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Context.— Point-of-care testing allows rapid analysis and short turnaround times. To the best of our knowledge, the present study assesses, for the first time, clinical, operative, and economic outcomes of point-of-care blood gas analysis in a nephrology department. Objective.— To evaluate the impact after implementing blood gas analysis in the nephrology department, considering clinical (differences in blood gas analysis results, critical results), operative (turnaround time, elapsed time between consecutive blood gas analysis, preanalytical errors), and economic (total cost per process) outcomes. Design.— A total amount of 3195 venous blood gas analyses from 688 patients of the nephrology department before and after point-of-care blood gas analyzer installation were included. Blood gas analysis results obtained by ABL90 FLEX PLUS were acquired from the laboratory information system. Statistical analyses were performed using SAS 9.3 software. Results.— During the point-of-care testing period, there was an increase in blood glucose levels and a decrease in pCO2, lactate, and sodium as well as fewer critical values (especially glucose and lactate). The turnaround time and the mean elapsed time were shorter. By the beginning of this period, the number of preanalytical errors increased; however, no statistically significant differences were found during year-long monitoring. Although there was an increase in the total number of blood gas analysis requests, the total cost per process decreased. Conclusions.— The implementation of a point-of-care blood gas analysis in a nephrology department has a positive impact on clinical, operative, and economic terms of patient care.

Background and Aims Changes on body composition have an impact on the survival of haemodialysis (HD) patients. The aim of the study was to determine the impact of the reduction of physical activity due to COVID19 lockdown on body composition in HD patients. Method Retrospective and observational study including 149 HD patients. Nutritional and Bioimpedance spectroscopy (BIS) data were recorded before and after COVID19 lockdown (mean of 148 ± 20 days between determinations). Results Over the 49 days of COVID19 lockdown, we observed a decrease in normohydrated weight (NHW) of 1.01 ± 3.59 kg mainly secondary to a reduction on total body water (TBW) 0.95 ± 3.78 L (extracellular water 0.45 ± 1.58 L and intracellular water 0.41 ± 2.36 L). There was also a small loss on lean tissue index (LTI) of 0.28 ± 2.42 kg/m2, with an increase of fat tissue index (FTI) 0f 0.02 ± 2.82 kg/m2. Twenty-three patients presented COVID19 infection, of which 21 required admission (median of 10 [4-16] days). Patients who presented COVID19 were older (70.7 ± 12.0 vs 64.9 ± 16.6 years, NS) with higher Charlson index (7.48 ± 2.77 vs 6.33 ± 2.65, p = 0.07). Patients with COVID19 infection presented a greater loss on LTI (-1.18 ± 3.15 bs -0.16 ± 2.30 kg/m2; p = 0.22), FTI (-0.41 ± 3.38 vs 0.06 ± 2.74 kg/m2; p = 0.54); BMI (-1.49 ± 2.14 vs -0.25 ± 0.96 kg/m2; p = < 0.01) and NHW (-4.00 ± 6.33 vs -0.62 ± 2.90 kg; p = < 0.01) compared to patients without COVID19 infection. The length of hospitalization was associated with greater loss of BMI and NHW, resulting, therefore, in overhydration. There also had lower serum phosphorus (3.6 ± 0.8 vs 5.2 ± 0.8 mg/dl; p = 0.01) and serum albumin (3.5 ± 0.4 vs 4.0 ± 0.1 g/dl; p = 0.01). Seven patients died during hospitalization. Deceased patients were older (78.4 ± 6.6 vs 67.4 ± 12.4 years; p = 0.01), presented higher comorbidity (estimated by Charlson index 10.0 [8.0-11.0] vs 6.5 [4.3-8.0]; p = 0.02) and were more overhydrated (3.4 ± 3.6 vs 1.9 ± 1.9; p = 0.34). Although not statistically different, they had lower LTI (10.4 ± 2.1 vs 12.0 ± 3.4 kg/m2; p = 0.18) and lower serum albumin (3.4 ± 0.6 vs 3.9 ± 0.4 g/dl; p = 0.08) compared to survivors. Patients who survived COVID19 infection had longer hospitalization (57% were discharged between twelfth and forty third day; mean hospitalization 14.6 ± 11.5 days). Deceased patients died within the first 12 days of hospitalization (6.8 ± 4.1 days). Conclusion COVID19 lockdown induced a weight reduction on HD patients due to decrease in total body water. COVID19 infection increased this reduction, inducing greater loss on lean and fat tissue composition. Moreover, COVID19 impact on body composition was magnified with the length of hospitalization.

