Cardiomyopathies are major causes of heart failure and sudden cardiac death, but nationwide epidemiological data from China remain limited. Following the inclusion of several cardiomyopathies in the first national rare disease list in 2018, contemporary trends in hospitalization and in-hospital outcomes have not been systematically evaluated. This study aimed to characterize national hospitalization patterns for cardiomyopathies from 2018 to 2022. We conducted a nationwide retrospective study using the Hospital Quality Monitoring System (HQMS), which covers secondary and tertiary hospitals across 31 provinces. Cardiomyopathies were identified using National Clinical Version 2.0 codes. We assessed age- and sex-standardized hospitalization rates, regional variation, comorbidities, device use, intensive care unit (ICU) admission, in-hospital mortality, length of stay, and hospital costs. Temporal trends were analyzed using Joinpoint regression to estimate the average annual percent change (AAPC). A total of 1,935,421 hospitalizations from 943,178 unique patients were included. The standardized hospitalization rate increased from 151.55 to 206.23 per 1,000,000 population (AAPC = 7.68
Renal fibrosis represents the core pathway through which chronic kidney disease progresses to end-stage renal failure. Therapeutic strategies targeting renal fibrosis provide an important approach for clinically mitigating the transition from acute kidney injury to chronic kidney disease. Macrophages, owing to their high heterogeneity in origin and phenotype, play a significant role in various stages of acute inflammation, tissue repair, and fibrosis, thereby holding substantial promise for the treatment of renal fibrosis. This review summarizes the pivotal role of renal macrophages in the transition from acute kidney injury to chronic kidney disease, with a focus on their temporal phenotypic reprogramming characteristics and the fibrotic microenvironment interaction network constituted by injured tubules, macrophages, and fibroblasts. On this basis, we systematically outline four core categories of macrophage-targeted intervention strategies: modulation of upstream signaling pathways, induction of macrophage phenotypic reprogramming, blockade of profibrotic factors and their downstream pathways, and selective depletion or engineered modification of profibrotic macrophage subsets. This review aims to provide new insights for future exploration of the timing, precision, and efficacy of macrophage-targeted therapies.
We report a rare case of peritoneal dialysis (PD)-associated peritonitis caused by Coxiella burnetii, an intracellular pathogen typically associated with Q fever. A 28-year-old female with lupus nephritis and end-stage kidney disease on PD presented with cloudy effluent and abdominal pain after consuming undercooked lamb. Despite initial empirical broad-spectrum antibiotic therapy (cefazolin/ceftazidime protocol), clinical symptoms persisted. Conventional bacterial, fungal, and mycobacterial cultures were negative. Metagenomic next-generation sequencing (mNGS) of the peritoneal effluent identified C. burnetii DNA. The treatment was transitioned to doxycycline and hydroxychloroquine resulted in clinical improvement and normalization of inflammatory markers. This case underscores the diagnostic value of mNGS in culture-negative peritonitis and emphasizes C. burnetii as an emerging pathogen in immunocompromised PD patients, particularly with zoonotic exposure histories.
Background Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disorder usually occurred in old-aged women. It can involve the kidneys, most commonly causing distal renal tubular acidosis (dRTA). Overt nephrogenic diabetes insipidus (NDI) is rare, and the simultaneous occurrence of both tubular defects as the presenting feature of pSS, particularly in adolescents, is exceptional and presents a challenging diagnostic puzzle. Case presentation: We present a case of a 17-year-old female presented with a 5-year history of polydipsia and polyuria (daily intake/output 6–7 L), who was found to have severe hypokalemia, hyperchloremic normal anion-gap metabolic acidosis and inappropriately alkaline urine, confirming distal RTA. Water deprivation test with desmopressin demonstrated a urine osmolality that remained below 150 mOsm/kg despite rising serum osmolality and lacked significant increase after desmopressin, establishing the diagnosis of NDI. Positive anti-SSA/SSB, objective sicca findings, and renal biopsy revealing active tubulointerstitial nephritis led to the diagnosis of pSS with dual tubular manifestations. Treatment with prednisone, mycophenolate mofetil, hydrochlorothiazide, and high-dose potassium supplements led to significant clinical and biochemical improvement. Conclusions This case illustrates a systematic approach to polyuria and hypokalemia in an adolescent, unraveling a rare dual tubulopathy as the initial manifestation of pSS. We propose that NDI in this setting may result from a “two-hit” mechanism: direct autoimmune injury to the collecting duct and profound hypokalemia-induced downregulation of aquaporin-2. Recognizing this association and initiating early immunosuppression can partially reverse tubular dysfunction and improve long-term outcomes.
