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Spurious hyperphosphatemia, also known as pseudo-hyperphosphatemia, refers to artifactually elevated serum phosphate values that do not correspond to their actual systemic levels. Vascular access poses a significant challenge for individuals undergoing hemodialysis (HD) due to chronic kidney disease, primarily attributed to the elevated incidence of complications, such as infections or thrombosis associated with catheter use. To mitigate clotting risk during the inter-dialysis intervals, in recent years, a strategy involving the application of concentrated heparin (recombinant tissue plasminogen activator (rt-PA) e.g. alteplase) has been used. These infusions have a very high content of phosphorus, and if it is not removed sufficiently before collecting blood samples through the central venous catheter, it can cause erroneously high phosphate levels. Here we report two cases of pseudo-hyperphosphatemia caused by contaminated blood samples obtained from an HD catheter from the pediatric nephrology department. Absurd values found in routine samples led us to investigate the reason for these results. Further investigations carried out by the laboratory biochemistry department showed that all the analytical checks and proficiency testing performance were within acceptable limits. We hypothesized that there was a pre-analytical error such as possible contamination with the high phosphate content of heparin and alteplase solution in the catheter, contributing to the increased phosphate levels in these samples.

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Purpose: To describe ocular findings in individuals with primary hyperoxaluria type 1 (PH1), focusing on the correlations between retinal anatomy and retinal function. To characterize the retinal alterations that occur at different disease stages by evaluating individuals with diverse degrees of renal impairment associated with PH1. Design: A cross-sectional study. Participants: Patients diagnosed with PH1 based on clinical criteria and genetic testing, treated in the Pediatric Nephrology Unit of the Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel between 2013 and 2021. Methods: The ophthalmological assessment included a slit-lamp biomicroscopy of the anterior and posterior segment or indirect ophthalmoscopy. Electroretinography was employed for assessment of the retinal function, and retinal imaging included spectral-domain OCT and fundus autofluorescence. A systematic evaluation of the disease stage was based on clinical criteria including physical examination, purposeful imaging (X-ray, echocardiography, and US abdomen), and laboratory tests as needed. Main Outcome Measures: Anatomical and functional assessment of the retina in patients with PH1, and the relationship between retinal dysfunction and kidney impairment. Results: A total of 16 eyes were examined in the study of 8 children ranging in age from 4 to 19 years. Four eyes (25%) showed normal structural and functional retinal findings, 8 eyes (50%) presented functional impairment in the absence of pathological structural findings, and 4 eyes (25%) had advanced retinal damage that manifested as significant morphological and functional impairment. There was no direct relationship between the severity of the renal disease and the severity of the retinal phenotype. Conclusions: Subjects with PH1 present varying severity levels of the retinal phenotype, with possible discrepancy between the clinical retinal morphology and the retinal function noted on electroretinography. These findings raise questions about the molecular basis of the retinal manifestations in PH1. The presence of functional impairment in the absence of evident crystal deposition in the retina suggests that, in addition to oxalate crystal accumulation, other biomolecular processes may play a role in the development of retinopathy.

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With the increasing threat of metabolic syndromes, a focus on maintaining kidney health from early- to mid-adulthood is necessary. This study elucidates mortality risk and years of life lost (YLLs) due to abnormal renal function. This was a retrospective, matched cohort study from health checkup data from 2000 to 2015. We identified 12,774 participants with abnormal renal function (eGFR < 60 mL/min/1.73 m2) and used propensity score matching to identify 25,548 participants with normal renal function (eGFR ≥ 60). YLLs were estimated using the life expectancy differences between the abnormal and matched normal cohorts. Cox models were used to estimate the adjusted mortality risk. The estimated life expectancy of participants with proteinuria and eGFR < 60 was 26.24 years, with a 95 % confidence interval of (23.96, 29.36), 17.62 (16.37, 18.78), and 11.70 (11.02, 12.46) for age groups of 30 - 54, 55 - 64, and 65 - 79 years, respectively. The estimated YLLs of participants with proteinuria and eGFR < 60, as compared with the matched normal cohort, were 17.86 (13.41, 20.36), 12.55 (11.41, 13.78), and 8.31 (7.47, 9.13) years for the three age groups, respectively. The Cox model estimates of mortality hazard ratios of participants having proteinuria and eGFR < 60 against matched referents were 5.29 (3.97, 7.05), 3.99 (3.34, 4.75), and 3.05 (2.62, 3.55) for the three age groups, respectively. Abnormal renal function shortens life expectancy, particularly in patients with proteinuria and in younger adults. Active health management of renal function can reduce the disease burden.

