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1.
Medicine (Baltimore) ; 99(38): e22300, 2020 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-32957390

RESUMO

INTRODUCTION: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. PATIENT CONCERNS: A 13-year-old female patient had a history of chronic renal failure, hypothyroidism, mental retardation, hypogonadotropic hypogonadism, obesity, and retinitis pigmentosa and was undergoing 4-hour hemodialysis 3 days a week. DIAGNOSIS: We diagnosed Bardet-Biedl syndrome based on the results of genetic tests. INTERVENTIONS: We performed renal transplantation under general anesthesia while considering the perioperative risks of airway obstruction and hypothermia. OUTCOMES: Multidisciplinary preoperative evaluation is crucial to avoid perioperative complications. The risk of an obstructed airway should be considered. Hypothyroidism is a rare consequence of Bardet-Biedl syndrome. Rocuronium and sugammadex are safe for anesthetic management during renal transplantation to address Bardet-Biedl syndrome. CONCLUSION: Safe anesthetic management can be achieved with the rigorous preoperative assessment of perioperative complications.


Assuntos
Anestesia Geral/métodos , Síndrome de Bardet-Biedl/complicações , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Anormalidades Múltiplas/etiologia , Adolescente , Feminino , Humanos , Falência Renal Crônica/diagnóstico por imagem , Falência Renal Crônica/etiologia , Cuidados Pré-Operatórios
2.
Medicine (Baltimore) ; 99(33): e21736, 2020 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-32872060

RESUMO

RATIONALE: Pilot studies have reported that patients with systemic lupus erythematosus (SLE) appear more likely to develop into neoplasia, especially lymphatic hyperplasia diseases. To our knowledge, this is the first case report of the concomitant onset of SLE and primary breast diffuse large B-cell lymphoma (PB-DLBCL). PATIENT CONCERNS: We reported an unusual case of the occurrence of primary breast diffuse large B-cell lymphoma in a 25-year-old female patient who had been diagnosed with SLE and treated with immunosuppressive drugs for about 4 years. She presented a 7-week history of a painless mass above the left breast and no history suggestive of any nipple discharge, fever, and weight loss. DIAGNOSIS: Ultrasonography of the breast showed that there was 1 mass in the left breast. After breast mass surgical resection, histopathological examinations were performed and revealed that it was primary breast diffuse large B-cell lymphoma. INTERVENTIONS: Treatment strategy with vincristine and dexamethasone was used to improve symptoms. However, the patient's renal function deteriorated and the blood potassium rose continuously and she and their family members refused the follow-up treatments. OUTCOMES: The patient died 8 months after she was discharged from the hospital. LESSONS: PB-DLBCL is a rare occurrence in SLE patients. Therefore, a careful examination is very important in SLE cohort, as activity of the disease and malignancy may mimic each other. Meanwhile, when symptoms cannot be explained or insensitive to treatment, the occurrence of malignant tumors must be highly considered.


Assuntos
Neoplasias da Mama/complicações , Mama/patologia , Falência Renal Crônica/etiologia , Lúpus Eritematoso Sistêmico/complicações , Linfoma Difuso de Grandes Células B/complicações , Adulto , Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/patologia , Neoplasias da Mama/terapia , Evolução Fatal , Feminino , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Radiografia , Ultrassonografia
3.
PLoS One ; 15(8): e0236998, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32790687

RESUMO

There are over 12,000 people with sickle cell disease (SCD) in the UK, and 4-12% of patients who develop Sickle Cell Nephropathy (SCN) progress to End Stage Renal Disease (ESRD). Renal transplantation offers the best outcomes for these patients with but their access to transplantation is often limited. Regular automated exchange blood transfusions (EBT) reduce the complications of SCD and may improve outcomes. However, concerns over alloimmunisation limit its widespread implementation. In this retrospective multicenter study, data were collected on 34 SCD patients who received a kidney transplant across 6 London Hospitals between 1997 and 2017. 20/34 patients were on an EBT program, pre or post renal transplantation. Overall patient and graft survival were inferior to contemporaneous UK data in the ESRD population as a whole, a finding which is well-recognised. However, patient survival (CI 95%, p = 0.0032), graft survival and graft function were superior at all time-points in those who received EBT versus those who did not. 4/20 patients (20%) on EBT developed de novo donor specific antibodies (DSAs). 3/14 patients (21%) not on EBT developed de novo DSAs. The incidence of rejection in those on EBT was 5/18 (28%), as compared with 7/13 (54%) not on EBT. In conclusion, our data, while limited by an inevitably small sample size and differences in the date of transplantation, do suggest that long-term automated EBT post renal transplant is effective and safe, with improvement in graft and patient outcomes and no increase in antibody formation or graft rejection.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/cirurgia , Transfusão Total , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Adulto , Anemia Falciforme/terapia , Terapia Combinada , Feminino , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Falência Renal Crônica/terapia , Londres , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
4.
Medicine (Baltimore) ; 99(29): e20678, 2020 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-32702818

