Plasmapheresis combined with rituximab treatment of a case of thrombotic thrombocytopenic purpura with Sjögren syndrome and renal impairment: A case report.

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by reduced activity of the von Willebrand factor-cleaving protease (ADAMTS13), which can be life-threatening. The patient reported in this case study also had concurrent Sjögren syndrome and renal impairment, presenting multiple symptoms and posing a great challenge in treatment. PATIENT CONCERNS: A 25-year-old woman in the postpartum period visited the hospital due to indifference in consciousness for more than 1 day following cesarean section 8 days prior. DIAGNOSIS: Notable decreases were observed in platelets, hemoglobin, creatinine, and ADAMTS13 levels. After a consultative examination by an ophthalmologist, she was diagnosed with retinal hemorrhage in the right eye and dry eye syndrome in both eyes. INTERVENTIONS: Having been diagnosed with TTP with Sjögren syndrome and renal impairment, she received repeated treatments with plasmapheresis combined with rituximab. OUTCOMES: Following treatment and during the follow-up period, the patient's platelet counts and bleeding symptoms significantly improved. LESSONS: TTP has a high mortality rate, and when combined with Sjögren syndrome and renal impairment, it poses an even greater challenge in treatment. However, after administering standard plasmapheresis combined with rituximab treatment, the treatment outcome is favorable.

Exploring pathogenesis and biomarkers through establishment of a rat model of male infertility with liver depression and kidney deficiency.

OBJECTIVES: To establish a rat model that accurately replicates the clinical characteristics of male infertility (MI) with Liver Depression and Kidney Deficiency (LD & KD) and investigate the pathogenesis. METHODS: After subjecting the rats to chronic restraint stress (CRS) and adenine treatment, a series of tests were conducted, including ethological assessments, evaluations of reproductive characteristics, measurements of biochemical parameters, histopathological examinations, and analyses of urinary metabolites. Additionally, bioinformatics predictions were performed for comprehensive analysis. RESULTS: Compared to the control, the model exhibited significant manifestations of MI with LD & KD, including reduced responsiveness, diminished frequency of capturing estrous female rats, and absence of mounting behavior. Additionally, the kidney coefficient increased markedly, while the coefficients of the testis and epididymis decreased significantly. Sperm counts and viabilities decreased notably, accompanied by an increase in sperm abnormalities. Dysregulation of reproductive hormone levels in the serum was observed, accompanied by an upregulation of proinflammatory cytokines expressions in the liver and kidney, as well as exacerbated oxidative stress in the penile corpus cavernosum and testis. The seminiferous tubules in the testis exhibited a loose arrangement, loss of germ cells, and infiltration of inflammatory cells. Furthermore, utilizing urinary metabolomics and bioinformatics analysis, 5 key biomarkers and 2 crucial targets most closely linked to MI were revealed. CONCLUSION: The study successfully established a clinically relevant animal model of MI with LD & KD. It elucidates the pathogenesis of the condition, identifies key biomarkers and targets, and provides a robust scientific foundation for the prediction, diagnosis, and treatment of MI with LD & KD.

Risk factors and outcomes of bloodstream infection from a urinary source in kidney transplant recipients.

BACKGROUND: Bacteriuria is common among kidney transplant recipients (KTR). Risk factors and outcomes associated with bloodstream infection due to a urinary source (BSIU) in KTR are poorly understood. METHODS: This single center case-control study from 2010 to 2022 compared KTR with BSIU to those with bacteria without bloodstream infection (BU). Multivariable logistic regression identified BSIU risk factors, and Cox models assessed its impact on graft failure. RESULTS: Among 3435 patients, who underwent kidney transplantation at Emory Hospital, 757 (22%) developed bacteriuria, among whom 142 (18.8%) were BSIU. Male sex, presence of Escherichia coli, Klebsiella pneumoniae, or Pseudomonas species in urine culture, urethral stricture, neuromuscular bladder disorder, and history of diabetes-induced renal failure were independently associated with increased odds of BSIU (Male sex: aOR 2.29, 95% CI 1.52, 3.47, E. coli: aOR 5.14, 95% CI 3.02, 9.13; K. pneumoniae aOR 3.19, 95% CI 1.65, 6.27, Pseudomonas spp aOR 3.06, 95% CI 1.25, 7.18; urethral stricture: 4.10, 95% CI 1.63, 10.3, neuromuscular bladder disorder aOR 1.98, 95% CI 1.09, 3.53, diabetes: aOR 1.64, 95% CI 1.08, 2.49). BSIU was associated with increased hazard of graft failure (HR 1.52, 95% CI 1.05, 2.20). CONCLUSION: Close monitoring is warranted for male KTR with bacteriuria, those with urine cultures positive for Pseudomonas spp, K. pneumoniae, or E. coli, as well as KTR with a history of diabetes-induced renal failure, urethral stricture, or neuromuscular bladder disorder due to their risk for developing BSIU. Future research should explore strategies to mitigate BSIU risk in these high-risk KTR and reduce the associated risk of long-term graft failure.