Background and Aims Thin basement membrane nephropathy (TBMN), the most common cause of persistent microhaematuria (mH), is due to mutations in genes codifying alfa-3 and alfa-4 collagen IV chains (COL4A4/COL4A3). Initially considered as a benign condition, subsequent studies have shown that an important number of patients develop proteinuria and CKD. We reported in a previous small study the presence of multicystic kidney disease (MCD) in some TBMD patients. In this study we aimed to evaluate the presence of MCD in a larger cohort of TBMD patients and analyze its association with renal outcomes. Method We collected 50 patients with a diagnosis of TBMD based on the presence of persistent mH (>5 erythocytes per high power field in more than 90% of urinary sediments and radiological examinations to exclude other causes of mH) and at least one first-degree relative with persistent mH. TBMD diagnosis was confirmed by renal biopsy (glomerular basement membrane thickness less than 150nm) in 18 patients and by genetic test (pathogenic mutations in COL4A3/COL4A4) in 6 patients. MCD was diagnosed by the presence of uncountable cysts on renal ultrasonography. Results Mean age at diagnosis was 43.7 years, 34% were males and 18% had hypertension. At baseline, serum creatinine (SCr) was 0.9 mg/dL, proteinuria 0.48 gr/24h and 9 patients (18%) had CKD (estimated glomerular filtration rate -eGFR- lower than Conclusion MCD is frequently observed in TBMD patients and is a risk factor for the progression of CKD.

Background and Aims The evolution of renal disease and its global impairment in patients diagnosed with ANCA-associated vasculitis (AAV) has been related to baseline C3 serum levels in several studies. The objective of this study is to evaluate in patients diagnosed with AAV and renal impairment the association between baseline serum C3 levels and the clinical outcomes. Method Our cohort included 56 patients diagnosed with AAV diagnosed from January 1986 to January 2018 in Hospital La Paz. According to C3 serum level measurements, patients were divided into low and normal concentration groups at onset. Histological, clinical and laboratory data were compared between groups. Results Among the 56 AAV cases, 58.9% were women and 96.5% Caucasian with an age mean and SD of 61.08 ± 15.11 years. Mean follow-up was 10.03 ± 7.02 years. MPO ANCAs were found in 69.8% of the patients and PR3 ANCAs in 22.64%. AAV diagnosis was earlier in PR3 AVV (47.59 ± 13.62 years) compared to MPO AVV (65.66 ± 11.53 years) (p=0.001). At diagnosis, baseline serum C3 was measured in 53 patients (94.6%) being lower in MPO AAV (112.79 ± 33.98) vs PR3 AAV (132.41 ± 20.69) (p=0.023). Low C3 was way more frequent in patients with higher percentage of glomeruli showing >50% of sclerosis (100% vs 32.5%) (p=0.007). Staphylococcus aureus nasal carriers were more frequent in patients with low C3 (27.2% vs 3.1%, p=0.045). 15 patients (27.77%) died during follow-up and patient survival median was 21.75 years (IC 95%: 15.41-28.08). 17 patients (31.48%) reached end-stage renal disease (ESRD) during follow-up and renal survival median was 20.1 years (IC 95%: 19.27-21.05). There were no statistical differences in the levels of baseline serum C3 according to sex, relapses, extrarenal manifestations, treatment, malignancy, cardiovascular events and time to ESRD or death. Conclusion Baseline serum C3 was lower in MPO AV, in Staphylococcus aureus nasal carriers and higher severity histological damage (glomerular sclerosis >50%). Despite previous evidence, in our cohort, time to ESRD and time to death seemed not to be affected.

Defective complement action is a cause of several human glomerular diseases including atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated vasculitis (ANCA), C3 glomerulopathy, IgA nephropathy, immune complex membranoproliferative glomerulonephritis, ischemic reperfusion injury, lupus nephritis, membranous nephropathy, and chronic transplant mediated glomerulopathy. Here we summarize ongoing clinical trials of complement inhibitors in nine glomerular diseases and show which inhibitors are used in trials for these renal disorders (http://clinicaltrials.gov).

