Poor Renal Outcome Research Articles

SUN-365 LUPUS NEPHRITIS: EPIDEMIOLOGICAL, CLINICAL, BIOLOGICAL, PATHOLOGICAL FINDINGS, OUTCOMES AND PROGNOSTIC FACTORS

Systemic lupus erythematosus is a multi-visceral autoimmune disease. Renal involvement is one of the most common and serious manifestations of this disease. The histological lesions are highly polymorphic and the renal biopsy remains crucial for the therapeutic management of lupus nephritis (LN). The aim of our investigation was to study the epidemiological, clinical, biological and histological characteristics, outcomes and to evaluate the therapeutic protocols used for lupus nephritis’ treatment and to identify predictive factors of renal prognosis in patients with lupus nephritis. It was a retrospective study including patients over 16 years old with lupus nephritis proved by kidney biopsy and followed up over a period of 17 years (1998 - 2015) in Internal Medicine Department of Charles Nicolle Hospital of Tunis. We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years with a maximum between 15 years and 45 years. The most frequent extra-renal manifestations were articular and dermatological manifestations (79%). Renal symptomatology was dominated by proteinuria noted in all patients, associated to a nephrotic syndrome in 68% of patients. At the time of diagnosis of LN, hematuria was present in 69% of patients and renal failure was present in half of cases. Immunologically, antinuclear antibody were positive in 89.1% of cases, anti DNA positive in 73.4% of cases, anti Sm positive in 79.8% of cases and Antiphospholipids were positive in 50% of cases, associated with an antiphospholipid syndrome in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (36.6%) isolated or associated with LN class V (17.7%). All patients received a corticosteroid for induction or maintenance treatment. It was associated with immunosuppressive treatment according to different treatment regimens. The median duration of follow-up was 81.2 months. Renal outcome was marked by complete and sustained remission in 36.7% of cases, incomplete remission with chronic kidney disease in 34.5% of cases, chronic renal failure in 28.7% of cases. At univariate analysis, we identified the young age below 35 years at the time of the discovery of LN, the male sex, increased serum creatinine at the time of biopsy, proliferative forms, the presence of histological signs of chronicity and lesions of thrombotic microangiopathy as predictive factors of poor renal outcomes. Lupus nephritis is one of the most common and serious manifestations of Systemic lupus erythematosus. The generalization of renal biopsy, the use of early codified therapeutic protocols and regular monitoring and evaluation of disease activity according to the appropriate scores can improve management and survival of patients with renal impairment.

The incidence, risk factors, and long-term outcomes of acute kidney injury in hospitalized diabetic ketoacidosis patients

BackgroundEmerging evidence has demonstrated that acute kidney injury (AKI) is an important risk factor associated with increased morbidity and mortality in diabetic ketoacidosis (DKA) patients. The current study aimed to investigate the incidence rate, risk factors, long-term renal outcomes, and mortality in DKA patients with AKI.MethodsA total of 179 patients diagnosed with DKA at Sun Yat-sen Memorial Hospital from January 2012 to January 2018 were included in the analysis. AKI was diagnosed according to the 2012 KDIGO criteria. Risk factors, long-term renal outcomes, and mortality were analyzed by logistic regression and Cox proportional hazards models.ResultsAmong 179 DKA patients, 98 patients (54.75%) were diagnosed as AKI. Aging; increased blood glucose, serum uric acid and white blood cells; decreased serum pH and albumin; coma; and preexisting chronic kidney disease (CKD) were risk factors of AKI in patients with DKA. During follow-up, DKA patients with AKI showed more than a two-fold decline in eGFR within 1 year after discharge from the hospital when compared with non-AKI DKA patients. Furthermore, AKI was also an independent risk factor for poor long-term renal outcomes and mortality in DKA patients.ConclusionsMultiple risk factors contribute to the development of AKI in DKA patients. AKI and advanced AKI stage are associated with rapid progressive CKD and long-term mortality in patients with DKA.

Upregulated Expression of Intestinal Antimicrobial Peptide HD5 Associated with Renal Function in IgA Nephropathy.

