Immunoglobulin A nephropathy （IgAN） is the primary glomerulonephritis with the highest incidence rate in the world. It is also the main cause of end-stage renal disease （ESRD） in China， which has brought heavy economic burden to the society and patient families. Traditional Chinese medicine （TCM） has certain advantages in treating IgAN. In TCM， IgAN is classified into consumptive disease， hematuria， and edema categories， with the location in the kidney and involving the lung， liver， and spleen. Professor Ren Jixue， a master of TCM， believes that kidney deficiency and spleen deficiency are the root causes of IgAN， and the throat is the source of the disease. He proposed the theory of throat-kidney correlation and used the method of tonifying kidney and invigorating spleen as well as detoxifying and relieving sore-throat to treat IgAN， achieving significant therapeutic effects. Studies have shown that IgAN is closely related to mucosal immune defense. IgAN patients often experience recurrent and gradually worsening symptoms due to mucosal infections， and polymeric Ig receptor （PIgR） is an important component of mucosal defense function. The lack of PIgR leads to the accumulation of IgA molecules in the mucosal lamina propria， and the molecules enter the bloodstream in large quantities and ultimately deposit in the kidneys， causing kidney damage. Complement regulatory protein complement receptor type 1 （CR1） exists on red blood cells and glomeruli and has the function of inhibiting the activation and differentiation of B cells， clearing immune complexes， and inhibiting excessive activation of the complement system. Therefore， regulating the immune defense function through the mucosal-renal axis mediated by PIgR-CR1 will be an important target for preventing and treating IgAN. Based on the theory of throat-kidney correlation， this article explores the effects and molecular mechanisms of tonifying kidney and invigorating spleen as well as detoxifying and relieving sore-throat in preventing and treating IgAN by regulating the mucosal-kidney axis mediated by PIgR-CR1. It provides effective theoretical support and a scientific basis for TCM prevention and treatment of IgAN based on the theory of throat-kidney correlation.

Immunoglobulin A nephropathy （IgAN） is the primary glomerulonephritis with the highest incidence rate in the world. It is also the main cause of end-stage renal disease （ESRD） in China， which has brought heavy economic burden to the society and patient families. Traditional Chinese medicine （TCM） has certain advantages in treating IgAN. In TCM， IgAN is classified into consumptive disease， hematuria， and edema categories， with the location in the kidney and involving the lung， liver， and spleen. Professor Ren Jixue， a master of TCM， believes that kidney deficiency and spleen deficiency are the root causes of IgAN， and the throat is the source of the disease. He proposed the theory of throat-kidney correlation and used the method of tonifying kidney and invigorating spleen as well as detoxifying and relieving sore-throat to treat IgAN， achieving significant therapeutic effects. Studies have shown that IgAN is closely related to mucosal immune defense. IgAN patients often experience recurrent and gradually worsening symptoms due to mucosal infections， and polymeric Ig receptor （PIgR） is an important component of mucosal defense function. The lack of PIgR leads to the accumulation of IgA molecules in the mucosal lamina propria， and the molecules enter the bloodstream in large quantities and ultimately deposit in the kidneys， causing kidney damage. Complement regulatory protein complement receptor type 1 （CR1） exists on red blood cells and glomeruli and has the function of inhibiting the activation and differentiation of B cells， clearing immune complexes， and inhibiting excessive activation of the complement system. Therefore， regulating the immune defense function through the mucosal-renal axis mediated by PIgR-CR1 will be an important target for preventing and treating IgAN. Based on the theory of throat-kidney correlation， this article explores the effects and molecular mechanisms of tonifying kidney and invigorating spleen as well as detoxifying and relieving sore-throat in preventing and treating IgAN by regulating the mucosal-kidney axis mediated by PIgR-CR1. It provides effective theoretical support and a scientific basis for TCM prevention and treatment of IgAN based on the theory of throat-kidney correlation.

