Objective:To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies.Methods:A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed.Results:A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common.Conclusions:Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.

Objective:To explore the current status of medication adherence to immunosuppressants in lung transplant recipients and to analyze its correlation with medication beliefs and perceived social support.Methods:This was a cross-sectional study. From June 2022 to April 2023, totally 233 lung transplant recipients who were followed up in the Department of Lung Transplantation at China-Japan Friendship Hospital were selected by convenience sampling. The Basel Assessment of Adherence to Immunosuppressive Medications Scale (BAASIS), the Chinese version of the Beliefs about Medicines Questionnaire, and the Perceived Social Support Scale (PSSS) were used for the survey. Multiple linear regression analysis was conducted to explore the correlation between medication adherence to immunosuppressants, medication beliefs, and perceived social support in these lung transplant recipients.Results:A total of 233 questionnaires were distributed, with 213 valid responses received (91.42%). The incidence of non-adherence to immunosuppressants among the 213 transplant recipients was 41.78% (89/213), with the most common issue being not taking medication on time (27.23%, 58/213). Multiple linear regression analysis showed that age and perceived social support were influencing factors of medication adherence ( P<0.05) . Conclusions:The current level of medication adherence to immunosuppressants in lung transplant recipients is relatively poor. Medication adherence is correlated with age and the level of perceived social support. Healthcare providers should pay attention to medication adherence in younger patients and enhance their perceived social support to increase adherence to immunosuppressive medications.

Peripheral blood eosinophilia is now becoming an increasingly prevalent scenario in clinical practice. Eosinophilia can be physiological or associated with many diseases involving multiple organ systems. Due to various etiologies and complex pathogenesis, the clinical characteristics of patients with eosinophilia can range from asymptomatic to severe organ dysfunction, and their prognosis may also vary greatly. At present, medical practitioners are not paying more attention to the peripheral eosinophil in routine blood tests. Therefore, this article aims to summarize the incidence of eosinophilia in healthy and diseased population in order to draw attention and serve as a reference for future research.

Peripheral blood eosinophilia is now becoming an increasingly prevalent scenario in clinical practice. Eosinophilia can be physiological or associated with many diseases involving multiple organ systems. Due to various etiologies and complex pathogenesis, the clinical characteristics of patients with eosinophilia can range from asymptomatic to severe organ dysfunction, and their prognosis may also vary greatly. At present, medical practitioners are not paying more attention to the peripheral eosinophil in routine blood tests. Therefore, this article aims to summarize the incidence of eosinophilia in healthy and diseased population in order to draw attention and serve as a reference for future research.

OBJECTIVE: To explore the genetic basis for two children with sporadic neurofibromatosis type 1 (NF1) complicated with nephrotic syndrome (NS). METHODS: Clinical data of the children were collected. Both children were subjected to high-throughput sequencing, and candidate variants were verified by Sanger sequencing. RESULTS: Both children had café-au-lait macules, subaxillary freckle and Lisch nodules. Child 1 also had congenital tibiofibular pseudarthrosis on the left side. Genetic testing revealed that child 1 has harbored a heterozygous c.844C>T variant in the exon 8 of the NF1 gene, whilst child 2 has harbored a heterozygous c.1246C>T variant in the exon 11 of the NF1 gene. Both children were diagnosed with NF1 and have developed pronounced proteinuria, hypoalbuminemia, hypercholesterolemia and pitting edema at the ages of 3 and 10, respectively. Renal biopsy of child 2 has revealed minimal change nephropathy, and the diagnosis of nephrotic syndrome was established. Child 1 was treated with glucocorticoid, and child 2 was treated with glucocorticoid in combination with mycophenolate mofetil. The NS was relieved with no recurrence during 1 year's follow-up. CONCLUSION NF1 combined with NS is rare in the clinical settings. The prognosis of children with NF1 combined with minimal change nephropathy is relatively good. Detection of NF1 gene variant can facilitate early identification and diagnosis of NF1.
Child ; Humans ; Neurofibromatosis 1/genetics* ; Nephrotic Syndrome/genetics* ; Nephrosis, Lipoid ; Glucocorticoids ; Genetic Testing