RESUMO

BACKGROUND: Diabetic nephropathy (DN) is among the common and serious complications of diabetes and is also a major cause of end-stage kidney disease. Early DN is also called diabetic microalbumin period, the main treatment is in the control of blood sugar on the basis of kidney protection and urine lowering protein. There are few effective methods of western medicine treatment, and most of them are accompanied by adverse reactions. But some studies have shown that traditional Chinese medicine has achieved the curative effect and has certain superiority. However, there are few systematic reviews on the treatment of traditional Chinese herbal medicine for early DN currently. Therefore, this study conducted a systematic review of clinical efficacy and safety of Chinese herbal medicine for the treatment of patients with early DN, aim to comprehensively analyze the role of traditional Chinese herbal medicine in the treatment of early DN. METHODS AND ANALYSIS: The protocol of this systematic review and meta-analysis was registered on the INPLASY website (https://inplasy.com/inplasy-2020-4-0139/) and INPLASY registration number is INPLASY202040139. A systematic literature search will be conducted in 3 English database and 4 Chinese databases with a language limitation of English and Chinese. Search for clinical research literature on Chinese herbal medicine treatment of DN published in domestic and foreign biomedical journals. The time is limited from January 2010 to February 2020. We will investigate heterogeneity across studies and publication bias. To assess the risk of bias and quality of the included studies, we will use the Cochrane Collaboration's ROB tool. According to the relevant standards in the Cochrane Intervention System Evaluation Manual, it will be divided into low risk, high risk, and unclear. We will also use the RevMan 5.3 software and Stata 13.0 software for meta-analysis of the effectiveness and symptom scores of DN proteinuria. ETHICS AND DISSEMINATION: The ethical considerations are not required because the systematic review is based on published studies. The systematic review and meta-analysis will be published in a peer-reviewed Journal.


Assuntos
Nefropatias Diabéticas/tratamento farmacológico , Medicamentos de Ervas Chinesas/uso terapêutico , Medicina Tradicional Chinesa/métodos , Complicações do Diabetes/epidemiologia , Nefropatias Diabéticas/classificação , Nefropatias Diabéticas/complicações , Feminino , Humanos , Falência Renal Crônica/etiologia , Masculino , Proteinúria/diagnóstico , Proteinúria/etiologia , Proteinúria/urina , Ensaios Clínicos Controlados Aleatórios como Assunto , Segurança , Sensibilidade e Especificidade , Resultado do Tratamento
5.
Am J Kidney Dis ; 76(3): 384-391, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32660897

RESUMO

RATIONALE & OBJECTIVE: Fibrinogen A α-chain amyloidosis (AFib amyloidosis) is a form of amyloidosis resulting from mutations in the fibrinogen A α-chain gene (FGA), causing progressive kidney disease leading to kidney failure. Treatment may include kidney transplantation (KT) or liver-kidney transplantation (LKT), but it is not clear what factors should guide this decision. The aim of this study was to characterize the natural history and long-term outcomes of this disease, with and without organ transplantation, among patients with AFib amyloidosis and various FGA variants. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 32 patients with AFib amyloidosis diagnosed by genetic testing in France between 1983 and 2014, with a median follow-up of 93 (range, 4-192) months, were included. RESULTS: Median age at diagnosis was 51.5 (range, 12-77) years. Clinical presentation consisted of proteinuria (93%), hypertension (83%), and kidney failure (68%). Manifestations of kidney disease appeared on average at age 57 (range, 36-77) years in patients with the E526V variant, at age 45 (range, 12-59) years in those with the R554L variant (P<0.001), and at age 24.5 (range, 12-31) years in those with frameshift variants (P<0.001). KT was performed in 15 patients and LKT was performed in 4. In KT patients with the E526V variant, recurrence of AFib amyloidosis in the kidney graft was less common than with a non-E526V (R554L or frameshift) variant (22% vs 83%; P=0.03) and led to graft loss less frequently (33% vs 100%). Amyloid recurrence was not observed in patients after LKT. LIMITATIONS: Analyses were based on clinically available historical data. Small number of patients with non-E526V and frameshift variants. CONCLUSIONS: Our study suggests phenotypic variability in the natural history of AFib amyloidosis, depending on the FGA mutation type. KT appears to be a viable option for patients with the most common E526V variant, whereas LKT may be a preferred option for patients with frameshift variants.


