RESUMO
IgG (mainly IgG3) is the most commonly involved isotype in proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). Here we describe the first series of PGNMID with deposition of monoclonal immunoglobulin light chain only (PGNMID-light chain). This multicenter cohort of 17 patients presented with nephritic or nephrotic syndrome with underlying hematologic conditions of monoclonal gammopathy of renal significance (71%) or multiple myeloma (29%). Monoclonal immunoglobulin was identified by serum and urine immunofixation in 65% and 73%, respectively, with abnormal serum free light chain in 83%, and a detectable bone marrow plasma cell clone in 88% of patients. Renal biopsy showed a membranoproliferative pattern in most patients. By immunofluorescence, deposits were restricted to glomeruli and composed of restricted light chain (kappa in 71%) and C3, with granular appearance and subendothelial, mesangial and subepithelial distribution by electron microscopy. Proteomic analysis in four cases of kappa PGNMID-light chain revealed spectra for kappa constant and variable domains, without evidence of Ig heavy chains; spectra for proteins of the alternative pathway of complement and terminal complex were detected in three. The classical pathway was not detected in three cases. After median follow up of 70 months, the renal response was dependent on a hematologic response and occurred in six of ten patients treated with plasma cell-directed chemotherapy but none of five patients receiving other therapies. Thus, PGNMID-light chain differs from PGNMID-IgG by higher frequency of a detectable pathogenic plasma cell clone. Hence, proper recognition is crucial as anti-myeloma agents may improve renal prognosis. Activation of an alternative pathway of complement by monoclonal immunoglobulin light chain likely plays a role in its pathogenesis.
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Glomerulonefrite Membranoproliferativa , Glomerulonefrite , Paraproteinemias , Anticorpos Monoclonais , Células Clonais , Glomerulonefrite Membranoproliferativa/diagnóstico , Humanos , Paraproteinemias/diagnóstico , Plasmócitos , ProteômicaRESUMO
Monoclonal gammopathy of renal significance (MGRS) regroups renal disorders caused by a monoclonal immunoglobulin without overt hematological malignancy. MGRS includes tubular disorders, glomerular disorders with organized deposits, and glomerular disorders with non-organized deposits, such as proliferative glomerulonephritis with monoclonal IgG deposits. Since glomerular involvement related to monotypic IgA deposits is poorly described we performed retrospective analysis and defined clinico-biological characteristics, renal pathology, and outcome in 19 referred patients. This analysis allowed distinction between 2 types of glomerulopathies, α-heavy chain deposition disease (5 patients) and glomerulonephritis with monotypic IgA deposits (14 patients) suggestive of IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits in 12 cases. Clinicopathologic characteristics of α-heavy chain deposition disease resemble those of the γ-heavy chain disease, except for a higher frequency of extra-capillary proliferation and extra-renal involvement. IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits should be differentiated from diseases with polytypic IgA deposits, given distinct clinical, histological, and pathophysiological features. Similarly to IgG-proliferative glomerulonephritis with monoclonal immunoglobulin deposits, overt hematological malignancy was infrequent, but sensitive serum and bone marrow studies revealed a subtle plasma cell proliferation in most patients with IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Anti-myeloma agents appeared to favorably influence renal prognosis. Thus, potential progression towards symptomatic IgA multiple myeloma suggests that careful hematological follow-up is mandatory. This series expands the spectrum of renal disease in MGRS.
