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IntroductionKimura’s disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%–18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD.Case reportA 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m2. Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin–angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels.ConclusionAlthough uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury.

BackgroundInsulin resistance and/or hyperinsulinemia are closely linked to adiposity, metabolic syndrome (MetS) and prolonged inflammatory processes.MethodsWe retrospectively analyzed 1,018 adult individuals with a mean age of 46 years (74% male) and classified them as: Metabolically normal: without any of the five criteria of the International Diabetes Federation (IDF) used for the diagnosis of MetS, plus normal fasting insulin (Men < 8 mU/L, Women < 10 mU/L); Level 1 MetS: with one or two IDF criteria, plus hyperinsulinemia (Men: ≥ 8 mU/L), and Women: ≥ 10 mU/L); Level 2 MetS: with three or more IDF criteria, plus hyperinsulinemia.ResultsThe mean values for fasting insulinemia in metabolically normal individuals was 4.6 ± 1.8 mU/L and 5.6 ± 2.3 mU/L, while their means for the Homeostatic Model Assessment for Insulin Resistance (HOMA-IR) were 1.0 and 1.2 for men and women, respectively. In addition, the mean values for insulin (and HOMA-IR) for individuals with two normal anthropometric parameters (body mass index and waist girth), or two normal anthropometric parameters plus no IDF criteria, were similar to the metabolically normal group. Based on the obtained mean + 2 SD, we established the following insulin (and HOMA-IR) values as diagnostic cut-offs for hyperinsulinemia: Men: ≥ 8 mU/L (≥ 1.5), and Women: ≥ 10 mU/L (≥ 2.0). The mean serum insulin was significantly higher for individuals with Level 1 MetS (approx. 9 mU/L for both genders) compared with metabolically normal individuals, as was the prevalence of hepatic steatosis, which was more evident in men. Thus, the presence of one or two abnormal IDF criteria, combined with hyperinsulinemia and/or raised HOMA-IR, suggests the presence of MetS and insulin resistance. Patients of both genders with Level 2 MetS had higher serum insulin and/or HOMA-IR values than Level 1, as well as a higher prevalence of hypertension and hepatic steatosis, being more pronounced among men. The process was progressive and proportional to the degree of hyperinsulinemia.ConclusionIt is proposed that intervention against MetS progression should be started in individuals with Level 1 MetS, rather than waiting for more criteria for diagnostic confirmation, which this should help to reduce the occurrence of known complications such as type 2 diabetes, atherosclerosis, hypertension, and chronic kidney disease, among others.

Abstract Background McArdle disease is a myopathy caused by mutations in PYGM gene that is characterized by reduced or absent activity of myophosphorylase. Reports of patients with concomitant McArdle disease and diabetes are scarce. We report a case of a patient with a late diagnosis of McArdle disease and we postulate that symptoms may be related to hypoinsulinemia. Case presentation This report describes the evolution of an elderly diabetic patient with confirmed diagnosis of McArdle’s disease based on the absence of myophosphorylase activity in the analysis of muscle biopsy, and a homozygous mutation in the PYGM gene. The variant – Chr11: 64.525 (p. Asn168*fs) has not been previously described. The diagnosis of McArdle disease was confirmed after two episodes of rhabdomyolysis, at 77 and 81 years of age, as the symptoms were, until then, discrete. The “second-wind phenomenon” was not spontaneously reported, but it was confirmed when directly questioned. We postulate that the later episodes of rhabdomyolysis occurred because of a progressive decrease in insulin production with a consequent reduction in the uptake of blood glucose by muscle cells, thus compromising the cellular energy balance. To our knowledge, this is the first report of recurrent rhabdomyolysis in an elderly diabetic patient with genetically proven McArdle disease. Our initial attempt to reduce insulin resistance with metformin and pioglitazone was not effective, possibly because of inadequate insulinemia. However, an improvement was evident after the administration of low doses of intermediate-acting insulin. Conclusions In view of the patient’s clinical evolution, we suggest the use of medication that reduces insulin resistance for patients with McArdle disease and type 2 diabetes, pre-diabetes or even normoglycemic metabolic syndrome.

ABSTRACT We produced this document to bring pertinent information to the practice of nephrology, as regards to the renal involvement with COVID-19, the management of acute kidney injury cases, and practical guidance on the provision of dialysis support.As information on COVID-19 evolves at a pace never before seen in medical science, these recommendations, although based on recent scientific evidence, refer to the present moment. The guidelines may be updated when published data and other relevant information become available.

