Articles

Objective: We set up a U.S. registry to examine prescription patterns and patient outcomes of repository corticotropin injection (Acthar® Gel) for childhood nephrotic syndrome. Methods: 18 participating U.S. pediatric centers performed retrospective review and prospective observation of patients < 21 years old with nephrotic syndrome treated with Acthar Gel. We captured baseline characteristics, drug regimen and duration, and disease response following treatment. Results: 46 patients, enrolled from 2015 to 2020 were included. 27 (58.7%) were male. 18 patients (39.1%) had a diagnosis of minimal change followed by focal segmental glomerulosclerosis in 16 patients (34.7%). Median age at start of treatment was 12.5 years (IQR 8.5-17.4) compared to 5.3 years at diagnosis (IQR 2.7-10.5 years). 52% were resistant to corticosteroids. The most common Acthar Gel regimen was 80IU twice a week with a median duration of 199 days (IQR 88-365). Among 37 patients with active disease, 18 (49%) were able to achieve partial or complete remission, though all patients that had a positive response were on other immunosuppressants concomitantly. Conclusion: We report the findings of the largest registry cohort of pediatric patients in the U.S. treated with Acthar Gel for clinically challenging cases of nephrotic syndrome. Acthar Gel was successful in inducing remission in approximately half of the patients with active disease at time of treatment. No predictors of response with respect to demographic data, age at start of Acthar Gel therapy, etiology of nephrotic syndrome, presence or absence of comorbidities, or steroid responsiveness was noted.

Background and objectives: An arteriovenous fistula is considered to be an ideal vascular access for patients receiving hemodialysis, its main limitation is its high failure rate to achieve maturation and long-term functionality loss. Multiple strategies have attempted to identify patients at risk. Bioelectrical impedance has shown to be a valuable resource in the determination of the hydration status, and the measurement of the phase angle through this method has demonstrated to be a good indicator of the nutritional state and its related as a general marker of survival. The objective of this study is to analyze the role of plasma albumin and phase angle measured through bioelectrical impedance as tools useful for predicting failure of arteriovenous fistulas. Materials and methods: prospective observational study, including 104 patients with chronic kidney disease receiving hemodialysis who underwent a native arteriovenous fistula during a period of 24 months. Analyzing its clinical characteristics, laboratory and phase angle through electrical bioimpedance, both univariate and multivariate analysis was performed both with logistic regression, furthermore calculation of coordinates and ROC curve to establish a better cut-off point. Results: of the variables that were analyzed only the phase angle measurement showed statistical significance OR 2.61 (1.6 – 4.4) p - value 0.001 for predicting arteriovenous fistula failure. In female patients with a phase angle value of 3.25 had a 90% sensibility and 53% specificity for male patients a value of 3.58 showed a sensibility of 84% and a specificity of 52% with ROC curve of 0.78. Conclusion: phase angle through bioimpedance is a useful parameter, helpful in predicting failure of native arteriovenous fistula, it is also an important tool for identifying patients at risk, in early stages prior to the construction of a vascular access. 

Introduction: Determination of dry weight is one of the daily goals to achieve in hemodialysis. The aim of this study was to validate the use of bioelectrical impedance analysis (BIA) in estimation of dry weight in a population of Senegalese chronic hemodialysis patients. Patients and methods: A 9-week cross-sectional study was carried out at the hemodialysis unit of Aristide Le Dantec University Hospital. Adult patients with no previous hospital history were included. The total body water (TBW) was measured with a single frequency bioelectric impedance foot-to-foot analyzer, before and after six successive hemodialysis sessions. These results were compared with those from clinical measurements with the Watson equation using a Student’s t-test and Bland-Altman analysis. Results: 264 measurements were made in 22 patients (46.6 years, 54.5% men, 92.3 months on dialysis, 62.7 kg mean dry weight). A significant reduction in weight (ΔWeight = 2.0 ± 1.1 kg; p < 0.0001) and in TBW measured by the BIA (ΔTBWBIA = 3.3 ± 1.0 liters; p < 0.0001)) or calculated by Watson’s equation (ΔTBWWatson = 0.5 ± 0.2 liter; p = 0.0001) was observed. There was a strong linear correlation and agreement between the 2 TBW measurements in pre-dialysis. In post-dialysis the concordance diagram indicated a bias = –2.2 and wide agreement limits. Conclusion: The BIA allows reproducible and reliable measurements and a fair estimate of the TBW in pre-dialysis.