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Glomerulonefrite por IGA/imunologia , Glomerulonefrite/imunologia , Doença das Cadeias Pesadas/imunologia , Imunoglobulina A/análise , Rim/imunologia , Mieloma Múltiplo/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/análise , Biópsia , Proliferação de Células , Diagnóstico Diferencial , Progressão da Doença , Feminino , Imunofluorescência , França , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/patologia , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/patologia , Doença das Cadeias Pesadas/tratamento farmacológico , Doença das Cadeias Pesadas/patologia , Humanos , Cadeias alfa de Imunoglobulina/análise , Cadeias gama de Imunoglobulina/análise , Rim/efeitos dos fármacos , Rim/ultraestrutura , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/patologia , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Fatores de TempoRESUMO
BACKGROUND: These last years, considerable attention has been given to renal issues following endovascular aortic repair but acute kidney injury (AKI) also remains one of the most frequent complications following open repair (OR). Since AKI definition has evolved, our aim was to review the etiology, incidence, classifications, and consequences of AKI after OR for intact abdominal aortic aneurysm (AAA). METHODS: A review of the English language literature published between 2004 and 2016 was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria. Studies regarding ruptured AAA, combination of emergency and elective repairs without results stratification, and thoracoabdominal aneurysms were excluded, as well as studies not using recent consensual AKI classifications. RESULTS: In total, 6 studies including 658 patients (394 from prospective studies and 264 from retrospective studies) used recent consensual classifications (risk injury failure loss end-stage renal disease [RIFLE], Acute Kidney Injury Network classification [AKIN], kidney disease-improving global outcomes [KDIGO], and Aneurysm Renal Injury Score [ARISe]) and were considered for analysis. After infrarenal clamping, AKI rates were quite similar between studies, observed in 24% cases when using the RIFLE classification, 20% cases with the AKIN criteria, and 26.3% cases when using the ARISe classification. Pooled rates of renal replacement therapy (RRT) and 30-day mortality were 0.3% and 1.4%, respectively. In the long term, no patient mandated chronic RRT. After suprarenal clamping, 2 retrospective studies used the RIFLE criteria to define the incidence of AKI, and the pooled AKI rate was 36.8%. The AKI rate was 26.5% in smaller retrospective study using the KDIGO criteria. Rates of RRT and 30-day mortality were 3.8% and 2.9%, respectively. In the long term, 1.2-3% of patients mandated chronic RRT. CONCLUSIONS: AKI is a common but generally transient complication after OR for AAA. Its incidence depends on definitions used, and few studies were found to use recent consensual AKI criteria. In the future, large prospective studies using consensual AKI definitions will facilitate comparison between repair strategies.
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Injúria Renal Aguda/epidemiologia , Aneurisma da Aorta Abdominal/cirurgia , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Injúria Renal Aguda/diagnóstico , Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/terapia , Aneurisma da Aorta Abdominal/diagnóstico por imagem , Aneurisma da Aorta Abdominal/mortalidade , Humanos , Incidência , Terapia de Substituição Renal , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/mortalidadeRESUMO
BACKGROUND: Chronic impaired renal function constitutes a major risk factor of morbi-mortality during the treatment of an abdominal aortic aneurism (AAA). The inflammatory state due to the AAA could result in a reduction in the muscular mass and an overestimation of the glomerular filtration rate (GFR) with the usual formulas. The objective of this study was to determine if the formulas used to evaluate the estimated GFR were adapted in patients with AAA. MATERIALS AND METHODS: Between August 2013 and November 2014, we conducted an exploratory study to evaluate the renal function before surgery for AAA in 28 patients. The renal function was evaluated by (1) the dosage of plasmatic creatinine, (2) the GFR estimated with the Cockroft-Gault, Modification of Diet in Renal Disease (MDRD), and chronic kidney disease epidemiology collaboration (CKD-EPI) formulas, (3) the creatinine clearance (CC), and (4) the direct measurement of the GFR with a reference method (iohexol clearance). Statistical analysis was carried out to compare and correlate the GFR estimated by the various formulas with the GFR measured by the reference technique. RESULTS: The study included 21 men (75%) and 7 women (25%), with a median age of 76 years (58-89). The measured GFR was correlated with the GFR estimated from the CKD-EPI (rho = 0.78, P < 0.0001), the MDRD (rho = 0.78, P < 0.0001), the Cockroft-Gault (rho = 0.65, P = 0.0002), and CC (rho = 0.86, P < 0.0001). However, there were important individual variations between estimated and measured GFR. As regards the detection of the patients presenting a GFR <60 mL/min/1.73 m2, the sensitivities of the CKD-EPI, MDRD, Cockroft-Gault formulas and CC were 64%, 64%, 71%, and 70%, respectively. Specificities were 71%, 79%, 57%, and 100%, respectively. The estimation of the GFR by the CKD-EPI formula had the lowest bias (-3.0). Bland-Altman plots indicated that the estimation of the GFR by the CKD-EPI formula had the best performance in comparison with the other methods. CONCLUSIONS: This study found a statistical correlation between the measurement of the GFR and the various formulas available to estimation the GFR among AAA patients. The CKD-EPI formula is most appropriate. However, there were important individual variations between the measurement and the estimations of the GFR. A larger scale study is necessary to determine the profile of the patients with a risk of error in the estimation of the GFR. The French recommendations on the evaluation of the renal function before AAA treatment remain based on serum creatinine and should be revalued.