Abstract Background Physical activity (PA) is typically lower on hemodialysis (HD) days. Albeit intradialytic inactivity is expected, it is unknown whether recovery after HD contributes to low PA. We investigated the impact of HD and post-HD period on granular PA relative to HD timing. Methods We used baseline data from the HDFIT trial conducted from August 2016 to October 2017. Accelerometry measured PA over 1 week in patients who received thrice-weekly high-flux HD (vintage 3 to 24 months), were clinically stable, and had no ambulatory limitations. PA was assessed on HD days (0 to ≤24 h after start HD), first non-HD days (> 24 to ≤48 h after start HD) and second non-HD day (> 48 to ≤72 h after start HD). PA was recorded in blocks/slices: 4 h during HD, 0 to ≤2 h post-HD (30 min slices), and > 2 to ≤20 h post-HD (4.5 h slices). Blocks/slices of PA were captured at concurrent/parallel times on first/second non-HD days compared to HD days. Results Among 195 patients (mean age 53 ± 15 years, 71% male), step counts per 24-h were 3919 ± 2899 on HD days, 5308 ± 3131 on first non-HD days (p

Mycophenolate rapidly substituted azathioprine (AZA) in transplant immunosuppression regimens since the 1990s, when early clinical trials indicated better outcomes, although opposite results were also observed. However, none of these trials used the well-established optimization methods for AZA dosing, namely, thiopurine methyltransferase pharmacogenetics combined with monitoring of the thiopurine metabolites 6-thioguanine nucleotides (6-TGN) and 6-methylmercaptopurine (6-MMP). Resistance to optimize AZA therapy remains today in transplant therapy, despite the fact that thiopurine metabolite testing is being used by other medical disciplines with evident improvement in clinical results. In a previous analysis, we found that active 6-TGN metabolites were not detectable in about 30% of kidney transplant patients under continuous use of apparently adequate azathioprine dosage, which demonstrates the need to monitor these metabolites for therapeutic optimization. Two of four case studies presented here exemplifies this fact. On the other hand, some patients have toxic 6-TGN levels with a theoretically appropriate dose, as seen in the other two case studies in this presentation, constituting one more important reason to monitor the AZA dose administered by its metabolites. This analysis is not intended to prove the superiority of one immunosuppressant over another, but to draw attention to a fact: there are thousands of patients around the world receiving an inadequate dose of azathioprine and, therefore, with inappropriate immunosuppression. This report is also intended to draw attention, to clinicians using thiopurines, that allopurinol co-therapy with AZA is a useful therapeutic pathway for those patients who do not adequately form active thioguanine metabolites.

Primary Effusion Lymphoma is an extremely rare and aggressive subtype of B-cell lymphoma, accounting for only

Introduction: Kimura’s disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%–18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD. Case report: A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m². Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin–angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels. Conclusion: Although uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury. [ABSTRACT FROM AUTHOR]

ABSTRACT Cardiovascular disease is the main cause of death in patients with chronic kidney disease (CKD). Several heart conditions have been associated with CKD, including myocardial and pericardial diseases. This paper describes a case of Dialysis-related constrictive pericarditis in a patient diagnosed with sudden hypotension during a hemodialysis session. A 65-year-old man diagnosed with hypertension, diabetes, obesity, and cirrhosis on hemodialysis for two years complained of symptoms during one of his sessions described as malaise, lipothymia, and confusion. The patient had a record of poor compliance with the prescribed diet and missed dialysis sessions. He was sluggish during the physical examination, and presented hypophonetic heart sounds, a blood pressure of 50/30mmHg, and a prolonged capillary refill time. The patient was referred to the intensive care unit and was started on antibiotics and vasoactive drugs. His workup did not show signs of infection, while electrocardiography showed low QRS-wave voltage. His echocardiogram showed signs consistent with a thickened pericardium without pericardial effusion. Cardiac catheterization showed equalization of diastolic pressures in all heart chambers indicative of constrictive pericarditis. The patient underwent a pericardiectomy. Examination of surgical specimens indicated he had marked fibrosis and areas of dystrophic calcification without evidence of infection, consistent with Dialysis-related constrictive pericarditis. Hypotension for unknown causes must be considered in the differential diagnosis of dialysis patients.