Purpose It was reported that gut-kidney axis may play an important role in IgA nephropathy (IgAN). Previous five GWASs of different populations for IgAN have discovered several genes related to intestinal immunity, including DEFA gene. However, the roles of the encoded proteins of DEFA5/6 which were called intestinal antimicrobial peptides HD5 and HD6 were not clear in kidney disease, such as IgAN. The purpose of this study was to clarify the association of HD5 and HD6 with IgAN. Methods We measured HD5 and HD6 in serum, urine, and kidney of IgAN patients and normal controls by ELISA, Western blot, and immunofluorescence. The association of HD5 or HD6 levels with clinical and pathologic phenotypes was analyzed. Results Serum levels of HD5 and HD6 were significantly higher in IgAN patients than those in normal controls. Baseline serum HD5 levels were significantly associated with eGFR (P = 0.002) and tubular atrophy/interstitial fibrosis (P = 0.002) and tubular atrophy/interstitial fibrosis (P = 0.002) and tubular atrophy/interstitial fibrosis (P = 0.002) and tubular atrophy/interstitial fibrosis (Conclusions In IgAN patients, an elevated serum HD5 level at the time of renal biopsy was associated with poor renal outcomes. HD5 rather than HD6 was probably associated with renal function of IgAN patients.

MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Sch\xf6nlein purpura nephritis

BackgroundHenoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy. Oxford classification of immunoglobulin A nephropathy pathology has been updated to the MEST-C score, but its application in HSPN remains unresolved.MethodsTwo hundred and thirteen patients with biopsy-proven HSPN were retrieved from the Seoul National University Hospital between 2000 and 2017. Renal outcome risks (i.e., end-stage renal disease or doubling of serum creatinine) were evaluated according to MEST-C scores after stratification by age: 113 children aged < 18 years (9.2 ± 3.6 years) and 100 adults aged ≥18 years (38.6 ± 18.3 years). We pooled our data with four previous cohort studies in which MEST or MEST-C scores were described in detail.ResultsTwenty-one child (19%) and 16 adult (16%) patients reached the renal outcome during the median follow-up periods of 12 years and 13 years, respectively (maximum 19 years). In children, M1 and T1/T2 scores revealed worse renal outcomes than did M0 and T0 scores, respectively, whereas the T score was the only factor related to worse outcomes in adult patients after adjusting for multiple clinical and laboratory variables. The pooled data showed that M1, S1, and T1/T2 in children and E1 and T1/T2 in adults were correlated with poorer renal outcomes than those of their counterpart scores.ConclusionsThe Oxford classification MEST-C scores can predict long-term renal outcomes in patients with HSPN.

The role of hypertriglyceridemia and treatment patterns in the progression of IgA nephropathy with a high proportion of global glomerulosclerosis.

Many studies have focused on finding predictors for mild IgAN progression. However, the cases of severe IgAN with a high proportion of global glomerulosclerosis have received inadequate attention. A group of 172 primary IgAN patients with 50-75% global glomerulosclerosis was studied retrospectively between April 2007 and October 2017. Patients were divided into three groups according to the serum triglyceride tertiles: < 1.42µmol/L (Group 1), 1.42-2.29µmol/L (Group 2), and > 2.29µmol/L (Group 3). Groups 1 and 2 comprised non-hypertriglyceridemia subjects, while Group 3 was defined as the hypertriglyceridemia (HTG) group. The patients were followed for 4-96months (median 39.43months). The study end point was defined as a 50% decline in estimated glomerular filtration rate (eGFR) or ESRD. A high proportion of global glomerulosclerosis is not absolutely correlated with severe clinical features and poor renal outcome. In our retrospective observation, eGFR decreased by less than 10% of the baseline during follow-up in 43.6% of the patients. However, in our patients with HTG, the cumulative renal survival rate was significantly lower compared to those without HTG. Multivariate Cox regression analysis also showed that triglyceride is an independent predictor of poor renal outcomes. Furthermore, in the HTG group, the cumulative renal survival rates were higher in patients treated with Tripterygium wilfordii Hook F (TwHF) compared to those without TwHF. A high proportion of global glomerulosclerosis combined with HTG at biopsy have better predictive validity for the disease progression of IgAN than global glomerulosclerosis alone. TwHF may partially affect the renal outcome of severe IgAN with HTG, and this may relate to its regulation of lipid metabolism and immunoinflammatory response.