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Aneurisma da Aorta Abdominal/cirurgia , Taxa de Filtração Glomerular , Rim/fisiopatologia , Modelos Biológicos , Modelos Estatísticos , Insuficiência Renal Crônica/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Aneurisma da Aorta Abdominal/complicações , Aneurisma da Aorta Abdominal/diagnóstico , Biomarcadores/sangue , Meios de Contraste/administração & dosagem , Creatinina/sangue , Feminino , Humanos , Iohexol/administração & dosagem , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Cuidados Pré-Operatórios , Estudos Prospectivos , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico , Reprodutibilidade dos Testes , Liberação de CirurgiaRESUMO
The ageing of the population is associated with an increase in the incidence of chronic renal failure. Management of the condition must be adapted to the specificities linked to ageing. With end-stage renal failure, haemodialysis is a substitution technique offered under certain conditions. The central role of the caregivers is to support and guide the elderly person along their care pathway. Faced with a reduced life expectancy, the aim of all concerned is to strike a balance between an effective treatment and quality of life.
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Gerenciamento Clínico , Falência Renal Crônica/terapia , Diálise Renal , Idoso , Dieta , Humanos , Qualidade de VidaRESUMO
BACKGROUND: Influenza-like illness (ILI) incidence estimates in individuals treated with immunosuppressants and/or biologics and/or corticosteroid for an autoimmune or chronic inflammatory disease are scarce. We compared the ILI incidence among immunocompromised population and the general population. METHOD: We conducted a prospective cohort study during the 2017-2018 seasonal influenza epidemic, on the GrippeNet.fr electronic platform, which allows the collection of epidemiological crowdsourced data on ILI, directly from the French general population. The immunocompromised population were adults treated with systemic corticosteroids, immunosuppressants, and/or biologics for an autoimmune or chronic inflammatory disease, recruited directly on GrippeNet.fr and also among patients of the departments of a single university hospital that were asked to incorporate GrippeNet.fr. The general population consisted of adults reporting none of the above treatments or diseases participating in GrippeNet.fr. The incidence of ILI was estimated on a weekly basis and compared between the immunocompromised population and the general population, during the seasonal influenza epidemic. RESULTS: Among the 318 immunocompromised patients assessed for eligibility, 177 were included. During the 2017-2018 seasonal influenza epidemic period, immunocompromised population had 1.59 (95% CI: 1.13-2.20) higher odds to experience an ILI episode, compared to the general population (N = 5358). An influenza vaccination was reported by 58% of the immunocompromised population, compared to 41% of the general population (p < 0.001). CONCLUSION: During a seasonal influenza epidemic period, the incidence of influenza-like illness was higher in patients treated with immunosuppressants, biologics, and/or corticosteroids for an autoimmune or chronic inflammatory disease, compared to the general population.
