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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 m 2 . 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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Barbosa, Neves, Mohrbacher, Abdo, Cavalcante, Menezes, Sato, Oliveira, Pereira, Bales, Frediani, Chocair and Cuvello-Neto.)

Competing Interests: Declaration of interests We declare no competing interests.

Purpose: The purpose of this study is to evaluate the safety and efficacy of the mechanical thrombectomy with the Indigo System in the treatment of thrombosed arteriovenous fistulas and grafts., Methods: A retrospective search of endovascular procedures performed from November 2018 to June 2020 was conducted. Inclusion criteria were: acute arteriovenous fistula or graft thrombosis that underwent endovascular mechanical thrombectomy with Indigo System. The following information was collected from each case: sex, age, fistula modality, fistula location, treatment modality, and outcomes. Endpoints evaluated were: technical and clinical success rates; primary, assisted primary, and secondary patency rates; complication rates., Results: Twenty-six mechanical thrombectomy procedures for declotting of arteriovenous fistula thrombosis, using the Indigo System, were performed in 22 patients. Technical and clinical success was achieved in 23/26 cases (88%). Mean follow-up was 9 months (range 11-539 days). The 6-month primary, primary assisted, and secondary patency rates were 71%, 86%, 93% and the 12-month primary, primary assisted, and secondary patency rates were 71%, 72%, 80%, respectively. No technical or device-related complications were observed during thrombectomy, however two venous ruptures occurred on the angioplasty of the underlying stenosis., Conclusion: In conclusion, vacuum-assisted thrombectomy of acutely thrombosed arteriovenous fistulas and grafts with Indigo System is safe and effective, providing good short term patency rates., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Primary Effusion Lymphoma is an extremely rare and aggressive subtype of B-cell lymphoma, accounting for only <1% of all cases of this neoplasm. It has a unique clinical presentation because it has a predilection for appearing in body cavities, such as the pleural space, pericardium and peritoneum. It mainly affects immunocompromised individuals and may also affect individuals in the Mediterranean region and in areas endemic for human herpesvirus 8 (HHV-8). Herein, we report the case of an 83-year-old immunocompetent male complaining of coughing, fever and progressive dyspnea for 3 days. His past medical history revealed a recurrent pleural effusion for the last three years, as well as losing weight and malaise. A subsequent investigation revealed a PEL diagnosis of the pleura.

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.

Background: Insulin resistance and/or hyperinsulinemia are closely linked to adiposity, metabolic syndrome (MetS) and prolonged inflammatory processes., Methods: We 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., Results: The 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., Conclusion: It 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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Chocair, de Menezes Neves, Sato, Mohrbacher, Oliveira, Pereira, Bales, da Silva, Duley and Cuvello-Neto.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Chocair, Neves, Mohrbacher, Neto, Sato, Oliveira, Barbosa, Bales, Silva, Cuvello-Neto and Duley.)

Peritoneal equilibration test (PET) is the gold standard for evaluating peritoneal transport, and measurement of the drain volume after 4-h dwell time with glucose 4.25% is a simple means of evaluating failure of ultrafiltration. The study objective was to verify if the measurement of the volume drained after 4 h dwell of icodextrin at 7.5% (ICO), has a better correlation with the parameters of PET. Patients in a peritoneal dialysis program (N = 35) underwent three procedures: PET; determination of the drain volume after a 4-h dwell with glucose 4.25%; and determination of the drain volume after a 4-h dwell with ICO. Among patients who were classified as high transporters, the ultrafiltration volume was greater after ICO use. The ICO ultrafiltration volume correlated negatively with the ratio between the 4- and 0-h dialysate glucose concentrations (D4/D0 ratio, r = -0.579; P = 0.002), correlating positively with the dialysate-to-plasma ratio for creatinine (D/PCr ratio, r = 0.474; P = 0.002). For ICO, the area under the receiver operating characteristic curve was 0.867 and 0.792 for the D/PCr and D4/D0 ratios (P < 0.0001 and P = 0.004, respectively), compared with 0.738 and 0.710 for glucose 4.25% (P = 0.020 and P = 0.041, respectively). A cut-off volume of 141 mL discriminated high/high-average transporters from low/low-average transporters. Volume drained after ICO use better predicts peritoneal transport patterns than does that drained after the use of glucose 4.25%., (© 2021 International Society for Apheresis, Japanese Society for Apheresis, and Japanese Society for Dialysis Therapy.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Neves, Sato, Mohrbacher, Ferreira, Oliveira, Pereira, Bales, Nardotto, Ferreira, Machado, Bassi, Silva-Júnior, Chocair and Cuvello-Neto.)

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.

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., Learning Points: We describe a case of COVID-19 and cytomegalovirus (CMV) co-infection in a critically ill patient.Clinical symptoms simulated mesenteric vascular involvement and intestinal obstruction.The successful management of invasive CMV colitis in a patient with COVID-19 with atypical symptoms is described., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2020.)