Assuntos
Amiloidose Familiar/cirurgia , Fibrinogênio/genética , Transplante de Rim , Transplante de Fígado , Adolescente , Adulto , Idoso , Amiloidose Familiar/genética , Amiloidose Familiar/patologia , Criança , Terapia Combinada , Progressão da Doença , Feminino , Seguimentos , Mutação da Fase de Leitura , França/epidemiologia , Estudos de Associação Genética , Humanos , Rim/patologia , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Falência Renal Crônica/terapia , Transplante de Rim/estatística & dados numéricos , Transplante de Fígado/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Mutação Puntual , Diálise Renal , Resultado do Tratamento , Adulto Jovem
6.
Am J Kidney Dis ; 76(3): 361-373, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32359822

RESUMO

RATIONALE & OBJECTIVE: Outcomes reported in trials involving patients with autosomal dominant polycystic kidney disease (ADPKD) are heterogeneous and rarely include patient-reported outcomes. We aimed to identify critically important consensus-based core outcome domains to be reported in trials in ADPKD. STUDY DESIGN: An international 2-round online Delphi survey was conducted in English, French, and Korean languages. SETTING & PARTICIPANTS: Patients/caregivers and health professionals completed a 9-point Likert scale (7-9 indicating critical importance) and a Best-Worst Scale. ANALYTICAL APPROACH: The absolute and relative importance of outcomes were assessed. Comments were analyzed thematically. RESULTS: 1,014 participants (603 [60%] patients/caregivers, 411 [40%] health professionals) from 56 countries completed round 1, and 713 (70%) completed round 2. The prioritized outcomes were kidney function (importance score, 8.6), end-stage kidney disease (8.6), death (7.9), blood pressure (7.9), kidney cyst size/growth (7.8), and cerebral aneurysm (7.7). Kidney cyst-related pain was the highest rated patient-reported outcome by both stakeholder groups. Seven themes explained the prioritization of outcomes: protecting life and health, directly encountering life-threatening and debilitating consequences, specificity to ADPKD, optimizing and extending quality of life, hidden suffering, destroying self-confidence, and lost opportunities. LIMITATIONS: Study design precluded involvement from those without access to internet or limited computer literacy. CONCLUSIONS: Kidney function, end-stage kidney disease, and death were the most important outcomes to patients, caregivers, and health professionals. Kidney cyst-related pain was the highest rated patient-reported outcome. Consistent reporting of these top prioritized outcomes may strengthen the value of trials in ADPKD for decision making.


Assuntos
Rim Policístico Autossômico Dominante/terapia , Adolescente , Adulto , África/epidemiologia , Idoso , Ásia/epidemiologia , Cuidadores/psicologia , Criança , Consenso , Técnica Delfos , Feminino , Pessoal de Saúde/psicologia , Humanos , Aneurisma Intracraniano/etiologia , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Medidas de Resultados Relatados pelo Paciente , Pacientes/psicologia , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/epidemiologia , Rim Policístico Autossômico Dominante/psicologia , Pesquisa Qualitativa , Qualidade de Vida , Autoimagem , Fatores Socioeconômicos , Estresse Psicológico , Adulto Jovem
7.
Transpl Infect Dis ; 22(4): e13286, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32279418

RESUMO

During the novel coronavirus pandemic, organ transplant recipients represent a frail susceptible category due to long-term immunosuppressive therapy. For this reason, clinical manifestations may differ from general population and different treatment approaches may be needed. We present the case of a 36-year-old kidney-transplanted woman affected by Senior-Loken syndrome diagnosed with COVID-19 pneumonia after a contact with her positive mother. Initial symptoms were fatigue, dry cough, and coryza; she never had fever nor oxygen supplementation. Hydroxychloroquine and lopinavir/ritonavir were started, and the antiviral drug was replaced with darunavir/cobicistat after 2 days for diarrhea. Immunosuppressant levels were closely monitored, and we observed very high tacrolimus trough levels despite initial dose reduction. The patient was left with steroid therapy alone. The peculiarity of clinical presentation and the management difficulties represent the flagship of our case report. We stress the need for guidelines in transplant recipients with COVID-19 infection with particular regard to the management of therapy.


Assuntos
Antivirais/efeitos adversos , Infecções por Coronavirus/tratamento farmacológico , Inibidores do Citocromo P-450 CYP3A/efeitos adversos , Rejeição de Enxerto/prevenção & controle , Imunossupressores/efeitos adversos , Transplante de Rim , Lopinavir/efeitos adversos , Pneumonia Viral/tratamento farmacológico , Ritonavir/efeitos adversos , Tacrolimo/efeitos adversos , Adulto , Antivirais/uso terapêutico , Betacoronavirus , Proteína C-Reativa/imunologia , Ciliopatias/complicações , Cobicistat/uso terapêutico , Resfriado Comum/etiologia , Resfriado Comum/fisiopatologia , Infecções por Coronavirus/complicações , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/fisiopatologia , Tosse/etiologia , Tosse/fisiopatologia , Darunavir/uso terapêutico , Desprescrições , Combinação de Medicamentos , Interações Medicamentosas , Inibidores Enzimáticos/uso terapêutico , Fadiga/etiologia , Fadiga/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hidroxicloroquina/uso terapêutico , Hospedeiro Imunocomprometido/imunologia , Interleucina-10/imunologia , Interleucina-1beta/imunologia , Interleucina-6/imunologia , Interleucina-8/imunologia , Doenças Renais Císticas/complicações , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Amaurose Congênita de Leber/complicações , Metilprednisolona/uso terapêutico , Atrofias Ópticas Hereditárias/complicações , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/imunologia , Pneumonia Viral/fisiopatologia , Índice de Gravidade de Doença
8.
Diabetes Res Clin Pract ; 163: 108135, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32259612