Objective To investigate the relationship between serum Rho-associated coiled coil containing protein kinase 2(ROCK2),syndecan-1(SDC-1),Apelin-13 and renal function and prognosis in patients with diabetes nephropathy.Methods From November 2019 to November 2021,153 patients with diabetic nephrop-athy admitted to the hospital were selected as the study group,and 153 patients with simple diabetes were se-lected as the simple diabetes group.Another 153 healthy individuals who underwent physical examinations during the same period were selected as the healthy control group.The levels of serum ROCK2,SDC-1 and Apelin-13 were determined by enzyme-linked immunosorbent assay.Pearson correlation analysis was used to analyze the correlations between the levels of serum ROCK2,SDC-1,and Apelin-13 and the clinical data of pa-tients with urinary nephropathy.Univariate and multivariate Logistic regression analyses were conducted to analyze the influencing factors of poor prognosis in patients with diabetic nephropathy.The receiver operating characteristic(ROC)curve was drawn to analyze the predictive value of serum ROCK2,SDC-1 and Apelin-13 for poor prognosis of patients with diabetes nephropathy.Results Compared with the healthy control group,the levels of serum ROCK2 and SDC-1 in the simple diabetes group and the study group increased,the level of Apelin-13 decreased,the estimated glomerular filtration rate(eGFR)decreased,and the ratio of urinary albu-min to creatinine(UACR)increased,and the differences were statistically significant(P＜0.05).The levels of serum ROCK2,SDC-1,Apelin-13 and UACR in the study group were higher than those in the simple diabe-tes group,while eGFR was lower than that in the simple diabetes group,and the differences were statistically significant(P＜0.05).Pearson correlation analysis indicated that the levels of serum ROCK2,SDC-1,and Apelin-13 in the study group were negatively correlated with eGFR(P＜0.05),and positively correlated with renal disease stage,creatinine,24-hour proteinuria,and UACR index(P＜0.05).Compared with the good prognosis group,the levels of serum ROCK2,DCC-1,Apelin-13 and UACR in the poor prognosis group in-creased(P＜0.05),and eGFR decreased(P＜0.05).Univariate and multivariate Logistic regression analyses indicated that the levels of serum ROCK2,SDC-1,and Apelin-13 were all influencing factors for the prognosis of patients(P＜0.05).ROC curve analysis indicated that the area under the curve(AUC)for the combined prediction of poor prognosis in patients by serum ROCK2,DCC-1,and Apelin-13 levels was significantly grea-ter than that by ROCK2(Z=2.854,P=0.004)and DCC-1(Z=2.426,P=0.015)and Apelin-13(Z=2.172,P=0.030)were predicted separately.Conclusion The serum ROCK2,DCC-1 and Apelin-13 levels in patients with diabetic nephropathy increase and are related to renal function.The combination of the three has a relatively high predictive efficacy for the prognosis of patients.

Objective:To investigate the clinicopathological characteristics, immunophenotype and differential diagnosis of atypical forms of microglandular hyperplasia of the cervix (AMGH).Methods:A total of 29 cases of AMGH diagnosed at the Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China from January 2010 to December 2024 were analyzed. Relevant clinical and pathological data of the patients were collected using the electronic medical record system and medical records copied from the outside hospitals. The patients were followed up.Results:Among the 29 cases, 28 were consultation cases, 22 (79%) of the 28 cases were considered as glandular neoplastic lesions by the original institutions. The nature of the lesion was uncertain in 1 case, the diagnosis was suspicious for AMGH in another 1 case, and only 4 cases were clearly diagnosed as AMGH. The median age of the 29 patients was 44 (43, 48) years. Eighteen (62%) of the 29 cases presented as cervical polyp. Twelve of the 16 tested cases were negative for human papillomavirus. The pathological presentation was complex and diverse, including solid, trabecular, cribriform, and papillary patterns, forming pseudo-invasive structures. The glandular epithelium and proliferating reserve cells had diverse morphologies, which presented with abundant eosinophilic cytoplasm or clear cytoplasm. Signet-ring or hobnail cells were also seen. The nuclear atypia was mild, with 0-7 mitotic figures per 10 HPF. Immature squamous metaplasia was noted. The stroma showed edema, myxoid change and hyaline degeneration, accompanied by infiltration of acute and chronic inflammatory cells. Immunohistochemistry demonstrated that p16 was negative in 8/16 of the cases or patchy positive in the other 8/16, Ki-67 positive rate was less than 10% in all 16 cases, p53 was wild phenotype (9/9), and carcinoembryonic antigen was negative in 4/5 cases and focally positive in 1/5 cases, while p63 was positive in 6/9 of the tested cases.Conclusions:AMGH is a benign non-neoplastic lesion of the cervical glands. Half of the cases occur in perimenopausal or postmenopausal women, often presenting as polypoid hyperplasia or localized cervical thickening/elevation with a friable, fragile texture. Microscopically, it may show a pseudoinvasive pattern, making it prone to misdiagnosis as a malignant lesion. Thus, differentiation from cervical adenocarcinoma, clear cell carcinoma and microglandular endometrioid carcinoma is required. Integration of clinical history, immunohistochemistry and molecular testing may aid in the differential diagnosis.