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Carnitine is a medically needful nutrient that contributes in the production of energy and the metabolism of fatty acids. Bioavailability is higher in vegetarians than in people who eat meat. Deficits in carnitine transporters occur as a result of genetic mutations or in combination with other illnesses such like hepatic or renal disease. Carnitine deficit can arise in diseases such endocrine maladies, cardiomyopathy, diabetes, malnutrition, aging, sepsis, and cirrhosis due to abnormalities in carnitine regulation. The exogenously provided molecule is obviously useful in people with primary carnitine deficits, which can be life-threatening, and also some secondary deficiencies, including such organic acidurias: by eradicating hypotonia, muscle weakness, motor skills, and wasting are all improved l-carnitine (LC) have reported to improve myocardial functionality and metabolism in ischemic heart disease patients, as well as athletic performance in individuals with angina pectoris. Furthermore, although some intriguing data indicates that LC could be useful in a variety of conditions, including carnitine deficiency caused by long-term total parenteral supplementation or chronic hemodialysis, hyperlipidemias, and the prevention of anthracyclines and valproate-induced toxicity, such findings must be viewed with caution.

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Chronic kidney disease (CKD) is a global health concern and public health priority. The condition often involves inflammation due to the accumulation of toxins and the reduced clearance of inflammatory cytokines, leading to gradual loss of kidney function. Because of the tremendous burden of CKD, finding effective treatment strategies against inflammation is crucial. Substantial evidence suggests an association between kidney disease and the inflammasome. As a well-known multiprotein signaling complex, the NLR family pyrin domain containing 3 (NLRP3) inflammasome plays an important role in inducing renal inflammation and fibrosis. Small molecule inhibitors targeting the NLRP3 inflammasome are potential agents for the treatment of CKD.The NLRP3 inflammasome activation amplifies the inflammation response, promoting pyroptotic cell death. Thus, it may contribute to the onset and progression of CKD, but the mechanism behind inflammasome activation in CKD remains obscure.In this review, we summarized recent findings on the role of the NLRP3 inflammasome in CKD and new strategies targeting the NLRP3 inflammasome.

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Gastric pneumatosis (GP) is a rare finding. It can be seen with both gastric emphysema (GE) and emphysematous gastritis (EG); however, both conditions present similarly and differentiating between the 2 is difficult radiographically. Moreover, the treatment is vastly different for both conditions, in which treatment for GE is focused on supportive care while treatment for EG may even involve gastrectomy. Making the distinction between GE and EG is crucial because GE has a benign clinical course, while EG carries significant mortality. Early endoscopy may be a useful tool in differentiating between the 2 conditions and to guide further management. Herein, we present a case series of 2 immunocompromised patients who presented with symptoms and radiographic evidence consistent with gastric pneumatosis. We found that early endoscopy assisted in risk stratification and helped guide our management strategy. We recommend consideration of endoscopic evaluation as part of ritualized evaluation of patients presenting with gastric pneumatosis.

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Cirrhosis predisposes to abnormalities in energy, hormonal, and immunological homeostasis. Disturbances in these metabolic processes create susceptibility to sarcopenia or pathological muscle wasting. Sarcopenia is prevalent in cirrhosis and its presence portends significant adverse outcomes including the length of hospital stay, infectious complications, and mortality. This highlights the importance of identification of at-risk individuals with early nutritional, therapeutic and physical therapy intervention. This manuscript summarizes literature relevant to sarcopenia in cirrhosis, describes current knowledge, and elucidates possible future directions.