RESUMO

AIMS: To evaluate the changes in cardiovascular risk factors (CVRFs) and cardiovascular disease (CVD) in patients with type 1 diabetes (T1D) and end-stage renal disease (ESRD) who were candidates for kidney-pancreas transplantation (KPTx) from 1999 to 2017. METHODS: Patients with T1D referred for KPTx evaluation were included. The cohort was divided into five groups according to the year of evaluation (1999-2002, 2003-2006, 2007-2010, 2011-2014 and 2015-2017). The control of CVRFs and the prevalence of prior CVD were evaluated. RESULTS: We evaluated 360 patients (64.4% men, age 38.9 ± 7.1 years). LDL-cholesterol <100 mg/dl increased from 22.7% to 76.9% (1999-2002 vs. 2015-2017; p < 0.001), as did the use of statins (from 24.7% to 74.5%; p < 0.001). Systolic blood pressure decreased from 138.8 ± 27.6 to 125.1 ± 27.9 mmHg (p = 0.001) and current smokers from 48% to 25% (p = 0.018). Intensive insulin treatment increased from 34.4% to 93.6% (p < 0.001). Diabetes duration before the initiation of renal replacement therapy increased from 23 ± 5.5 to 26.9 ± 8.9 years (p = 0.001). Overall, 30.3% had previous CVD, without significant changes over time (p = 0.699), albeit patients were older and had longer diabetes duration. CONCLUSIONS: Patients with T1D and ESRD referred for KPTx have better control of CVRFs over time, which might lead to a decrease in cardiovascular events.


Assuntos
Doenças Cardiovasculares/etiologia , Diabetes Mellitus Tipo 1/complicações , Falência Renal Crônica/etiologia , Transplante de Rim/métodos , Transplante de Pâncreas/métodos , Adulto , Feminino , História do Século XX , História do Século XXI , Humanos , Masculino , Fatores de Risco
9.
Am J Kidney Dis ; 76(1): 141-143, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32240718

RESUMO

In December 2019, an outbreak of coronavirus disease 2019 (COVID-19) due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) began in China and spread rapidly worldwide. It is unknown whether hemodialysis patients represent a distinct group of patients with certain characteristics that may make them susceptible to infection or severe disease. In this case report, we describe the clinical and epidemiologic features of COVID-19 infection in 201 maintenance hemodialysis patients in Zhongnan Hospital of Wuhan University, including 5 maintenance hemodialysis patients who contracted COVID-19 infection. Of the 5 patients with COVID-19 infection, one had a definite history of contact with an infected person. The age range of the patients was 47 to 67 years. Diarrhea (80%), fever (60%), and fatigue (60%) were the most common symptoms. Lymphopenia occurred in all patients. Computed tomography of the chest showed ground glass opacity in the lungs of all patients. Up to February 13, 2020, none of the patients had developed severe complications (acute respiratory distress syndrome, shock, or multiple organ dysfunction) or died.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Falência Renal Crônica/etiologia , Pneumonia Viral/complicações , Diálise Renal/métodos , Idoso , China/epidemiologia , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Surtos de Doenças , Feminino , Humanos , Falência Renal Crônica/terapia , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
10.
BMC Med ; 18(1): 66, 2020 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-32234061

RESUMO

BACKGROUND: Preterm delivery is an independent risk factor for maternal cardiovascular disease. Little is known about the association between preterm delivery and maternal renal function. This study aimed to examine whether women who experience preterm delivery are at increased risk of subsequent chronic kidney disease (CKD) and end-stage kidney disease (ESKD). METHODS: Using data from the Swedish Medical Birth Register, singleton live births from 1973 to 2012 were identified and linked to data from the Swedish Renal Register and National Patient Register (up to 2013). Gestational age at delivery was the main exposure and treated as a time-dependent variable. Primary outcomes were maternal CKD or ESKD. Cox proportional hazard regression models were used for analysis. RESULTS: The dataset included 1,943,716 women who had 3,760,429 singleton live births. The median follow-up was 20.6 (interquartile range 9.9-30.0) years. Overall, 162,918 women (8.4%) delivered at least 1 preterm infant (< 37 weeks). Women who had any preterm delivery (< 37 weeks) were at increased risk of CKD (adjusted hazard ratio (aHR) 1.39, 95% CI 1.32-1.45) and ESKD (aHR 2.22, 95% CI 1.90-2.58) compared with women who only delivered at term (≥ 37 weeks). Women who delivered an extremely preterm infant (< 28 weeks) were at increased risk of CKD (aHR 1.84, 95% CI 1.52-2.22) and ESKD (aHR 3.61, 95% CI 2.03-6.39). The highest risk of CKD and ESKD was in women who experienced preterm delivery + preeclampsia (vs. non-preeclamptic term deliveries, for CKD, aHR 2.81, 95% CI 2.46-3.20; for ESKD, aHR 6.70, 95% CI 4.70-9.56). However, spontaneous preterm delivery was also associated with increased risk of CKD (aHR 1.32, 95% CI 1.25-1.39) and ESKD (aHR 1.99, 95% CI 1.67-2.38) independent of preeclampsia or small for gestational age (SGA). CONCLUSIONS: Women with history of preterm delivery are at increased risk of CKD and ESKD. The risk is higher among women who had very preterm or extremely preterm deliveries, or whose preterm delivery was medically indicated. Women who experience spontaneous preterm delivery are at increased risk of long-term renal disease independent of preeclampsia or SGA. Preterm delivery may act as a risk marker for adverse maternal renal outcomes.