Objective:To investigate the clinicopathological characteristics, immunophenotype and differential diagnosis of atypical forms of microglandular hyperplasia of the cervix (AMGH).Methods:A total of 29 cases of AMGH diagnosed at the Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China from January 2010 to December 2024 were analyzed. Relevant clinical and pathological data of the patients were collected using the electronic medical record system and medical records copied from the outside hospitals. The patients were followed up.Results:Among the 29 cases, 28 were consultation cases, 22 (79%) of the 28 cases were considered as glandular neoplastic lesions by the original institutions. The nature of the lesion was uncertain in 1 case, the diagnosis was suspicious for AMGH in another 1 case, and only 4 cases were clearly diagnosed as AMGH. The median age of the 29 patients was 44 (43, 48) years. Eighteen (62%) of the 29 cases presented as cervical polyp. Twelve of the 16 tested cases were negative for human papillomavirus. The pathological presentation was complex and diverse, including solid, trabecular, cribriform, and papillary patterns, forming pseudo-invasive structures. The glandular epithelium and proliferating reserve cells had diverse morphologies, which presented with abundant eosinophilic cytoplasm or clear cytoplasm. Signet-ring or hobnail cells were also seen. The nuclear atypia was mild, with 0-7 mitotic figures per 10 HPF. Immature squamous metaplasia was noted. The stroma showed edema, myxoid change and hyaline degeneration, accompanied by infiltration of acute and chronic inflammatory cells. Immunohistochemistry demonstrated that p16 was negative in 8/16 of the cases or patchy positive in the other 8/16, Ki-67 positive rate was less than 10% in all 16 cases, p53 was wild phenotype (9/9), and carcinoembryonic antigen was negative in 4/5 cases and focally positive in 1/5 cases, while p63 was positive in 6/9 of the tested cases.Conclusions:AMGH is a benign non-neoplastic lesion of the cervical glands. Half of the cases occur in perimenopausal or postmenopausal women, often presenting as polypoid hyperplasia or localized cervical thickening/elevation with a friable, fragile texture. Microscopically, it may show a pseudoinvasive pattern, making it prone to misdiagnosis as a malignant lesion. Thus, differentiation from cervical adenocarcinoma, clear cell carcinoma and microglandular endometrioid carcinoma is required. Integration of clinical history, immunohistochemistry and molecular testing may aid in the differential diagnosis.