Glomerular microangiopathy (GMA) is a group of diseases characterized pathologically by thrombotic microangiopathy (TMA) changes in the glomerulus without detectable microthrombi or intima edema of small arteries on renal biopsy. Few studies have focused on the clinicopathological characteristics and prognosis of patients with GMA. In this single-center retrospective cohort study, we summarized the clinical and pathological data of patients diagnosed with GMA between January 2005 and July 2023 at the Peking Union Medical College Hospital. Treatment and prognosis were also analyzed. A total of 32 patients diagnosed with GMA were included in this study. They were 62.5% male, with a mean age of 46 ± 17 years. The most common primary diseases were idiopathic multicenter Castleman disease (iMCD) (31.2%), POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome (21.9%), and exposure to anti-vascular endothelial growth factor (VEGF) agents (21.9%). All the patients exhibited various degrees of proteinuria (2.22 g/d (IQR 0.94, 3.50)). Acute kidney injury (AKI) complicated by significantly elevated inflammatory markers was observed in 13 patients, mostly iMCD patients (n = 8). In renal pathology, patients with iMCD showed marked endothelial cell proliferation, whereas patients with POEMS syndrome displayed prominent mesangial cell proliferation. Treatments targeting primary diseases could improve proteinuria and renal function. Patients with GMA primarily presented with proteinuria and AKI. The common primary diseases included iMCD, POEMS syndrome, and exposure to anti-VEGF agents. GMA usually responds well to treatment of primary diseases. Renal pathology is essential for diagnosis. It is important to distinguish GMA from TMA that may respond to anti-C5 therapy.
Objective To investigate the relationships of various types of renal vascular lesions with the clinical,pathological,and prognostic features of lupus nephritis (LN). Method A retrospective analysis was performed on the clinical and pathological data of LN patients treated in the Peking Union Medical College Hospital over the past 10 years to explore the relationships of different vascular lesions with clinical and pathological features and analyze the effects of renal vascular lesions on kidney prognosis from multiple perspectives. Results Among 644 enrolled LN patients,females accounted for 82.6%,and LN Ⅳ/Ⅳ+Ⅴ (60.4%) was the predominant pathological type.According to the types of renal vascular lesions,the patients were classified into four groups:atherosclerosis (AS)(n=171,26.6%),thrombotic microangiopathy (TMA)(n=78,12.1%),uncomplicated immune complex deposition (ICD)(n=29,4.5%),and lupus vasculopathy (LV)(n=19,3.0%). No renal vascular lesion (NRVL)(n=347,53.9%) was taken as the control group.The average age of patients in the AS group was higher than that in the other groups (all P<0.001).The TMA (P=0.009,P=0.025) and LV groups (P=0.007,P=0.005) had lower levels of complement C3,and higher disease activity index of systemic lupus erythematosus and incidence of acute kidney injury (both P<0.001) than the NRVL and AS groups.The TMA (P<0.001),LV (P<0.001),and AS (P=0.037) groups had lower estimated glomerular filtration rate (eGFR) than the NRVL group,and the eGFR of the TMA group was lower than that of the LV,AS,and ICD groups (all P<0.001).The renal pathological activity index (AI) of each renal vascular lesion group was higher than that of the NRVL group (all P<0.001),and the AI value of the TMA and LV groups was higher than that of the AS group (both P<0.001).There was no significant difference in AI value between TMA and LV groups (P=0.675),while the proportion of fibrinoid necrosis in the LV group was higher than that in the TMA group (P=0.012).The TMA group had the highest degree of chronicity and higher chronic index than the AS (P=0.021),LV (P<0.001),ICD (P<0.001),and NRVL (P<0.001) groups.The AS group had the secondary highest degree of chronicity and higher median chronic index than the NRVL group (P=0.004).The TMA group had the lowest 1-year clinical remission rate and the highest proportion of annual eGFR decline >5 mL/(min·1.73 m2).Both TMA and AS groups showed lower long-term renal survival rates than the NRVL group (P<0.001,P=0.023) respectively. Conclusions Intrarenal vascular lesions are strongly associated with clinical severity and prognosis in LN.LV correlates with acute kidney injury and severe active renal injury,while TMA is the most severe subtype and characterized by high pathological activity,chronicity,poor treatment response,and adverse outcomes.AS,the most common type,does not affect treatment response but predicts unfavorable long-term renal survival.