Characteristics and clinical outcomes of patients with kidney failure of unknown aetiology from ANZDATA registry.

INTRODUCTION: Kidney failure of unknown aetiology (uESKD) is also heavily location dependent varying between 27% in Egypt to 54% in Aguacalientes, Mexico. There is limited information about the characteristics of people with uESKD in Australia and New Zealand, as well as their clinical outcomes on kidney replacement therapy. METHODS: Data on people commencing kidney replacement therapy 1989-2021 were received from the Australia and New Zealand Dialysis and Transplant (ANZDATA) registry. Primary exposure was cause of kidney failure-uESKD or non-uESKD (known-ESKD). Primary outcome was mortality. Secondary outcome was kidney transplantation. Dialysis and transplant cohorts were analysed separately. Cox Proportional Hazards Regression models were used to evaluate correlations between cause of kidney failure and mortality risk. Subgroup analyses were completed to compare mortality risk in people with uESKD to those with diabetic nephropathy, autosomal dominant polycystic kidney disease (ADPKD), glomerular disease and other kidney diseases. RESULTS: This study included 60,448 people on dialysis and 20,859 transplant recipients. 1-year, 3-year and 5-year mortality rates in people with uESKD on dialysis were 31.6%, 58.7% and 77.2%, respectively. 1-year, 3-year and 5-year mortality rates in transplant recipients with uESKD were 2.8%, 13.8% and 24.0%, respectively. People with uESKD on dialysis had a higher mortality risk compared to those without uESKD on univariable and multivariable analyses (adjusted hazard ratio [AHR] 1.10, 95% CI 1.06-1.16, p<0.001). Transplant recipients with uESKD have a higher mortality risk compared to those without uESKD on univariable and multivariable analyses (AHR 1.17, 95% CI 1.01-1.35, p<0.05). People with uESKD had similar likelihood of kidney transplantation compared to people with known-ESKD. CONCLUSION: People with uESKD on kidney replacement therapy have higher mortality risk compared to people with other kidney diseases. Further studies are required to identify contributing factors to these findings.

Is hemodiafiltration superior to high-flow hemodialysis in reducing all-cause and cardiovascular mortality in kidney failure patients? A meta-analysis of randomized controlled trials.

INTRODUCTION: Hemodiafiltration (HDF) and high-flux hemodialysis (hf-HD) are different methods of kidney replacement therapy (KRT) used for the treatment of kidney failure patients. A debate has raged over the last decade about the survival benefit of patients with the use of HDF compared with hf-HD, but with divergent results from randomized controlled trials. Therefore, this study aimed to perform a meta-analysis to compare HDF and hf-HD regarding all-cause and cardiovascular mortality. METHODS: PubMed and Cochrane databases were searched until July 19, 2023, for randomized clinical trials comparing HDF and hf-HD in patients on maintenance dialysis. A meta-analysis was performed using Stata 16.1, applying fixed or random effect models according to the heterogeneity between studies. FINDINGS: Of the 496 studies found, five met the inclusion criteria. Compared with the hf-HD group, the risk ratio (RR) for all-cause mortality with HDF use was 0.76 (95% CI: 0.67-0.88, I2 = 0%). HDF was associated with lower cardiovascular mortality, although the sensitivity analysis showed that the result differed between scenarios. Subgroup analysis showed lower all-cause mortality among patients without diabetes in the HDF group compared with hf-HD (RR 0.66, 95% CI: 0.51-0.81, I2 = 0%), but not in diabetic patients (RR = 0.89, 95% CI: 0.65-1.12, I2 = 0.0%). A subgroup analysis considering convection volumes was not performed, but the studies with the highest weight in the meta-analysis described convection volume as more than 20 L/session. DISCUSSION: More clinical studies considering critical risk factors, such as advanced age and preexisting cardiovascular disease, are needed to confirm the supremacy of HDF over hf-HD on the survival of patients treated by these two forms of kidney replacement therapy.

Survival of Infants With Severe Congenital Kidney Disease After ECMO and Kidney Support Therapy.

Congenital kidney failure not only affects the homeostatic functions of the kidney, but also affects neonatal respiratory integrity. Until recently, extracorporeal membrane oxygenation (ECMO) support was not used in this population because the need for ECMO clearly established nonviability. Since 2016, 31 neonates have been admitted to the NICU at Children's of Alabama with congenital kidney failure. Five patients were placed on ECMO for severe respiratory distress unresponsive to conventional interventions. We evaluated neonates with congenital kidney failure and pulmonary hypoplasia/hypertension refractory to conventional therapies who received ECMO support within the first 9 postnatal days. We describe the pre and postnatal diagnoses, ECMO course details, dialysis modalities, complications, procedures, and long-term outcomes of these patients. All 5 patients received kidney support therapy by postnatal day 7. Diagnoses included posterior urethral valves, bilateral renal dysplasia, and autosomal recessive polycystic kidney disease. Gestational age ranged from 35.6 to 37.1 weeks. Birth weight ranged from 2740 to 3140 g. Days on ECMO ranged from 4 to 23. Four survived and are living today. Pulmonary hypertension resolved in surviving patients. Three surviving patients require no oxygen support, and 1 patient requires nocturnal oxygen. Three survivors received a kidney transplant, and 1 awaits transplant evaluation. Patients with congenital kidney failure with severe pulmonary hypoplasia/pulmonary hypertension no longer warrant a reflexive assignment of nonviability. Meticulous ECMO, respiratory, nutritional, and kidney support therapies may achieve a favorable long-term outcome. Further investigation of strategies for optimal outcome is needed.

