Gitelman syndrome（GS）is a salt-losing kidney disease caused by dysfunction of the Na-Cl cotransporter（NCC）in the distal tubular of the kidney. It often occurs after the age of 6. The main clinical features include hypokalemia，metabolic alkalosis，hypomagnesemia，hypocalciuria，hyperrenin-hyperaldosteronemia，and normal or low blood pressure. The child patient may show symptoms such as thirst，muscle weakness or cramps，salt craving，fatigue，etc.，which are prone to missed diagnosis and misdiagnosis. In severe cases，epileptic seizures，rhabdomyolysis，prolonged QT interval and abnormal rhythm may occur. Diagnosis requires the exclusion of conditions that can easily cause hypokalemic alkalosis，such as diuretic use and Bartter syndrome. Genetic testing for biallelic mutations in the SLC12A3 gene can confirm the diagnosis.Lifelong supplementation of potassium chloride and magnesium is required，along with regular re-examination and treatment adjustment，as well as monitoring of adverse drug reactions and complications. Therefore，Clinicians need to identify and manage GS.

Hand degloving injury represents one of the most severe forms of hand trauma, characterised by challenging treatment and a complex prognostic outcome. It is crucial to effectively utilise the degloved tissues in emergency or primary repair of a hand degloving injury. This consensus provides a comprehensive review of the existing literature on definition, classification, emergency assessment, debridement, judgment of skin viability, in situ repair of the degloved skin, and adjunctive treatment for degloving injury of hand. Based on conclusion of both domestic and international experiences, this expert consensus on the classification of hand degloving injury and the emergency repair with the avulsed skin is established, aiming to provide a guidance to surgeons on standardised treatment strategy and improve the management of hand degloving injury.

BACKGROUND: The available evidence regarding the benefits of percutaneous coronary intervention (PCI) on patients receiving dialysis with coronary artery disease (CAD) is limited and inconsistent. This study aimed to evaluate the association between PCI and clinical outcomes as compared with medical therapy alone in patients undergoing dialysis with CAD in China. METHODS: This multicenter, retrospective study was conducted in 30 tertiary medical centers across 12 provinces in China from January 2015 to June 2021 to include patients on dialysis with CAD. The primary outcome was major adverse cardiovascular events (MACE), defined as a composite of cardiovascular death, non-fatal myocardial infarction, and non-fatal stroke. Secondary outcomes included all-cause death, the individual components of MACE, and Bleeding Academic Research Consortium criteria types 2, 3, or 5 bleeding. Multivariable Cox proportional hazard models were used to assess the association between PCI and outcomes. Inverse probability of treatment weighting (IPTW) and propensity score matching (PSM) were performed to account for potential between-group differences. RESULTS: Of the 1146 patients on dialysis with significant CAD, 821 (71.6%) underwent PCI. After a median follow-up of 23.0 months, PCI was associated with a 43.0% significantly lower risk for MACE (33.9% [ n  = 278] vs . 43.7% [ n  = 142]; adjusted hazards ratio 0.57, 95% confidence interval 0.45-0.71), along with a slightly increased risk for bleeding outcomes that did not reach statistical significance (11.1% vs . 8.3%; adjusted hazards ratio 1.31, 95% confidence interval, 0.82-2.11). Furthermore, PCI was associated with a significant reduction in all-cause and cardiovascular mortalities. Subgroup analysis did not modify the association of PCI with patient outcomes. These primary findings were consistent across IPTW, PSM, and competing risk analyses. CONCLUSION This study indicated that PCI in patients on dialysis with CAD was significantly associated with lower MACE and mortality when comparing with those with medical therapy alone, albeit with a slightly increased risk for bleeding events that did not reach statistical significance.
Humans ; Percutaneous Coronary Intervention/methods* ; Male ; Female ; Coronary Artery Disease/drug therapy* ; Retrospective Studies ; Renal Dialysis/methods* ; Middle Aged ; Aged ; China ; Proportional Hazards Models ; Treatment Outcome