BACKGROUND: Cardiovascular (CV) disease is the leading cause of morbidity and mortality in adults with type 2 diabetes (T2D). The aim of this study was to determine the CV risk in Chinese patients with T2D based on the 2019 European Society of Cardiology (ESC) and the European Association for the Study of Diabetes (EASD) guidelines on diabetes, pre-diabetes, and CV diseases. METHODS: A total of 25,411 patients with T2D, who participated in the study of China Cardiometabolic Registries 3B study, were included in our analysis. We assessed the proportions of patients in each CV risk category according to 2019 ESC/EASD guidelines. RESULTS: Based on the 2019 ESC/EASD guidelines, 16,663 (65.6%), 1895 (7.5%), and 152 (0.6%) of patients were included in "very high risk," "high risk," and "moderate risk" categories, respectively. The proportions of patients in each category varied based on age, sex, body mass index, and duration. While 58.7% (9786/16,663) of elderly patients were classified to "very high risk" group, 89.6% (3732/4165) of patients with obesity were divided into "very high risk" group. Almost all patients with a duration of diabetes >10 years had "very high risk" or "high risk." However, 6701 (26.4%) of Chinese T2D patients, who had shorter duration, and one or two risk factors, could not be included in any category (the "unclear risk" category). CONCLUSIONS In China, most patients with T2D have "very high" or "high" CV risk based on 2019 ESC/EASD guidelines. However, the risk of patients in "unclear risk" group needs to be further classified.
Adult ; Aged ; Cardiovascular Diseases/epidemiology* ; Cross-Sectional Studies ; Diabetes Mellitus, Type 2 ; Heart Disease Risk Factors ; Humans ; Risk Factors

Chinese medicine enema is an effective guiding method to treat diabetic kidney disease, a common chronic complication of diabetes, by regulating the function of the intestines to increase clearing and lower turbidity. We reviewed the treatment of diabetic kidney disease (DKD) with traditional Chinese medicine enemas and found that commonly used traditional Chinese medicines included Rhei Radix Et Rhizoma (Rheum palmatum L.), Ostrea concha (Ostrea gigas Thunberg), Taraxaci Herba (Taraxacum mongolicum Hand.-Mazz.), Astmgali Radix (Astragalus membranaceus Bge. var.mongholicus(Bge.) Hsiao), Salviae Miltiorrhizae Radix Et Rhizoma (Salvia miltiorrhiza Bge.), and Aconiti Lateralis Radix Praeparata (Aconitum carmichaelii Debx). The mechanism of the adjuvant therapeutic effect on DKD may relate to regulating intestinal flora, inhibiting inflammation and oxidative stress and protecting against kidney injury.

