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1.
Ren Fail ; 46(2): 2386146, 2024 Dec.
Article in English | MEDLINE | ID: mdl-39091091

ABSTRACT

The significance of glomerular IgM deposit intensity in IgA Nephropathy (IgAN) remained ambiguous and requires further research. Patients with biopsy-proven IgAN in our hospital from January 2018 to May 2023 were recruited into this retrospective single-center study. Patients who presented with positive IgM deposit were included in IgM + cohort while patients with negative IgM deposit were included in IgM- cohort. Of the IgM+, patients whose IF intensity of IgM deposits exceeded 1+ formed IgM-H cohort while patients whose IF intensity of IgM deposits was equal to 1+ consisted IgM-L cohort. Pairwise comparisons were performed among these cohorts to determine clinical disparities, following the propensity score matching process. Among 982 IgAN patients, 539 patients presented with positive IgM deposit. The Kaplan-Meier analysis showed that the IgM deposit did not contribute adversely to the outcomes (eGFR decreased from the baseline ≥ 50% continuously or reached end-stage renal disease). However, the Cox regression analysis showed that increased intensity of IgM deposit was an independent risk factor (p = 0.03) in IgM+. The IgM-H exhibited more pronounced segmental glomerulosclerosis (p = 0.02) than the IgM-L, which may also be associated more directly with higher urine protein levels (p = 0.02). Moreover, our generalized linear mixed model demonstrated a remarkably higher urine albumin/creatinine ratio (p < 0.01) and serum creatinine (p = 0.04) levels as well as lower serum albumin (p < 0.01) level in IgM-H persistently during the 5-year follow-up. This study concluded that increased intensity of glomerular IgM deposits may contribute adversely to clinicopathologic presentation and outcome in those IgM + patients.


Subject(s)
Glomerular Filtration Rate , Glomerulonephritis, IGA , Immunoglobulin M , Kidney Glomerulus , Humans , Immunoglobulin M/blood , Male , Glomerulonephritis, IGA/immunology , Female , Retrospective Studies , Adult , Follow-Up Studies , Kidney Glomerulus/pathology , Kidney Glomerulus/immunology , Middle Aged , Risk Factors , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/immunology , Kaplan-Meier Estimate , Disease Progression , Biopsy , Clinical Relevance
2.
Front Immunol ; 15: 1427943, 2024.
Article in English | MEDLINE | ID: mdl-39050843

ABSTRACT

Background: Pemphigoid diseases constitute a group of autoimmune blistering disorders characterized by subepithelial blistering. The association between pemphigoid diseases and both end-stage kidney disease (ESKD) and its treatment is notable. However, there is limited evidence about the management of pemphigoid diseases in patients with ESKD. This systematic review compiled case reports and relevant studies, summarized the underlying mechanisms of pemphigoid diseases in patients with ESKD, and summarized the efficacy of various therapies. Methods: A systematic search of PubMed and Embase was performed for articles published between 1982 to June 2, 2024. Results: Fifty-three case reports and eight relevant studies were included. Triggers for pemphigoids in patients with ESKD included materials used to treat ESKD, immune dysregulation of patients with ESKD, and rejection of renal allograft. Treatment for these patients included removing triggers, as well as administering of corticosteroids, mycophenolate mofetil (MMF), tetracyclines, rituximab, methotrexate, dapsone, azathioprine, cyclosporine, intravenous immunoglobin (IVIG), plasmapheresis, and Janus kinase inhibitors. Conclusion: Removing triggers is the most effective strategy. Despite their suboptimal efficacy, corticosteroids remain the most commonly used agents in this patient population. MMF, tetracyclines, and rituximab are less used but with benefits. There are significant adverse effects associated with methotrexate treatment. Other treatment may also be beneficial and require further investigation. These findings may enable clinicians to optimize the therapeutic approach for these patients.


Subject(s)
Kidney Failure, Chronic , Pemphigoid, Bullous , Humans , Pemphigoid, Bullous/therapy , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/immunology , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/complications , Immunosuppressive Agents/therapeutic use , Immunosuppressive Agents/adverse effects , Kidney Transplantation/adverse effects
3.
Adv Kidney Dis Health ; 31(4): 275-289, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39084753

ABSTRACT

Focal segmental glomerular sclerosis (FSGS) is a histological lesion characterized by sclerosis in sections (segmental) of some glomeruli (focal) in association with podocyte injury. Historically, FSGS has often been characterized as a disease, but it is a heterogeneous entity based on etiology, clinical course, and therapeutic approach. A unifying feature is podocyte injury and loss, which can be primary or the result of secondary maladaptive responses to glomerular stressors. FSGS has been demonstrated over time to carry a large health burden and remains a leading glomerular cause of ESRD globally. Recent clinical practice guidelines highlight the unmet scientific need for better understanding of disease pathogenesis, particularly for immunologic etiologies, as well as more targeted therapeutic drug development. In this review, we will discuss the current FSGS classification scheme, pathophysiologic mechanisms of injury, and treatment guidelines, along with emerging and investigational therapeutics.