Association between serum alkaline phosphatase and renal outcome in patients with type 2 diabetes mellitus

This retrospective study included 299 patients with type 2 diabetes mellitus and biopsy-confirmed diabetic nephropathy (DN) to investigate the prognostic value of alkaline phosphatase (ALP) for renal outcome. Cox proportional hazards models were used to estimate the hazard ratios (HRs) for the serum ALP level on renal outcome, which was defined as end-stage renal disease (ESRD) or a 50% decline in estimated glomerular filtration rate (eGFR) from baseline. The median baseline ALP was 80 IU/L with an interquartile range of 64–97 IU/L. Serum ALP was negatively associated with eGFR but positively associated with proteinuria and renal interstitial fibrosis. During a median follow-up period of 23 months, ESRD or a 50% declined in the eGFR occurred in 156 (52.2%) patients. The highest quartile of ALP was significantly associated with poor renal outcome, as defined above (HR 2.38, 95% confidence interval [CI] 1.09–5.17), when adjusted for sociodemographics, baseline eGFR, proteinuria, liver function parameters, parathyroid hormone levels, and renal pathological findings. Each standard deviation higher in the natural log-transformed ALP was associated with a 25% increased risk for poor renal outcome. Additionally, there was a graded increase in the risk for poor renal outcome with higher ALP in patients with nephrotic-range proteinuria. However, no significant associations were observed between serum ALP levels and renal outcome in patients with non-nephrotic-range proteinuria. In conclusion, an elevated ALP level was independently associated with poor renal outcome in patients with type 2 diabetes mellitus and nephrotic-range proteinuria after multivariate adjustment.

Association of unhealthy dietary behaviors with renal function decline in patients with diabetes

ObjectiveBalanced nutrition is important for patients with diabetes, and nutrition might well influence diabetes-related complications, although there is limited evidence for this supposition at present. Consequently, we investigate the association...

P-glycoprotein Expressing-B cell associated Active True Renal Lupus Vasculitis in Lupus Nephritis

True renal lupus vasculitis (TRLV), a rare form of vascular lesion usually associated with proliferative lupus nephritis (LN), is resistant to conventional treatments and associated with poor renal outcome among renal vasculopathies. Evidence suggests the involvement of P-glycoprotein (P-gp) expressing-activated B cells in the pathogenesis and treatment resistance of TRLV through the production of autoantibodies and direct infiltration into the inflammatory lesion of small vessels. Therapies targeting activated B cells might overcome refractory TRLV. Identification of the subsets of peripheral activated B cells that express P-gp in TRLV patients might help the selection of suitable treatment strategy.

Poor Renal and Cardiovascular Outcomes in Patients with Biopsy-Proven Diabetic Nephropathy

Background: Despite the high mortality of cardiovascular disease (CVD) in diabetic patients with renal injury, few studies have compared cardiovascular characteristics and outcomes between patients with diabetic nephropathy (DN) and non-diabetic renal disease (NDRD). Methods: A total of 326 type 2 diabetes mellitus patients with renal biopsy were assigned to DN and NDRD groups. Echocardiography and Doppler ultrasound were performed to evaluate left ventricular hypertrophy (LVH) and peripheral atherosclerosis disease (PAD). Renal and cardiovascular survival rates were compared between the DN and NDRD groups by Kaplan-Meier analysis. Risk factors for renal and cardiovascular events in DN patients were identified by a Cox proportional hazards model. Results: In total, 179 patients entered the DN group (54.9%) and 147 made up the NDRD group (45.1%). The presence of diabetic retinopathy, family history of diabetes, and dependence on insulin therapy were associated with the presence of DN. DN patients had more CVD with more severe LVH and PAD. Poorer renal (log-rank χ² = 26.534, p < 0.001) and cardiovascular (log-rank χ² = 16.257, p < 0.001) prognoses were seen in the DN group. DR (HR 1.539, 95% CI 1.332–1.842), eGFR (HR 0.943, 95% CI 0.919–0.961), and 24-h proteinuria (HR 1.211, 95% CI 1.132–1.387) were identified as risk factors for renal endpoints. Age (HR 1.672, 95% CI 1.487–1.821), HbA1C (HR 1.398, 95% CI 1.197–1.876), and 24-h proteinuria (HR 1.453, 95% CI 1.289–1.672) were associated with cardiovascular endpoints. Conclusion: Patients with DN had more severe CVD along with poorer renal and cardiovascular prognoses than those with NDRD.