Assuntos
Falência Renal Crônica/etiologia , Nascimento Prematuro/fisiopatologia , Adulto , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , Fatores de Risco , Adulto Jovem
11.
Am J Surg Pathol ; 44(8): 1031-1039, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32271189

RESUMO

Acquired cystic disease of kidney-associated renal cell carcinoma (ACD-RCC) is a distinct subtype of renal cell carcinoma with unique morphologic and clinicopathologic features. Generally, ACD-RCC is regarded as an indolent tumor; however, prognostic and outcomes data have been conflicted by the limited and relatively low number of cases with patient follow-up or adverse events. In this study, we focused on the histology of metastatic lesions and identifying prognostic factors associated with metastatic progression. From 32 cases in the cohort, 9 patients had metastasis [ACD-RCC (M+)] and 23 patients were without metastasis [ACD-RCC (M-)]. The median age of patients was 52 years; right side, n=10; left side, n=18; bilateral, n=4; median tumor size=2.6 cm; median hemodialysis duration=17 y; and the median duration of follow-up was 50 mo. Immunohistochemistry showed ACD-RCC to be racemase positive and CK7 negative to focally positive within tumor cells, with consistent positivity for renal histogenesis-associated markers (PAX8 and RCC antigen); S100A1 was a less reliable marker at metastatic sites. All metastatic ACD-RCC except 2 cases involved lymph nodes (para-aortic, renal hilar, subclavicular). Overall, 6/9 (67%) had visceral metastasis to sites including lung (n=3), liver (n=3), bone (n=5), stomach (n=1), and brain (n=1). In total, 5/9 (56%) metastatic tumors had distinctive cystic growth pattern at the metastatic site; intriguingly metastatic tumors had intrametastatic oxalate crystal deposition, a pathognomonic feature associated with primary tumors. Four of nine (44%) patients with ACD-RCC (M+) had fatal outcomes due to metastatic disease. Clinically significant adverse prognostic features associated with metastasis [median follow-up 47 mo, ACD-RCC (M+) vs. ACD-RCC (M-), 50 mo] included: duration of hemodialysis (≥20 vs. <20 y, P=0.0085) and tumor necrosis (P=0.049). Because of sufficient overlap between these parameters, the study was not able to identify parameters that would be reliable in further management strategies, in clinical settings. Our data indicate that ACD-RCC is a tumor which has distinct metastatic potential with nodal and visceral tropism and proclivity for cystic morphology at metastatic sites; this is the first report of the presence of oxalate crystals in metastatic tumors. Our data suggest that ACD-RCC patients with prolonged hemodialysis and tumoral coagulative necrosis require additional surveillance in view of the association of these parameters with metastatic progression.


Assuntos
Carcinoma de Células Renais/secundário , Doenças Renais Císticas/complicações , Falência Renal Crônica/etiologia , Neoplasias Renais/patologia , Adulto , Idoso , Biomarcadores Tumorais/análise , Carcinoma de Células Renais/química , Carcinoma de Células Renais/etiologia , Carcinoma de Células Renais/terapia , Cristalização , Feminino , Humanos , Japão , Doenças Renais Císticas/diagnóstico , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Neoplasias Renais/química , Neoplasias Renais/etiologia , Neoplasias Renais/terapia , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Ácido Oxálico/análise , Prognóstico , Diálise Renal , Fatores de Risco
12.
Clin Nephrol ; 93(5): 251-261, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32228829