Subject(s)
Glomerulosclerosis, Focal Segmental , Podocytes , Glomerulosclerosis, Focal Segmental/pathology , Glomerulosclerosis, Focal Segmental/therapy , Humans , Podocytes/pathology , Kidney Failure, Chronic/pathology , Kidney Failure, Chronic/etiology , Kidney Glomerulus/pathology , Kidney Glomerulus/physiopathology
4.
Transplantation ; 108(8): 1782-1792, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-39042770

ABSTRACT

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage kidney disease and frequently recurs after kidney transplantation. Recurrent FSGS (rFSGS) is associated with poor allograft and patient outcomes. Bleselumab, a fully human immunoglobulin G4 anti-CD40 antagonistic monoclonal antibody, disrupts CD40-related processes in FSGS, potentially preventing rFSGS. METHODS: A phase 2a, randomized, multicenter, open-label study of adult recipients (aged ≥18 y) of a living or deceased donor kidney transplant with a history of biopsy-proven primary FSGS. The study assessed the efficacy of bleselumab combined with tacrolimus and corticosteroids as maintenance immunosuppression in the prevention of rFSGS >12 mo posttransplantation, versus standard of care (SOC) comprising tacrolimus, mycophenolate mofetil, and corticosteroids. All patients received basiliximab induction. The primary endpoint was rFSGS, defined as proteinuria (protein-creatinine ratio ≥3.0 g/g) with death, graft loss, or loss to follow-up imputed as rFSGS, through 3 mo posttransplant. RESULTS: Sixty-three patients were followed for 12 mo posttransplantation. Relative decrease in rFSGS occurrence through 3 mo with bleselumab versus SOC was 40.7% (95% confidence interval, -89.8 to 26.8; P = 0.37; absolute decrease 12.7% [95% confidence interval, -34.5 to 9.0]). Central-blinded biopsy review found relative (absolute) decreases in rFSGS of 10.9% (3.9%), 17.0% (6.2%), and 20.5% (7.5%) at 3, 6, and 12 mo posttransplant, respectively; these differences were not statistically significant. Adverse events were similar for both treatments. No deaths occurred during the study. CONCLUSIONS: In at-risk kidney transplant recipients, bleselumab numerically reduced proteinuria occurrence versus SOC, but no notable difference in occurrence of biopsy-proven rFSGS was observed.


Subject(s)
Antibodies, Monoclonal, Humanized , Glomerulosclerosis, Focal Segmental , Immunosuppressive Agents , Kidney Transplantation , Recurrence , Humans , Kidney Transplantation/adverse effects , Glomerulosclerosis, Focal Segmental/drug therapy , Glomerulosclerosis, Focal Segmental/immunology , Male , Female , Middle Aged , Adult , Immunosuppressive Agents/therapeutic use , Immunosuppressive Agents/adverse effects , Antibodies, Monoclonal, Humanized/therapeutic use , Antibodies, Monoclonal, Humanized/adverse effects , Treatment Outcome , Tacrolimus/therapeutic use , Tacrolimus/adverse effects , Graft Survival/drug effects , Drug Therapy, Combination , Adrenal Cortex Hormones/therapeutic use , Secondary Prevention/methods , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/prevention & control , Kidney Failure, Chronic/etiology
5.
Exp Clin Transplant ; 22(6): 459-464, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39072518

ABSTRACT

Here, we describe an interesting case of a patient with the duplication of inferior vena cava, high-positioned bifurcation of the abdominal aorta with transposition of iliac arteries, and right renal aplasia associated with end-stage renal disease who underwent kidney transplant. In this case, the patient with anorectal malformations with a vaginal fistula was prepared and underwent a kidney transplant. During the surgery, we discovered duplicated inferior vena cava and transposed iliac arteries. After the surgery, computed tomography angiography revealed the inferior vena cava duplication with the 2 connections between the right and left inferior vena cava with the formation of an anomalous circle, high-positioned bifurcation of the abdominal aorta at the level of the L2 vertebral body, and transposition of right and left iliac arteries. Also, we observed the right kidney aplasia and absence of blood circulation in the left native kidney. In our case, a delayed diagnosis of pyelonephritis resulted in the progression to end-stage renal disease that necessitated a kidney transplant, during which we found these anomalies. We confirmed the asymptomatic course of these anomalies, diagnosed only during radiological imaging or surgical intervention. Patients with congenital anomalies of the kidney and urinary tract should undergo complete investigations before surgical decisions. Diagnosis of this pathology in the preoperative period, especially in transplant patients, will alert the surgery team in advance of the operation and allow preparation for the intraoperative difficulties that are typically associated with anomalies such as inferior vena cava transposition or aplasia.


