Purpose: Patients with neurogenic bladder (NB) have higher risk of developing chronic kidney disease (CKD). Due to their lower muscle mass, the estimated glomerular filtration rate (eGFR) using creatinine (Cr) may be overestimated and delay the diagnosis of renal failure. The study compare eGFR calculated by different formulas based on creatinine and/or cystatin C (CysC) in children with NB, and the differences between patients with lower muscle mass (underdeveloped lower limbs) and with independent gait (less muscle depletion). Methods: The eGFR was calculated in all pediatric patients with NB and CKD stage 1 and 2, using different formulas: CKiD-Cr, CKiD-CysC, CKiD combined-Cr/CysC, Zappitelli-CysC and Zappitelli combined-Cr/CysC. Results: Forty-seven patients were evaluated, 74.5% in CKD stage 1, with median age of 14.1 years, 59.6% had lipo/myelomeningocele. When compared with CKiD-Cr, CysC-based formulas showed lower eGFR (p<0.05), (CKiD-CysC (p<0.001), Zappitelli-CysC (p<0.001), CKiD -Cr/CysC (p<0.001) and Zappitelli combined-Cr/CysC (p<0.05)). When CKiD-CysC was used, 68% of patients moved to a more advanced CKD stage. In patients without independent gait, with lower muscle mass (55.3%), the median eGFR using CKiD-Cr and CKiD combined-Cr/CysC were higher (p<0.05). No differences were found between the two groups using the others CysC-based formulas. Conclusion: In patients with NB and poor muscle mass, CKiD-Cr formula may overestimate renal function. CysC-based formulas seem more reliable in these patients, especially in those with greater muscular atrophy.
Background and Aims Patients with type 2 diabetes mellitus (T2DM), may have various underlying causes contributing to kidney disease beyond diabetic nephropathy (DN). In such cases, a kidney biopsy (KB) can provide a definite diagnosis and allow for tailored treatment options. The aim of this study is to evaluate non diabetic kidney disease (NDKD) in T2DM patients and identify data to support KB indications. Method This is a retrospective observational study that included patients with T2DM who were submitted to a native KB between 2011 and 2022. We collected demographic, clinical and laboratory data at the date of biopsy. KB indication was considered in order to include the patients in the first encountered criteria defined sequentially as the presence of (1) nephrotic syndrome, (2) low or rapidly declining estimated glomerular filtration rate (eGFR), (3) nephrotic proteinuria and (4) hematuria. Results We analysed 72 patients with T2DM that were submitted to KB (Table 1). All except one patient had hypertension and 38 patients were screened for diabetic retinopathy (DR), which was present in 23 patients (60%). The criteria for KB was in most of the patients (59.7%) a low or rapidly declining eGFR, followed by nephrotic proteinuria (19.4%), nephrotic syndrome (16.7%) and hematuria (4.2%). KB showed 50% (n = 36) of patients with NDKD, 12.5% (n = 9) with NDKD and DKD and 37.5% (n = 27) with isolated NDKD. Among these patients, hypertensive nephrosclerosis (19.4%), focal segmental glomerulosclerosis (13.8%), acute interstitial nephritis (13.8%), membranous nephropathy (11.2%) and IgA nephropathy (5.6%), were the most prevalent diagnosis. Patients with DR had a higher prevalence of DKD with a positive predictive value (PPV) of 87%. On the other hand, in the absence of DR, DKD was only absent in 66.7%. Patients submitted to KB for the criteria of low or rapid declining eGFR had significantly more NDKD (p = 0.016). DKD with or without NDKD was found in patients with higher levels of albumin to creatinine ratio (p = 0.001) and HbA1c (p = 0.05). Conclusion Our data showed that NDKD is prevalent in T2DM patients, and given its potentially treatable nature, KB should be considered in T2DM patients, especially in those with low or rapid declining eGFR. DR supports the diagnosis of DKD (PPV of 87%), but alone is insufficient to exclude other causes. Patients with DKD had higher levels of albuminuria and HbA1c. Overall, more than half of the patients had hematuria, without any correlation with any group.
Hemodialysis central venous catheter (CVC) insertion can be complicated in patients with anomalous vessel anatomy. In such cases detailed knowledge of thoracic vessel anatomy is necessary to identify the exact location of the catheter. Central venous placement under ultrasound control has significantly reduced the complications associated with blind puncture and allows an appropriate puncture of the desired vessel, but the CVC can still get misplaced if it follows an anomalous route. Herein, we report a case of dialysis catheter placed into a left sided superior vena cava, only diagnosed after CT scan study.