IgG deposits in the mesangium and capillary loops predict poor renal outcome in patients with IgA nephropathy: a single-center retrospective study

Background Glomerular IgG deposition in patients with IgA nephropathy (IgAN) has been shown to be associated with poor renal survival; however, most published studies to date are too small-scale and inconsistent to provide guidance for clinical practice. Methods Based on renal biopsy findings, 742 patients were divided into the following groups: (i) IgA deposition alone (IgA) vs IgA + IgG deposition (IgA + IgG) and (ii) IgG co-deposition confined to the mesangium vs mesangium + capillary loops (CLs). The clinicopathological variables at biopsy and renal outcome were assessed. Results Of the 742 patients, 182 had IgG co-deposition and 51 had IgG deposits in the mesangium + CLs. Patients with IgG co-deposition were associated with severe clinical and pathological lesions, especially those with a location of IgG deposits in the mesangium +CLs. Kaplan–Meier analysis revealed that a lower renal cumulative survival rate was present in both patients with IgG co-deposition and those with a location of IgG deposits in the mesangium + CLs (all p < 0.05). Moreover, patients with a higher intensity of glomerular IgG deposits or C3 deposits or C1q deposits were also associated with a lower survival rate. A multivariate Cox regression model identified the location of IgG deposits in the mesangium + CLs as an independent risk factor for poor prognosis (HR, 2.11; 95% CI: 1.06–4.18; p = 0.005). Conclusions Glomerular IgG co-deposition and the location of glomerular IgG deposits in the mesangium + CLs were both associated with adverse renal outcomes, but only the location of glomerular IgG deposits in the CLs was an independent risk factor for poor prognosis in IgAN.

Diagnostic and Risk Factors for Complement Defects in Hypertensive Emergency and Thrombotic Microangiopathy.

Hypertensive emergency can cause thrombotic microangiopathy (TMA) in the kidneys with high rates of end-stage renal disease (ESRD) and vice versa. The conundrum of hypertension as the cause of TMA or consequence of TMA on the background of defects in complement regulation remains difficult. Patients with hypertensive emergency and TMA on kidney biopsy were tested for ex vivo C5b9 formation on the endothelium and rare variants in complement genes to identify complement-mediated TMA. We identified factors associated with defects in complement regulation and poor renal outcomes. Massive ex vivo C5b9 formation was found on resting endothelial cells in 18 (69%) out of 26 cases at the presentation, including the 9 patients who carried at least one rare genetic variant. Thirteen (72%, N=18) and 3 (38%, N=8) patients with massive and normal ex vivo complement activation, respectively, progressed to ESRD (P=0.03). In contrast to BP control, inhibition of C5 activation prevented ESRD to occur in 5 (83%, N=6) patients with massive ex vivo complement activation. TMA-related graft failure occurred in 7 (47%, N=15) donor kidneys and was linked to genetic variants. The assessment of both ex vivo C5b9 formation and screening for rare variants in complement genes may categorize patients with hypertensive emergency and TMA into different groups with potential therapeutic and prognostic implications. We propose an algorithm to recognize patients at the highest risk for defects in complement regulation.

Positive Fluid Balance and Association with Post-Traumatic Acute Kidney Injury

Acute kidney injury (AKI) is common in severely injured trauma patients and is associated with poor outcomes. A positive fluid balance is associated with AKI and poor long-term renal outcomes among general ICU and cardiac surgery patients. Currently, the optimal endpoint of resuscitation of severely injured trauma patients is unknown, which may result in excess fluid administration. We hypothesized that positive fluid balance is common after severe trauma and is associated with increased AKI development. A cohort study of adult (≥16 years old) trauma patients requiring ICU admission from January 2017 to June of 2017 was conducted. Patients were excluded for early death, rhabdomyolysis, or previous history of end-stage renal disease or congestive heart failure. Acute kidney injury within 7 days of admission was defined according to Kidney Disease Improving Global Outcomes creatinine-based criteria. Univariate and multivariable analyses were performed. Of 364 patients, 74% were male. The median age was 41 years (interquartile range [IQR] 27 to 59 years), and the median Injury Severity Score (ISS) was 18 (IQR 10 to 29). Positive fluid balance (>2 L) was observed in 49% of patients. Acute kidney injury was diagnosed in 105 (29%) patients. After adjustment, there was an increased risk of AKI with a positive fluid balance >2 L (relative risk [RR] 1.98 [95% CI 1.24 to 3.17]). Additionally, the risk of AKI incrementally increased by 1.22 with each liter fluid positive above a zero balance (95% CI 1.11 to 1.34). Positive fluid balance in excess of 2 L at 48 hours occurs in half of severely injured trauma patients, and fluid positivity is independently and incrementally associated with AKI development. Fluid responsiveness should be investigated as an end point of post-traumatic resuscitation to prevent unnecessary fluid administration and subsequent AKI.