Subject(s)
Aorta, Abdominal , Kidney Failure, Chronic , Kidney Transplantation , Vascular Malformations , Vena Cava, Inferior , Humans , Vena Cava, Inferior/abnormalities , Vena Cava, Inferior/diagnostic imaging , Vena Cava, Inferior/surgery , Female , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/diagnosis , Treatment Outcome , Aorta, Abdominal/abnormalities , Aorta, Abdominal/surgery , Aorta, Abdominal/diagnostic imaging , Vascular Malformations/surgery , Vascular Malformations/complications , Vascular Malformations/diagnostic imaging , Aortography , Computed Tomography Angiography , Abnormalities, Multiple/surgery , Phlebography/methods , Incidental Findings , Iliac Artery/surgery , Iliac Artery/abnormalities , Iliac Artery/diagnostic imaging , Adult , Pyelonephritis/surgery , Pyelonephritis/etiology , Pyelonephritis/diagnosis , Pyelonephritis/diagnostic imaging , Predictive Value of Tests
6.
Int J Mol Sci ; 25(10)2024 May 07.
Article in English | MEDLINE | ID: mdl-38791134

ABSTRACT

We report the histological changes over time for a patient with infection-related glomerulonephritis (IRGN) that developed in a transplanted kidney. A 47-year-old man had undergone renal transplantation 3 years ago for end-stage kidney disease (ESKD). After several episodes of acute rejection, the patient was in a stable CKD condition. The abrupt development of severe microscopic hematuria and renal dysfunction was observed approximately 2 weeks after the onset of a phlegmon in his right leg. An allograft biopsy showed prominent glomerular endocapillary proliferation on light microscopy, granular C3 deposition on immunofluorescent microscopy, and subepithelial electron-dense deposits on electron microscopy, suggesting IRGN accompanied by moderate interstitial fibrosis and tubular atrophy (IFTA). Positive glomerular staining for nephritis-associated plasmin receptor (NAPlr) and plasmin activity, which are biomarkers of bacterial IRGN, supported the diagnosis. Although the infection was completely cured with antibiotic therapy, renal dysfunction persisted. A re-biopsy of the allograft 2 months later revealed resolution of the glomerular endocapillary proliferation and negative staining for NAPlr/plasmin activity, with worsening IFTA. We showed, for the first time, the chronological changes in infiltrating cells and histological markers of IRGN in transplanted kidneys. Glomerular changes, including NAPlr/plasmin activity staining, almost disappeared after the cessation of infection, while interstitial changes continuously progressed, contributing to ESKD progression.


Subject(s)
Allografts , Glomerulonephritis , Kidney Transplantation , Humans , Male , Kidney Transplantation/adverse effects , Middle Aged , Glomerulonephritis/pathology , Glomerulonephritis/etiology , Kidney Failure, Chronic/pathology , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/surgery , Kidney Glomerulus/pathology , Kidney Glomerulus/metabolism , Biopsy , Kidney/pathology
7.
BMJ Case Rep ; 17(5)2024 May 13.
Article in English | MEDLINE | ID: mdl-38740443

ABSTRACT

Alport syndrome and autosomal dominant polycystic kidney disease are monogenic causes of chronic kidney disease and end-stage kidney failure. We present a case of a man in his 60s with progressive chronic kidney disease, bilateral sensorineural hearing loss and multiple renal cysts. Genetic analysis revealed a heterozygous variant in COL4A3 (linked to Alport syndrome) and in the GANAB gene (associated with a milder form of autosomal dominant polycystic kidney disease). Although each variant confers a mild risk of developing end-stage kidney disease, the patient presented a pronounced and accelerated progression of chronic kidney disease, which goes beyond what would be predicted by adding up their individual effects. This suggests a potential synergic effect of both variants, which warrants further investigation.