BACKGROUND Acute kidney injury (AKI) is a common and serious complication of major abdominal surgery. However, predictive models specific to pancreatic surgery remain scarce. AIM To develop and validate an interpretable machine learning model for early prediction of postoperative AKI following pancreatic surgery. METHODS Adults undergoing pancreaticoduodenectomy or distal or total pancreatectomy from 2014 to 2024 were retrospectively analyzed. AKI was defined by the kidney disease Improving global outcomes creatinine-based criteria. After matching, data from 2014-2021 trained seven models using Boruta/least absolute shrinkage and selection operator selection and five-fold cross-validation. Data from 2022-2024 were utilized for validation. Model performance was evaluated by the area under the receiver operating characteristic curve (AUROC); Shapley Additive Explanations were used for interpretation, and an online calculator was developed. RESULTS Among the 4216 eligible patients, 230 (5.5%) developed postoperative AKI. The categorical boosting model showed the best performance in the training cohort (AUROC = 0.803) and maintained a robust prediction in the validation cohort (AUROC = 0.751). Shapley Additive Explanations analysis highlighted operative time, postoperative serum creatinine level, intensive care unit admission after surgery, postoperative white blood cell count, and intraoperative red blood cell transfusion as key features for predicting postoperative AKI. CONCLUSION The developed models showed satisfactory performance for predicting postoperative AKI in patients undergoing major pancreatic surgery. They may facilitate early high-risk identification and inform perioperative management strategies.
BackgroundDiabetic kidney disease (DKD) and diabetic retinopathy (DR) represent two major microvascular complications of diabetes mellitus (DM). Previous studies have suggested that renin-angiotensin system inhibitors (RASi) exert protective effects on both DKD and DR. However, their specific impact on retinal microvascular parameters (RMPs), as well as the association between changes in fundus microvasculature and alterations in renal clinical parameters, remains unclear. This pilot study aimed to quantitatively assess the short-term effects of RASi on retinal microvasculature in patients with DKD using an artificial intelligence (AI)-based analysis of ultra-wide-field (UWF) fundus images.MethodsIn this prospective cohort study, 27 patients with DKD were enrolled between July 2023 and September 2024. UWF fundus images were acquired at baseline and 12 weeks after initiation of RASi therapy. A validated deep learning AI model was employed to segment retinal vessels and quantify RMPs, including fractal dimension (Df) and tortuosity (TORT), in both the central and peripheral retinal regions. Statistical analyses for pre- and post-treatment comparisons were performed using a linear mixed-effects model with patient ID as a random intercept. or a Wilcoxon signed-rank test, as appropriate. Changes in these parameters post-treatment were analyzed and correlated with alterations in clinical renal indicators.ResultsAmong the enrolled patients, 21 patients (77.8%) were male, with a mean age of 55.7 ± 14.2 years, a mean diabetes duration of 9.9 ± 6.6 years, a baseline estimated glomerular filtration rate (eGFR) of 73.7 ± 18.5 mL/min/1.73 m², and a median proteinuria of 0.57 (0.25, 0.97) g/24h. Fifteen patients (55.6%) had diabetic retinopathy (DR). After 12 weeks of RASi treatment, significant decreases were observed within the UWF images in venous Df (adjusted p = 0.0389) for the overall cohort, and venous TORT (adjusted p = 0.0496) for No-DR and NPDR groups. These significant changes were not observed in parameters derived from the central retinal region.ConclusionRASi therapy might be associated with retinal peripheral venous alterations, providing clues to a vascular- and topography-specific therapeutic response and shedding light on a potential imaging biomarker for diabetes management.
