BMC Nephrology最新文献

Background: Patients with C3 glomerulopathy (C3G) often have a history of infection, which implies that infection may lead to abnormal activation of the complement alternative pathway (CAP) and induce the development of C3G. However, patients with postinfectious glomerulonephritis (PIGN) often have a low serum C3 concentration and positive glomerular C3 staining, consistent with the activation of the CAP. PIGN, especially if it involves simultaneous IgA deposition, is often difficult to differentiate from C3G.

Case presentation: In this study, we report the consequences of Mycoplasma pneumoniae (MP) infection in a 66-year-old male Chinese patient, who developed persistent hypocomplementemia, gross hematuria, and rapidly progressive glomerulonephritis. The findings of the histologic examination of an initial renal biopsy were consistent with a diagnosis of IgA-dominant postinfectious glomerulonephritis. The sample was negative for Gd-IgA1 staining. After treatment with antibiotics, glucocorticoids, and mycophenolate mofetil, the patient's serum creatinine decreased from a peak of 387 µmol/L to 195 µmol/L prior to discharge, and there was a partial response in his urinary protein concentration. After 2 months, his serum C3 concentration had returned to normal. However, owing to reinfection with MP the patient's serum creatinine rapidly increased again to 475.07 µmol/L, and this was accompanied by a decrease in serum C3 concentration (> 8 months) and positivity for C3 nephritis factor. Examination of both renal biopsies showed stronger immunostaining for C3 than for IgA in the glomeruli.

Conclusion: Thus, MP infection can cause sustained activation of the CAP, leading to C3G. For patients with MP infection, if there is an ongoing decrease in complement C3 levels and a progressive increase in serum creatinine, it is crucial to be vigilant for possible C3G and to consider the use of immunosuppressive therapy in conjunction with anti-infective treatment to prevent the ongoing activation of the CAP.

Background: Pregnancy-related Acute kidney injury (PR-AKI) accounts for approximately 15% of maternal mortality, with 10-30% progressing to end stage renal disease (ESRD). However, there are no comparative studies of obstetric and non-obstetric AKI. This study compares the outcomes of both groups with short-term follow-up to day 90.

Materials and methods: This prospective observational study was conducted over 1.5 years, enrolling 260 cases divided into non-obstetric and obstetric AKI groups.

Inclusion criteria: Non-obstetric group - patients > 18 years with AKI; Obstetric group - pregnant or up to 42 days postpartum with AKI, as per KDIGO criteria. Patients with known Chronic kidney disease (CKD) or transplant were excluded. Demographics, clinical profiles and relevant investigations (including renal biopsy) were analysed. Outcomes assessed at days 7, 30, and 90 for complete recovery, dialysis dependency, CKD progression, and mortality.

Results: Of 260 patients, 83.4% were in non-obstetric group while 16.6% were in the obstetric group. Sepsis was leading cause of AKI (51.5%), affecting 47.7% of non-obstetric and 74.4% of obstetric patients. Renal biopsies (12.3% of cases) predominantly showed acute tubular injury, lupus nephritis, Minimal change disease, Focal segmental glomerulosclerosis, ANCA-associated Glomerulonephritis (GN), IgA nephropathy, and Membranoproliferative GN. In Obstetric AKI, acute cortical necrosis and thrombotic microangiopathy (TMA) were common biopsy findings. At 3-months follow-up, complete recovery was higher in the non-obstetric group (40.5% vs. 33.3%), with the obstetric group having more progression to CKD and dialysis dependency. Mortality was higher in non-obstetric AKI (50.4% vs. 33.3%), likely due to underlying comorbidities.

Conclusion: Non-obstetric AKI showed higher early mortality but better long-term recovery, while obstetric AKI had poorer renal outcomes and a higher risk of progression to CKD. Early detection and intervention are critical for improving outcomes.

Objective: Blood pressure fluctuations during dialysis, including intradialytic hypotension (IDH) and intradialytic hypertension (IDHTN), are common complications among patients undergoing maintenance hemodialysis. Early prediction of IDH and IDHTN can help reduce the occurrence of these fluctuations. With the development of artificial intelligence, machine learning and deep learning models have become increasingly sophisticated in the field of hemodialysis. Utilizing machine learning to predict blood pressure fluctuations during dialysis has become a viable predictive method.