RESUMO

BACKGROUND: In Brazil, schistosomiasis is caused only by Schistosoma mansoni, occurring in the northeastern and southeastern regions. Schistosomiasis primarily affects the liver and gastrointestinal tract, although the kidneys can also be affected, mainly in the form of glomerulopathies. Here, we describe the characteristics of patients with schistosomiasis-associated glomerulopathies, including treatment and renal outcomes. MATERIALS AND METHODS: This was a retrospective analysis of patients diagnosed with schistosomiasis-associated glomerulopathy between 2002 and 2017. Clinical, biochemical, and histopathological (kidney biopsy) data were evaluated. RESULTS: Of the 24 patients evaluated, 19 (79.1%) were male and 16 (66.4%) were White. The mean age was 38.58 ± 9.83 years. We observed the hepatosplenic form of schistosomiasis in 15 patients (68.1%), nephrotic-nephritic syndrome in 13 (54.1%), hematuria in 20 (83.3%), and hypertension in 18 (75.0%). Renal histology showed a predominance of membranoproliferative pattern (n = 17/70.8%). On immunofluorescence, 19 patients (82.6%) showed immunoglobulin M (IgM) expression, 10 (43.4%) showed IgM+IgG expression, and 1 (4.3%) showed a "full house" pattern. The median follow-up time was 59.70 months, by the end of which 9 patients (37.5%) had developed end-stage renal disease (ESRD). Baseline serum creatinine was higher among the patients who developed ESRD than among those who did not (1.99 ± 1.08 vs. 1.34 ± 0.46 mg/dL, p = 0.05). CONCLUSION: Our study is one of the rare clinical studies on schistosomiasis-associated glomerulopathy with a long follow-up and renal endpoints, showing that one third of our patients, independent of their histological form, progress to dialysis.
.


Assuntos
Glomerulonefrite Membranoproliferativa/etiologia , Glomérulos Renais/patologia , Esquistossomose mansoni/complicações , Adolescente , Adulto , Feminino , Glomerulonefrite Membranoproliferativa/patologia , Hematúria/etiologia , Humanos , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/etiologia , Estudos Retrospectivos , Adulto Jovem
13.
Rev Med Suisse ; 16(683): 412-416, 2020 Feb 26.
Artigo em Francês | MEDLINE | ID: mdl-32129019

RESUMO

Over the last decades, an increasing number of cases of chronic and end-stage kidney disease has been observed in Central America and Asia. This kidney disease mainly affects young farmers without classic renal risk factors. The clinical presentation includes a progressive decrease of the glomerular filtration rate, minimal proteinuria and the presence of tubulo-interstitial nephritis at renal biopsy. A close link with global warming is suspected for this disease, called (according to its location) meso-american nephropathy, Sri Lanka nephropathy or chronic kidney disease of unknown etiology. Others have suggested that intake of water contaminated with pesticides may be responsible. This article provides an overview of this new kidney disease. Measures to prevent acute kidney injury during heat waves in Switzerland are also discussed.


Assuntos
Aquecimento Global , Nefropatias/epidemiologia , Nefropatias/etiologia , Rim/patologia , Rim/fisiopatologia , América Central/epidemiologia , Humanos , Nefropatias/prevenção & controle , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Falência Renal Crônica/prevenção & controle , Sri Lanka/epidemiologia , Suíça/epidemiologia
14.
Ann Rheum Dis ; 79(6): 713-723, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32220834

RESUMO

OBJECTIVE: To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN). METHODS: Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements. RESULTS: The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease. CONCLUSIONS: We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.


Assuntos
Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Sociedades Médicas , Antirreumáticos/uso terapêutico , Azatioprina/uso terapêutico , Inibidores de Calcineurina/uso terapêutico , Quimioterapia Combinada , Europa (Continente) , Taxa de Filtração Glomerular , Glucocorticoides/uso terapêutico , Humanos , Hidroxicloroquina/uso terapêutico , Falência Renal Crônica/etiologia , Falência Renal Crônica/terapia , Nefrite Lúpica/complicações , Nefrite Lúpica/patologia , Nefrite Lúpica/fisiopatologia , Ácido Micofenólico/uso terapêutico , Proteinúria/etiologia , Proteinúria/terapia
15.
Am J Kidney Dis ; 76(2): 265-281, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32220510

RESUMO

Systemic lupus erythematosus is a multisystem autoimmune disease that commonly affects the kidneys. Lupus nephritis (LN) is the most common cause of kidney injury in systemic lupus erythematosus and a major risk factor for morbidity and mortality. The pathophysiology of LN is heterogeneous. Genetic and environmental factors likely contribute to this heterogeneity. Despite improved understanding of the pathogenesis of LN, treatment advances have been few and risk for kidney failure remains unacceptably high. This installment in the Core Curriculum of Nephrology provides an up-to-date review of the current understanding of LN epidemiology, pathogenesis, diagnosis, and treatment. Challenging issues such as the management of LN in pregnancy, timing of transplantation, and the evolving role of corticosteroid use in the management of LN are discussed. We review the currently accepted approach to care for patients with LN and highlight deficiencies that need to be addressed to better preserve long-term kidney health and improve outcomes in LN.