Renal function modifies the association between hemoconcentration and outcomes in hospitalized heart failure patients.

Evidence-based management of decongestion is lacking in hospitalized heart failure (HHF) patients, especially in patients with impaired renal function. Hemoconcentration is an objective measure of decongestion that portends a favorable prognosis and guides management in HHF patients with preserved renal function. We aim to investigate whether it remains a prognosticator in patients with renal impairment, and to refine the identification of subpopulations who will benefit from hemoconcentration-guided therapy. HHF patients admitted to Heart Failure Center of Fuwai Hospital were consecutively included from December 2006 to June 2018. Patient characteristics were depicted. Relationships between in-hospital hemoconcentration, worsening renal function (WRF), and one-year all-cause mortality were investigated in the total population and compared between renal function groups using survival analysis and cubic splines, with a special focus on renal function-based interactions. The association was further validated in sensitivity analyses. Clinically relevant cut-offs and subpopulations were identified by subpopulation treatment effect pattern plots (STEPP) and subgroup analysis. 3661 participants (30.4% with impaired renal function) were included. Hemoconcentration, reflected by an in-hospital increase in hemoglobin, hematocrit, or a relative reduction in estimated plasma volume from baseline to discharge, was predictive of decreased one-year mortality in the total cohort despite its correlation with higher WRF incidence. The prognostic value of hemoconcentration differed in patients with impaired and preserved renal function. Hemoconcentration was related to a favorable prognosis in patients with preserved renal function (HR, 0.69; 95% CI, 0.53-0.90; P = 0.007), especially in young male patients with New York Heart Association functional class III-IV, reduced ejection fraction, and baseline eGFR > 75 mL/min/1.73m2. Contrarily, impaired renal function patients experienced a higher incidence of WRF, and hemoconcentration was no longer related to outcome (HR, 0.90; 95% CI, 0.64-1.26; P = 0.545), with findings consistent in all clinically relevant subgroups. In HHF patients, the prognostic value of hemoconcentration differs by renal function, and the clinical utility of hemoconcentration is contingent on preserved renal function.

Risk and prediction of kidney failure early after liver transplantation.

BACKGROUND: Kidney disease is common after liver transplantation (LT), but postoperative kidney failure is difficult to predict. Current guidelines recommend simultaneous liver-kidney transplantation (SLKT) in patients with pre-LT estimated glomerular filtration rate (eGFR) below 30-40 mL/min, which might be too liberal. The aim of this study was to evaluate the risk of kidney failure after LT. We also assessed the predictive ability of pretransplantation eGFR using various equations. METHODS: This single-center study included patients undergoing primary LT 2006-2020. Patients undergoing simultaneous liver-kidney transplantations or on dialysis before LT were analysed separately. We calculated 5 different eGFR equations measured just before LT and assessed their predictive ability using Kaplan-Meier cumulative incidence estimates. RESULTS: Among 556 LT patients with a median follow-up of 5.0 years (IQR 2.0-8.5), 20 developed kidney failure during follow-up, 7 of them within 1-year post LT. Six of these 7 suffered from major perioperative complications. Depending on the eGFR equation used, the incidence of kidney failure within 1-year was 3.9-6.7% at pre-LT eGFR-values <30 mL/min, 1.2-3.1% at eGFR 30-60 mL/min, and 0.6-0.9% at eGFR >60 mL/min. CONCLUSIONS: Kidney failure within 1-year post-LT could not be reliably predicted by pre-LT eGFR. However, kidney failure was uncommon even in patients with severely reduced pre-LT glomerular filtration rate (eGFR <30 mL/min), and extremely rare in patients unaffected by major perioperative complications. Our data prompts further consideration regarding the guidelines for SLKT in patients with a reduced preoperative eGFR.

Prognosis of patients with renal failure one year following non-ST-segment elevation myocardial infarction treated with percutaneous coronary intervention.

