Aline Grosskopf Monich, John J Bissler, Fellype Carvalho Barreto
{"title":"Tuberous Sclerosis Complex and the kidneys: what nephrologists need to know.","authors":"Aline Grosskopf Monich, John J Bissler, Fellype Carvalho Barreto","doi":"10.1590/2175-8239-JBN-2024-0013en","DOIUrl":"10.1590/2175-8239-JBN-2024-0013en","url":null,"abstract":"<p><p>Tuberous sclerosis complex (TSC) is an autosomal dominant disease characterized by the development of hamartomas in the central nervous system, heart, skin, lungs, and kidneys and other manifestations including seizures, cortical tubers, radial migration lines, autism and cognitive disability. The disease is associated with pathogenic variants in the TSC1 or TSC2 genes, resulting in the hyperactivation of the mTOR pathway, a key regulator of cell growth and metabolism. Consequently, the hyperactivation of the mTOR pathway leads to abnormal tissue proliferation and the development of solid tumors. Kidney involvement in TSC is characterized by the development of cystic lesions, renal cell carcinoma and renal angiomyolipomas, which may progress and cause pain, bleeding, and loss of kidney function. Over the past years, there has been a notable shift in the therapeutic approach to TSC, particularly in addressing renal manifestations. mTOR inhibitors have emerged as the primary therapeutic option, whereas surgical interventions like nephrectomy and embolization being reserved primarily for complications unresponsive to clinical treatment, such as severe renal hemorrhage. This review focuses on the main clinical characteristics of TSC, the mechanisms underlying kidney involvement, the recent advances in therapy for kidney lesions, and the future perspectives.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240013"},"PeriodicalIF":1.3,"publicationDate":"2024-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11239183/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Valerie A Luyckx, Katherine R Tuttle, Dina Abdellatif, Ricardo Correa-Rotter, Winston W S Fung, Agnès Haris, Li-Li Hsiao, Makram Khalife, Latha A Kumaraswami, Fiona Loud, Vasundhara Raghavan, Stefanos Roumeliotis, Marianella Sierra, Ifeoma Ulasi, Bill Wang, Siu-Fai Lui, Vassilios Liakopoulos, Alessandro Balducci
{"title":"Mind the gap in kidney care: translating what we know into what we do.","authors":"Valerie A Luyckx, Katherine R Tuttle, Dina Abdellatif, Ricardo Correa-Rotter, Winston W S Fung, Agnès Haris, Li-Li Hsiao, Makram Khalife, Latha A Kumaraswami, Fiona Loud, Vasundhara Raghavan, Stefanos Roumeliotis, Marianella Sierra, Ifeoma Ulasi, Bill Wang, Siu-Fai Lui, Vassilios Liakopoulos, Alessandro Balducci","doi":"10.1590/2175-8239-JBN-2024-E007en","DOIUrl":"10.1590/2175-8239-JBN-2024-E007en","url":null,"abstract":"<p><p>Historically, it takes an average of 17 years for new treatments to move from clinical evidence to daily practice. Given the highly effective treatments now available to prevent or delay kidney disease onset and progression, this is far too long. Now is the time to narrow the gap between what we know and what we do. Clear guidelines exist for the prevention and management of common risk factors for kidney disease, such as hypertension and diabetes, but only a fraction of people with these conditions are diagnosed worldwide, and even fewer are treated to target. Similarly, the vast majority of people living with kidney disease are unaware of their condition, because it is often silent in the early stages. Even among patients who have been diagnosed, many do not receive appropriate treatment for kidney disease. Considering the serious consequences of kidney disease progression, kidney failure, or death, it is imperative that treatments are initiated early and appropriately. Opportunities to diagnose and treat kidney disease early must be maximized beginning at the primary care level. Many systematic barriers exist, ranging from the patient to the clinician to the health systems to societal factors. To preserve and improve kidney health for everyone everywhere, each of these barriers must be acknowledged so that sustainable solutions are developed and implemented without further delay.