Peritoneal dialysis-associated peritonitis is the primary cause of technical failure and infection-related mortality in PD patients. Culture-negative peritonitis poses unique management challenges. The reported incidence of culture-negative peritonitis varies substantially across regions and centers, likely reflecting differences in patient characteristics, microbiological practices, and treatment protocols. In this study, we aimed to investigate the risk factors, treatment strategies, and clinical outcomes of culture-negative peritonitis in a large peritoneal dialysis center in northern China. In this single-center retrospective cohort study, we included consecutive peritonitis episodes from January 2013 to December 2023. We assessed patient demographics, medical history, peritonitis presentation, antibiotic use, and treatment pathways. The primary outcomes were culture-negativity and outcomes of medical cure. Logistic regression was used to identify independent risk factors for culture negativity and failure to achieve a medical cure. The overall incidence of peritonitis during the study period was 0.15 episodes per patient-year and the culture-negative peritonitis rate was 25.5
Background:Long-term management strategies for IgA Nephropathy (IgAN) remain insufficiently studied. This study aims to address this gap by comparing patients with persistent proteinuria who did not receive additional treatment with those whose persistent proteinuria led to on-demand immunosuppressive treatment. Methods:This retrospective cohort study included 264 propensity score-matched patients with IgAN treated between January 2003 and December 2016, with a follow-up through August 2024. Patients with proteinuria ≥1g/day without a 40% decline in eGFR over the previous 3 months, despite adequate supportive therapy, were considered eligible for on-demand immunosuppressive therapy during follow-up. Effective regimens-including glucocorticoids, cyclophosphamide, or mycophenolate mofetil, alone or in combination with other immunosuppressive drugs-were considered distinct treatment courses only if separated by at least six months. Survival was analyzed using the Kaplan-Meier estimator, and the primary composite endpoint was analyzed using the Cox proportional hazards model. Results:Over a median follow-up of 9.9 years (interquartile range, 7.3-12.8 years), 65 of 264 (25%) experienced the primary composite endpoint. Risk was lower among patients who consistently received on-demand immunosuppressive therapy compared with those who did not (10/132 [8%] vs. 55/132 [42%]; hazard ratio, 0.11; 95% CI, 0.05-0.21; p < 0.001). The incidence of the primary endpoint increased progressively with the number of missed on-demand immunosuppressive therapy: 27/34 (79%), 17/34 (50%), and 11/64 (17%) among patients who missed 3 or more, 2, or 1 treatment(s) respectively. Of the 281 missed on-demand treatment opportunities, 107 (38%) were attributable to patient-related factors and 174 (62%) to physician-related factors. Conclusions:On-demand immunosuppressive therapy is associated with improved long-term outcomes in patients with IgAN.