OBJECTIVE: To explore the clinical and genetic characteristics of a patient with hypertrophic cardiomyopathy as the initial manifestation of Mucopolysaccharidosis type Ⅲ A (MPS Ⅲ A). METHODS: A female patient with MPS Ⅲ A who was admitted to the Affiliated Hospital of Jining Medical University in January 2022 and her family members (seven individuals from three generations) were selected as the study subjects. Clinical data of the proband were collected. Peripheral blood samples of the proband was collected and subjected to whole exome sequencing. Candidate variants were verified by Sanger sequencing. Heparan-N-sulfatase activity was determined for the disease associated with the variant site. RESULTS: The proband was a 49-year-old woman, for whom cardiac MRI has revealed significant thickening (up to 20 mm) of left ventricular wall and delayed gadolinium enhancement at the apical myocardium. Genetic testing revealed that she has harbored compound heterozygous variants in exon 17 of the SGSH gene, namely c.545G>A (p.Arg182His) and c.703G>A (p.Asp235Asn). Based on guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be pathogenic (PM2_Supporting +PM3+PP1Strong+PP3+PP4; PS3+PM1+PM2_Supporting +PM3+PP3+PP4). Sanger sequencing confirmed that her mother was heterozygous for the c.545G>A (p.Arg182His) variant, whilst her father, sisters and her son were heterozygous for the c.703G>A (p.Asp235Asn) variant. Determination of blood leukocyte heparan-N-sulfatase activity suggested that the patient had a low level of 1.6 nmol/(g·h), whilst that of her father, elder and younger sisters and son were all in the normal range. CONCLUSION The compound heterozygous variants of the SGSH gene probably underlay the MPS ⅢA in this patient, for which hypertrophic cardiomyopathy is an associated phenotype.
Female ; Humans ; Cardiomyopathy, Hypertrophic ; Contrast Media ; East Asian People ; Gadolinium ; Mucopolysaccharidosis III ; Mutation ; Pedigree ; Male ; Middle Aged

Since the implementation of the Measures for the Management of Radiation Workers’ Occupational Health in November 2007, it has played an extremely important role in protecting the occupational health of radiation workers. There are more than 700 000 radiation workers in about 100 000 workplaces with potential radiation exposure, as well as a large number of miners exposed to high levels of radon. As the radiation health monitoring project suggests, measures of occupational health management such as personal dose monitoring and occupational health examination of radiation workers have been widely implemented and achieved good results in the protection of radiation workers. However, the risks of chromosomal aberration and specific turbidity of the eye lens of radiation workers have increased in high-risk positions such as interventional radiology, nuclear medicine, and industrial flaw detection. The control of high radon exposure in miners needs to be strengthened. It is necessary to adapt to the new situation in view of new challenges and actively promote the revision of the Measures for the Management of Radiation Workers’ Occupational Health, so as to further improve the occupational health management of radiation workers in China.

Objective To investigate the effect of tonifying kidney and promoting pharynx prescription on IgA nephropathy model rats and the mechanism of regulating the TLR4 signaling pathway.Methods After SD rats were randomly divided into groups,the rat model of IgA nephropathy was replicated.After the medication,urinary RBC,UTP,blood creatinine,urea nitrogen,renal tissue TLR4,MyD88,NF-κB,MCP-1 expression,C1GALT1 and cosmoc mRNA expression in peripheral blood,TLR4,NF-κB and IL-6 in serum were observed.Results ①Compared with the blank group,urinary RBC,UTP,and renal tissue TLR4,MyD88 and NF-κB,MCP-1 expression,C1GALT1 and cosmoc mRNA expression in peripheral blood,TLR4,NF-κB and IL-6 in serum increased in the model group(P<0.05).②Compared with the model group,UTP,urinary RBC,renal tissue TLR4,MyD88 and NF-κB,MCP-1 expression,C1GALT1 and cosmoc mRNA expression in peripheral blood,the content of TLR4 and NF-κB,IL-6 were decreased(P<0.05)in tonifying kidney and promoting pharynx prescription group;There was no significant change in renal function.Conclusions ①The tonifying kidney and promoting pharynx prescription can effectively reduce hematuria and proteinuria in rats with IgA nephropathy.②The tonifying kidney and promoting pharynx prescription can reduce renal injury by improving the immune response mediated by TLR4 Signal transduction system induced by mucosal infection and reducing abnormal glycosylation IgA1 by adjusting key enzyme C1GalT1 and molecular companion Cosmc in IgA1 glycosylation process.