Objective:To explore the level of unmet need during chemotherapy for gastric cancer patients, analyze its dynamic trajectory and impact on the economic toxicity of chemotherapy.Methods:Convenience sampling was used to select 385 gastric cancer patients who received chemotherapy from June 2022 to December 2023 at the First Affiliated Hospital of Zhengzhou University. The level of unmet need for patients' first chemotherapy cycle (C 1) , second chemotherapy cycle (C 2) and third chemotherapy cycle (C 3) and economic toxicity at the end of chemotherapy were investigated separately. The latent class growth model (LCGM) was used to analyze classes of unmet need trajectories and to compare differences in patient economic toxicity across classes. Results:Finally 368 patients completed all data collection, and the effective response rate of survey respondents was 95.58%. As chemotherapy progressed, patients' unmet need scores gradually increased, and the unmet need scores of C 1, C 2, and C 3 were (35.54±5.44) , (47.32±6.45) , and (58.19±11.81) , respectively, which were categorized into three latent classes of medium-low level rapid increase group, medium level gradual increase group, and medium level stable group. There were 122 cases (33.15%) in medium-low level rapid increase group, 167 cases (45.38%) in medium level gradual increase group, and 79 cases (21.47%) in medium level stable group. The lowest economic toxicity score was found in medium-low level rapid increase group, followed by medium level gradual increase group, and the highest in medium level stable group, and the difference in economic toxicity scores among the three groups was statistically significant ( P<0.05) . The percentage of patients with positive economic toxicity in the three groups was 45.90% (56/122) , 28.14% (47/167) , and 18.99% (15/79) , respectively, with a statistically significant difference ( P<0.05) . Conclusions:Unmet needs of patients during chemotherapy for gastric cancer show a moderate to high level and gradually increase with the course of chemotherapy. The trajectory can be divided into three latent classes of medium-low level rapid increase group, medium level gradual increase group, and medium level stable group. There are differences in economic toxicity for patients with different classes of unmet need trajectories, with patients in increase or rapid increase groups facing severe economic toxicity.

Allogeneic haemopoietic stem cell transplantation (allo-HSCT)-related nephrotic syndrome is a rare complication, recognized as a clinical manifestation of chronic graft versus host disease (GVHD). T cell dysfunction is thought to play a significant role in the pathogenesis of allo-HSCT-related nephrotic syndrome, but the precise mechanism remains unclear. This paper reported a case of X-linked adrenoleukodystrophy (X-ALD) who had good control of the disease after allo-HSCT, but developed proteinuria and progressed to nephrotic syndrome after immunosuppressive therapy was tapered. Kidney biopsy revealed secondary membranous nephropathy, which responded well to treatment with glucocorticoids and tacrolimus. Limited literature exist on allo-HSCT-related nephrotic syndrome in children. This study provides a comprehensive summary of its mechanism, clinical features, pathology, diagnosis,and treatment, offering valuable insights for diagnosing and managing allo-HSCT-related nephrotic syndrome in pediatric patients.

This article reports a case of membranous nephropathy in an adolescent accompanied by monoclonal IgG1-κ deposition. The 16-year-old female patient was hospitalized for experiencing proteinuria and hematuria for more than 20 days. The patient had a history of mycoplasma infection and acute kidney injury, and renal pathology revealed glomerular membrane lesions accompanied by crescent formation. Electron microscopy showed electron dense deposits in the subepithelial and mesangial regions, and immunofluorescence demonstrated monotypic IgG1-κ deposits in the glomerulus. Bone marrow examination did not find any abnormal plasma cells, nor were there significant abnormalities in serum or urine free light chain κ/λ ratio. The diagnosis was proliferative glomerulonephritis characterized by membranous lesions with monoclonal IgG1-κ deposits. This disease is rare in children and adolescents, and currently there is limited understanding of its mechanism, with limited clinical treatment experience. This article aims to provide clinical insights through case analysis and literature review.