Objective Renal Fanconi syndrome(FS)is a rare renal manifestation of primary Sjögren's syndrome(pSS).This study aims to analyze the clinical and prognostic characteristics of patients with pSS-associ-ated renal FS(pSS-FS)and provide insights for clinical management.Methods Patients diagnosed with pSS-FS via renal biopsy at Peking Union Medical College Hospital from 1993 to 2024 were enrolled.Data collected in-cluded age,sex,clinical symptoms(xerostomia,xerophthalmia,skin purpura,arthralgia,polyuria,and systemic symptoms),laboratory findings[serum immunoglobulin G(IgG)and IgM,complement(C3,C4),antinuclear antibody,anti-Sjögren's syndrome-associated antigen A antibody(SSA),anti-SSB antibody,24-hour urinary pro-tein quantification,tubular proteinuria,serum creatinine,serum electrolytes],treatment,and follow-up informa-tion.Systematic assessments included the EULAR Sjögren's Syndrome Disease Activity Index(ESSDAI)score,pulmonary involvement(including non-infectious interstitial pneumonia,pulmonary fibrosis,pulmonary hyperten-sion,etc.),hematological involvement(anemia,leukopenia,thrombocytopenia),etc.Efficacy evaluations en-compassed improvements in immunological parameters,renal function,and tubular function.Group comparisons were performed using chi-square/Fisher's exact tests,t-tests,or non-parametric tests.Changes over time were an-alyzed using paired t-tests,and repeated measures during follow-up were assessed using mixed linear models.Results A total of 38 patients with pSS-FS were included,with 37(97.4%)being female.The median age at pSS diagnosis was 43(37,57)years.Xerostomia(76.3%)and xerophthalmia(71.1%)were the predominant clinical symptoms.The most common renal tubular dysfunctions were generalized aminoaciduria(96.9%),tubular proteinuria(96.0%),and hypokalemia(94.7%).The median eGFR was 52.57(32.04,76.10)mL/(min·1.73 m2),with60.5%(23/38)of patients having an eGFR below60 mL/(min·1.73 m2).After six months of immunosuppressive therapy,including moderate-to-high-dose glucocorticoids,significant improvements were observed in immunological parameters(improvement rate:69.2%),renal tubular function(89.5%),and renal function(44.4%).Following immunosuppressive treatment,the median eGFR increased from 54.95(33.06,76.10)mL/(min·1.73 m2)to 65.56(56.24,83.58)mL/(min·1.73 m2).Compared to patients with normal or mildly impaired baseline eGFR[≥60 mL/(min·1.73 m2)],those with significantly de-creased baseline eGFR[<60 mL/(min·1.73 m2)]were older(46 years vs.37 years),had higher ESSDAI scores(16 vs.13),higher 24-hour urinary protein levels(1.16 g/d vs.0.48 g/d),but lower positivity rates of serum anti-SSA antibody(36.4%vs.86.7%)and anti-SSB antibody(22.7%vs.73.3%).Moreover,this group showed more pronounced renal function improvement after 6 months(58.8%vs.20.0%)and 12 months(66.7%vs.25.0%)of immunosuppressive therapy.Conclusions This study reports the clinical characteristics of the lar-gest single-center cohort of pSS-FS patients internationally,characterized by varying degrees of proximal renal tu-bular dysfunction and renal impairment.Timely initiation of immunosuppressive therapy,including glucocorticoids,is crucial,particularly for patients with significantly reduced eGFR,who may experience more substantial renal function improvement.
The use of single-cell combinatorial indexing sequencing via droplet microfluidics presents an attractive approach for balancing cost, scalability, robustness and accessibility. However, existing methods often require tailored protocols for individual modalities, limiting their automation potential and clinical applicability. To address this, we introduce UDA-seq, a universal workflow that integrates a post-indexing step to enhance throughput and systematically adapt existing droplet-based single-cell multimodal methods. UDA-seq was benchmarked across various tissue and cell types, enabling several common multimodal analyses, including single-cell co-assay of RNA and VDJ, RNA and chromatin, and RNA and CRISPR perturbation. Notably, UDA-seq facilitated the efficient generation of over 100,000 high-quality single-cell datasets from three dozen frozen clinical biopsy specimens within a single-channel droplet microfluidics experiment. Downstream analysis demonstrated the robustness of this approach in identifying rare cell subpopulations associated with clinical phenotypes and exploring the vulnerability of cancer cells. UDA-seq incorporates a post-indexing step to enhance the throughput of droplet-based single-cell multimodal sequencing, enabling efficient large-scale single-cell analysis.