Pregnant women are a group of people in a special period, once sudden cardiac arrest (CA) occurs, it will threaten the life of both mother and child. It has become a great challenge for hospital, doctors and nurses to minimize maternal mortality during pregnancy. All the efforts should ensure the safety of both mother and child throughout the perinatal period. Because difference of the cardiopulmonary resuscitation strategies for common CA patients of the same age, the resuscitation strategies for CA patients during pregnancy need consider the patient's gestational age and fetal condition. Different resuscitation techniques, such as manual left uterine displacement (MLUD), will involve perimortem cesarean delivery (PMCD). At the same time, drugs should be reasonably used for different causes of CA during pregnancy, such as hypoxemia, hypovolemia, hyperkalemia or hypokalemia and other electrolyte disorders and hypothermia in 4Hs, as well as thrombosis, pericardial tamponade, tension pneumothorax and toxicosis in 4Ts. In view of the fact that many causes of CA in pregnancy are preventable, it is more necessary to introduce guidelines for CA in pregnancy in line with our national conditions for clinical guidance. This paper systematically reviewed the pathophysiological characteristics of CA during pregnancy, the high-risk factors of CA during pregnancy, and identified the correct resuscitation methods and prevention and treatment strategies of CA during pregnancy.

Objective:To explore the detection rate, clinical characteristics of vulvar squamous intraepithelial lesion (SIL).Methods:Women diagnosed with vulvar high-grade squamous intraepithelial lesions (HSIL) through colposcopy-guided biopsy from January 1, 2018 to August 31, 2022 in Obstetrics and Gynecology Hospital of Fudan University were included in a 1∶1 ratio with patients diagnosed with vulvar low-grade squamous intraepithelial lesions (LSIL) during the same period. Clinical characteristics including human papillomavirus (HPV) infection rate, genotype, cytology result, colposcopy impression, and lesion location were retrospectively analyzed.Results:(1) The proportion of vulvar SIL detected by colposcopy-guided biopsy increased annually from 2018 to 2022, with rates of 1.64% (740/45 057), 2.34% (1 110/47 402), 2.68% (1 108/41 335), 3.26% (1 536/47 078), 3.31% (667/20 155), with an average rate of 2.57% (5 161/201 027). (2) A total of 1 096 cases of vulvar HSIL and 1 096 cases of vulvar LSIL were included. The overall infection rate of HPV was 92.7% (1 993/2 150), with higher infection rate in vulvar HSIL patients than that in vulvar LSIL patients [96.0% (1 012/1 054) vs 89.5% (981/1 096); χ2=33.62, P<0.001]. Among vulvar HSIL patients, the common HPV genotype from high to low were HPV 16 (66.7%), HPV 52 (14.3%), and HPV 58 (10.0%). For vulvar LSIL patients, the most common HPV genotype were respectively HPV 16 (24.9%), HPV 6 (20.1%) and HPV 52 (17.1%). The overall sensitivity rate of cytology was 53.6%, with no significance difference between vulvar LSIL and HSIL groups (54.3% vs 52.9%; χ2=0.40, P=0.526). The accuracy of colposcopy impression for vulvar HSIL was lower than that for vulvar LSIL [40.2% (163/405) vs 81.7% (380/465); χ2=158.72, P<0.001]. About 57.3% (1 257/2 192) of the patients had concomitant cervical and vaginal lesions, with a higher rate in vulvar HSIL group than that in vulvar LSIL group [62.6% (686/1 096) vs 52.1% (571/1 096); χ2=24.67, P<0.001]. Unifocal lesion was the main type, with no significance difference between vulvar LSIL and HSIL groups [81.4% (381/468) vs 82.5% (386/468); χ2=0.18, P=0.671]. The most common lesion locations were the posterior commissure, followed by labia minora, vaginal vestibule, labia majora, perianal and clitoris. Conclusions:The detection rate of vulvar SIL under colposcopy is about 3%, and the infection rate of HPV is 92.7%. Vulvar SIL, especially vulvar HSIL, is likely to cause concomitant cervical and vaginal lesions. The accuracy of colposcopy in diagnosing vulvar HSIL is low. Therefore a comprehensive and careful examination of the vulva is necessary and suspicious vulvar lesions should be undergone colposcopy-guided biopsy for diagnosis.