Assuntos
Corticosteroides/uso terapêutico , Fatores Imunológicos/uso terapêutico , Falência Renal Crônica/terapia , Transplante de Rim , Nefrite Lúpica , Diálise Renal , Imunidade Adaptativa/imunologia , Distribuição por Idade , Autoanticorpos/imunologia , Autoimunidade/imunologia , Biópsia , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Imunidade Inata/imunologia , Imunossupressores/uso terapêutico , Quimioterapia de Indução , Falência Renal Crônica/etiologia , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/epidemiologia , Nefrite Lúpica/patologia , Nefrite Lúpica/fisiopatologia , Quimioterapia de Manutenção , Masculino , Ácido Micofenólico/uso terapêutico , Gravidez , Complicações na Gravidez , Urinálise
16.
Curr Urol Rep ; 21(2): 14, 2020 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-32166462

RESUMO

PURPOSE OF REVIEW: Nowadays many ESRD patients awaiting kidney transplantation have known unsuitable iliac vessels for vascular anastomosis, due to severe atheromatosis, occupied iliac fossa, or other uncommon vascular abnormalities. In these cases, orthotopic kidney transplantation (OKT) could be the solution. RECENT FINDINGS: Since the update on OKT published in 2010, no more large series have been reported. Some small series or case reports being described in the literature. The orthotopic position has shown good recipient and graft results with acceptable complication rate in selected patients. This technique permits the possibility of kidney transplantation, in patients unfit for heterotopic kidney transplantation (HKT), and consequently the avoidance of the dialysis treatment. In this paper, we review what is new in the literature, analyzing indications, technique, and results of this surgical approach.


Assuntos
Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Humanos , Falência Renal Crônica/etiologia , Falência Renal Crônica/patologia , Seleção de Pacientes
17.
Transplant Proc ; 52(2): 608-613, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32085860

RESUMO

BACKGROUND: A higher prevalence of bladder cancer is reported in solid organ recipients, and advanced cancer requires radical cystectomy combined with urinary diversion. Surgery is technically challenging in kidney transplant recipients because of urinary tract abnormalities. Here, we describe the use of a robot-assisted approach in a kidney transplant recipient. CASE PRESENTATION: The etiology of the patient's end-stage renal disease was bilateral hypoplastic kidney. The patient started to receive hemodialysis at 19 years of age and underwent living-related kidney transplant at 23 years of age. Thirteen years later, he was diagnosed with invasive urothelial carcinoma and underwent robot-assisted radical cystectomy with extracorporeal neobladder construction under open laparotomy. Surgery was indicated to enhance suture flexibility and dissection of the peribladder tissues. Although the patient had an intraperitoneal infection caused by leakage from the vesicourethral anastomosis site and required drainage of the abscess, his condition stabilized after antibiotic treatment. CONCLUSION: This case outlines the effectiveness of the robot-assisted approach in patients with urinary tract abnormalities, such as kidney transplant recipients.


Assuntos
Cistectomia/métodos , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Procedimentos Cirúrgicos Robóticos/métodos , Derivação Urinária/métodos , Carcinoma de Células de Transição/complicações , Carcinoma de Células de Transição/cirurgia , Humanos , Falência Renal Crônica/etiologia , Masculino , Resultado do Tratamento , Neoplasias da Bexiga Urinária/complicações , Neoplasias da Bexiga Urinária/cirurgia , Adulto Jovem
18.
Transplant Proc ; 52(2): 614-618, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32057496

RESUMO

Renal transplantation is an ever-growing therapeutic option for patients with end-stage renal disease due to lupus nephritis. Outcomes for these patients are comparable to those of patients receiving renal transplantation for other causes. A known complication for these patients is recurrence of lupus nephritis in the renal graft (recurrent lupus nephritis [RLN]). Although disease severity at the time of recurrence is usually milder, a small number of cases have been reported to progress to allograft failure. There is a trend toward preemptive renal transplantation in patients with lupus nephritis, as more favorable outcomes have been observed with this treatment modality. While clinicians usually seek clinical remission of lupus prior to proceeding with renal transplantation, no guidelines are established regarding how often to check for serologic activity of lupus in patients with end-stage renal disease due to lupus nephritis and whether these serologic markers should be taken into account when deciding on the timing of transplantation. We present a case of early RLN co-occurring with acute cellular rejection 15 days after renal transplantation. The patient had been in clinical remission for more than 5 months prior to transplantation but had a rise in anti-double-stranded DNA antibody titers and a decrease in complement C3 level at the time of surgery. Although additional studies are needed to establish the extent to which serologic markers of lupus correlate with renal graft dysfunction, this case suggests hypocomplementemia and high double-stranded DNA antibody titers may be a risk factor for early RLN.