OBJECTIVE: Kidney failure is highly prevalent in patients with non-ST-elevation myocardial infarction (NSTEMI). The aim of the study was to evaluate the prognostic significance of baseline renal function regarding in-hospital and 1-year mortality among patients with NSTEMI and treated with percutaneous coronary intervention (PCI). METHODS: Data were obtained from the Polish Registry of Acute Coronary Syndromes (PL-ACS) and included 47,052 NSTEMI patients treated with PCI between 2017 and 2021. The cumulative incidence of all-cause mortality during the 1-year follow-up was presented using the Kaplan-Meier curves. The multivariable Cox regression model was created to adjust the relationship between eGFR (as a spline term) and all-cause mortality for potential confounders. RESULTS: After considering the exclusion criteria, 20,834 cases were evaluated, with a median eGFR of 72.7 (IQR 56.6-87.5) mL/min/1.73 m2. The median age was 69 (62-76) years. The study comprised 4,505 patients with normal (90-120), 10,189 with mild (60-89), 5,539 with moderate (30-59), and 601 with severe eGFR impairment (15-29). Lower eGFR was associated with worse baseline clinical profile and longer in-hospital delay to coronary angiography. There was a stepwise increase in the crude all-cause death rates across the groups at 1 year. The Cox regression model with a spline term revealed that the relationship between eGFR and the risk of death at 1 year was non-linear (reverse J-shaped), and the risk was the lowest in patients with eGFR∼90 mL/min/1.73 m2. CONCLUSIONS: There is a J-curve relationship between the eGFR value and 1-year all-cause mortality in patients with NSTEMI and treated with PCI.

Propensity score-matched comparison of total arterial off- and on-pump coronary artery bypass with complete revascularization.

Little is known regarding the long-term (> 10 years) outcomes and risk factors of total arterial coronary artery bypass grafting (CABG). This study evaluated the long-term outcomes and risk factors for all-cause mortality and major adverse cardiac and cerebrovascular events (MACCEs) following total arterial on-pump CABG (ONCAB) or off-pump CABG (OPCAB) with complete revascularization. This retrospective cohort analysis enrolled patients with stable angina who underwent total arterial CABG with complete revascularization in our institute between July 2000 and June 2019. The endpoints were all-cause mortality and MACCE incidence, including a comparison between OPCAB and ONCAB. Long-term (10-year) outcomes were analyzed using propensity score-matched pairs, and risk factors were evaluated using univariate and multivariate analyses. Overall, 401 patients who underwent primary total arterial CABG were classified into the OPCAB (n = 269) and ONCAB (n = 132) groups. Using propensity score matching (PSM), 88 patients who underwent OPCAB were matched with 88 patients who underwent ONCAB. The mean follow-up period was 7.9 ± 6.3 years. No significant difference in all-cause mortality (hazard ratio, 1.04; 95% confidence interval, 0.53-2.04; p = 0.9138) and MACCE incidence (hazard ratio, 1.06; 95% confidence interval, 0.68-1.65; p = 0.7901) was observed between the two groups. Renal failure requiring dialysis was a significant risk factor for mortality (p < 0.0001) and MACCEs (p = 0.0003). Long-term outcomes of total arterial OPCAB and ONCAB with complete revascularization showed similar findings using PSM. Renal failure requiring dialysis was a significant risk factor for mortality and morbidity.Journal standard instruction requires an unstructured abstract; hence the headings provided in abstract were deleted. Kindly check and confirm.Thank you for your kindness.Clinical registration number 5598, Tokyo Women's Medical University Hospital.

One-Year Results of a Low-Profile Endograft in Acute, Complicated Type B Aortic Dissection.

BACKGROUND: The safety and effectiveness of the RelayPro endograft (Terumo Aortic) was assessed for the treatment of acute, complicated type B aortic dissection (TBAD). METHODS: A prospective pivotal trial analyzed a primary end point of all-cause mortality at 30 days. Secondary end points included technical success, major adverse events (disabling stroke, renal failure, and paraplegia/paralysis), endoleaks, patency, rupture, device integrity, false lumen perfusion, reinterventions, aortic expansion, and migration evaluated to 5 years. RESULTS: The study involved 22 United States centers and enrolled 56 patients (mean age, 59.5 ± 11.4 years) from 2017 to 2021; of whom, 73.2% were men and 53.6% were African American. TBAD was complicated by malperfusion of the kidneys (51.8%), lower extremities (35.7%), and viscera (33.9%), and rupture (10.7%). Dissection extended proximally to zones 1/2 (14.3%) and zone 3 (78.6%) and distally to the iliac arteries (67.3%). Most procedures were percutaneous (85.5%). Technical success was 100%. Median hospitalization was 7 days (interquartile range, 5-12 days). All-cause mortality at 30 days was 1.8% (1 of 56; upper 95% CI, 8.2%; P < .0001). Seven major adverse events occurred in 6 patients (10.7%), consisting of paraplegia (n = 3), paraparesis (n = 2), disabling stroke (n = 1), and renal failure (n = 1). All paraplegia/paraparesis resolved with lumbar drainage. Kaplan-Meier analysis estimated a freedom from major adverse events of 89.1% at each interval from 30 days to 3 years. There was 1 endoleak (Type Ia), 2 retrograde dissections, and aortic diameter growth occurred in 2. There has been no rupture, fistula, component separation, patency loss, stenosis, kinking, twisting, bird beak, loss of device integrity, or fracture. CONCLUSIONS: RelayPro is safe and effective in acute, complicated TBAD. Follow-up is ongoing to evaluate longer-term outcomes and durability.