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Pregnancy after simultaneous pancreas and kidney transplantation (SPKT) carries a high risk of maternal and fetal complications. We report the case of a 39-year-old woman with three consecutive pregnancies with favorable outcomes after SPKT. Within the first year of SPKT, the patient had a spontaneous pregnancy. At 32 weeks of gestation, she underwent an emergency cesarean section (CS) due to severe preeclampsia and HELLP syndrome. The infant was of average birth weight and was transferred to the neonatal intensive care unit for further management. A second unplanned pregnancy occurred almost nine months after the first. The antenatal assessments for fetal growth, blood glucose, and blood pressure were normal throughout follow-up. Early in her pregnancy, the patient developed an uneventful retinopathy of the left eye. At 37 weeks of gestation, she underwent an elective CS due to a short inter-pregnancy interval and delivered a healthy baby with an average birth weight. At the age of 39 years, the patient had a third unplanned pregnancy. She was diagnosed with seronegative antiphospholipid syndrome. She suffered from bilateral vitreous hemorrhage and was managed successfully with a minimally invasive laser treatment combined with an intravitreal injection of anti-vascular endothelial growth factor during her third trimester. At 35 weeks of gestation, the patient presented with labor pain and underwent an emergency CS and delivered a healthy baby with an average birth weight. Pregnancy after SPKT requires a multidisciplinary approach with a careful workup.

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Guidelines consider radial access a relative contraindication in patients with end-stage renal disease as part of a vessel preservation strategy. Radial access distal to a hemodialysis fistula, what we term transradial-transfistula access, offers a solution to radially access this population without affecting their vessel preservation plan. (Level of Difficulty: Advanced.).

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Direct-acting oral anticoagulants (DOACs) represent the standard for preventing stroke and systemic embolization (SSE) in patients with atrial fibrillation (AF). There is limited information for patients ≥ 80 years. We report a retrospective analysis of AF patients ≥ 80 years prescribed either a US Food and Drug Administration (FDA)-approved reduced (n = 514) or full dose (n = 199) DOAC (Dabigatran, Rivaroxaban, or Apixaban) between January 1st, 2011 (first DOAC commercially available) and May 31st, 2017. The following multivariable differences in baseline characteristics were identified: patients prescribed a reduced dose DOAC were older (p < 0.001), had worse renal function (p = 0.001), were more often prescribed aspirin (p = 0.004) or aspirin and clopidogrel (p < 0.001), and more often had new-onset AF (p = 0.001). SSE and central nervous system (CNS) bleed rates were low and not different (1.02 vs 0 %/yr and 1.45 vs 0.44 %/yr) for the reduced and full dose groups, respectively. For non-CNS bleeds, rates were 10.89 vs 4.15 %/yr (p < 0.001, univariable) for the reduced and full doses, respectively. The mortality rate was 6.24 vs 1.75 %/yr (p = 0.001, univariable) for the reduced and full doses. Unlike the non-CNS bleed rate, mortality rate differences remained significant when adjusted for baseline characteristics. Thus, DOACs in patients ≥ 80 with AF effectively reduce SSE with a low risk of CNS bleeding, independent of DOAC dose. The higher non-CNS bleed rate and not the mortality rate is explained by the higher risk baseline characteristics in the reduced DOAC dose group. Further investigation of the etiology of non-CNS bleeds and mortality is warranted.

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Calciphylaxis is a rare, life-threatening vascular disease, which predominantly affects patients with chronic renal failure treated by dialysis. Penile calciphylaxis is an extremely rare condition, a severe manifestation of calciphylaxis, which is associated with poor prognosis and high mortality rate. Diagnosis and management are challenging and still debatable. We present a case with penile calciphylaxis on whom an arterial bypass to the deep dorsal penile vein was performed. Although, in this case, the method was not permanently successful, the histology showed a cluster of neovascularization after the operation. Deep dorsal arterialization might be a proper technique in well-selected patients.