Introduction: The kidney may be affected by coronavirus (COVID-19) in the setting of acute kidney injury (AKI). Data about AKI in intensive care unit (ICU) patients in Latin America are scarce. We aimed to evaluate the risk of AKI, dialysis (HD), and death in ICU COVID-19 patients in a Brazilian center.Methods: Analysis from medical records of COVID-19 patients in a Brazilian center.Results: A total of 95 patients were analyzed. There was male predominance (64.2%), median age: 64.9 years, and previous history of hypertension and diabetes in 51.6 and 27.4%, respectively. AKI was diagnosed in 54 (56.8%) patients, and 32 (59.2%) of them required HD. Mortality rate was 17.9%. AKI patients when compared with no-AKI were more frequently hypertensive/diabetic and more often needed organ support therapies. Workups depicted more anemia, lymphopenia, and higher levels of inflammatory markers and higher mortality. Comparing patients who had undergone death to survivors, they were older, more frequently diabetic, and had worse SAPS3 and SOFA scores and need for organ support therapies, AKI, and HD. Multinomial logistic regression revealed that hypertension (p = 0.018) and mechanical ventilation (p = 0.002) were associated with AKI; hypertension (p = 0.002), mechanical ventilation (p = 0.008), and use of vasopressor (p = 0.027) to HD patients; and age >65 years (p = 0.03) and AKI (p = 0.04) were risk factors for death.Conclusions: AKI was a common complication of ICU COVID-19 patients, and it was more frequent in patients with hypertension and need of organ support therapies. As well as age >65 years, AKI was an independent risk factor for death.

COVID-19 is a severe disease that has reached pandemic status. To the best of our knowledge, we describe the first case of COVID-19 and cytomegalovirus (CMV) co-infection in a critically ill patient. We discuss the challenge of establishing the diagnosis as well as the management of tissue-invasive gastrointestinal CMV infection (TI-GI CMV) simulating vascular involvement and intestinal obstruction in a critically ill patient.

Abstract Background End stage renal disease (ESRD) patients require a renal replacement therapy (RRT) to filter accumulated toxins and remove excess water, which are associated with impaired physical function. Hemodialysis (HD) removes middle-molecular weight (MMW) toxins less efficiently compared to hemodiafiltration (HDF); we hypothesized HDF may improve physical function. We detailed the design and methodology of the HDFIT protocol that is testing whether changing from HD to HDF effects physical activity levels and various outcomes. Methods HDFIT is a prospective, multi-center, unblinded, randomized control trial (RCT) investigating the impact of dialysis modality (HDF verses HD) on objectively measured physical activity levels, self-reported quality of life, and clinical/non-clinical outcomes. Clinically stable patients with HD vintage of 3 to 24 months without any severe limitation ambulation were recruited from sites throughout southern Brazil. Eligible patients were randomized in a 1:1 ratio to either: 1) be treated with high volume online HDF for 6 months, or 2) continue being treated with high-flux HD. This study includes run-in and randomization visits (baseline), 3- and 6-month study visits during the interventional period, and a 12-month observational follow up. The primary outcome is the difference in the change in steps per 24 h on dialysis days from baseline to the 6-month follow up in patients treated with HDF versus HD. Physical activity is being measured over one week at study visits with the ActiGraph (www.actigraphcorp.com). For assessment of peridialytic differences during the dialysis recovery period, we will analyze granular physical activity levels based on the initiation time of HD on dialysis days, or concurrent times on non-dialysis days and the long interdialytic day. Discussion In this manuscript, we provide detailed information about the HDFIT study design and methodology. This trial will provide novel insights into peridialytic profiles of physical activity and various self-reported, clinical and laboratory outcomes in ESRD patients treated by high volume online HDF versus high-flux HD. Ultimately, this investigation will elucidate whether HDF is associated with patients having better vitality and quality of life, and less negative outcomes as compared to HD. Trial registration Registered on ClinicalTrials.gov on 20 April 2016 (NCT02787161).