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Produtos Biológicos , Crowdsourcing , Influenza Humana , Viroses , Adulto , Humanos , Imunossupressores/uso terapêutico , Influenza Humana/tratamento farmacológico , Influenza Humana/epidemiologia , Influenza Humana/prevenção & controle , Estudos de Coortes , Estudos Prospectivos , Corticosteroides/uso terapêutico , Doença Crônica , França/epidemiologiaRESUMO
BACKGROUND: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined. METHODS: We conducted an observational cohort study using data from 35 sites in two European countries. Clinical, biologic, imaging, and histopathologic data; treatment modalities; and outcomes were collected from medical records. Logistic regression was performed to identify the possible factors related to an eGFR ≤30 ml/min per 1.73 m 2 at the last follow-up. Cox proportional hazards model was performed to assess the factors associated with the risk of relapse. RESULTS: We studied 101 adult patients with IgG4-related disease with a median follow-up of 24 (11-58) months. Of these, 87 (86%) patients were male, and the median age was 68 (57-76) years. Eighty-three (82%) patients had IgG4-related kidney disease confirmed by kidney biopsy, with all biopsies showing tubulointerstitial involvement and 16 showing glomerular lesions. Ninety (89%) patients were treated with corticosteroids, and 18 (18%) patients received rituximab as first-line therapy. At the last follow-up, the eGFR was below 30 ml/min per 1.73 m 2 in 32% of patients; 34 (34%) patients experienced a relapse, while 12 (13%) patients had died. By Cox survival analysis, the number of organs involved (hazard ratio [HR], 1.26; 95% confidence interval [CI], 1.01 to 1.55) and low C3 and C4 concentrations (HR, 2.31; 95% CI, 1.10 to 4.85) were independently associated with a higher risk of relapse, whereas first-line therapy with rituximab was protective (HR, 0.22; 95% CI, 0.06 to 0.78). At their last follow-up, 19 (19%) patients had an eGFR ≤30 ml/min per 1.73 m 2 . Age (odd ratio [OR], 1.11; 95% CI, 1.03 to 1.20), peak serum creatinine (OR, 2.74; 95% CI, 1.71 to 5.47), and serum IgG4 level ≥5 g/L (OR, 4.46; 95% CI, 1.23 to 19.40) were independently predictive for severe CKD. CONCLUSIONS: IgG4-related kidney disease predominantly affected middle-aged men and manifested as tubulointerstitial nephritis with potential glomerular involvement. Complement consumption and the number of organs involved were associated with a higher relapse rate, whereas first-line therapy with rituximab was associated with lower relapse rate. Patients with high serum IgG4 concentrations (≥5 g/L) had more severe kidney disease.
Assuntos
Doença Relacionada a Imunoglobulina G4 , Nefrite Intersticial , Adulto , Pessoa de Meia-Idade , Humanos , Masculino , Idoso , Feminino , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Rituximab/efeitos adversos , Estudos de Coortes , Prognóstico , Rim/patologia , Nefrite Intersticial/patologia , Imunoglobulina G , Recidiva , Estudos RetrospectivosRESUMO
BACKGROUND: Receiving the diagnosis of kidney failure has a major impact on patients. Yet, the way in which this diagnosis should be delivered is not formally taught within our medical curriculum. To fill this gap we set up a training course of kidney failure diagnosis delivery for nephrology trainees since 2016. This study assessed the effectiveness of this educational intervention. METHODS: The primary outcome was change in the empathy score immediately after the training session and several months afterward, based on the Jefferson Scale of Physician Empathy (JSPE). Self-reported change in clinical practice was also evaluated. As control groups, we assessed empathy levels in untrained nephrology trainees (n = 26) and senior nephrologists (n = 71). Later on (>6 months) we evaluated participants' perception of changes in their clinical practice due to the training. RESULTS: Six training sessions permitted to train 46 trainees. Most respondents (76%) considered the training to have a durable effect on their clinical practice. Average empathy scores were not significantly different in pre-trained trainees (average JSPE: 103.7 ± 11.4), untrained trainees (102.8 ± 16.4; P = 0.81) and senior nephrologists (107.2 ± 13.6; P = 0.15). Participants' empathy score significantly improved after the training session (112.8 ± 13.9; P = 0.003). This improvement was sustained several months afterwards (average JSPE 110.5 ± 10.8; P = 0.04). CONCLUSION: A single 4-hour training session can have long lasting impact on empathy and clinical practice of participants. Willingness to listen, empathy and kindness are thought to be innate and instinctive skills, but they can be acquired and should be taught.