Assessing survival post-kidney transplantation in Australia: A multivariable prediction model.

AIM: Kidney transplantation remains the preferred standard of care for patients with kidney failure. Most patients do not access this treatment and wide variations exist in which patients access transplantation. We sought to develop a model to estimate post-kidney transplant survival to inform more accurate comparisons of access to kidney transplantation. METHODS: Development and validation of prediction models using demographic and clinical data from the Australia and New Zealand Dialysis and Transplant Registry. Adult deceased donor kidney only transplant recipients between 2000 and 2020 were included. Cox proportional hazards regression methods were used with a primary outcome of patient survival. Models were evaluated using Harrell's C-statistic for discrimination, and calibration plots, predicted survival probabilities and Akaike Information Criterion for goodness-of-fit. RESULTS: The model development and validation cohorts included 11 302 participants. Most participants were male (62.8%) and Caucasian (79.2%). Glomerulonephritis was the most common cause of kidney disease (45.6%). The final model included recipient, donor, and transplant related variables. The model had good discrimination (C-statistic, 0.72; 95% confidence interval (CI) 0.70-0.74 in the development cohort, 0.70; 95% CI 0.67-0.73 in the validation cohort and 0.72; 95% CI 0.69-0.75 in the temporal cohort) and was well calibrated. CONCLUSION: We developed a statistical model that predicts post-kidney transplant survival in Australian kidney failure patients. This model will aid in assessing the suitability of kidney transplantation for patients with kidney failure. Survival estimates can be used to make more informed comparisons of access to transplantation between units to better measure equity of access to organ transplantation.

Low non-relapse mortality and good haematological and renal responses after autologous haematopoietic stem cell transplantation in multiple myeloma patients with renal insufficiency at transplant: A prospective Société Francophone de Greffe de Moelle-Thérapie Cellulaire observational study.

High-dose melphalan followed by autologous haematopoietic stem cell transplantation is widely used in newly diagnosed multiple myeloma (MM) patients as upfront therapy. However, the safety and efficacy of transplantation in patients with renal insufficiency (RI) are controversial. We followed a multicentre (16 SFGM-TC centres) prospective cohort of 50 newly diagnosed MM patients with a serum creatinine clearance of <40 mL/min at transplantation. Patients received a recommended dose of melphalan of 140 mg/m2. The primary end-point was the non-relapse mortality at Day 100. One death occurred during the first 100 days post-transplant. The median time to neutrophil engraftment was 12 days and to platelet engraftment was 13 days. The haematological response improved in 69% of patients, with best responses from partial response (PR) to very good partial response (VGPR) (10%), from PR to complete response (CR)/stringent complete response (sCR) (16%), from VGPR to CR/sCR (39%) and from CR to sCR (2%). At 2 years, the overall survival was 84%, the progression-free survival was 70% and the cumulative incidence of relapse was 20%. The renal response improved in 59% of patients, with the best renal responses post-transplant being minimal (9%), partial (2%) and complete (48%). Autologous transplantation was safe and effective in myeloma patients with RI at transplant.

Treatment Location Variation for Chronic Limb-Threatening Ischemia in Patients With Kidney Failure.

INTRODUCTION: End-stage kidney disease (ESKD) is an established risk factor for chronic limb-threatening ischemia (CLTI). Procedural location for ESKD patients has not been well described. This study aims to examine variation in index procedural location in ESKD versus non-ESKD patients undergoing peripheral vascular intervention for CLTI and identify preoperative risk factors for tibial interventions. METHODS: Chronic limb-threatening ischemia (CLTI) patients were identified in the Vascular Quality Initiative (VQI) peripheral vascular intervention dataset. Patient demographics and comorbidities were compared between patients with and without ESKD and those undergoing index tibial versus nontibial interventions. A multivariable logistic regression evaluating risk factors for tibial intervention was conducted. RESULTS: A total of 23,480 procedures were performed on CLTI patients with 13.6% (n = 3154) with ESKD. End-stage kidney disease (ESKD) patients were younger (66.56 ± 11.68 versus 71.66 ± 12.09 y old, P = 0.019), more often Black (40.6 versus 18.6%, P < 0.001), male (61.2 versus 56.5%, P < 0.001), and diabetic (81.8 versus 60.0%, P < 0.001) than non-ESKD patients. Patients undergoing index tibial interventions had higher rates of ESKD (19.4 versus 10.6%, P < 0.001) and diabetes (73.4 versus 57.5%, P < 0.001) and lower rates of smoking (49.9 versus 73.0%, P < 0.001) than patients with nontibial interventions. ESKD (odds ratio (OR) 1.67, 95% confidence interval (CI) 1.52-1.86, P < 0.001), Black race (OR 1.19, 95% CI 1.09-1.30, P < 0.001), and diabetes (OR 1.82, 95% CI 1.71-2.00, P < 0.001) were risk factors for tibial intervention. CONCLUSIONS: Patients with ESKD and CLTI have higher rates of diabetes and tibial disease and lower rates of smoking than non-ESKD patients. Tibial disease was associated with ESKD, diabetes, and Black race.