Tacrolimus is an immunosuppressant that was clinically used for organ transplantation in the 1990s. In the early 2000s, tacrolimus began to be used to treat pediatric kidney diseases in China. This article reviews the therapeutic effects, clinical dosages, and treatment methods of tacrolimus in the treatment of steroid-resistant, steroid-dependent, frequently relapsing, different pathological types, and monogenic mutation-related childhood nephrotic syndrome. It explores the clinical guiding role of machine learning in tacrolimus treatment for childhood nephrotic syndrome, aiming to provide references for the clinical research and application of tacrolimus in pediatric kidney diseases.

Objective:To investigate the association between anti-rituximab antibodies (ARA) and relapse after rituximab (RTX) therapy in children with frequently relapsing or steroid-dependent nephrotic syndrome (FRNS or SDNS).Methods:A retrospective cohort study was conducted. Clinical and laboratory data were collected from 48 FRNS or SDNS children treated with RTX in the Department of Pediatrics, General Hospital of Eastern Theater Command, between April 2024 and October 2024. Data included RTX dosing frequency, relapse events, peripheral CD20? B-cell counts, and ARA levels. With a 6-month observation period after the last RTX therapy, the children were divided into an ARA-positive group and an ARA-negative group based on ARA test results. Chi-square test, independent sample t-test, or Mann-Whitney U test were used to compare relapse rates and laboratory indicators between the two groups. The predictive value of ARA levels for relapse was evaluated using univariate receiver operating characteristic (ROC) curve analysis. Results:Among the 48 children (36 males, 12 females), the age of disease onset was 3.5 (2.0, 6.0) years, the ages at the first and last RTX treatments were 7.0 (5.0, 12.0) years and 9.5 (7.0, 13.0) years, respectively. The overall ARA positive rate was 29% (14/48). The relapse rate in the ARA-positive group was significantly higher than that in the negative group ( P<0.05). The ARA level was 0.01 (0.01, 5.88) μg/L, and all 12 children with ARA levels >5.88 μg/L relapsed. ROC curve analysis showed that ARA levels predicted relapse after RTX treatment in FRNS or SDNS children with an area under the curve (AUC) of 0.73, sensitivity of 0.50, specificity of 1.00, and an optimal cut-off value of 5.02 μg/L. All children received single-dose RTX therapy, with no significant difference in treatment frequency between the two groups ( P>0.05). At 3 months after the last rituximab therapy, CD20? B cell counts were significantly higher in the ARA-positive group ( P<0.05). During follow-up, 15% (7/48) of the children experienced infusion-related adverse reactions, with no significant difference in incidence between the two groups ( P>0.05). Conclusion:ARA is significantly associated with relapse in FRNS or SDNS children after RTX therapy.

Epicardial adipose tissue(EAT)is a type of fat tissue that is closely adjacent to the coronary arteries and myocardium,and it caused physiological and pathological changes to the body through the secretion of autocrine and paracrine active factors.EAT is regarded as a diagnostic marker and a potential therapeutic target for cardiovascular diseases,and it is of great significance to segment and quantify EAT.This article introduced the evolution of the EAT segmentation and quantification methods from the aspects of traditional imaging,atlas,and artificial intelligence.Furthermore,it reviewed the research progresses on automatically quantified EAT indices in the diagnosis and treatment of cardiovascular diseases.

The clinical data of a case of congenital anomalies of the kidney and urinary tract(CAKUT) complicated with ichthyosis diagnosed at Department of Pediatrics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University in November 2023 were retrospectively analyzed.The patient, male, 12 years old, exhibited left renal agenesis, right renal dysplasia, renal insufficiency, proteinuria, and ichthyosis.Whole-exome sequencing identified a microdeletion of approximately 1.80 Mb at p22.31 of the X-chromosome, encompassing the ANOS1 and STS genes.Additionally, a heterozygous missense mutation in the EHMT1 gene (c.3664C>A, exon26) on chromosome 9 was detected.The father is clinically normal and did not carry either variant.The mother has proteinuria and was found to carry the same X-chromosome microdeletion as the proband.