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Empatia , Falência Renal Crônica/diagnóstico , Relações Médico-Paciente , Estudos Transversais , Educação Médica , Feminino , Humanos , Masculino , Satisfação do Paciente , MédicosRESUMO
BACKGROUND: The radiological or interventional use of contrast medium exposes patients to a risk of contrast-induced nephropathy. Pre-existing kidney failure is a major risk factor. Point-of-Care Capillary blood creatinine tests are promising; their speed might help to optimize treatment decisions and patient care in these situations. METHODS: The objective of the present study was to assess the ability of a new point of care capillary blood creatinine test (Stat Sensor X-press, Nova Biomedical Cooperation, Waltham, MA, USA) to diagnose kidney failure, relative to a standard lab-based plasma creatinine assay. A total of 113 patients 33 women (29.2%) were included. The capillary blood creatinine concentration was significantly correlated with the plasma creatinine concentration in both men (Pearson's r [95% Confidence Interval (CI)] = 0.84 [0.75 - 0.89]; p<0.001) and women (Pearson's r [95%CI] = 0.95 [0.89 - 0.97]; p<0.001). The test's diagnostic performance was satisfactory, its sensitivity was 70% [35 - 93] in women and 78% [52 - 94] in men, and its specificity was 91% [72 - 99] in woman and 93% [84 - 98] in men. CONCLUSION: Rapid Point-of Care Capillary creatinine test is an easy-to-use, accurate tool for detecting kidney failure before a patient is exposed to procedures involving contrast medium. The POC test performed less well in patients over the age of 75 and in patients with high plasma creatinine level.
RESUMO
Acute kidney injury is a major cause of in-hospital morbidity and mortality because of the serious nature of the underlying illnesses and the high incidence of complications. The two major causes of acute kidney injury that occur in the hospital are prerenal disease and acute tubular necrosis. Acute tubular necrosis has a histological definition, even if a kidney biopsy is rarely performed. Kidney injuries occurring during acute tubular necrosis are underlined by different pathophysiological mechanisms that emphasize the role of hypoxia on the tubular cells such as apoptosis, cytoskeleton disruption, mitochondrial function and the inflammation mediated by innate immune cells. The microcirculation and the endothelial cells are also the targets of hypoxia-mediated impairment. Repair mechanisms are sometimes inadequate because of pro-fibrotic factors that will lead to chronic kidney disease. Despite all the potential therapeutic targets highlighted by the pathophysiological knowledge, further works remain necessary to find a way to prevent these injuries.
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Injúria Renal Aguda , Necrose Tubular Aguda , Injúria Renal Aguda/terapia , Células Endoteliais , Humanos , Necrose Tubular Aguda/etiologia , Necrose Tubular Aguda/terapia , Mitocôndrias , NecroseRESUMO
The classical theory of sodium metabolism considers mostly its role on the extracellular volume according to a daily response to the variations of salt intake, correlated to the variations of water volume. Recent works consider sodium tissular storage. This non-osmotic pool could play a role in blood pressure regulation and in immunity mechanisms. The regulation modalities could be more complex, organised over the long term, with a modification of the sodium-water relationship. The aim of this article is to give a new insight on sodium metabolism, based on recent works, especially on the role and regulation of non osmotic tissular sodium.
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Sódio/metabolismo , HumanosRESUMO
Kidney transplantation is the best treatment for the patient with end stage kidney disease in term of increasing the survival rate, reducing complications, improving quality of live and its lower cost compared to peritoneal dialysis or hemodialysis. However, the number of patients waiting for kidney transplantation is growing day by day and the gap between demand and supply is still huge. This situation is even more complicated in developing countries where the lack of legislation, infrastructure and government involvement is common. Some national transplantation programs have been implemented, with the support of the International Society for Transplantation and the International Society of Nephrology, in order to increase the transplantation activity of these countries in accordance with the Istanbul Declaration on organ trafficking and transplant tourism.
Assuntos
Falência Renal Crônica , Transplante de Rim , Nefrologia , Humanos , Falência Renal Crônica/cirurgia , Doadores Vivos , Diálise Renal , Taxa de SobrevidaRESUMO
BACKGROUND: The objectives were to characterize Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease 2019 (COVID-19) in patients with acute kidney injury (AKI). METHODS: Kidney biopsy samples in two Caucasian patients and one African with COVID-19 AKI were investigated. RESULTS: All patients had a high-level non-selective glomerular proteinuria. SARS-CoV-2 samples by real-time polymerase chain reaction (RT- PCR) assay were all-negative, as well as for virus particles in the kidney by electron microscopy. The three patients and patients with other AKI did not differ significantly with regard to angiotensin-converting enzyme 2 and transmembrane protease serine 2 kidney staining. CONCLUSIONS: The kidney damage particularly in Caucasians in COVID-19 seems to be an AKI, possibly by the systemic inflammatory response.