REBOA Improves Outcomes in Hybrid Surgery for Ruptured Abdominal Aortic Aneurysms.

BACKGROUND: Despite advancements in diagnostic methods and emergency interventions, mortality rates of ruptured abdominal aortic aneurysm (rAAA) continue to remain high. To address this issue, the resuscitative endovascular balloon occlusion of the aorta (REBOA) technique has been designed to provide temporary control of bleeding. We aimed to compare the impact of the REBOA technique during open aortic surgery for rAAA. METHODS: Between January 2014 and November 2021, 53 consecutive patients (46 males, 7 females; mean age 71.9 ± 7.9 years; range 51-89 years) who underwent emergency open aortic surgery for rAAA were retrospectively analyzed. Patients were divided into REBOA (21 patients) and non-REBOA (32 patients) groups. The primary outcomes were postoperative 24-hr and 30-day mortality. The secondary outcomes were intensive care unit (ICU) stay, in-hospital stay, bleeding, postoperative renal failure, bowel ischemia, and transient ischemic attack (TIA)/stroke rate. RESULTS: The REBOA group showed a significant reduction in mortality rates at both 24 hr (9.5% vs. 37.5%, P = 0.029) and 30 days (14.2% vs. 43.7%, P = 0.035) compared to the non-REBOA group. In-hospital stay (12.8 ± 3.48 vs. 15.6 ± 4.74 days, P = 0.02) and ICU stay (2.42 ± 2.08 vs. 5.09 ± 5.79 days, P = 0.048) were shorter among the REBOA group. Total procedure time and bleeding were reduced among the REBOA group without significant differences in terms of postoperative renal failure, bowel ischemia, and TIA/stroke rate. CONCLUSIONS: The REBOA group demonstrated significantly improved survival rates compared to the non-REBOA group, without a significant difference in complication rates. REBOA is considered a less invasive option compared to the traditional method for open aortic cross-clamping. This study demonstrated that the use of REBOA may be considered as a first-line treatment option for open surgery in cases of rAAA particularly when an off-the-shelf endovascular aneurysm repair device is not suitable.

Alfaepoetina para o tratamento de pacientes com doença falciforme apresentando declínio da função renal e piora dos níveis de hemoglobina / Epoetin alfa for the treatment of patients with sickle cell disease presenting with declining kidney function and worsening hemoglobin levels