OBJECTIVE：To stud y the effects of different processed products of Whitmania pigra on hemorheology and coagulation indexes in acute blood stasis model rats. METHODS ：SD rats were randomly divided into blank group ，model group ， aspirin group ，W. pigra hang-dried product low- ，medium- and high-dose groups ，W. pigra talcum powder-ironed product low- ， medium- and high-dose groups ，W. pigra wine bran-processed product low- ，medium- and high-dose groups ，with 6 rats in each group. Except for blank group ，other groups received subcutaneous injection of epinephrine hydrochloride and ice water bath for 15 d to induce acute blood stasis model. From the 8th day of modeling ，rats in aspirin group were given aspirin 0.2 g/kg intragastrically. Rats in each dose group of W. pigra processed products were given relevant medicine 0.35，1.4，3.5 g/kg intragastrically（calculated by crude drug ）. Rats in blank group and model group were given constant volume of normal saline intragastrically， once a day ， for consecutive 8 days. Hemorheology indexes as whole blood viscosity （high， medium and low shearrate ），plasma viscosity ，erythrocyte com deformation index ，erythrocyte aggregation index ，hematocrit， and blood coagulation indexes as prothrombin time （PT）， mail：wcl19960125@163.com activated partial prothrombin time （APTT），thrombin time （TT）were determined. RESU LTS：Compared with blank group ，whole blood viscosity under different shear rates ，plasma viscosity ， erythrocyte aggregation index and hematocrit of model group were increased significantly ，while erythrocyte deformation index was significantly decreased ，PT，TT and APTT were significantly shortened （P＜0.01）. Compared with model group ，whole blood viscosity under different shear rates ，plasma viscosity ，erythrocyte aggregation index and hematocrit of aspirin group and W. pigra hang-dried product ，talcum powder-ironed product ，wine bran-processed product high-dose groups were decreased significantly ， while erythrocyte deformation index were significantly increased ，and PT （only W. pigra talcum powder-ironed products high-dose group），APTT（except for W. pigra hang-dried products high-dose group ）and TT were prolonged significantly. The whole blood viscosity of W. pigra hang-dried product medium-dose group under low shear rate ，and those of W. pigra talcum powder-ironed product low-dose ，wine bran-processed product medium-dose groups under low and medium shear rates were decreased significantly. Erythrocyte deformation index of W. pigra talcum powder-ironed product medium-dose group was increased significantly ，while erythrocyte aggregation index was decreased significantly ，and PT ，TT were prolonged significantly. APTT of W. pigra hang-dried product medium-dose group was prolonged significantly. Hematocrit of W. pigra wine bran-processed product low-dose group was decreased significantly （P＜0.05 or P＜0.01）. CONCLUSIONS ： W. pigra hang-dried， talcum powder-ironed and wine bran-processed product can effectively improve hemorheology indexes and prolong blood coagulation time.

OBJECTIVE: To detect genetic variant in a sib-pair with Finnish type congenital nephrotic syndrome (CNF). METHODS: Clinical data of the sib-pair was reviewed. Coding regions of the NPHS1 gene was analyzed for the sib-pair and both parents. RESULTS: The sister and brother respectively developed severe proteinuria 1 month and 28 days after birth, in addition with low serum albumin, hypercholesterolemia and severe edema, which were suggestive of CNF. Genetic testing identified that the sib-pair has both carried two heterozygous variants of NPHS1 gene, namely c.2605G>C (p.P869>A) and c.-61G>A, for which their father and mother were heterozygous carriers. CONCLUSION The c.2605G>C (p.869P>A) and c.-61G>A variants of the NHPS1 gene probably underlay the CNF in both sibs. The c.2605G>C(p.869P>A) was unreported previously.
Adult ; Female ; Humans ; Infant, Newborn ; Male ; Membrane Proteins/genetics* ; Mutation ; Nephrotic Syndrome/genetics* ; Siblings

OBJECTIVE: To explore the influencing factors of depressive symptom of employees in a mould-producing enterprises using two occupational stress models. METHODS: A total of 457 employees in a mould-producing enterprise were selected as study subjects using cluster sampling method. The Center for Epidemiological Study-Depression Scale, the Chinese version of Job Demand Content Questionnaire and the Effort-Reward Imbalance Questionnaire were used to investigate the level of depressive symptom and occupational stress by the job demand control(JDC) model and effort reward imbalance(ERI) model. RESULTS: Among the 457 employees, the median score of depressive symptom was 11.00, the detection rate of depressive symptom in the subjects was 23.4%(107/457). The incidence of occupational stress by the JDC and ERI models was 78.3%(358/457) and 62.6%(286/457), respectively. The rate of depressive symptom was statistically higher in the high occupational stress group than that of the low occupational stress group by the ERI model(26.6% vs 18.1%, P<0.05). The multivariate logistic regression analysis showed that both marital status and monthly income were influencing factors of depressive symptom by the JDC model(P<0.05). The marital status, monthly income and occupational stress were influencing factors of depressive symptom by the ERI model(P<0.05). The risk of depressive symptom was statistically higher in the high occupational stress group than that of the low occupational stress group(P<0.05). CONCLUSION: Employees in mould-producing enterprise have some depressive symptom. The marital status, monthly income and occupational stress by the ERI model are the main influencing factors of depressive symptom.