Subject(s)
Collagen Type IV , Nephritis, Hereditary , Polycystic Kidney, Autosomal Dominant , Humans , Nephritis, Hereditary/genetics , Nephritis, Hereditary/complications , Nephritis, Hereditary/diagnosis , Male , Polycystic Kidney, Autosomal Dominant/genetics , Polycystic Kidney, Autosomal Dominant/complications , Collagen Type IV/genetics , Middle Aged , Autoantigens/genetics , Disease Progression , Kidney Failure, Chronic/genetics , Kidney Failure, Chronic/etiology , Hearing Loss, Sensorineural/genetics , Hearing Loss, Sensorineural/diagnosis
8.
Pediatr Nephrol ; 39(9): 2641-2643, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38597973

ABSTRACT

Acquired cystic kidney disease (ACKD) can occur in patients with chronic kidney disease and kidney failure, and its incidence increases with the duration of dialysis. In adults, ACKD is less common in the pre-dialysis group (~ 7%), but its incidence can be as high as 80% for those who are on dialysis for more than ten years. There is, however, very little information about the prevalence of ACKD in children. We report a case of malignant transformation of ACKD following a kidney transplant, highlighting the importance of surveillance of the native kidneys in paediatric patients who have been in long-term kidney replacement therapy.


Subject(s)
Carcinoma, Renal Cell , Incidental Findings , Kidney Diseases, Cystic , Kidney Neoplasms , Kidney Transplantation , Humans , Kidney Diseases, Cystic/diagnosis , Kidney Diseases, Cystic/complications , Kidney Diseases, Cystic/etiology , Kidney Neoplasms/complications , Kidney Neoplasms/diagnosis , Kidney Neoplasms/etiology , Kidney Transplantation/adverse effects , Adolescent , Carcinoma, Renal Cell/complications , Carcinoma, Renal Cell/diagnosis , Carcinoma, Renal Cell/surgery , Male , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/etiology , Female
9.
Sci Rep ; 14(1): 8278, 2024 04 09.
Article in English | MEDLINE | ID: mdl-38594302

ABSTRACT

Focal segmental glomerulosclerosis (FSGS) is a common pathological form of nephrotic syndrome. This study analyzed the value of pathological lesions and clinical prognosis of different segmental glomerulosclerosis ratios in FSGS. Two hundred and six FSGS patients were collected from Dec 2013 to Apr 2016. The patients were divided into two groups according to the proportion of glomerular segmental sclerosis: F1 (SSR ≤ 15%, n = 133) and F2 (SSR > 15%, n = 73). The clinical and pathological data were recorded and analyzed, and statistical differences were observed between the serum uric acid level and the percentage of chronic renal failure. The pathological results showed significant differences in interstitial fibrosis and tubular atrophy (IFTA), degree of mesangial hyperplasia, vascular lesions, synaptopodin intensity, and foot process effacement between the two groups. Multivariate logistic regression analysis showed significant differences in creatinine (OR: 1.008) and F2 group (OR: 1.19). In all patients, the prognoses of urine protein and serum creatinine levels were statistically different. Multivariate Cox regression analysis revealed that F2 (hazard ratio: 2.306, 95% CI 1.022-5.207) was associated with a risk of ESRD (end stage renal disease). The proportion of segmental glomerulosclerosis provides a guiding value in the pathological diagnosis and clinical prognosis of FSGS.


Subject(s)
Glomerulosclerosis, Focal Segmental , Kidney Failure, Chronic , Renal Insufficiency, Chronic , Humans , Glomerulosclerosis, Focal Segmental/pathology , Uric Acid , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/diagnosis
10.
Clin Exp Med ; 24(1): 70, 2024 Apr 05.
Article in English | MEDLINE | ID: mdl-38578316

ABSTRACT

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is an autoimmune disease that involves inflammation of blood vessels. There is increasing evidence that platelets play a crucial role not only in hemostasis but also in inflammation and innate immunity. In this study, we explored the relationship between platelet count, clinical characteristics, and the prognosis of patients with AAV. We divided 187 patients into two groups based on their platelet count. Clinicopathological data and prognostic information were retrospectively gathered from medical records. Univariate and multivariate regression analyses were used to identify risk factors for prognosis, including end-stage renal disease (ESRD) and mortality. The cutoff point for platelet count was set at 264.5 × 109/L, as determined by the receiver operating characteristic (ROC) curve for predicting progression to ESRD in patients with AAV. We observed patients with low platelet count (platelets < 264.5 × 109/L) had lower leukocytes, hemoglobin, complement, acute reactants, and worse renal function (P for eGFR < 0.001). They were also more likely to progress to ESRD or death compared to the high platelet count group (platelets > 264.5 × 109/L) (P < 0.0001, P = 0.0338, respectively). Low platelet count was potentially an independent predictor of poor renal prognosis in the multivariate regression analysis [HR 1.670 (95% CI 1.019-2.515), P = 0.014]. Lower platelet count at diagnosis is associated with more severe clinical characteristics and impaired renal function. Therefore, platelet count may be an accessible prognostic indicator for renal outcomes in patients with AAV.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Kidney Failure, Chronic , Humans , Retrospective Studies , Platelet Count , Prognosis , Kidney/pathology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/etiology , Inflammation/complications , Severity of Illness Index
11.
Eur Rev Med Pharmacol Sci ; 28(7): 2805-2816, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38639520