INTRODUÇÃO: A doença falciforme (DF) se refere a um grupo de hemoglobinopatias nas quais a mutação na hemoglobina (Hb) associada à falcização das hemácias é co-herdada com mutações em outras beta globinas. A doença evolui com vaso-oclusão e outras complicações crônicas, graves e multissistêmicas. Uma das principais complicações da DF ­ que ocorre especialmente em pessoas com os genótipos HbSS e HbSbeta0 ­ é a nefropatia falciforme, que pode evoluir desde quadros assintomáticos até doença crônica renal (DRC). Além do tratamento padrão com hidroxiureia e transfusões sanguíneas, diretrizes internacionais recomendam o tratamento com agentes estimulantes da eritropoiese (AEE), como a alfaepoetina para pacientes com comprometimento renal. O uso desse medicamento está consolidado para tratamento da anemia em DRC em pacientes sem DF, estando incorporada ao SUS como pó para solução injetável e em solução injetável para o tratamento desta condição clínica. PERGUNTA: Alfaepoetina em associação ao cuidado-padrão comparada ao cuidado-padrão, é eficaz, efetiva, segura, custo-efetiva e viável economicamente para o tratamento de adultos com DF que apresentam comprometimento renal associado à piora do quadro de anemia? EVIDÊNCIAS CLÍNICAS: Foram incluídos oito estudos observacionais que apresentaram os resultados por meio de análises antes/depois de parâmetros clínicos e hematológicos. O uso de alfaepoetina esteve associado à melhora significativa estatisticamente na concentração de Hb (variação de 4 a 32,8% de aumento nos níveis de Hb comparados aos valores da linha de base, p<0,05), nos níveis percentuais de Hb-F (a diferença variou de 5,2 a 17,1% entre os estudos, p<0,05) e na redução da necessidade de transfusões sanguíneas (resultados quantitativos não reportados). Não houve aumento de crises vaso-oclusivas (CVO) ou tromboembolismo venoso (TEV), sugerindo que o tratamento com alfaepoetina pode ser seguro (resultados quantitativos não reportados). A certeza na evidência pelo GRADE foi muito baixa para todos os desfechos, com preocupações relacionadas ao risco de viés e imprecisão. Ao considerar apenas a evidência para um subgrupo de pacientes (n=4) com comprometimento renal para o desfecho de concentração de Hb (aumento de 29,0%) a certeza na evidência permanece muito baixa, apesar de não haver, neste caso, rebaixamento da qualidade da evidência por evidência indireta. AVALIAÇÃO ECONÔMICA (AE): Foi elaborada uma análise de custo-efetividade/utilidade a partir de modelo de árvore de decisão com horizonte temporal de um ano para avaliar as consequências da alfaepoetina na redução da necessidade de transfusões sanguíneas em termos de custos e ano de vida ajustado por qualidade (QALY). A análise demonstrou que a alfaepoetina + cuidado padrão para o tratamento de adultos com DF apresentando declínio da função renal e piora dos níveis de hemoglobina, quando comparado ao cuidado padrão, apresenta modesto benefício clínico (incremental de 0,033 QALY e redução de custo - R$ 11.564). A alfaepoetina permaneceu como alternativa dominante nas simulações realizadas nas análises de sensibilidade. ANÁLISE DE IMPACTO ORÇAMENTÁRIO (AIO): O tamanho da população elegível foi estimado por demanda aferida combinada à demanda epidemiológica, sendo identificado em média 5.274 pacientes por ano. A taxa de difusão de alfaepoetina variou de 10% a 50% entre primeiro e último anos. O custo direto com aquisição da alfaepoetina variou de R$ 806.129 no primeiro ano a R$ 4.853.242 no quinto ano de incorporação. A AIO construída, atrelada à análise de custoefetividade, sugeriu uma economia de R$ 96.545.791 acumulada em cinco anos. Esta economia é decorrente da efetividade do medicamento em reduzir a necessidade de transfusões frequentes, uma vez que este procedimento está associado a altos custos, como os custos da terapia de quelação de ferro. As análises de sensibilidade reforçaram estes resultados. MONITORAMENTO DO HORIZONTE TECNOLÓGICO: Foram identificadas 2 tecnologias para o potencial tratamento de pessoas com doença falciforme, apresentando declínio da função renal e piora dos níveis de hemoglobina. Crizanlizumabe, anti-P selectina, com registro na Anvisa, EMA e FDA. Voxelotor, registrada na EMA e FDA. PERSPECTIVA DO PACIENTE: Foi aberta Chamada Pública nº 35/2023 para inscrição de participantes para a perspectiva do paciente, durante o período de 21 a 27/09/2023, e 11 pessoas se inscreveram, mas nenhuma atendia aos critérios da chamada. A Secretaria-Executiva da Conitec realizou uma busca ativa junto a especialistas, associações de pacientes e Centros de Tratamento para identificar um usuário do SUS que pudesse fazer o relato na reunião da Conitec. Em sua fala, o participante informou que há 10 anos apresentou elevação nas taxas de potássio, necessitando fazer, na época, uso do medicamento denominado "sorcal" (poliestirenossulfonato de cálcio). Há cerca de cinco anos começou a fazer uso da alfaepoetina. O medicamento, segundo ele, mantém a insuficiência renal controlada sem provocar efeitos adversos. Há algum tempo, entretanto, vem apresentando queda nas taxas de hemoglobina, necessitando receber transfusões de sangue a cada dois meses, procedimento que até então não era rotineiro. Não sabe dizer se o declínio das taxas de hemoglobina se deve ao uso da alfaepoetina. RECOMENDAÇÃO PRELIMINAR DA CONITEC: Os membros do Comitê de Medicamentos presentes na 16ª Reunião Extraordinária da Conitec, realizada no dia 01 de novembro de 2023, deliberaram por unanimidade, que a matéria fosse disponibilizada em consulta pública com recomendação preliminar favorável à incorporação ao SUS da alfaepoetina (rHuePO, eritropoietina humana recombinante) para adultos com doença falciforme, com comprometimento renal associado à piora do quadro de anemia. Discutiu-se a necessidade de estabelecer, no contexto da atualização do PCDT, critérios objetivos de declínio da função renal, queda de hemoglobina e níveis de eritropoetina endógena para compor critérios de uso do medicamento. Sugeriu-se o uso de critérios relativos, como queda de 25% ou mais na TFGe em relação ao valor basal. Ademais, frente às incertezas quanto às evidências de benefício, especialmente no longo prazo, recomendou-se que sejam definidos no PCDT da DF, além dos critérios para uso, critérios de interrupção do tratamento na ausência de benefício clínico. CONSULTA PÚBLICA: A consulta pública nº 56 foi realizada entre os dias 26/12/2023 e 15/01/2024 e recebeu uma contribuição técnico-científica e oito contribuições de experiência e opinião. A contribuição técnico-científica foi emitida pela Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH), que se posicionou favoravelmente à incorporação, destacando que a tecnologia irá impactar positivamente a qualidade de vida dos pacientes, possibilitando a redução das transfusões de hemácias e da quelação de ferro, e, consequentemente, reduzindo custos associados às complicações. Especialistas participantes do processo de atualização do PCDT da DF contribuíram apresentado critérios de uso e interrupção do tratamento, tendo em vista as discussões realizadas durante a reunião de apresentação da demanda. Em relação às contribuições recebidas pelo formulário de experiência e opinião, observou-se que em uma delas havia um anexo, o qual, por se tratar de artigo científico, foi encaminhado para ser incorporado à análise das contribuições de natureza técnico-científica. Todas as contribuições concordaram com a recomendação preliminar da Conitec, que foi favorável à incorporação da tecnologia avaliada. A eficácia da tecnologia, a melhora da qualidade de vida e a importância do acesso por meio do SUS foram mencionados como pontos a favor da incorporação. Como resultados positivos e facilidades da tecnologia em avaliação, foram mencionadas a eficácia do medicamento e a importância de estar disponível no SUS. Quanto aos efeitos negativos, ao lado da consideração da inexistência de efeitos negativos, foi mencionada a dificuldade de acesso ao medicamento. RECOMENDAÇÃO FINAL DA CONITEC: Após apreciação das contribuições recebidas na Consulta Pública, os membros do Comitê de Medicamentos presentes na 126ª Reunião Ordinária da Conitec deliberaram, por unanimidade, recomendar a incorporação da alfaepoetina para o tratamento de pacientes com doença falciforme apresentando declínio da função renal e piora dos níveis de hemoglobina conforme Protocolo Clínico do Ministério da Saúde. Foi assinado o Registro de Deliberação nº 871/2024. DECISÃO: incorporar, no âmbito do Sistema Único de Saúde - SUS, a alfaepoetina para o tratamento de pacientes com doença falciforme apresentando declínio da função renal e piora dos níveis de hemoglobina, conforme Protocolo Clínico do Ministério da Saúde, publicada no Diário Oficial da União, nº 66, seção 1, página 109, em 05 de abril de 2024.