Methods: Our study included data from 67,524 hemodialysis sessions conducted at Ningbo No.2 Hospital and Xiangshan First People's Hospital from August 1, 2019, to September 30, 2023. 47,053 sessions were used for model training and testing, while 20,471 sessions were used for external validation. We collected 45 features, including general information, vital signs, blood routine, blood biochemistry, and other relevant data. Data not meeting the inclusion criteria were excluded, and feature engineering was performed. The definitions of IDH and IDHTN were clarified, and 10 machine learning algorithms were used to build the models. For model development, the dialysis data were randomly split into a training set (80%) and a testing set (20%). To evaluate model performance, six metrics were used: accuracy, precision, recall, F1 score, ROC-AUC, and PR-AUC. Shapley Additive Explanation (SHAP) method was employed to identify eight key features, which were used to develop a clinical application utilizing the Streamlit framework.

Results: Statistical analysis showed that IDH occurred in 56.63% of hemodialysis sessions, while the incidence of IDHTN was 23.53%. Multiple machine learning models (e.g., CatBoost, RF) were developed to predict IDH and IDHTN events. XGBoost performed the best, achieving ROC-AUC scores of 0.89 for both IDH and IDHTN in internal validation, with PR-AUC scores of 0.95 and 0.78, and high accuracy, precision, recall, and F1 scores. The SHAP method identified pre-dialysis systolic blood pressure, BMI, and pre-dialysis mean arterial pressure as the top three important features. It has been translated into a convenient application for use in clinical settings.

Conclusion: Using machine learning models to predict IDH and IDHTN during hemodialysis is feasible and provides clinically reliable predictive performance. This can help timely implement interventions during hemodialysis to prevent problems, reduce blood pressure fluctuations during dialysis, and improve patient outcomes.

Autosomal Dominant Polycystic Kidney Disease (ADPKD) affects up to 70 000 people in the UK and the most common inherited cause of end-stage kidney disease (ESKD). It is generally a late-onset multisystem disorder characterised by bilateral kidney cysts, liver cysts and an increased risk of intracranial aneurysms. Approximately 50% of people with ADPKD reach ESKD by age 60. Disease-associated pain, discomfort, fatigue, emotional distress and, impaired mobility can impact health-related quality of life. The approval of tolvaptan, a vasopressin V2 receptor antagonist, has greatly advanced the care for people with ADPKD, shifting the focus from general chronic kidney disease management to targeted therapeutic approaches. While guidance from NICE and SMC provides a foundational framework, this is not clear or comprehensive enough to offer practical guidance for healthcare professionals in real-world settings. This commentary expands on the previous United Kingdom Kidney Association (UKKA) commentary in 2016 with an updated evidence base, the incorporation of real-world data and expert opinion to provide practical guidance to healthcare professionals. Through co-development with people affected by ADPKD, it now incorporates valuable patient perspectives and offers practical recommendations for the UK kidney community seeking to harmonise the quality of care of all people with ADPKD.

Background: Syphilis, a sexually transmitted disease, presents with a wide range of clinical manifestations. As the global rate of syphilis infection continues to rise, so does the incidence of syphilis-associated nephritis. Characterized by diverse clinical and pathological features, the disease shows a good response to penicillin treatment. This article presents the case of a 46-year-old Chinese male patient exhibiting edema, hematuria, proteinuria, and a tendency towards rapidly progressive glomerulonephritis (RPGN). The patient was diagnosed with membranoproliferative glomerulonephritis (MPGN), with syphilis being a likely etiology and co-infection with Hepatitis B virus (HBV). He was treated with benzathine penicillin for three weeks, followed by low-dose glucocorticoids and mycophenolate mofetil (MMF) for subsequent treatment, leading to a significant improvement in his condition.

Conclusion: Highlighting the significance of syphilis as a cause of nephritis and emphasizing the importance of timely diagnosis and treatment can greatly alleviate the patient's condition. Additionally, the role of Hepatitis B virus as a contributing factor in the development of nephritis should not be overlooked.

Clinical trial number: Not applicable.