ABSTRACT

OBJECTIVE: Identifying reliable predictors of mortality in end-stage renal disease patients is crucial for patient outcomes. Aortic knob width is a radiographic parameter used to assess cardiovascular diseases and atherosclerosis. This study investigated the association between aortic knob width and mortality in hemodialysis patients. PATIENTS AND METHODS: The study included data collected between 2007 and 2022 from 103 patients aged between 18 and 85 who had been undergoing hemodialysis treatment for at least one year. Patients were divided into two groups: survivors and deceased. The aortic knob width was measured using a posterior-anterior chest radiograph after midweek hemodialysis. The relationship between aortic knob width and mortality was investigated. RESULTS: Deceased patients had significantly larger aortic knob widths compared with survivors. The deceased group's hemodialysis (HD) duration was shorter, median age was older, Kt/V, hemoglobin, and albumin levels were lower, and the frequency of patients with hypertension, diabetes, and aortic wall calcification was higher. Aortic knob width greater than 37.98 mm was identified as a predictor of mortality in hemodialysis patients. Survival rates for aortic knob width <37.98 mm are 98.1% for 1 year and 64.9% for 15 years. For aortic knob width larger than 37.98 mm, survival rates are 88% for three years, 68% for five years, 45.2% for ten years, and 25% for fifteen years. The most important risk factors for increased aortic knob width were age, male sex, aortic calcification, and hypertension. CONCLUSIONS: Age, male gender, aortic calcification, and hypertension are the primary risk factors for increased aortic knob width in hemodialysis patients. Aortic knob width greater than 37.98 mm, which can be measured simply and rapidly using posterior-anterior chest radiography, may be a predictor of mortality. Graphical Abstract: https://www.europeanreview.org/wp/wp-content/uploads/Graphical-Abstract-10.jpg.


Subject(s)
Atherosclerosis , Cardiovascular Diseases , Hypertension , Kidney Failure, Chronic , Humans , Male , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over , Aorta/diagnostic imaging , Atherosclerosis/etiology , Cardiovascular Diseases/etiology , Renal Dialysis , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/etiology , Risk Factors
12.
J Nephrol ; 37(4): 1051-1061, 2024 May.
Article in English | MEDLINE | ID: mdl-38512370

ABSTRACT

BACKGROUND: Phospholipase A2 receptor (PLA2R)-associated membranous nephropathy accounts for the majority of membranous nephropathy; however, few studies have determined the prognostic impact and clinical application of renal pathologic change on this disease. METHODS: A retrospective cohort study of 262 patients with PLA2R-associated membranous nephropathy was conducted. The total renal chronicity score calculated according to the degree of glomerulosclerosis, interstitial fibrosis, tubular atrophy, and arteriosclerosis was applied to evaluate renal chronicity. Baseline bias was adjusted by inverse probability weight when assessing the prognostic impact of chronicity, and multiple parameters were used to evaluate the application value of renal chronicity. RESULTS: During a median follow-up of 24.5 months, renal outcome (kidney function deterioration and/or end-stage kidney disease) was observed in 22 (8.40%) patients. Not only did a higher total renal chronicity score independently predict renal outcome [odds ratio (OR): 1.562, 95% confidence interval (CI) 1.073-2.273, P = 0.020], but non-minimal chronicity was also an independent risk factor for renal outcome (OR: 3.170, 95% CI 1.040-9.659, P = 0.042). Moreover, the membranous nephropathy risk classification in the Kidney Disease: Improving Global Outcomes (KDIGO) guideline integrated with non-minimal chronicity showed improvements in categorical net reclassification (0.174, 95% CI 0.012-0.335, P = 0.035), continuous net reclassification (0.462, 95% CI 0.087-0.838, P = 0.016), and integrated discrimination (0.019, 95% CI 0.003-0.035, P = 0.020) compared to the original classification. CONCLUSIONS: Renal chronicity is closely associated with renal outcomes in PLA2R-associated membranous nephropathy, and combining the KDIGO risk classification with chronicity scores may provide a more accurate prognostic prediction.