ABSTRACT Objective To examine the relationship between serum carotenoid levels and cardiovascular‐kidney‐metabolic (CKM) syndrome in a representative sample of US adults. Methods Data from the fasting subsample of the NHANES 2017–2018 were analyzed using a survey‐weighted approach to ensure the findings are representative of the broader US adult population. Serum levels of α‐carotene, β‐carotene, β‐cryptoxanthin, lutein/zeaxanthin, and lycopene were measured using high‐performance liquid chromatography. CKM syndrome stages were defined according to the 2023 American Heart Association guidelines, with advanced CKM syndrome categorized as stages 3 or 4. Associations between serum carotenoids and advanced CKM syndrome were assessed using logistic regression and weighted quantile sum (WQS) regression. Results The study included 1671 adults aged 20 years and older, with a mean age of 48.7 years and a gender distribution of 50.9% female and 49.1% male. Higher serum levels of α‐carotene, β‐carotene, α‐cryptoxanthin, lutein/zeaxanthin, and lycopene were inversely associated with advanced CKM syndrome. Specifically, compared to the lowest quartile, the highest quartile of α‐carotene had an odds ratio (OR) of 0.29 (95% CI: 0.16–0.55), β‐carotene 0.35 (95% CI: 0.16–0.78), α‐cryptoxanthin 0.23 (95% CI: 0.11–0.49), lutein/zeaxanthin 0.26 (95% CI: 0.14–0.48), and lycopene 0.58 (95% CI: 0.35–0.98). However, β‐cryptoxanthin did not show a significant association. Moreover, the combined effect of all carotenoids was significantly negatively correlated with advanced CKM syndrome (OR = 0.67, 95% CI: 0.53–0.86), with lutein/zeaxanthin contributing the most (44.56%). Conclusions Elevated serum carotenoid levels are inversely associated with the prevalence of advanced CKM syndrome in a dose‐dependent manner, with this association remaining consistent across diverse demographic and health subgroups.
Light and heavy chain deposition disease (LHCDD) is a clonal plasma cell or monoclonal B-cell dyscrasia characterized by deposition of monoclonal immunoglobulin light and heavy chains. LHCDD mainly belongs to monoclonal gammopathy of renal significance (MGRS), including a spectrum of kidney disorders caused by a monoclonal protein (M-protein) secreted by a small plasma cell clone or other B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma or other B-cell malignancies. It may also occur as a renal complication of overt multiple myeloma. We report a 27-year-old man who presented clinically with chronic nephritic syndrome and was diagnosed with LHCDD confirmed by renal biopsy, accompanied by hypocomplementemia and bronchiolitis obliterans (BO). Notably, he initially developed acquired cutis laxa (CL) four years before renal dysfunction. Progressive dermatologic manifestations prompted repeat skin biopsies, revealing deposition of γ1 heavy chains, restrictive lambda light chains and complement components (C3, C4 and C1q) along dermal elastic fibers, establishing monoclonal gammopathy of dermatologic significance (MGODS) before systemic involvement. This case illustrates a rare constellation of MGRS, MGODS, and BO in a young adult and provides unique histologic and serologic evidence of classical complement pathway activation. Our findings support a potential immune-mediated mechanism underlying tissue injury in both renal and extrarenal manifestations of monoclonal gammopathy, highlighting the diagnostic value of early tissue biopsy and the importance of complement assessment in such cases.
A 20-year-old male patient presented to the Department of Dermatology of Peking Union Med-ical College Hospital with complaints of an 8-year history of facial scarring,swelling of the lower limbs,and a 4-year history of scalp thickening.Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears,ciliary body hirsutism,blepharoptosis,and cutis verticis gyrate.Both lower limbs were swollen,especially the knees and ankles.The skin of the palms and soles of the feet was keratinized and thickened.Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands,distal ulna and radius,tibia and fibula,distal femurs,and metatar-sals.Genetic testing revealed two variants in SLCO2A1,c.1658T>A and c.96+4A>C.Through multidiscipli-nary consultation,we confirmed the diagnosis of pachydermoperiostosis.