Background: Hyperphosphatemia is deemed to be an asymptomatic silent killer, its high prevalence in patients undergoing hemodialysis (HD) is correlated mainly with malnutrition and mortality. Dietitian's renal nutrition education has a major impact on chronic kidney disease patients' knowledge, and attitude towards nutrition guidelines. However, a large number of factors are acting as barriers to the appropriate practices of Lebanese dietitians in renal therapy. This study evaluated the knowledge, attitudes, and practices (KAP) of licensed dietitians (LDs) regarding hyperphosphatemia management in patients undergoing HD.

Methods: A total of 408 LDs from Mount-Lebanon Governorate in Lebanon participated in this study. A 52-item online questionnaire was used to assess nutritional phosphorus' KAP of all LDs, in compliance with dietetic practices with KDOQI guidelines updated version 2020 and identified the factors preventing dietitians from dealing with renal patients undergoing HD, e.g., nutrition care. The data was analyzed using SPSS version 25.

Results: There was a significant association between KAP levels, and almost all sociodemographic characteristics evaluated. Only 2% of dietitians applied all KDOQI guidelines, 64% attained poor and moderate knowledge, and 60% had a positive attitude towards renal care. Working in a clinical field was a common predictor of positive knowledge (adjusted OR = 2.453, 95% CI 1.244-4.836), positive attitude (adjusted OR = 1.900, 95% CI 1.300-2.541) and positive practice (adjusted OR = 0.192, 95% CI 0.184-0.491) while HD/hospital-based field increased the odds for positive knowledge (adjusted OR = 4.520, 95% CI 1.189-17.182). LDs, compared to registered dietitians, had lower odds of positive knowledge (adjusted OR = 0.390, 95% CI 0.231-0.658) and positive attitude (adjusted OR = 0.270, 95% CI 0.154-0.471). Lack of training was the main reason preventing the appropriate dietetic practices regarding hyperphosphatemia management in patients undergoing HD.

Conclusion: The Ministry of Public Health (MOPH) should be asked to endorse the integration of renal nutrition programs in the Lebanese curriculum, to aid in the empowerment of dietitians from different backgrounds towards renal therapy, in order to enhance the knowledge and attitude regarding nutritional guidelines of poorly supported Lebanese patients undergoing HDhemodialysis. Other stakeholders may include the Syndicate of Dietitians in Lebanon.

Background: Biobanks that hold blood, urine and kidney tissue are key for translational nephrology research but are few and have limited availability. We describe the organization, baseline characteristics, and generalizability of a low-cost national biobank.

Materials and methods: Eight Norwegian hospitals participated in this multi-center, prospective cohort study and biobank initiative. Patients referred for routine clinical native kidney biopsies were eligible for inclusion, starting September 2020. Extensive information on medical history and risk factors were collected into an encrypted on-line database by the treating nephrologist. A comprehensive standardized panel of blood and urine tests were analyzed in the clinical routine and registered along with the full histology report. Extra urine and blood samples were collected, aliquoted and prepared locally within two hours, frozen at -80 C, and later sent to a central government-funded biorepository together with remaining kidney biopsy material.

Results: By September 2023, a total of 633 patients were included out of 1050 eligible patients. Mean age was 52.6 years (SD 18.7), 384 (61%) were men, and participants displayed a wide spectrum of kidney disease with mean estimated glomerular filtration rate (eGFR) 53 mL/min/1.73m². The most frequent biopsy indications were progressive chronic kidney disease (CKD) of unknown cause, acute kidney disease, and isolated hematuria/proteinuria. The most frequent diagnoses were IgA nephropathy (21%), arterionephrosclerosis (13%), and diabetes nephropathy (9%). Biopsy indications and diagnoses were similar to the spectrum typically seen in Norway and other western countries, and similar population level kidney health measures were demonstrated for Norway, United Kingdom, and USA.

Discussion: We demonstrate the feasibility of establishing a large national kidney biopsy biobank across a variety of clinical and histopathologic diagnoses. Blood and urine were stored, accompanied by kidney tissue, at a moderate cost due to a combination of a dedicated nephrology workforce, routine clinical care, and established biobank facilities.

Background: Immunosuppressive agents, although indispensable in the treatment of chronic kidney diseases (CKD), could compromise the patient's immune function. The risk factor for in-hospital mortality in immunocompromised CKD patients with severe infections remain elusive.