Subject(s)
Glomerulonephritis, Membranous , Receptors, Phospholipase A2 , Humans , Glomerulonephritis, Membranous/complications , Glomerulonephritis, Membranous/diagnosis , Male , Female , Retrospective Studies , Middle Aged , Adult , Prognosis , Kidney/physiopathology , Kidney/pathology , Risk Factors , Kidney Failure, Chronic/etiology , Predictive Value of Tests , Disease Progression , Atrophy , Fibrosis , Biopsy , Severity of Illness Index
13.
Semin Nephrol ; 44(1): 151497, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38485643

ABSTRACT

Recent advances in the treatment of plasma cell disorders (PCDs) have provided a wealth of therapy alternatives and improved overall survival tremendously. Various types of PCDs are associated with kidney injury and end-stage kidney disease in a considerable number of patients. Kidney transplantation (KTx) is the best option for renal replacement therapy in select patients in terms of both quality of life parameters and overall survival. Even with modern therapies, all PCDs carry the risk of hematologic progression, whereas histologic recurrence and graft loss are other prevailing concerns in these patients. The risk of mortality is also higher in some of these disorders compared with KTx recipients who suffer from other causes of kidney disease. Unlike solid cancers, there is no well-defined "waiting time" after hematologic remission before proceeding to KTx. Thus, clinicians are usually reluctant to recommend KTx to patients who develop end-stage kidney disease due to PCDs. This review aims to provide the current evidence on KTx outcomes in patients with monoclonal gammopathy of renal significance and multiple myeloma. Although immunoglobulin light chain amyloidosis is a monoclonal gammopathy of renal significance subtype, KTx outcomes in this group are mentioned in another chapter of this issue.


Subject(s)
Kidney Failure, Chronic , Kidney Transplantation , Multiple Myeloma , Paraproteinemias , Humans , Multiple Myeloma/complications , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/etiology , Paraproteinemias/complications
14.
Front Immunol ; 15: 1354101, 2024.
Article in English | MEDLINE | ID: mdl-38495894

ABSTRACT

Beyond the direct benefit that a transplanted organ provides to an individual recipient, the study of the transplant process has the potential to create a better understanding of the pathogenesis, etiology, progression and possible therapy for recurrence of disease after transplantation while at the same time providing insight into the original disease. Specific examples of this include: 1) recurrence of focal segmental glomerulosclerosis (FSGS) after kidney transplantation, 2) recurrent autoimmunity after pancreas transplantation, and 3) recurrence of disease after orthotopic liver transplantation (OLT) for cirrhosis related to progressive steatosis secondary to jejuno-ileal bypass (JIB) surgery. Our team has been studying these phenomena and their immunologic underpinnings, and we suggest that expanding the concept to other pathologic processes and/or transplanted organs that harbor the risk for recurrent disease may provide novel insight into the pathogenesis of a host of other disease processes that lead to organ failure.


Subject(s)
Glomerulosclerosis, Focal Segmental , Kidney Failure, Chronic , Kidney Transplantation , Transplants , Humans , Neoplasm Recurrence, Local/complications , Kidney Transplantation/adverse effects , Kidney Failure, Chronic/etiology
15.
Lancet ; 403(10433): 1279-1289, 2024 Mar 30.
Article in English | MEDLINE | ID: mdl-38492578

ABSTRACT

BACKGROUND: Individuals with rare kidney diseases account for 5-10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. METHODS: People aged 0-96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan-Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m2 or more to first eGFR of less than 30 mL/min per 1·73 m2 (the therapeutic trial window). FINDINGS: Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9-16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32-0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. INTERPRETATION: Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3-5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. FUNDING: RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.


Subject(s)
Kidney Failure, Chronic , Renal Insufficiency, Chronic , Renal Insufficiency , Humans , Glomerular Filtration Rate , Kidney , Kidney Failure, Chronic/epidemiology , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/etiology , Radar , Rare Diseases , Registries , Renal Insufficiency/epidemiology , Renal Insufficiency, Chronic/epidemiology , Renal Insufficiency, Chronic/therapy , Renal Insufficiency, Chronic/complications , United Kingdom/epidemiology , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over
16.
Ren Fail ; 46(1): 2324079, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38425087