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e2024E007"},"PeriodicalIF":1.3,"publicationDate":"2024-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11239182/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141590319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Practices of dietitians in dialysis units in Brazil: nutritional assessment and intervention.","authors":"Viviane de Oliveira Leal, Denise Mafra","doi":"10.1590/2175-8239-JBN-2024-0112en","DOIUrl":"10.1590/2175-8239-JBN-2024-0112en","url":null,"abstract":"","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240112"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11379405/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142143059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transition between modalities of renal replacement therapy.","authors":"Thyago Proença de Moraes, Caio Pellizzari","doi":"10.1590/2175-8239-JBN-2024-E008en","DOIUrl":"10.1590/2175-8239-JBN-2024-E008en","url":null,"abstract":"","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e2024E008"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11379404/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142143060","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Erratum: Acute kidney injury: the experience of a tertiary center of Pediatric Nephrology.","authors":"","doi":"10.1590/2175-8239-JBN-2024-0012eren","DOIUrl":"10.1590/2175-8239-JBN-2024-0012eren","url":null,"abstract":"<p><p>[This corrects the article doi: 10.1590/2175-8239-JBN-2024-0012en] [This corrects the article doi: 10.1590/2175-8239-JBN-2024-0012pt].</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240012er"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11318159/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141897454","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gabriel Assis Lopes do Carmo, Mariana Paiva Oliveira, Anna Luiza Lino Campos, Bráulio Roberto Gonçalves Marinho Couto, Lilian Pires de Freitas do Carmo, Tiago Lemos Cerqueira, Camila Alencar Monteiro de Souza, Yan Lopes Goll, Vitor Santos de Souza, Mariana Oliveira Guimarães Vieira, Pedro Alves Soares Vaz de Castro, Pedro Augusto Botelho Lemos, Ana Cristina Simões E Silva
{"title":"COVID-19 Computed tomography patterns in renal replacement therapy patients.","authors":"Gabriel Assis Lopes do Carmo, Mariana Paiva Oliveira, Anna Luiza Lino Campos, Bráulio Roberto Gonçalves Marinho Couto, Lilian Pires de Freitas do Carmo, Tiago Lemos Cerqueira, Camila Alencar Monteiro de Souza, Yan Lopes Goll, Vitor Santos de Souza, Mariana Oliveira Guimarães Vieira, Pedro Alves Soares Vaz de Castro, Pedro Augusto Botelho Lemos, Ana Cristina Simões E Silva","doi":"10.1590/2175-8239-JBN-2023-0029en","DOIUrl":"10.1590/2175-8239-JBN-2023-0029en","url":null,"abstract":"<p><strong>Introduction: </strong>Lung diseases are common in patients with end stage kidney disease (ESKD), making differential diagnosis with COVID-19 a challenge. This study describes pulmonary chest tomography (CT) findings in hospitalized ESKD patients on renal replacement therapy (RRT) with clinical suspicion of COVID-19.</p><p><strong>Methods: </strong>ESKD individuals referred to emergency department older than 18 years with clinical suspicion of COVID-19 were recruited. Epidemiological baseline clinical information was extracted from electronic health records. Pulmonary CT was classified as typical, indeterminate, atypical or negative. We then compared the CT findings of positive and negative COVID-19 patients.</p><p><strong>Results: </strong>We recruited 109 patients (62.3% COVID-19-positive) between March and December 2020, mean age 60 ± 12.5 years, 43% female. The most common etiology of ESKD was diabetes. Median time on dialysis was 36 months, interquartile range = 12-84. The most common pulmonary lesion on CT was ground glass opacities. Typical CT pattern was more common in COVID-19 patients (40 (61%) vs 0 (0%) in non-COVID-19 patients, p < 0.001). Sensitivity was 60.61% (40/66) and specificity was 100% (40/40). Positive predictive value and negative predictive value were 100% and 62.3%, respectively. Atypical CT pattern was more frequent in COVID-19-negative patients (9 (14%) vs 24 (56%) in COVID-19-positive, p < 0.001), while the indeterminate pattern was similar in both groups (13 (20%) vs 6 (14%), p = 0.606), and negative pattern was more common in COVID-19-negative patients (4 (6%) vs 12 (28%), p = 0.002).</p><p><strong>Conclusions: </strong>In hospitalized ESKD patients on RRT, atypical chest CT pattern cannot adequately rule out the diagnosis of COVID-19.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20230029"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11300031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140174700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Carolina de Lima, Ana Claudia Siqueira Marques, Rodrigo Bueno de Oliveira