Association of Mineral Bone Disorder With Decline in Residual Kidney Function in Incident Hemodialysis Patients.

Abnormalities of mineral bone disorder (MBD) parameters have been suggested to be associated with poor renal outcome in predialysis patients. However, the impact of those parameters on decline in residual kidney function (RKF) is uncertain among incident hemodialysis (HD) patients. We performed a retrospective cohort study in 13,772 patients who initiated conventional HD during 2007 to 2011 and survived 6 months of dialysis. We examined the association of baseline serum phosphorus, calcium, intact parathyroid hormone (PTH), and alkaline phosphatase (ALP) with a decline in RKF. Decline in RKF was assessed by estimated slope of renal urea clearance (KRU) over 6 months from HD initiation. Our cohort had a mean ± SD age of 62 ± 15 years; 64% were men, 57% were white, 65% had diabetes, and 51% had hypertension. The median (interquartile range [IQR]) baseline KRU level was 3.4 (2.0, 5.2) mL/min/1.73 m2 . The median (IQR) estimated 6-month KRU slope was -1.47 (-2.24, -0.63) mL/min/1.73 m2 per 6 months. In linear regression models, higher phosphorus categories were associated with a steeper 6-month KRU slope compared with the reference category (phosphorus 4.0 to <4.5 mg/dL). Lower calcium and higher intact PTH and ALP categories were also associated with a steeper 6-month KRU slope compared with their respective reference groups (calcium 9.2 to <9.5 mg/dL; intact PTH 150 to <250 pg/mL; ALP <60 U/L). The increased number of parameter abnormalities had an additive effect on decline in RKF. Abnormalities of MBD parameters including higher phosphorus, intact PTH, ALP and lower calcium levels were independently associated with decline in RKF in incident HD patients. © 2019 American Society for Bone and Mineral Research. © 2019 American Society for Bone and Mineral Research.

Urinary chemokine C-X-C motif ligand 16 and endostatin as predictors of tubulointerstitial fibrosis in patients with advanced diabetic kidney disease.

Interstitial fibrosis and tubular atrophy (IFTA) is a well-recognized risk factor for poor renal outcome in patients with diabetic kidney disease (DKD). However, a noninvasive biomarker for IFTA is currently lacking. The purpose of this study was to identify urinary markers of IFTA and to determine their clinical relevance as predictors of renal prognosis. Seventy patients with biopsy-proven isolated DKD were enrolled in this study. We measured multiple urinary inflammatory cytokines and chemokines by multiplex enzyme-linked immunosorbent assay in these patients and evaluated their association with various pathologic features and renal outcomes. Patients enrolled in this study exhibited advanced DKD at the time of renal biopsy, characterized by moderate to severe renal dysfunction [mean estimated glomerular filtration rate (eGFR) 36.1 mL/min/1.73 m2] and heavy proteinuria (mean urinary protein:creatinine ratio 7.8 g/g creatinine). Clinicopathologic analysis revealed that higher IFTA scores were associated with worse baseline eGFR (P < 0.001) and poor renal outcome (P = 0.002), whereas glomerular injury scores were not. Among measured urinary inflammatory markers, C-X-C motif ligand 16 (CXCL16) and endostatin showed strong correlations with IFTA scores (P = 0.001 and P < 0.001, respectively), and patients with higher levels of urinary CXCL16 and/or endostatin experienced significantly rapid renal progression compared with other patients (P < 0.001). Finally, increased urinary CXCL16 and endostatin were independent risk factors for poor renal outcome after multivariate adjustments (95% confidence interval 1.070-3.455, P = 0.029). Urinary CXCL16 and endostatin could reflect the degree of IFTA and serve as biomarkers of renal outcome in patients with advanced DKD.

Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study

ObjectiveTo describe the differences in clinical characteristics and outcome between adult- and childhood-onset biopsy-proven IgA vasculitis (IgAV) in North America. Patients and MethodsPatients with IgAV diagnosed from January 1, 1997, through December 31, 2016, were retrospectively identified. Data were abstracted from direct medical record review. Kaplan-Meier methods were used to estimate survival rates. ResultsA total of 243 patients with IgAV were included (227 [93.4%] white, 141 [58.0%] male); 174 patients were adults (≥21 years), and 69 were younger than 21 years. Compared with patients younger than 21 years, adults at baseline more frequently had ulcerative skin lesions (19 [10.9%] vs 1 [1.4%]; P=.02) and nephrotic-range proteinuria (21 of 96 [21.9%] vs 1 of 38 [2.6%]; P=.007) but less commonly had abdominal pain (59 [33.9%] vs 42 [60.9%]; P<.001), ischemic gastrointestinal tract involvement (18 [10.3%] vs 14 [20.3%]; P=.04), and arthralgias (66 [37.9%] vs 42 [60.8%]; P<.001). During 389 person-years of follow-up, 29 deaths were observed. Five-year survival rates for patients aged younger than 21, 21 to 50, and 51 years or older were 100%, 94%, and 40%, respectively. In comparison to data from the United States life tables for whites, patients 51 years or older at diagnosis had a greater than 7-fold increased risk of mortality (standardized mortality, 7.60 [95% CI, 5.01-11.06]; P<.001). ConclusionIgA vasculitis in adults is associated with more severe skin/kidney involvement and poorer renal outcome. Among adults with IgAV, patients aged 51 years or older at diagnosis have significantly higher mortality (P<.001).

Serum uric acid is an independent predictor of renal outcomes in patients with idiopathic membranous nephropathy.

Accumulating evidence suggests that a relationship exists between serum uric acid (UA) and the progression of chronic kidney disease (CKD), but information regarding idiopathic membranous nephropathy (IMN) is limited. Patients with renal biopsy-confirmed diagnosis of IMN between 2009 and 2017 were identified. The demographic and clinical data recorded at the time of renal biopsy were considered the baseline values. The included cases were separated into three groups based on tertiles of the baseline serum UA level, and the relationship between serum UA and poor renal outcome was investigated by receiver operating characteristic (ROC) and time-event analyses. The primary endpoint was poor renal outcome, which was defined as a decrease in the estimated glomerular filtration rate to 50% of the baseline level or progression to end-stage renal disease during the follow-up. Of 989 cases, 572 eligible patients were included. During a median of 18months of follow-up, 45 (7.9%) patients progressed to the primary endpoint. Both baseline serum UA and time-averaged UA levels could be used for discrimination of renal outcomes, but the difference was not significant (p value = 0.6). Our Cox regression analysis further demonstrated that baseline serum UA was an independent predictor of poor renal outcome in IMN patients, and subgroup analysis revealed a gender difference in the predictive effect of serum UA. Our study demonstrated that baseline serum UA was an independent predictor of poor renal outcome in patients with IMN, and a gender difference in the predictive effect was observed in our cohort.

PKD1-associated autosomal dominant polycystic kidney disease with glomerular cysts presenting with nephrotic syndrome caused by focal segmental glomerulosclerosis

BackgroundAutosomal dominant polycystic kidney disease (ADPKD) may manifest non-nephrotic range proteinuria, but is rarely complicated with nephrotic syndrome. Limited number of reports describe the histology of ADPKD with nephrotic syndrome in detail.Case presentationWe encountered a 23-year-old man with polycystic kidney disease (PKD) with small kidney volume and nephrotic syndrome, which eventually progressed to end-stage renal disease. Renal histology showed typical focal segmental glomerulosclerosis and remarkable glomerular cyst formation, but did not reveal tubular cysts. PKD1 mutation was detected in him and his father, who also had PKD with small kidney volume.ConclusionsIn contrast to tubular cysts which develop along ADPKD progression, glomerular cysts may likely be associated with ADPKD with slower volume progression manifesting small kidney volume. Although previous investigations report that ADPKD with smaller kidney volume is attributed to slower decline in renal function, coexistence of nephrotic-range proteinuria implies complication of other glomerular diseases and needs histological evaluation since it may lead to poor renal outcome.

Outcome and Prognosis of Patients With Lupus Nephritis Submitted to Renal Transplantation