ABSTRACT

BACKGROUND: Patients with end-stage kidney disease (ESKD) receiving peritoneal dialysis (PD) or haemodyalisis (PD) appear to be less physically active than healthy persons, a situation that could lead to reductions in quality of life. The aim of the present study was to assess and compare physical activity and health-related quality of life in renal patients on HD and PD programs. METHODS: In May 2020, 130 patients (106 HD and 24 PD) were enrolled in a study of chronic dialysis programs. All participants received a questionnaire containing information on demographics, treatment, and co-morbidities. Physical activity was assessed using the International Physical Activity Questionnaire (IPAQ) short form, and quality of life was measured using the Kidney Disease Quality of Life-Short Form 12 (KDQOL-SF-12) questionnaire comprising mental (MCS) and physical components (PCS). Non-parametric statistical tests were executed with 0.05 as the level of significance. RESULTS: The physical activity of patients treated in both HD and PD programs could be considered as low, without a statistically significant difference between the two modalities. For the quality of life measures, we found a significant (p = .004) difference regarding Physical Component Summary (PCS) scores, with higher PCS scores in patients treated in the PD programme compared to HD. Furthermore, higher physical activity levels were associated with better quality of life parameters in both groups. CONCLUSION: This study confirms the importance of physical activity among dialysis patients with ESKD, suggesting that greater activity could be associated with a better quality of life.


Subject(s)
Kidney Failure, Chronic , Quality of Life , Humans , Hungary , Renal Dialysis/adverse effects , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/etiology , Exercise
17.
Braz J Cardiovasc Surg ; 39(2): e20220436, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38426701

ABSTRACT

INTRODUCTION: The aim of this study was to assess the impact of aortic angulation (AA) on periprocedural and in-hospital complications as well as mortality of patients undergoing Evolut™ R valve implantation. METHODS: A retrospective study was conducted on 264 patients who underwent transfemoral-approach transcatheter aortic valve replacement with self-expandable valve at our hospital between August 2015 and August 2022. These patients underwent multislice computer tomography scans to evaluate AA. Transcatheter aortic valve replacement endpoints, device success, and clinical events were assessed according to the definitions provided by the Valve Academic Research Consortium-3. Cumulative events included paravalvular leak, permanent pacemaker implantation, new-onset stroke, and in-hospital mortality. Patients were divided into two groups, AA ≤ 48° and AA > 48°, based on the mean AA measurement (48.3±8.8) on multislice computer tomography. RESULTS: Multivariable logistic regression analysis was performed to identify predictors of cumulative events, utilizing variables with a P-value < 0.2 obtained from univariable logistic regression analysis, including AA, age, hypertension, chronic renal failure, and heart failure. AA (odds ratio [OR]: 1.73, 95% confidence interval [CI]: 0.89-3.38, P=0.104), age (OR: 1.04, 95% CI: 0.99-1.10, P=0.099), hypertension (OR: 1.66, 95% CI: 0.82-3.33, P=0.155), chronic renal failure (OR: 1.82, 95% CI: 0.92-3.61, P=0.084), and heart failure (OR: 0.57, 95% CI: 0.27-1.21, P=0.145) were not found to be significantly associated with cumulative events in the multivariable logistic regression analysis. CONCLUSION: This study demonstrated that increased AA does not have a significant impact on intraprocedural and periprocedural complications of patients with new generation self-expandable valves implanted.


Subject(s)
Aortic Valve Stenosis , Heart Failure , Heart Valve Prosthesis , Hypertension , Kidney Failure, Chronic , Transcatheter Aortic Valve Replacement , Humans , Transcatheter Aortic Valve Replacement/methods , Aortic Valve/surgery , Aortic Valve Stenosis/surgery , Retrospective Studies , Treatment Outcome , Prosthesis Design , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/surgery , Heart Failure/surgery , Hypertension/etiology
18.
BMC Nephrol ; 25(1): 65, 2024 Feb 23.
Article in English | MEDLINE | ID: mdl-38395753

ABSTRACT

BACKGROUND: We devoted ourselves to proving that the initial transthoracic echocardiography score (TTES) had predictive significance for patients with continuous ambulatory peritoneal dialysis (CAPD). METHODS: In this retrospective analysis, 274 CAPD patients who had PD therapy were recruited sequentially. TTE exams were performed three months following the start of PD therapy. All patients were divided into two groups based on the strength of their TTES levels. TTES's predictive value for CAPD patients was then determined using LASSO regression and Cox regression. RESULTS: During a median of 52 months, 46 patients (16.8%) died from all causes, and 32 patients (11.7%) died from cardiovascular disease (CV). The TTES was computed as follows: 0.109 × aortic root diameter (ARD, mm) - 0.976 × LVEF (> 55%, yes or no) + 0.010 × left ventricular max index, (LVMI, g/m2) + 0.035 × E/e' ratio. The higher TTES value (≥ 3.7) had a higher risk of all-cause death (hazard ratio, HR, 3.70, 95% confidence index, 95%CI, 1.45-9.46, P = 0.006) as well as CV mortality (HR, 2.74, 95%CI 1.15-19.17, P = 0.042). Moreover, the TTES had an attractive predictive efficiency for all-cause mortality (AUC = 0.762, 95%CI 0.645-0.849) and CV mortality (AUC = 0.746, 95%CI 0.640-0.852). The introduced nomogram, which was based on TTES and clinical variables, exhibited a high predictive value for all-cause and CV mortality in CAPD patients. CONCLUSION: TTES is a pretty good predictor of clinical outcomes, and the introduced TTES-based nomogram yields an accurate prediction value for CAPD patients.