Background and Aims Chronic volume overload is a major contributor to cardiovascular mortality in patients receiving maintenance hemodialysis (HD). Hence, accurately assessing volume status in this population is crucial. Bioelectrical impedance analysis (BIA) is a validated, non-invasive, and straightforward bedside technique that estimates total body water (TBW), extracellular water (ECW), intracellular water (ICW), and over hydration (OH). However, it is not suitable for use in patients with limb amputation or metallic medical devices. On the other hand, lung ultrasound (LUS) is used to evaluate extravascular lung water (EVLW) and has been shown to predict all-cause mortality and cardiac events in HD patients. Although a 28-zone LUS is the reference standard for LUS studies, recent studies have shown that an 8-zone LUS protocol can be as accurate. The aim of this study was to compare the performance of an 8-zone LUS protocol with bioelectrical impedance analysis to evaluate hydration status in an outpatient haemodialysis unit. Method Adult patients under thrice-weekly 4h HD sessions using high-flux membrane dialyzers for at least 1 month were prospectively enrolled between June and August 2022. We excluded patients with systemic infections, advanced neoplasia, metallic medical devices, decompensated cirrhosis, and limb amputations. The dry weight (DW) was established by the attending nephrologist, blind to the results. All measurements were performed in the first session of the week. BIA was assessed before HD using a portable whole body BIA device (BCM—Fresenius Medical Care D GmbH) and the OH was normalized to ECW. LUS using 8-zone protocol was performed before and after HD, with patients in the near-to-supine or supine position using a 2–5 MHz convex probe (Acuson X150®, Siemens, Germany), and the total number of B lines was recorded. Results A total of 37 patients with median age of 60 (IQR 15) years, 75% males, 65% with residual diuresis >300 mL/24 h were included. The median HD vintage was 9 (IQR 10) months, and median body mass index (BMI) of 22.7 (IQR 9) Kg/m2. At the beginning of HD, the median systolic blood pressure (BP) was 140 (IQR 24) mmHg and the median diastolic BP was 79 (IQR 16) mmHg. The median interdialytic weight gain was 4.2% (IQR 2.8) and median ultrafiltration (UF) was 2600 (IQR 1300) mL. The BIA showed median TBW of 38.2 (IQR 11) L, ECW 18.2 (IQR 7.73) L, ICW 20.8 (IQR 6.55) L, and normalized OH 0.138 (IQR 0.147). There was a positive correlation between BMI, TBW (r = 0.375, p = 0.045), and ECW (r = 0.486, p = 0.006), but not with ICW (r = 0.061, p = 0.755), nor normalized OH (r = 0.14, p = 0.468). Systolic BP, but not diastolic, was correlated with the water volume measured in both compartments (ECW: r = 0.498, p = 0.005; ICW: r = 0.421, p = 0.023, TBW: r = 0.423, p = 0.022). The 8-zone LUS showed a statistically significative (p<0.001) reduction in the number of B lines between pre and post HD evaluation (16.5 (IQR 17.25) lines to 8.5 (IQR 10.25) lines). Total evaluation time was under 8 minutes. When comparing LUS before HD with BIA assessment we found that the number of B lines correlated with the normalized OH (r = 0.454, p = 0.018), ECW (r = 0.501, p = 0.007), TBW (r = 0.418, p = 0.030), but not ICW (r = 0.239, p = 0.23). The total number of B lines post HD was also correlated with the ECW (r = 0.568, p = 0.002), TBW (r = 0.465, p = 0.017), and normalized OH (r = 0.408, p = 0.039). Conclusion The 8-zone LUS protocol provides a quick and efficient way to evaluate patients prior to HD sessions. Our study reveals a strong correlation between the total number of B lines determined by the 8-zone protocol and BIA parameters such as TBW, ECW, and normalized OH. This demonstrates that the 8-zone protocol can effectively be used in routine evaluations of HD patients. Although these techniques reflect different over-fluid compartments, they have a complementary role for fluid overload determination and dry weight guidance.
Background and Aims Focal and segmental glomerulosclerosis (FSGS) is a histological pattern that is defined by the presence of segmental sclerosis of at least one glomerulus in the kidney biopsy. However, the etiological classification of this lesion remains challenging in clinical practice. FSGS can be divided into primary/idiopathic, genetic or secondary. Nowadays here is no biochemical marker that allows clinicians to distinguish between these categories nor a pathognomonic feature associated to a particular subtype of FSGS. Also, genetic testing is not suitable for all patients. Although it is crucial to determine the cause of FSGS, this is not easily done in clinical practice. Herein we present a series of patients with histologic diagnosis of FSGS in which clinical and histological criteria were applied in order to establish the etiologic classification of FSGS. Method A retrospective analysis of 359 patients who performed kidney biopsy from January 2010 to December 2018 was performed. Patients with histological features of FSGS were identified. Clinical, laboratorial and pathological data were collected, including treatment and outcome. Patients were classified as having genetic FSGS if they accomplished at least one of the following: a) nephrotic syndrome resistant to corticosteroids; b) non-nephrotic proteinuria or nephrotic syndrome with normal serum albumin; c) non-nephrotic proteinuria or nephrotic syndrome with focal foot process effacement or d) non-nephrotic proteinuria with diffuse foot process effacement. The remaining patients were classified as secondary FSGS (if they presented a disease known to be associated with FSGS) or primary FSGS. Each group was divided according to treatment with corticoid, other immunosuppressive agents or and RAAS blockage. Renal outcomes were assessed (progression to ESKD). Results A total of 359 kidney biopsies were performed between January 2010 to December 2018, 66 with a histological pattern of FSGS. 65% (N=43) were males, 71% (N=47) were Caucasian and 23% (N=15) were black. 74% (N=49) had past medical history of hypertension, 26% (N=17) diabetes mellitus and 5% (N=3) had HIV. The majority of patients were classified as having secondary FSGS (52%, N=34), 23% (N=15) were classified as primary and as 25% (N=17) genetic/idiopathic. From the genetic group 58.8% (N=10) were treated with corticoid, 12% (N=2) with other IS agents and 71% (N=12) with ACE/ARB. 29% (N=5) evolved to ESKD (median time to dialysis 11 IQR 1-30). In the primary FSGS group 40% (N=6) were treated with corticoid, 7% (N=1) with other IS agents and 80% (N=12) with ACE/ARB. 27% (N=4) evolved to ESKD (median time to dialysis 0 months, IQR 0-33). In the genetic group 59% (N=10) were treated with corticoid, 12% (N=2) with other IS agents and 71%(N=12) with ACE/ARB. 29% (N=5) evolved to ESKD (median time to dialysis 11 months, IQR 1-30). Conclusion FSGS is a very prevalent glomerular disease and its correct etiologic classification aids in better treatment choice. Although its etiology is not straightforward in most of the patients, some clinical and histological characteristics aid in FSGS classification.