Methods: We conducted a retrospective analysis of the clinical data of CKD patients who received immunosuppressive agents and presented severe infections. The cohort comprised 272 patients, among whom 73 experienced mortalities during their hospitalization. Logistic regression was employed on the training set to identify key feature variables and construct a predictive model for in-hospital mortality among immunocompromised CKD patients following severe infections. To facilitate clinical application, we constructed a nomogram to visually represent the predictive model.

Results: Our findings indicate that ventilator use, vasoactive drug administration, elevated lactate dehydrogenase (LDH), total bilirubin (TBIL) levels, and persistent lymphopenia(PL) are effective predictors of in-hospital mortality in immunocompromised patients with severe infections. These variables were subsequently incorporated to construct a robust prognostic model. Our model demonstrated excellent discriminative ability (AUC = 0.959, 95% CI, 0.924-0.994), significantly outperforming the Sequential Organ Failure Assessment (SOFA) score (AUC = 0.878, 95% CI, 0.825-0.930) and quick Pitt Bacteremia Score (qPBS) (AUC = 0.897, 95% CI, 0.846-0.947). Calibration curve analysis and the Hosmer-Lemeshow (HL) test corroborate the concordance of our model with empirical observations. Furthermore, decision curve analysis (DCA) underscores the superior clinical utility of our predictive model when compared to the SOFA score and qPBS score. Most importantly, our results showed that PL is the most important predictor of in-hospital mortality in immunocompromised patients following severe infection.

Conclusion: Our findings highlight PL as the most significant predictor of in-hospital mortality in immunocompromised CKD patients. A clinical prediction model incorporating PL as a key variable exhibited robust performance in terms of diagnostic accuracy and clinical utility.

Background: Electronic (e-)alerts for rising serum creatinine values are increasingly used as clinical indicators of acute kidney injury (AKI). The aim of this study was to investigate to what degree AKI episodes, as identified using e-alerts, correlated with coding for AKI in the hospital record for a national cohort of hospitalised children and examine whether coding corresponded with 30-day mortality after an AKI episode.

Methods: A cross-section of AKI episodes based on alerts issued for children under 18 years in England during 2017 were linked to hospital records. Multivariable logistic regression was used to examine patient and clinical factors associated with AKI coding. Agreement between coding and 30-day mortality was examined at hospital level.

Results: 6272 AKI episodes in 5582 hospitalised children were analysed. Overall, coding was poor (19.7%). Older age, living in the least deprived quintile (odds ratio (OR) 1.4, 95% Confidence Interval (CI) 1.1, 1.7) and higher peak AKI stage (stage 1 reference; stage 2 OR 2.0, 95% CI 1.7, 2.4; stage 3 OR 8.6, 95% CI 7.1, 10.6) were associated with higher likelihood of coding in the hospital record. AKI episodes during birth admissions were less likely to be coded (OR 0.4, 95% CI 0.3, 0.5). No correlation was seen between coding and 30-day mortality.

Conclusions: The proportion of AKI alert-identified episodes coded in the hospital record is low, suggesting under-recognition and underestimation of AKI incidence. Understanding the reasons for inequalities in coding, variation in coding between hospitals and how alerts can enhance clinical recognition is needed.

Anderson-Fabry disease (AFD) is a multisystem X-linked lysosomal storage disorder caused by a deficiency in the enzyme α-galactosidase A (α-Gal A). This deficiency results in the intracellular accumulation of glycosphingolipids, primarily uncleaved globotriaosylceramide (Gb3) and its deacylated form, lyso-globotriaosylceramide (Lyso-Gb3), leading to progressive organ damage and functional impairment. The diagnostic evaluation for AFD involves clinical assessment and family history, supported by biochemical testing (α-Gal A enzyme activity and Lyso-Gb3 levels) and genetic analysis of the GLA gene. In cases of unexplained renal impairment or when genetic analysis is inconclusive, kidney biopsy is often required to confirm the diagnosis and guide targeted treatments. However, histological findings in kidney biopsies may sometimes be nonspecific, complicating the diagnostic process. This article aims to provide an updated perspective on the role of kidney biopsy in AFD, illustrating two cases that exemplify its pivotal role in confirming or excluding the suspected disease, proving to be both decisive and confounding in this complex clinical setting.