Atheroembolic disease (AED), or Cholesterol Crystals Embolism, is a systemic disease presented as a complication of severe atherosclerosis [1], where older age, male sex, diabetes hypercholesterolemia, smoking and hypertension [2], are the main risk factors for the development of Atherosclerosis, it is known that spontaneous atherosclerotic renal disease is rare in the absence of any vascular intervention [3], and in the absence of anticoagulant [4], or the absence of calcified aorta, with the most common presentation of the disease is subacute kidney injury progress into renal dysfunction occurs in like a staircase pattern and the renal dysfunction is usually observed several weeks after a possible intervention, caused by dislodging the micro cholesterol plaques from a major artery, and start showering multiple organs causing micro and macro embolic phenomena. In our case, we report acute kidney injury on a previously stable kidney disease in a female with diabetes mellitus type 2 presented with severe anemia, dyspnea, massive fluid overload with bilateral pleural effusion, patient had a history of multiple IV contrast exposures, with peripheral vascular occlusive disease (PVOD), required amputation of right below the knee amputation, presented during the COVID-19 pandemic, found with nephrotic syndrome, a kidney biopsy has shown cholesterol crystal embolization compatible with Athero-embolic Disease with severe Diabetic Nephropathy.

In Portugal, around 2500 patients with end-stage chronic kidney disease (CKD stage 5) start a renal replacement therapy (RRT) for the first time each year [1]. They have four main treatment options: kidney transplantation (TX); haemodialysis (HD); peritoneal dialysis (PD) and conservative treatment (CT). RRT selection is quite complex due to the specificities of each option and to their profound effect on patient’s quality of life. Patients must play a decisive role in the choice of treatment modality and select the option that best suits to their values and needs.

Background: Acute kidney injury (AKI) is a major health problem affecting millions of people worldwide. Effective preventative and therapeutic treatments remain to be produced. We aim to determine the association between blood glucose and mortality in critical patients with AKI. Method: This cohort study included 18,703 patients with AKI. The exposure of interest was baseline blood glucose. The outcome was 30-day mortality. Multivariable Cox regression analyses and smooth curve fitting were adopted to assess the independent association between blood glucose and 30-day mortality. Results: We identified 18,703 consecutive individuals with AKI. The average age of the participants was 66.8 ± 16.0 years, and about 42.7% of them were female. The overall 30-day mortality was 16.9%. Through the multivariate COX regression model and smooth curve fitting, we observed that the correlation between blood glucose and 30-day mortality is nonlinear. An inflection point was found at about 5.93 mmol/L. On the left side of inflection point, the effect size was 0.81 (HR: 0.81, 95% CI 0.74-0.89, p < 0.001). On the right side of inflection point, the effect size was 1.02 (HR: 1.02,95% CI 1.01-1.03, p < 0.001). Conclusion: Our study suggested that, among patients with AKI, there was a nonlinearity relationship between blood glucose and mortality in patients with AKI. The optimal of blood glucose associated with the lowest risk of 30-day mortality was around 5.93 mmol/L. 