Hemodialysis Access: US for Preprocedural Mapping and Evaluation of Maturity and Access Dysfunction.

Patients with kidney failure require kidney replacement therapy. While renal transplantation remains the treatment of choice for kidney failure, renal replacement therapy with hemodialysis may be required owing to the limited availability and length of time patients may wait for allografts or for patients ineligible for transplant owing to advanced age or comorbidities. The ideal hemodialysis access should provide complication-free dialysis by creating a direct connection between an artery and vein with adequate blood flow that can be reliably and easily accessed percutaneously several times a week. Surgical arteriovenous fistulas and grafts are commonly created for hemodialysis access, with newer techniques that involve the use of minimally invasive endovascular approaches. The emphasis on proactive planning for the placement, protection, and preservation of the next vascular access before the current one fails has increased the use of US for preoperative mapping and monitoring of complications for potential interventions. Preoperative US of the extremity vasculature helps assess anatomic suitability before vascular access creation, increasing the rates of successful maturation. A US mapping protocol ensures reliable measurements and clear communication of anatomic variants that may alter surgical planning. Postoperative imaging helps assess fistula maturation before cannulation for dialysis and evaluates for early and late complications associated with arteriovenous access. Clinical and US findings can suggest developing stenosis that may progress to thrombosis and loss of access function, which can be treated with percutaneous vascular interventions to preserve access patency. Vascular access steal, aneurysms and pseudoaneurysms, and fluid collections are other complications amenable to US evaluation. ©RSNA, 2023 Supplemental material is available for this article. Test Your Knowledge questions for this article are available through the Online Learning Center.

Safety, Effectiveness and Pitfalls of Carbon Dioxide Routine Use as a Contrast Agent for Endovascular Abdominal Aortic Repair.

BACKGROUND: Despite the evidence of good performance, carbon dioxide (CO2) routine employment as a contrast agent for endovascular procedures is far from being adopted with its use currently limited to patients with renal impairment and known allergy to iodinated contrast medium (ICM). The purpose of our study is to evaluate the safety and effectiveness of CO2 guided endovascular abdominal aortic aneurysm repair (EVAR) in a standard population and to assess the rationale for a future widespread use. METHODS: We retrospectively collected data of every patient who underwent CO2 guided standard EVAR from September 2020 to May 2021 and compared them with the data of every patient who underwent EVAR using ICM from December 2019 to August 2020 in our unit. The selection of the contrast medium was not based on any preoperative factor as the contrast medium was routinely used in every patient in both periods. The primary end point of the study was the technical success rate. Secondary end points were the early and late complication rates, radiation exposure and renal function impairment. RESULTS: 49 patients underwent ICM guided EVAR and 52 patients underwent CO2 guided EVAR in our unit in the time frames specified above. The technical success rate was 100% in both groups with no accidental coverage of any target vessel. Intraoperative endoleaks were observed in 14% of ICM patients and 25% of CO2 patients. The radiation exposure was higher in the CO2 group if compared to the ICM group (311.48 vs. 159.86 median mGy/cm2 - P < 0.001). The incidence of postoperative acute kidney injury was low and similar in the 2 groups. No significant worsening over time of the renal function has been reported in both groups. CONCLUSIONS: EVAR can be safely performed under CO2 guidance without the integration of any quantity of ICM but with an increase in radiation exposure. The nephroprotective role of CO2 guided EVAR in a standard population is unclear and the same role in renal impaired patients should be validated with further studies on selected populations.