Background Dialysis patients are typically inactive and their physical activity (PA) decreases over time. Uremic toxicity has been suggested as a potential causal factor of low PA in dialysis patients. Post-dilution high-volume online hemodiafiltration (HDF) provides greater higher molecular weight removal and studies suggest better clinical/patient-reported outcomes compared with hemodialysis (HD). Methods HDFIT was a randomized controlled trial at 13 clinics in Brazil that aimed to investigate the effects of HDF on measured PA (step counts) as a primary outcome. Stable HD patients (vintage 3–24 months) were randomized to receive HDF or high-flux HD. Treatment effect of HDF on the primary outcome from baseline to 3 and 6 months was estimated using a linear mixed-effects model. Results We randomized 195 patients (HDF 97; HD 98) between August 2016 and October 2017. Despite the achievement of a high convective volume in the majority of sessions and a positive impact on solute removal, the treatment effect HDF on the primary outcome was +538 [95% confidence interval (CI) −330 to 1407] steps/24 h after dialysis compared with HD, and was not statistically significant. Despite a lack of statistical significance, the observed size of the treatment effect was modest and driven by steps taken between 1.5 and 24.0 h after dialysis, in particular between 20 and 24 h (+197 steps; 95% CI −95 to 488). Conclusions HDF did not have a statistically significant treatment effect on PA 24 h following dialysis, albeit effect sizes may be clinically meaningful and deserve further investigation. [ABSTRACT FROM AUTHOR]

Aim: Fatigue in haemodialysis (HD) patients can be captured in quality of life questionnaires and by the dialysis recovery time (DRT) question. The associations between fatigue and measured physical activity has not been explored until the present. We tested our hypothesis that the patient perception of chronic and post dialysis fatigue would be associated with lower physical activity. Methods: This study was a cross sectional evaluation of baseline data from HD patients recruited in the HDFIT trial. Vitality scores from the Kidney Disease Quality of Life (KDQOL‐36) and the dialysis recovery time (DRT) question were used as indicators of chronic and post dialysis fatigue, respectively. Granular physical activity was measured by accelerometers as part of the study protocol. Results: Among 176 patients, Vitality score was 63 ± 21 and the DRT was ≤30 minutes in 57% of patients. The mean number of steps was 5288 ± 3540 in 24 hours after HD and 953 ± 617 in the 2‐hour post‐HD period. The multivariable analysis confirmed Vitality scores were associated with physical activity in the 24‐hour post‐HD period. In contrast, DRT was not associated with physical activity captured by the accelerometer in the period immediately (2 hours) after the HD session. Conclusion: Chronic fatigue was negatively associated with step counts, while patient perception of post‐dialysis fatigue was not associated with physical activity. These patterns indicate limitations in interpretation of DRT. Since physical activity is an important component of a healthy life, our results may partially explain the associations between fatigue and poor outcomes in HD patients. SUMMARY AT A GLANCE: This cross‐sectional study evaluates the association between patient perception of chronic and post‐dialysis fatigue and physical activity. Vitally was found to be associated with physical activity, while dialysis recovery time was not shown to be associated with physical activity within the two hours after a haemodialysis session. [ABSTRACT FROM AUTHOR]

Background: Physical activity (PA) is typically lower on hemodialysis (HD) days. Albeit intradialytic inactivity is expected, it is unknown whether recovery after HD contributes to low PA. We investigated the impact of HD and post-HD period on granular PA relative to HD timing.Methods: We used baseline data from the HDFIT trial conducted from August 2016 to October 2017. Accelerometry measured PA over 1 week in patients who received thrice-weekly high-flux HD (vintage 3 to 24 months), were clinically stable, and had no ambulatory limitations. PA was assessed on HD days (0 to ≤24 h after start HD), first non-HD days (> 24 to ≤48 h after start HD) and second non-HD day (> 48 to ≤72 h after start HD). PA was recorded in blocks/slices: 4 h during HD, 0 to ≤2 h post-HD (30 min slices), and > 2 to ≤20 h post-HD (4.5 h slices). Blocks/slices of PA were captured at concurrent/parallel times on first/second non-HD days compared to HD days.Results: Among 195 patients (mean age 53 ± 15 years, 71% male), step counts per 24-h were 3919 ± 2899 on HD days, 5308 ± 3131 on first non-HD days (p < 0.001), and 4926 ± 3413 on second non-HD days (p = 0.032). During concurrent/parallel times to HD on first and second non-HD days, patients took 1308 and 1128 more steps (both p < 0.001). Patients took 276 more steps and had highest rates of steps/hour 2-h post-HD versus same times on first non-HD days (all p < 0.05). Consistent findings were observed on second non-HD days.Conclusions: PA was higher within 2-h of HD versus same times on non-HD days. Lower PA on HD days was attributable to intradialytic inactivity. The established PA profiles are of importance to the design and development of exercise programs that aim to increase activity during and between HD treatments.Trial Registration: HDFIT was prospectively registered 20 April 2016 on ClinicalTrials.gov (NCT02787161). [ABSTRACT FROM AUTHOR]