This stydy aimed to evaluate the epidemiological and clinical profile and outcome of patients with lupus nephritis (LN) submitted to renal transplantation. Retrospective cohort study based on the records of 35 LN patients submitted to renal transplantation at a single center in Brazil between July 1996 and May 2016. The Kaplan-Meier method was used to estimate 6-month, 1-year and 5-year graft survival. The sample included 38 transplantations (3 of which retransplantations). The mean age at the time of SLE diagnosis was 23.7 ± 9.0 years. Most patients were female (94.7%) and 68.4% were non-Caucasian. Twenty-two (57.9%) underwent renal biopsy prior to transplantation. The mean time from SLE diagnosis to transplantation was 10.3 ± 6.4 years. The mean pre-transplantation dialysis time was 3.8 ± 3.7 years. The grafts came from living related (n = 11) or deceased (n = 27) donors. Three (7.9%) patients experienced acute rejection in the first year. Graft and patient survival rates were, respectively, 97.1% and 100% at 6 months, 84.9% and 96.9% at 1 year, and 76.3% and 92.5% at 5 years. One (2.6%) patient had SLE recurrence. Venous thrombosis (p = 0.017) and antiphospholipid syndrome (APS) (p = 0.036) were more prevalent in patients with graft loss. In our cohort of LN patients submitted to renal transplantation, the 5-year survival rate was high, and APS was an important predictor of poor renal outcome (graft loss).

Recurrent acute kidney injury: predictors and impact in a large population-based cohort.

This study examined the impact of recurrent episodes of acute kidney injury (AKI) on patient outcomes. The Welsh National electronic AKI reporting system was used to identify all cases of AKI in patients ≥18 years of age between April 2015 and September 2018. Patients were grouped according to the number of AKI episodes they experienced with each patient's first episode described as their index episode. We compared the demography and patient outcomes of those patients with a single AKI episode with those patients with multiple AKI episodes. Analysis included 153 776 AKI episodes in 111528 patients. Of those who experienced AKI and survived their index episode, 29.3% experienced a second episode, 9.9% a third episode and 4.0% experienced fourth or more episodes. Thirty-day mortality for those patients with multiple episodes of AKI was significantly higher than for those patients with a single episode (31.3% versus 24.9%, P < 0.001). Following a single episode, recovery to baseline renal function at 30 days was achieved in 83.6% of patients and was significantly higher than for patients who had repeated episodes (77.8%, P < 0.001). For surviving patients, non-recovery of renal function following any AKI episode was significantly associated with a higher probability of a further AKI episode (33.4% versus 41.0%, P < 0.001). Furthermore, with each episode of AKI the likelihood of a subsequent episode also increased (31.0% versus 43.2% versus 51.2% versus 51.7% following a first, second, third and fourth episode, P < 0.001 for all comparisons). The results of this study provide an important contribution to the debate regarding the need for risk stratification for recurrent AKI. The data suggest that such a tool would be useful given the poor patient and renal outcomes associated with recurrent AKI episodes as highlighted by this study.

Plasma galactose-deficient immunoglobulin A1 and loss of kidney function in patients with immunoglobulin A vasculitis nephritis.

Immunoglobulin A (IgA) vasculitis nephritis (IgAV-N) is the most common secondary IgA nephropathy (IgAN). Many studies have demonstrated that galactose-deficient IgA1 (Gd-IgA1) in the IgA1 hinge region is associated with the development and also progression of primary IgAN. In this study, we aimed to evaluate the roles of Gd-IgA1 in kidney disease progression in a large Chinese cohort of IgAV-N patients. This cohort study enrolled 112 patients with IgAV-N, 15 patients with IgA vasculitis (IgAV) without kidney involvement and 108 patients with IgAN. Plasma IgA1 and Gd-IgA1 levels at kidney biopsy were measured by enzyme-linked immunosorbent assay. The primary endpoint was a 30% decline in estimated glomerular filtration rate or end-stage renal disease or death. The levels of Gd-IgA1 in IgAV-N and IgAN patients were higher than in healthy controls (mean ± SD, 302.86 ± 54.93 U/mL versus 303.16 ± 59.43 U/mL versus 281.30 ± 43.74 U/mL, respectively; P = 0.047), as well as compared with those with IgAV without kidney involvement (272.65 ± 53.14 U/mL; P = 0.036). After adjusting clinical data, higher levels of Gd-IgA1 were found to be independently associated with a greater risk for kidney failure [hazard ratio (HR) = 1.703 per 1 SD, 95% confidence interval (CI) 1.233-2.352; P = 0.001]. Compared with the first Gd-IgA1 quartile group (as reference), the fourth Gd-IgA1 quartile group retained a predictive value for poor renal outcome (HR = 3.740, 95% CI 1.204-11.619; P = 0.023). These data indicate that Gd-IgA1 levels were similarly elevated in adult patients with IgAN and those with IgAV-N. Moreover, increased Gd-IgA1 levels were associated with both the development and progression of IgAV-N, as observed in IgAN.