Background: Lupus Nephritis (LN) occurs in approximately half of all patients with Systemic Lupus Erythematosus (SLE) and it is the most common cause of morbidity and mortality in patients with SLE. Factors associated with poor renal outcome vary among studies, and researches coming from Brazil are scarce. Objectives: To identify the prognostic factors associated to the development of Chronic Kidney Disease (CKD) and End Stage Renal Disease (ESRD) in LN patients followed in a tertiary hospital. Design and Settings: We conducted a retrospective cohort study set in a tertiary hospital in Fortaleza, Ceará, Brazil. Methods: We compiled a total of 214 LN patients diagnosed between 1983 and 2015. Data was collected from medical records and further analyzed using logistic regression. Results: LN prevalence was 53.9%. The cohort had a mean follow-up of 11.2 years (SD ± 7.2 years). At the end of follow-up, 93 of 197 patients (47.2%) had CKD, and 49 of 191 (25.6%) were on regular dialysis. The main factors associated for developing CKD after logistic regression analysis were the following predictors: hypertension (HR 2.80; 95% CI 1.30-6.01; p = 0.008), time between diagnosis of SLE and diagnosis of LN (HR 0.98; 95% CI 0.97-0.99; p = 0.009) and discontinuation of medications (HR 2.41; 95% CI 1.08-5.37; p = 0.03). Conclusion: Hypertension, discontinuation of medications, and time between diagnosis of SLE and diagnosis of LN are independent variables associated with the development of CKD and ESDR in our study. 

Diffuse alveolar hemorrhage (DAH) is a rare complication of systemic lupus erythematosus (SLE) and carries a high mortality. It was first described by Osler in 1904 as the most devastating pulmonary complication of SLE. We describe a case of a 23-year-old girl recently diagnosed with SLE associated by a class III nephritis treated with oral corticoids and mycophenolate mofetil who developed a Diffuse Alveolar Hemorrhage DAH a few days later. The early diagnosis and the aggressive therapy allowed us to have a favorable outcome.

Introduction: Sodium-glucose cotransporter 2 inhibitors such as empagliflozin (EMPA) protect against diabetic kidney disease. Prostaglandin E2 (PGE2) the main renal product of cyclooxygenase-2, inhibits vasopressin (AVP)-water reabsorption in the collecting duct (CD). The novelty of this study is that for the first time, we examined if EMPA affects the renal PGE2/EP receptor system and determined if CD responses to EMPA prevent water loss. Methods: Four groups of adult male mice were studied after 6 weeks of treatment: control (db/m), db/m+EMPA (10 mg/kg/day in chow), type 2 diabetic diabetic/dyslipidemia (db/db), and db/db+EMPA. Tubules were microdissected for quantitative polymerase chain reaction (qPCR) and CD water transport was measured in response to AVP, with or without PGE2. Results: Hyperglycemia and albuminuria were attenuated by EMPA. Renal mRNA expression for COX, PGE synthase, PGE2 (EP) receptor subtypes, CD AVP V2 receptors and aquaporin-2 was elevated in db/db mice, but unchanged by EMPA. Urine PGE2 levels increased in db/db but were unchanged by EMPA. AVP-water reabsorption was comparable in db/m and db/m+EMPA, and equally attenuated to 50% by PGE2. In db/db mice, AVP-water reabsorption was reduced by 50% compared to non-diabetic mice, and this reduction was unaffected by EMPA. In db/db mice, AVP-stimulated water transport was more significantly attenuated with PGE2 (62%), compared to non-diabetic mice, but this attenuation was reduced in response to EMPA, to 28%. Conclusion: In summary, expression of renal PGE2/EP receptors is increased in db/db mice, and this expression is unaffected by EMPA. However, in diabetic CD, PGE2 caused a greater attenuation in AVP-stimulated water reabsorption, and this attenuation is reduced by EMPA. This suggests that EMPA attenuates diabetes-induced excess CD water loss.

Background: Thrombotic microangiopathy (TMA) is a rare and life-threatening complication of prostate carcinoma. Whether plasma exchange has a role in treatment remains a subject of debate. Here we present a case followed by a systematic review of the literature on this subject. Case report: We describe a 69-year old patient presenting with TMA, which was associated with an underlying metastatic prostate carcinoma. We conducted a search of similar cases in literature. Results: Our patient was treated and responded well on plasma exchange. Systematic review of the literature showed 17 additional cases of TMA associated with prostate carcinoma of which eleven were treated with plasma exchange with mostly good response. Conclusion: Based on current data we cannot exclude a potential role for plasma exchange in prostate cancer associated TMA.