RESUMO
Cardiovascular mortality in patients with chronic kidney disease remains a major problem. The uremic toxins among which the molecules of middle molecular weight are counted contribute significantly to this high mortality, alongside the traditional risk factors. They generate and maintain a chronic inflammatory state called low-level chronic inflammatory state. A growing interest in these molecules has been noted for some years and the uremic toxins associated with this cardiovascular mortality are currently identified: FGF23, cytokines, pentraxin-3 and recently light chains. The existence of an interaction between uremic toxins, inflammation and/or oxidative stress and cardiovascular mortality is well reported in the various epidemiological studies. While the use of anti-oxidative therapies and/or antibodies against uremic toxins or their site of action have not yet yielded a real benefit, hopes are turning to the use of new hemodialysis membranes medium cut-off (MCO), which have the advantage of purifying the uremic toxin middle molecules without a significant loss of albumin. However, additional works are needed to demonstrate the use of these membranes will lead to modulate the morbi-mortality in the dialysis patients.
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Doenças Cardiovasculares/complicações , Falência Renal Crônica/complicações , Toxinas Biológicas/sangue , Fator de Crescimento de Fibroblastos 23 , Fatores de Crescimento de Fibroblastos/sangue , Humanos , Cadeias Leves de Imunoglobulina/sangue , Inflamação/etiologia , Inflamação/prevenção & controle , Interleucina-6/sangue , Falência Renal Crônica/terapia , Membranas Artificiais , Diálise Renal , Fator de Necrose Tumoral alfa/sangue , Uremia/complicações , Microglobulina beta-2/sangueRESUMO
The incidence of malignant melanoma has increased over the past two decades. A combined BRAF/MEK inhibitor regimen has been shown to lead to prolonged survival and progression-free survival in patients with metastatic BRAF V600-mutant melanoma. Different nephrotoxic effects have been described, among them hyponatremia. The goal of the present narrative review was to understand the pathophysiological mechanisms driving hyponatremia when using selective BRAF inhibitors and/or MEK inhibitors in order to propose potential strategies to prevent or to treat this side effect. Several mechanisms of kidney injury have been suggested including changes in glomerular and tubular function. However, the precise mechanisms of hyponatremia remain unknown. Our hypothesis is that BRAF/MEK inhibitors lead to hyponatremia and water retention (so-called dilution hyponatremia) by activating aquaporin 2 (AQP2) trafficking from its intracellular compartment to the luminal cell membrane, and by activating ENaC channel. Therefore, we recommend treating the hyponatremia related to BRAF/MEK inhibitors with restriction of fluid intake.
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Hiponatremia/tratamento farmacológico , Hiponatremia/fisiopatologia , Melanoma/tratamento farmacológico , Melanoma/fisiopatologia , Inibidores de Proteínas Quinases/uso terapêutico , Humanos , Hiponatremia/complicações , Hiponatremia/diagnóstico , Melanoma/complicações , Quinases de Proteína Quinase Ativadas por Mitógeno/antagonistas & inibidores , Quinases de Proteína Quinase Ativadas por Mitógeno/metabolismo , Modelos Biológicos , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Proteínas Proto-Oncogênicas B-raf/metabolismoRESUMO
Profound deficit of the body fluid composition regulation system is present at the end stage kidney disease, leading to the increase the risk of acute or chronic volume overload, which impacts the morbidity and mortality in these patients. Pulmonary ultrasound by its ability to estimate extrapulmonary water at an infraclinical stage has helped to make progress in this area. Line B is the element of fundamental semiology that reflects the presence of water in the pulmonary alveoli. The alteration of left ventricular function and the increase of pulmonary capillary permeability are the determining factors in the genesis of subclinical pulmonary congestion and are positively correlated with B-lines. Because of its non-invasive nature, its ease of use, its intra- and interoperability reproducibility and its ease of learning, nephrologists can be efficiently and quickly trained to use it to measure pulmonary congestion. Recent data have shown an epidemiological association between B-lines and mortality in end stage kidney disease patients. The causal role of subclinical pulmonary congestion assessed by these B lines in the genesis of detrimental events is being evaluated by a randomized, multicentre, open-label European clinical trial (Lung water by ultra-sound guided treatment [LUST] trial). The clinical usefulness of pulmonary ultrasound in the management of subclinical pulmonary congestion in patients with end stage kidney disease remains to be determined, but it could be considered from now as an additional tool to improve the management of this congestion, possibly by complementing bioimpedancemetry data.