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The main treatment for renal anemia in end-stage renal disease (ESRD) patients on hemodialysis is erythropoiesis (EPO). EPO hyporesponsiveness (EH) in dialysis patients is a common clinical problem, which is poorly understood. Recent searches reported that gut microbiota was closely related to the occurrence and development of ESRD. This study aims to explore the changes in gut microbiota between ESRD patients with different responsiveness to EPO treatment. We compared the gut microbiota from 44 poor-response (PR) and 48 good-response (GR) hemodialysis patients treated with EPO using 16S rDNA sequencing analysis. The results showed that PR patients displayed a characteristic composition of the gut microbiome that clearly differed from that of GR patients. Nine genera (Neisseria, Streptococcus, Porphyromonas, Fusobacterium, Prevotella_7, Rothia, Leptotrichia, Prevotella, Actinomyces) we identified by Lasso regression and ROC curves could excellently predict EH. In contrast, five genera (Faecalibacterium, Citrobacter, Bifidobacterium, Escherichia-Shigella, Bacteroides) identified by the same means presented a protective effect against EH. Analyzing the correlation between these biomarkers and clinical indicators, we found that gut microbiota may affect response to EPO through nutritional status and parathyroid function. These findings suggest that gut microbiota is altered in hemodialysis patients with EH, giving new clues to the pathogenesis of renal anemia.

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Objective: Chronic kidney disease and renal failure are common in patients being considered for left ventricular assist device support. We sought to evaluate the outcomes of patients undergoing left ventricular assist device implantation with preoperative dialysis and those with new-onset postoperative renal failure requiring dialysis. Methods: All patients (n = 14,090) undergoing primary left ventricular assist device implantation who were listed in the Interagency Registry for Mechanically Assisted Circulatory Support database (2014-2019) were evaluated. Landmark analysis then stratified patients alive at 1 month by preoperative dialysis and at 1 month postoperatively, preoperative dialysis only, postoperative dialysis only, and no dialysis. Results: Of 14,090 patients undergoing left ventricular assist device implantation, patients on dialysis (400%, 3%) preoperatively had significantly higher mortality at 1 month (18% vs 6%, P < .0001). However, of patients on preoperative dialysis, 131 (32.8%) no longer required dialysis at 1 month postoperatively and had long-term survival similar to patients who never required dialysis (no dialysis vs recovered, P = .13). Long-term survival was significantly worse in patients with persistent dialysis and new dialysis at 1 month postoperatively (P < .0001). Time to first stroke, major nondevice infection, any bleeding event, and gastrointestinal bleeding were all worse in patients on preoperative or postoperative dialysis (all P < .0001). Device infection, malfunction, or thrombosis was not associated with dialysis status (P > .05). Negative predictors of recovery include biventricular assist device (odds ratio, 0.20) and inotropes 1 week postimplant (odds ratio, 0.19). Conclusions: Preoperative renal failure is associated with 3 times higher mortality and worse morbidity in patients receiving a left ventricular assist device. However, one-third of patients with preoperative dialysis will recover renal function postimplant with similar long-term survival and quality of life as those without dialysis.

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While analysis of the bacterial microbiome has become routine, that of the fungal microbiome is still hampered by the lack of robust databases and bioinformatic pipelines. Here, we present FunOMIC, a pipeline with built-in taxonomic (1.6 million marker genes) and functional (3.4 million non-redundant fungal proteins) databases for the identification of fungi. Applied to more than 2,600 human metagenomic samples, the tool revealed fungal species associated with geography, body sites, and diseases. Correlation network analysis provided new insights into inter-kingdom interactions. With this pipeline and two of the most comprehensive fungal databases, we foresee a fast-growing resource for mycobiome studies.