C3 glomerulopathy (C3G) is a severe kidney disease, which is caused by defective regulation of the alternative complement pathway. Disease pathogenesis is heterogeneous and is caused by both autoimmune and genetic factors. Here we characterized IgG autoantibodies derived from 33 patients with autoimmune C3 glomerulopathy. Serum antibodies from all 33 patients as well as purified IgGs bound to the in vitro assembled C3-convertase. Noteworthy, two groups of antibodies were identified: group 1 with strong (12 patients) and group 2 with weak binding C3-convertase autoantibodies (22 patients). C3Nef, as evaluated in a standard C3Nef assay, was identified in serum from 19 patients, which included patients from group 1 as well as group 2. The C3-convertase binding profile was independent of C3Nef. Group 1 antibodies, but not the group 2 antibodies stabilized the C3-convertase, and protected the enzyme from dissociation by Factor H. Also, only group 1 antibodies induced C3a release. However, both group 1 and group 2 autoantibodies bound to the C5-convertase and induced C5a generation, which was inhibited by monoclonal anti-C5 antibody Eculizumab in vitro. In summary, group 1 antibodies are composed of C3Nef and C5Nef antibodies and likely over-activate the complement system, as seen in hemolytic assays. Group 2 antibodies show predominantly C5Nef like activities and stabilize the C5 but not the C3-convertase. Altogether, these different profiles not only reveal a heterogeneity of the autoimmune forms of C3G (MPGN), they also show that in diagnosis of C3G not all autoimmune forms are identified and thus more vigorous autoantibody testing should be performed.

Background: Congenital nephrogenic diabetes insipidus (NDI) is a rare condition characterized by severe polyuria, due to the inability of the kidneys to concentrate urine in response to arginine vasopressin (AVP). In the majority of the cases, the disease shows an X‐linked inherited pattern, although an autosomal recessive inheritance was also observed. Methods: We report a patient with a severe NDI diagnosed during the neonatal period. Because the patient was female without a family history of congenital NDI, her disease was thought to exhibit an autosomal recessive form. Results: A full mutation analysis of AVP receptor 2 (AVPR2; MIM#300538) gene showed no mutations. However, direct Sanger sequencing of the aquaporin 2 (AQP2) revealed an apparently homozygous mutation at nucleotide position NM_000486.5:c.374C>T (p.Thr125Met) in exon 2. Further customized multiplex ligation‐dependent probe amplification (MLPA), single‐nucleotide polymorphism (SNP) array analysis, and long‐range polymerase chain reaction (PCR) followed by Sanger sequencing showed a heterozygous exonic deletion comprising exons 2, 3, and partially 4 of AQP2. Conclusion: This is the first case of a compound heterozygote patient with a missense mutation involving NM_000486.5:exon2:c.374C>T (p.Thr125Met) and a gross deletion of at least exons 2, 3, and partially 4 on the AQP2 to present with a severe NDI phenotype, This study was supported in part by grants from the Research Activity Intensification Program (Programa Intensificación Actividad Investigadora) (IdiPAZ and Agencia Laín‐Entralgo/CM) to R.P. ISCIII RETIC REDINREN RD16/0009 FEDER FUNDS, and (Programa Intensificación Actividad Investigadora) (IdiPAZ and FIBHULP) to J.N.

Background: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episo des of fever and polyserositis and by the onset of reactive amyloid-associated amyloidosis. Amyloidosis due to familial Mediterranean fever can lead to end-stage renal disease, culminating in kidney transplantation for some patients. In this study, we report the clinical outcome of two brothers with familial Mediterranean fever who were the inadvertent donor and recipient, respectively, of a kidney. Subsequently, they were diagnosed with renal amyloidosis secondary to familial Mediterranean fever and were successfully treated with anakinra and colchicine. Case presentation: Two brothers with familial Mediterranean fever and renal amyloidosis were the inadvertent donor and recipient, respectively, of a kidney. The recipient had presented recurrent acute febrile episodes of familial Mediterranean fever, developed nephrotic syndrome secondary to amyloidosis and needed bilateral nephrectomy and chronic dialysis. His elder brother, in apparent good health, donated his left kidney to his brother. Immediately after the kidney transplantation, both the donor and recipient presented massive proteinuria, impaired renal function and elevated serum amyloid A levels. Biopsies of the brothers' kidneys showed amyloidosis. Genetic studies thereafter revealed a homozygous variant for the MEFV gene (NM_000243.2.c.2082G > A; p.M694I) in both brothers. At this point, both the donor and recipient were treated with colchicine and anakinra, resulting in improved renal function, decreased proteinuria, undetectable serum amyloid A levels and stable renal function at 62 months of follow-up and no major adverse effects. Conclusions: In familial Mediterranean fever, analyses of the MEFV gene should be performed in potential live kidney donors from a direct family member (either between siblings or between parents and children). In addition, genetic studies are required when consanguinity is suspected between members involved in the living transplant. Finally, anakinra could be a safe adjuvant therapy combined with colchicine for patients with familial Mediterranean fever and amyloidosis, including those with successful kidney transplantation, This study was supported in part by grants from the Research Activity Intensification Program (Programa Intensificación Actividad Investigadora) (IdiPAZ and Agencia Laín-Entralgo/CM) to R.P. ISCIII RETIC REDINREN RD16/0009 FEDER FUNDS