keyword
https://read.qxmd.com/read/38527446/kidney-failure-secondary-to-hereditary-xanthinuria-due-to-a-homozygous-deletion-of-the-xdh-gene-in-the-absence-of-overt-kidney-stone-disease
#1
Pedro Lisboa Gonçalves, Hugo Diniz, Isabel Tavares, Sofia Dória, Juan Dong, McKenna Kyriss, Lynette Fairbanks, João Paulo Oliveira
Hereditary xanthinuria (HXAN) is a rare metabolic disorder that results from mutations in either the xanthine dehydrogenase (XDH) or the molybdenum cofactor sulfurase genes (MOCOS), respectively defining HXAN type I and type II. Hypouricemia, hypouricosuria, and abnormally high plasma and urine levels of xanthine, causing susceptibility to xanthine nephrolithiasis and deposition of xanthine crystals in tissues, are the metabolic hallmarks of HXAN. Several pathogenic variants in the XDH gene have so far been identified in patients with HXAN type I, but the clinical phenotype associated with the whole deletion of the human XDH gene is unknown...
March 25, 2024: Nephron
https://read.qxmd.com/read/37682181/survival-and-prognosis-analysis-of-systemic-lupus-erythematosus-patients-with-pulmonary-hypertension-a-systematic-review-and-meta-analysis
#2
JOURNAL ARTICLE
Jianguo Yang, Fuyu Zhou, Xinpeng Zhou, Yuying Sun, Xueping Lun, Jiaojiao Cao, Bing Fan
BACKGROUND: The study aimed to evaluate survival rates and prognosis in systemic lupus erythematosus (SLE) patients with pulmonary hypertension (PH) using meta-analysis. METHODS: PubMed, EMBASE, Cochrane central register of controlled trials, China National Knowledge Infrastructure, China Science and Technology Journal Database, Wan-Fang Database, and Chinese biomedical database were searched. Information and data were screened and extracted by 2 researchers. The obtained data were analyzed using the R software meta package...
September 8, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/37597335/clinical-and-biochemical-footprints-of-inherited-metabolic-diseases-xiv-metabolic-kidney-diseases
#3
JOURNAL ARTICLE
Anke Schumann, Ulla T Schultheiss, Carlos R Ferreira, Nenad Blau
Kidney disease is a global health burden with high morbidity and mortality. Causes of kidney disease are numerous, extending from common disease groups like diabetes and arterial hypertension to rare conditions including inherited metabolic diseases (IMDs). Given its unique anatomy and function, the kidney is a target organ in about 10% of known IMDs, emphasizing the relevant contribution of IMDs to kidney disease. The pattern of injury affects all segments of the nephron including glomerular disease, proximal and distal tubular damage, kidney cyst formation, built-up of nephrocalcinosis and stones as well as severe malformations...
August 12, 2023: Molecular Genetics and Metabolism
https://read.qxmd.com/read/37506667/fatal-cerebrovascular-accident-in-a-captive-red-panda-ailurus-fulgens-fulgens-with-concurrent-amdoparvovirus-infection
#4
JOURNAL ARTICLE
Cecilia Gola, Pavel Kvapil, Urska Kuhar, Josué Diaz-Delgado, Charles E Alex, Justine Shotton, Sarah J Smith, Sai Fingerhood
We report the pathological and molecular findings in an adult male Himalayan red panda (Ailurus fulgens fulgens) whose death was attributed to parenchymal brain haemorrhage (PBH) of the thalamus. Post-mortem examination revealed severe, acute PBH and intraventricular haemorrhage with major involvement of the thalamus, as well as scattered chronic microinfarctions. Vascular disease in the brain and other organs was suggestive of systemic hypertension. Histological lesions included arteriolar hyalinosis and varying degrees of arteriosclerosis, arterial tunica media hypertrophy and hyperplasia and infiltration of arterial walls by lipid-laden macrophages...
July 26, 2023: Journal of Comparative Pathology
https://read.qxmd.com/read/36668916/prevalence-of-jc-and-bk-polyomavirus-infection-in-patients-with-chronic-kidney-disease-in-the-state-of-par%C3%A3-brazil
#5
JOURNAL ARTICLE
Scheila do Socorro Vasconcelos Ávila da Costa, Jacqueline Cortinhas Monteiro, Ana Paula do Vale Viegas, Keyla Santos Guedes de Sá, Silvia Regina da Cruz, Sandra Souza Lima, Izaura Maria Vieira Cayres Vallinoto, Igor Brasil Costa, Antonio Carlos Rosário Vallinoto
The polyomaviruses that infect humans, JC virus (JCV) and BK virus (BKV), can establish persistent infections in the cells that make up the renal system, causing nephritis and BKV-associated nephropathy in up to 10% of renal transplant patients, and of these, 90% lose the graft and return for hemodialysis. This study aimed to determine the prevalence of polyomaviruses (PyV) in the population with chronic kidney disease (CKD), classified into three groups (conservative, dialysis, and transplanted) and a control group...