Background and Aims IgA nephropathy (IgAN) is one of the most prevalent glomerulopathies worldwide with broad variable clinical presentation and extremely heterogeneous risk of progressive CKD and ESKD. It was recommended risk stratifying patients in order to target immunosuppressive treatment to high-risk patients. The OXFORD MEST classification and, more recently, a new international risk-prediction tool for IgAN (IgANPT) help to predict kidney outcomes and stratify patient risk, eventually aiding in treatment decision. Here, we analyzed a single center cohort of IgAN to investigate if treatment decisions were accurately accomplished using individualized risk from the IgANPT. Method A retrospective analysis of all kidney biopsies performed from January 2010 to December 2019 in a Nephrology Department was performed and adult patients with IgAN diagnosis were selected. The presence of IgA vasculitis and Henoch-Schönlein purpura as well as lack of follow-up data were exclusion criteria. Clinical, laboratorial and pathological data were collected, including treatment and kidney outcome. Risk of kidney progression decline was assessed by using the on-line web-based calculator of the IgANPT. Results A total of 33 patients met the study criteria, with median age at diagnosis of 58 (IQR 38 - 68) years-old, mostly male (84,8%, N=28) and all Caucasian. At biopsy time 60,6% (N=20) had hypertension, only 15,5% (N=5) diabetes mellitus and 57,6% (N=19) were under ACE inhibitors/ARBs. Median GFR at biopsy time was 57 (IQR 20 - 78,5) ml/min/1.73m2. More than half of the patients (57,6%, N=19) had GFR <60ml/min/1.73m2. All patients had proteinuria (UACR > 300mg/g), with 18,2% (N=6) in the nephrotic range. Hematuria was present in almost all patients. Overall MEST classification was: 51,5% (N= 17) with M1 score, 39,4% (N=13) E1, 66,7% (N=22) S1 and 45,5% (N=15) had T ≥1 score (2 patients with T2). Crescents were present in 27,3% of the biopsies (N=9). Near one-half of the patients (45,5%, N=15) presented IgA deposits on both mesangium and capillary wall. Around one-third of patients (30,3%, N=10) progressed to ESKD during follow up. According to the risk of kidney disease progression in 60-months calculated with IgANPT, patients were stratified into low/intermediate risk in 21,2% (N=7) of cases (mean predicted risk < 4,7%) and high risk in 78,8% (N=26) of cases (mean predicted risk > 4,7%). Using the chi-squared test the high-risk group was associated with progression to ESKD(p=0,019). On the contrary none of the low-risk group progressed to ESKD. The majority of patients received supportive treatment (87,9%, N= 29), mainly with ACEi/ARB (N=31) and omega (15,2%, N=5). Immunosuppressive treatment included mainly corticosteroids (51,5%, N=17), with other options being (12,1% N=4): cyclophosphamide (N=2), ciclosporin (N=1) and MMF (N=1). From the group of low/intermediate risk patients, only two (28,5%) received immunosuppressive treatment with either oral or intravenous corticosteroids. On the other hand, from the high-risk group, more than one third of patients (38,4%, N=10) did not receive immunosuppressive treatment. Conclusion A better prediction of kidney outcomes and consequent better patient selection for IS therapy may be challenging in IgA nephropathy. In spite of the existent tools, it is still difficult to determine which patients benefit from immunosuppressive treatment. In this case series we stratified our patients by using the IgANPT identifying who was at higher risk of progression to ESKD, in whom, a more aggressive treatment could have potentially benefit avoiding kidney disease progression. It was also important to stratify patients at low-risk, in whom immunosuppressive treatment could carry risk of adverse events with no additional advantage in kidney outcomes.
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