{"title":"An image of a giant brown tumor due to severe secondary hyperparathyroidism.","authors":"Ana Carolina de Lima, Ana Claudia Siqueira Marques, Rodrigo Bueno de Oliveira","doi":"10.1590/2175-8239-JBN-2024-0083en","DOIUrl":"10.1590/2175-8239-JBN-2024-0083en","url":null,"abstract":"","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240083"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11398717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142142982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maria Lúcia Vaz, Lúcio Requião-Moura, José Medina Pestana
{"title":"Hospital do Rim: 25 years as the global leader in kidney transplants.","authors":"Maria Lúcia Vaz, Lúcio Requião-Moura, José Medina Pestana","doi":"10.1590/2175-8239-JBN-2024-0076en","DOIUrl":"10.1590/2175-8239-JBN-2024-0076en","url":null,"abstract":"","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240076"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11318454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transitioning to peritoneal dialysis: it does not matter where you come from.","authors":"Diogo Francisco, Andreia Carnevale, Gonçalo Ávila, Ana Rita Calça, Patrícia Matias, Patrícia Branco","doi":"10.1590/2175-8239-JBN-2023-0139en","DOIUrl":"10.1590/2175-8239-JBN-2023-0139en","url":null,"abstract":"<p><strong>Introduction: </strong>Patients with end-stage renal disease (ESRD) frequently change renal replacement (RRT) therapy modality due to medical or social reasons. We aimed to evaluate the outcomes of patients under peritoneal dialysis (PD) according to the preceding RRT modality.</p><p><strong>Methods: </strong>We conducted a retrospective observational single-center study in prevalent PD patients from January 1, 2010, to December 31, 2017, who were followed for 60 months or until they dropped out of PD. Patients were divided into three groups according to the preceding RRT: prior hemodialysis (HD), failed kidney transplant (KT), and PD-first.</p><p><strong>Results: </strong>Among 152 patients, 115 were PD-first, 22 transitioned from HD, and 15 from a failing KT. There was a tendency for ultrafiltration failure to occur more in patients transitioning from HD (27.3% vs. 9.6% vs. 6.7%, p = 0.07). Residual renal function was better preserved in the group with no prior RRT (p < 0.001). A tendency towards a higher annual rate of peritonitis was observed in the prior KT group (0.70 peritonitis/year per patient vs. 0.10 vs. 0.21, p = 0.065). Thirteen patients (8.6%) had a major cardiovascular event, 5 of those had been transferred from a failing KT (p = 0.004). There were no differences between PD-first, prior KT, and prior HD in terms of death and technique survival (p = 0.195 and p = 0.917, respectively) and PD efficacy was adequate in all groups.</p><p><strong>Conclusions: </strong>PD is a suitable option for ESRD patients regardless of the previous RRT and should be offered to patients according to their clinical and social status and preferences.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20230139"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11287956/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140891174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hashimoto's thyroiditis masquerading as acute tubular injury and rhabdomyolysis.","authors":"Gerry George Mathew, Varadharajan Jayaprakash","doi":"10.1590/2175-8239-JBN-2024-0022en","DOIUrl":"10.1590/2175-8239-JBN-2024-0022en","url":null,"abstract":"<p><p>Hashimoto's thyroiditis manifesting as hypothyroidism has been implicated in glomerular disorders due to autoantibody formation. Here we present the case of a 26-year-old male without any comorbidities presenting with easy fatiguability and weight gain for 2 months. He was found to have a creatinine of 2.1 mg/dL with a history of rhinitis treated with anti-histaminic three days prior to the hospital visit. He had symptoms of intermittent myalgia for the past two weeks. On laboratory evaluation, he was found to have raised CPK, elevated TSH, low normal T4, and positive anti-TPO and anti-Tg antibodies. Neck ultrasound revealed linear echogenic septations in the thyroid gland. Renal biopsy revealed acute tubular injury. Appropriate thyroxine supplementation was started and his creatinine decreased to 1.2 mg/dL after 1 month. It is important that clinicians should be aware of this rare kidney presentation in Hashimoto's thyroiditis.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"46 3","pages":"e20240022"},"PeriodicalIF":1.3,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11318353/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}