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Linezolid is an oxazolidinone antibiotic. Linezolid-associated lactic acidosis has been reported in 6.8% of linezolid-treated patients. Lactic acidosis is associated with poor clinical outcomes, with high blood lactate levels resulting in organ dysfunction and mortality. This case report describes the development of lactic acidosis in a 64-year-old Chinese woman who had received 33 days of treatment with antituberculosis drugs and 28 days of treatment with oral linezolid for tuberculous meningitis. Severe lactic acidosis was reversed by withdrawing antituberculosis drugs and using continuous venovenous hemodiafiltration (CVVH). When the patient's condition was stable, she was transferred to the infectious disease department, and antituberculosis drugs, with the exception of linezolid, were reintroduced. This did not result in recurrence of lactic acidosis. The causal relationship between lactic acidosis and linezolid was categorized as 'probable' on the Adverse Drug Reaction Probability Scale. This case demonstrates that CVVH has potential as an alternative to discontinuation of linezolid alone for rapid reversal of linezolid-associated severe lactic acidosis.

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Drug-induced nephrotoxicity remains a common problem after exposure to medications and diagnostic agents, which may be heightened in the kidney microenvironment and deteriorate kidney function. In this study, the toxic effects of fourteen marked drugs with the individual chemical structure were evaluated in kidney cells. The quantitative structure-activity relationship (QSAR) approach was employed to investigate the potential structural descriptors of each drug-related to their toxic effects. The most reasonable equation of the QSAR model displayed that the estimated regression coefficients such as the number of ring assemblies, three-membered rings, and six-membered rings were strongly related to toxic effects on renal cells. Meanwhile, the chemical properties of the tested compounds including carbon atoms, bridge bonds, H-bond donors, negative atoms, and rotatable bonds were favored properties and promote the toxic effects on renal cells. Particularly, more numbers of rotatable bonds were positively correlated with strong toxic effects that displayed on the most toxic compound. The useful information discovered from our regression QSAR models may help to identify potential hazardous moiety to avoid nephrotoxicity in renal preventive medicine.

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Objective: Pericardial effusion and pericarditis are responsible for 3-5% of deaths due to tamponade, fatal arrhythmia and heart failure. Improvement in dialysis methods has allowed the initiation of the treatment at early stages. This study is designed to evaluate the prevalence of pericardial effusion and its predisposing factors among end-stage renal disease (ESRD) patients undergoing dialysis. Methods: This is a cross-sectional study that included patients from the two Medical Centers in (XXX). These patients were presented with ESRD with a GFR <10 cc/min and were under long-term dialysis, also called chronic hemodialysis. The echocardiography was performed and patients with pericarditis and/or pericardial effusion due to non-uremic causes or dialysis were excluded. Results: Of 132 patients included, mild pericardial effusion was observed in 17(12.9%) patients, 8(6.1%) patients were presented with moderate and 1 (0.7%) had severe pericardial effusion. Among females, 9(15.8%) showed pericardial effusion whereas, it was reported in 8(10.7%) males, with no statistically significant difference. Furthermore, no significant difference was seen in the ages or etiologies of patients with or without pericardial effusion (50.5 ± 15.5 vs 52.8 ± 16.1, respectively). Conclusion: Our study reports that echocardiography among dialysis patients is likely to determine the effectiveness of the dialysis procedure and can be a cost-effective approach. Futher studies regarding laboratory parameters are required in this area.

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Skeletal changes are a common complication in patients with chronic kidney disease and traditionally labelled as renal osteodystrophy. Uremic leontiasis ossea is a rare and severe form of renal osteodystrophy with characteristic overgrowth of the craniofacial bones. Imaging, in particular computed tomography, is valuable for the diagnosis and management of such rare condition. Uremic leontiasis ossea has distinctive imaging features with significant overgrowth of the jaw and characteristic internal serpiginous tunneling. The recognition of its radiological appearance and abrupt management are essential to avoid its devastating esthetic and functional impairments.