Background:Gitelman syndrome (GS) is a rare inherited salt-losing tubulopathy caused by dysfunction of the thiazide-sensitive sodium-chloride cotransporter (NCC). Hyperuricemia is frequently observed in patients with GS, yet its risk factors and associations with GS remain unclear. This study aimed to investigate the percentage, clinical characteristics, and genetic mechanisms in GS patients with hyperuricemia. Methods:We reanalyzed the GS cohort at Peking Union Medical College Hospital, investigated the baseline clinical, laboratory, and genetic data of GS patients, and utilized the Illumina Human Asian Screening Array-24+v1.0 gene chip and IMPUTE2 for single nucleotide polymorphism (SNP) analysis. Results:The average serum uric acid of 132 GS patients was 352.3 ± 96.9 μmol/l (5.9 ± 1.6 mg/dl), with 21.2% hyperuricemia [mean 486.1 μmol/l (8.2 mg/dl)] and two cases of gout. The GS patients with hyperuricemia had higher body mass index (BMI) (24.3 ± 3.9 vs 21.7 ± 3.5 kg/m2, P = .001), lower fractional excretion of uric acid (FEUA) (median 4.22% vs 6.17%, P < .001), and urinary calcium/creatinine ratio (0.05 vs 0.12 mmol/mmol, P = .004). In GS patients with or without hyperuricemia, the impairment of NCC function indicated by the value of increase of chloride excretion fraction (△FECl) in the hydrochlorothiazide test was median 0.49% vs 0.82% (P = .10). We focused on the SLC12A3 genotype and 33 SNPs linked to uric acid levels and found no significant genetic differences between patients with or without hyperuricemia. Conclusions:Hyperuricemia is common in patients with GS in China, associated with elevated BMI and potentially more severe NCC dysfunction.
Severe gestational hypertriglyceridemia can lead to life-threatening pancreatitis with high maternal and fetal mortality, yet there are no established guidelines for managing these patients. We report two cases of severe gestational hypertriglyceridemia managed through a multidisciplinary approach. With the collaboration of obstetricians, endocrinologists, and nephrologists, we utilized a novel plasmapheresis circuit—combining centrifugation and filtration plasmapheresis (CFPP)—and performed regular sessions via peripheral venous access without the use of plasma or albumin. This treatment effectively lowered triglycerides in an attempt to prevent gestational pancreatitis, with no major maternal or fetal complications. We also conducted a systematic review of published reports on regular apheresis for severe gestational hypertriglyceridemia, identifying twenty-one additional cases. In these cases, treatment was generally administered twice weekly or every other week, with therapeutic plasmapheresis being the most frequently employed modality. The mean triglyceride reduction rate was 45.1
The immunofluorescence (IF) tests for renal tissues are crucial in glomerular disease diagnosis. We optimized and prospectively validated our multiple deep learning model-assisted diagnostic program for glomerular diseases previously developed. In 2022, we developed an artificial intelligent-assisted immunofluorescence image diagnosis system based on a convolutional neural network (CNN), consisting of glomeruli identification (YOLOv5 algorithm), IF intensity, deposition appearance, location classifier (Swin Transformer model), and decision adjustment trained with more than 5000 slides. With the continuous algorithm optimization, it could output IgA nephropathy (IgAN), idiopathic membranous nephropathy (IMN), anti-glomerular basement membrane antibody disease (anti-GBM), membranoproliferative glomerulonephritis (MPGN), post-streptococcal glomerulonephritis (PSGN), C3 nephritis, and manual review reminder (Fig. 1) by putting the positive IF images. In the prospective validation experiment, patients in Peking Union Medical College Hospital (PUMCH, n = 1286) included IgAN, IMN, lupus nephritis (LN), anti-GBM disease, and other glomerular diseases classified as unidentified diseases requiring further manual review, such as diabetic nephropathy, secondary MN. The accuracy of this program for diagnosing IgAN, IMN, LN, and anti-GBM was 93.2%, 94.6%, 93.9%, and 99.7%, with sensitivity of 88%, 89.6%, 72.8%, 85.7%, and specificity of 98.6%, 96.2%, 99.1%, 99.8%, respectively. The corresponding F1 scores were 0.99, 0.88, 0.95, and 0.75. For those unidentified diseases requiring manual review, the accuracy and specificity of this program were 85.1% and 87.2%, which is better than the Junior pathologists. The assisted diagnostic program based on multiple deep learning networks performs well in diagnosing glomerular diseases, shedding light on the future application perspective.