Subject(s)
Cardiovascular Diseases , Kidney Failure, Chronic , Peritoneal Dialysis, Continuous Ambulatory , Humans , Peritoneal Dialysis, Continuous Ambulatory/adverse effects , Prognosis , Retrospective Studies , Echocardiography , Kidney Failure, Chronic/diagnostic imaging , Kidney Failure, Chronic/therapy , Kidney Failure, Chronic/etiology
19.
Int J Rheum Dis ; 27(2): e15079, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38396352

ABSTRACT

OBJECTIVE: Given limited regional data, we investigate the state-wide epidemiology, renal and patient outcomes for lupus nephritis (LN) in Western Australia (WA). METHODS: Patients hospitalized with incident SLE (≥2 diagnostic codes in the state-wide WA Health Hospital Morbidity Data Collection) in the period 1985-2015 were included (n = 1480). LN was defined by the presence of glomerulonephritis and/or raised serum creatinine. Trends over three study decades for annual incidence rate (AIR)/100.000 population, mortality (MR), and end-stage renal disease (ESRD) rates/100 person years were analyzed by least square regression and compared with a matched control group (n = 12 840). RESULTS: Clinical evidence of LN developed in 366 SLE patients (25.9%) after a median disease duration of 10 months (IQR 0-101) with renal biopsy performed in 308 (84.2%). The AIR for LN (0.63/100.000) did not change significantly over time (R2 = .11, p = .85), while point prevalence reached 11.9/100.000 in 2015. ESRD developed in 14.1% (n = 54) of LN patients vs. 0.2% in non-LN SLE patients and 0.05% in controls (all p ≤ 0.01). ESRD rates increased over time in LN patients (0.4 to 0.7, R2 = .52, p = .26). The odds ratio for death was 8.81 (CI 3.78-22.9) for LN and 6.62 (CI 2.76-17.9) for non-LN SLE patients compared to controls and MR for LN patients increased over time (1.3 to 2.2, R2 = .84, p = .26). CONCLUSIONS: The incidence rate of LN in WA remained unchanged over 30 years. A lack of improvement in renal failure and mortality rates illustrates the pressing need for better long-term treatment options and/or strategies in LN.


Subject(s)
Glomerulonephritis , Kidney Failure, Chronic , Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Lupus Nephritis/diagnosis , Lupus Nephritis/epidemiology , Lupus Nephritis/drug therapy , Incidence , Kidney/pathology , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/epidemiology , Kidney Failure, Chronic/etiology , Glomerulonephritis/pathology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/complications , Retrospective Studies
20.
Exp Clin Transplant ; 22(Suppl 1): 60-65, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38385374

ABSTRACT

Renal transplant is the best procedure for patients with end-stage renal disease. Although an ideal kidney transplant should survive for the lifetime of each recipient, there may be a need for a second, third, or even a fourth retransplant. The outcomes of these kidney allografts, surgical approaches, immunology issues, and drug therapies warrant greater focus. Pediatric kidney retransplant is even more important because these patients are more immunologically responsive to donor antigens and because they need longer allograft survival. Although kidney retransplant provides a survival advantage for patients who would otherwise remain on the wait list and/or hemodialysis, careful patient selection is crucial for second, third, and fourth renal transplants. Despite the shortage of donor organs, outcomes, manageable complications, and economic considerations support earlier kidney retransplants rather than delayed retransplants. Preoperative vascular imaging, appropriate induction therapy, regular monitoring of renal function, and regular surveillance for malignancy and infection are more important in the retransplanted kidneys than in cases of first kidney transplants. The lack of robust data on optimal clinical management of these retransplant recipients has contributed to substantial variations in clinical practice among different centers. In this review, we discuss medical and surgical approaches in the cases of second and third kidney transplants.


Subject(s)
Kidney Failure, Chronic , Kidney Transplantation , Humans , Child , Kidney Transplantation/adverse effects , Tissue Donors , Renal Dialysis , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/etiology , Reoperation , Graft Survival
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