of life (QOL), psychological status, and visceral sensitivity, and explore the formation mechanism of IBD-IBS. Methods: Forty-seven patients with Crohn’s disease in remission, 24 ulcerative colitis in remission, 26 IBS, and 20 healthy controls were included in the study. The abdominal pain, QOL, anxiety, and depression were evaluated through questionnaires. Visceral sensitivity was measured by rectal balloon distension. The serum levels of 5-hydroxytryptamine (5-HT) and nerve growth factor (NGF) were measured by enzyme-linked immunosorbent assay. The expressions of tryptase, 5-HT, NGF, and related receptors in colonic tissues were detected by immunohistochemistry and western blot. Results: Prevalence of IBS-like symptoms in Crohn’s disease and ulcerative colitis patients in clinical remission was 29.8% and 50.0%, respectively. The QOL was lower, the anxiety/depression scores were higher in IBD-IBS patients than those without IBS-like symptoms. Additionally, patients with IBD-IBS existed visceral hypersensitivity. Besides, abdominal pain was associated with poor QOL, visceral hypersensitivity, anxiety, and depression in IBD-IBS patients. The number of mast cells (MCs) and expressions of 5-HT, NGF, and related receptors were higher in IBD-IBS patients than those with no such symptoms. The serum levels of 5-HT and NGF positively correlated with abdominal pain and visceral hypersensitivity. Conclusion IBD-IBS patients may have low QOL and psychological abnormalities, as wells as visceral hypersensitivity which may be related to increased 5-HT and NGF levels released from activated mast cells.

Background/Aims: Some inflammatory bowel disease (IBD) patients in remission suffer from irritable bowel syndrome (IBS)-like symptoms (IBD-IBS). The pathogenesis has not yet been elucidated. The study aim is to evaluate relationships among quality of life (QOL), psychological status, and visceral sensitivity, and explore the formation mechanism of IBD-IBS. Methods: Forty-seven patients with Crohn’s disease in remission, 24 ulcerative colitis in remission, 26 IBS, and 20 healthy controls were included in the study. The abdominal pain, QOL, anxiety, and depression were evaluated through questionnaires. Visceral sensitivity was measured by rectal balloon distension. The serum levels of 5-hydroxytryptamine (5-HT) and nerve growth factor (NGF) were measured by enzyme-linked immunosorbent assay. The expressions of tryptase, 5-HT, NGF, and related receptors in colonic tissues were detected by immunohistochemistry and western blot. Results: Prevalence of IBS-like symptoms in Crohn’s disease and ulcerative colitis patients in clinical remission was 29.8% and 50.0%, respectively. The QOL was lower, the anxiety/depression scores were higher in IBD-IBS patients than those without IBS-like symptoms. Additionally, patients with IBD-IBS existed visceral hypersensitivity. Besides, abdominal pain was associated with poor QOL, visceral hypersensitivity, anxiety, and depression in IBD-IBS patients. The number of mast cells (MCs) and expressions of 5-HT, NGF, and related receptors were higher in IBD-IBS patients than those with no such symptoms. The serum levels of 5-HT and NGF positively correlated with abdominal pain and visceral hypersensitivity. Conclusion IBD-IBS patients may have low QOL and psychological abnormalities, as wells as visceral hypersensitivity which may be related to increased 5-HT and NGF levels released from activated mast cells.

Objective:Mesenteric fat hypertrophy is present in about a quarter of Crohn′s disease (CD) patients and it can be easily detected by bowel ultrasound (US). The purpose of this research was to assess the correlation between mesenteric fat hypertrophy and behavior and activity of CD.Methods:A total of 89 CD patients who admitted to the First Affiliated Hospital of Nanjing Medical University from August 2018 to November 2019 were recruited in this study. The total CD patients were divided into two groups depending on with or without mesenteric fat hypertrophy by US tests. Crohn′s disease activity index (CDAI), simplified endoscopic score for Crohn′s disease (SES-CD), serum inflammatory indicators and fecal calprotectin were assessed.Results:Mesenteric fat hypertrophy was significantly associated with stricturing behavior (B2, P<0.01). CDAI ( P=0.002) , blood platelet ( P=0.001) , C-reactive protein ( P=0.024) , fecal calprotectin ( P=0.004) and bowel wall thickness ( P<0.01) in patients with mesenteric fat hypertrophy were significantly higher than those without, but not the erythrocyte sedimentation rate ( P=0.110) and SES-CD ( P=0.115) . Serum albumin ( P=0.001) in patients with mesenteric fat hypertrophy was lower than that in patients without mesenteric fat hypertrophy. Conclusion:Mesenteric fat hypertrophy is correlated with intestinal stenosis and disease activity in patients with Crohn′s disease.

A novel method was proposed for the quantification of nitrite ion in aqueous samples by the combination of an advanced quantitative fluorescence model with ratiometric fluorescence indicator 2 , 3-diaminonaphthalene . The proposed method was tentatively applied to direct quantitative determination of nitrite ion in turbid environmental water samples containing both scatterers and absorbers; and its performance was thoroughly investigated and evaluated. Experimental results showed that the proposed method could realize accurate quantitative determination of nitrite ion in environmental water samples with recovery rates in the range of 90. 8%-103. 0%, which were comparable to the corresponding values of HPLC-DAD experiments. The limit of detection and limit of quantification of the proposed method were estimated to be 1 . 9 and 5. 8 nmo/L, respectively.