Assuntos
Anticorpos Antinucleares/sangue , Complemento C3/metabolismo , Transplante de Rim , Nefrite Lúpica/imunologia , Nefrite Lúpica/cirurgia , Rejeição de Enxerto , Humanos , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Nefrite Lúpica/patologia , Masculino , Recidiva , Fatores de Risco , Transplante Homólogo , Adulto Jovem
19.
Clin Nephrol ; 93(5): 243-250, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32101518

RESUMO

AIMS: We aim to describe the clinical and histological findings in patients with the finding of any tubular oxalate deposits in kidney biopsy specimens. BACKGROUND: The prevalence, manifestation, and outcome of secondary oxalate nephropathy have not been extensively studied. MATERIALS AND METHODS: In this retrospective cohort study, we analyzed the clinical and histological findings in all patients with the finding of any tubular oxalate deposits in kidney biopsy specimens between July 1, 2017, and December 31, 2018, at Northwell Health Pathology Department (Manhasset, NY, USA). RESULTS: The prevalence of oxalate deposition on a kidney biopsy was 4.07% (25/615), and in 88% of cases was a major finding. Prior to biopsy, oxalate was anticipated in only 1 case. The etiology of oxalosis was clarified retrospectively in 14 cases, most commonly due to GI surgery (n = 10) and increased oxalate intake (n = 4). In 11 cases, etiology remained unknown, although at least 3 cases were exposed to antibiotics associated with secondary oxalosis. There was no significant clinical/pathological or survival difference between known vs. unknown cause groups. The overall 3-month renal survival rate was 76.0 ± 8.5%. Multivariate Cox regression showed that creatinine at the time of biopsy (HR: 1.79, 95% CI: 0.71 - 4.51), background histological chronicity change (HR: 1.82, 95% CI: 0.70 - 4.72) and oxalate density (HR: 2.27, 95% CI: 0.49 - 10.55) are associated with end-stage kidney disease. CONCLUSION: Oxalate deposition is common but rarely anticipated biopsy finding. Nephrologists need to consider surgical history and other secondary causes of oxalosis as causes of acute kidney injury and chronic kidney disease.


Assuntos
Rim/metabolismo , Rim/patologia , Oxalatos/metabolismo , Idoso , Biópsia , Cristalização , Feminino , Humanos , Hiperoxalúria/complicações , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
20.
N Engl J Med ; 382(7): 622-631, 2020 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-32053298

RESUMO

BACKGROUND: More effective and safer treatments are needed for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. METHODS: We conducted a randomized trial with a 2-by-2 factorial design to evaluate the use of plasma exchange and two regimens of oral glucocorticoids in patients with severe ANCA-associated vasculitis (defined by an estimated glomerular filtration rate of <50 ml per minute per 1.73 m2 of body-surface area or diffuse pulmonary hemorrhage). Patients were randomly assigned to undergo plasma exchange (seven plasma exchanges within 14 days after randomization) or no plasma exchange (control group). Patients were also randomly assigned to follow either a standard-dose regimen or a reduced-dose regimen of oral glucocorticoids. Patients were followed for up to 7 years for the primary composite outcome of death from any cause or end-stage kidney disease (ESKD). RESULTS: Death from any cause or ESKD occurred in 100 of 352 patients (28.4%) in the plasma-exchange group and in 109 of 352 patients (31.0%) in the control group (hazard ratio, 0.86; 95% confidence interval [CI], 0.65 to 1.13; P = 0.27). The results were similar in subgroup analyses and in analyses of secondary outcomes. We also assessed the noninferiority of a reduced-dose regimen of glucocorticoids to a standard-dose regimen, using a noninferiority margin of 11 percentage points. Death from any cause or ESKD occurred in 92 of 330 patients (27.9%) in the reduced-dose group and in 83 of 325 patients (25.5%) in the standard-dose group (absolute risk difference, 2.3 percentage points; 90% CI, -3.4 to 8.0), which met the criterion for noninferiority. Serious infections at 1 year were less common in the reduced-dose group than in the standard-dose group (incidence rate ratio, 0.69; 95% CI, 0.52 to 0.93), but other secondary outcomes were similar in the two groups. CONCLUSIONS: Among patients with severe ANCA-associated vasculitis, the use of plasma exchange did not reduce the incidence of death or ESKD. A reduced-dose regimen of glucocorticoids was noninferior to a standard-dose regimen with respect to death or ESKD. (Funded by the U.K. National Institute for Health Research and others; PEXIVAS Current Controlled Trials number, ISRCTN07757494; ClinicalTrials.gov number, NCT00987389.).


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Glucocorticoides/administração & dosagem , Falência Renal Crônica/prevenção & controle , Troca Plasmática , Administração Oral , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/mortalidade , Terapia Combinada , Ciclofosfamida/uso terapêutico , Relação Dose-Resposta a Droga , Feminino , Glucocorticoides/efeitos adversos , Humanos , Imunossupressores/uso terapêutico , Incidência , Quimioterapia de Indução , Nefropatias/complicações , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Troca Plasmática/efeitos adversos , Rituximab/uso terapêutico
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