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Falência Renal Crônica/complicações , Pulmão/diagnóstico por imagem , Edema Pulmonar/etiologia , Diálise Renal/métodos , Ultrassonografia/métodos , Humanos , Falência Renal Crônica/terapia , Edema Pulmonar/terapia , Diálise Renal/efeitos adversos , Desequilíbrio Hidroeletrolítico/etiologia , Desequilíbrio Hidroeletrolítico/terapiaRESUMO
Although responsibility is a fundamental determinant in medical practice, physicians are generally unfamiliar with its principles. The same is true for disclosure requirements and requests for compensation in the event of physical injury. We report on a representative survey of iatrogenic complications that may arise after the implementation of vascular access for haemodialysis and that illustrate's the physician's responsibility and obligation to inform the patient. Vascular access steal syndrome is a serious complication of arteriovenous fistulas, and physicians may not be sufficiently aware of the likelihood of its occurrence. Diabetes (via medial calcific sclerosis) and placement in the brachial artery (with excessively high flow rates) are the main risk factors. The precariousness of vascular status in dialysis patients threatens to increase the incidence of this complication. The therapeutic challenge is to resolve ischemic events while maintaining vascular access. The presence of gangrene of the fingers is a formal indication for surgery. The borderline between therapeutic risk (the risk inherent in a medical procedure and which cannot be controlled) and liability for injury is blurred. The French Patient's Rights Act (voted on March 4th, 2002) emphasizes the physician's duty to inform the patient of treatment-associated risks and the fact that the physician now bears the burden of proof. We suggest that a patient information sheet on the benefits and risks of vascular access should be published on the French Society of Nephrology, Dialysis and Transplantation's website.
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Derivação Arteriovenosa Cirúrgica/efeitos adversos , Dedos/irrigação sanguínea , Doença Iatrogênica , Isquemia/etiologia , Humanos , Consentimento Livre e Esclarecido , Imperícia , Educação de Pacientes como Assunto , Papel do Médico , Diálise Renal/efeitos adversosRESUMO
We present the case of a family whose members have high levels of serum calcium (hypercalcaemia) by loss of function of the enzyme vitamin D 24-hydroxylase due to bi-allelic mutations in the CYP24A1 gene: c.443 T>C (p.Leu148Pro) and c.1187 G>A (p.Arg396Gln). 24-VITD hydroxylase is a key player in regulating the circulating calcitriol, its tissue concentration and its biological effects. Transmission is recessive. The estimated prevalence of stones in the affected subjects is estimated between 10 and 15%. The loss of peripheral catabolism of vitamin D metabolites in patients with an inactivating mutation of CYP24A1 is responsible for persistent high levels of 1,25-dihydroxyvitamin D especially after sun exposure and a charge of native vitamin D. Although there are currently no recommendations (French review) on this subject, this disease should be suspected in association with recurrent calcium stones with nephrocalcinosis, and a calcitriol-dependent hypercalcaemia with adapted low parathyroid hormone levels. Resistance to corticosteroid therapy distinguishes it from other calcitriol-dependent hypercalcemia. A ratio of 25-hydroxyvitamin D/24.25 hydroxyvitamin D>50, is in favor of hypercalcemia with vitamin D deficiency 24-hydroxylase. Genetic analysis of CYP24A1 should be performed at the second step. The current therapeutic management includes the restriction native vitamin D supplementation and the limitation of sun exposure. Biological monitoring will be based on serum calcium control and modulation of parathyroid hormone concentrations.