December 23, 2022: Tropical Medicine and Infectious Disease
https://read.qxmd.com/read/36571535/-acute-myocardial-infarction-in-a-young-woman-with-systemic-lupus-erythematosus
#6
JOURNAL ARTICLE
Giuliana A Tresenza, Luciana J Puente, Carlos J Colla, Miguel F Albornoz, Lucía R Kazelian
We repor a case of acute ST elevation myocardial infarction in a 22-year-old patient with SLE, hypertension and nephropathy who underwent successful coronary angioplasty to a middle third of the left anterior descending artery. She evolved without signs of heart failure however, due to the delay in diagnosis, she presented severe deterioration of ventricular function. ST segment elevation myocardial infarction is a very rare event in young premenopausal women, but compared to the general population, patients with lupus have at least a 50% higher risk of suffering it regardless their age...
2022: Medicina
https://read.qxmd.com/read/36409367/chronic-kidney-disease-in-patients-with-childhood-onset-systemic-lupus-erythematosus
#7
JOURNAL ARTICLE
Ana P Sakamoto, Clovis A Silva, Aline G Islabão, Glaucia V Novak, Beatriz Molinari, Paulo K Nogueira, Rosa M R Pereira, Claudia Saad-Magalhães, Gleice Clemente, Daniela P Piotto, Nadia E Aikawa, Ana C Pitta, Vitor C Trindade, Simone Appenzeller, Luciana M Carvalho, Carlos N Rabelo-Junior, Adriana R Fonseca, Flavio R Sztajnbok, Maria C Santos, Blanca E Bica, Evaldo G Sena, Ana J Moraes, Melissa M Fraga, Teresa C Robazzi, Paulo F Spelling, Iloite M Scheibel, Andre S Cavalcanti, Erica N Matos, Luciano J Guimarães, Flavia P Santos, Licia M H Mota, Eloisa Bonfá, Maria T Terreri
BACKGROUND: Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients. METHODS: We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients...
June 2023: Pediatric Nephrology
https://read.qxmd.com/read/36293172/cathepsin-s-inhibition-suppresses-experimental-systemic-lupus-erythematosus-associated-pulmonary-arterial-remodeling
#8
JOURNAL ARTICLE
Tzung-Hai Yen, Wan-Jing Ho, Yung-Hsin Yeh, Ying-Ju Lai
Patients with systemic lupus erythematosus (SLE) associated with pulmonary arterial hypnertension (PAH) receive targeted therapy for PAH to decrease pulmonary arterial systolic pressure and significantly prolong their survival. Cysteine cathepsin proteases play critical roles in the progression of cardiovascular disease. Inhibition of cathepsin S (Cat S) has been shown to improve SLE and lupus nephritis. However, the effect of Cat S inhibitors on SLE-associated PAH (SLE-PAH) remains unclear, and there is no animal model for translational research on SLE-PAH...
October 14, 2022: International Journal of Molecular Sciences
https://read.qxmd.com/read/36063165/-lupus-nephritis
#9
JOURNAL ARTICLE
Johanna Mucke, Matthias Schneider
Lupus nephritis (LN) is one of the most frequent organ manifestations of systemic lupus erythematosus. Urine analysis is suitable for screening and proteinuria or an active sediment with acanthocytes can be indicative for LN. The gold standard for confirming the diagnosis is a kidney biopsy. The type and extent of the histological alterations are decisive for treatment. The LN is histologically classified into six classes, whereby classes III, IV and V in particular require immunosuppressive treatment. The treatment of LN consists of the administration of hydroxychloroquine, angiotensin-converting enzyme (ACE) inhibitors for nephroprotection and further antihypertensive drugs in cases of arterial hypertension...
February 2023: Zeitschrift Für Rheumatologie
https://read.qxmd.com/read/36027365/course-of-preeclampsia-in-chronic-kidney-disease-ckd
#10
JOURNAL ARTICLE
Maria Alekseeva, Ksenia Demyanova, Natalya Kozlovskaya, Yulia Korotchaeva, Ayana Chegodaeva, Sergey Apresyan, Zhanna Kobalava
OBJECTIVE: The aim of the study is to analyze the incidence and characteristics of PE in patients (pts) with CKD. DESIGN AND METHOD: A retrospective analysis of 60 case histories of pregnant women with CKD 1-4 stages followed in the nephrology center for pregnant women from 2018-2021 was carried out. 20 of them developed PE: CKD 1 - 7/23 (30,4%), CKD 2 - 3/10 (30%), CKD 3 - 7/23 (30,4%), CKD 4 - 3/4 (75%), then the course of their pregnancy was analyzed. Indicators of proteinuria (PU) and blood pressure (BP) were assessed at the time of the first visit to the center and further, at the time of PE...
June 1, 2022: Journal of Hypertension
https://read.qxmd.com/read/35902128/acute-kidney-injury-in-patients-with-liver-disease
#11
JOURNAL ARTICLE
Giuseppe Cullaro, Swetha Rani Kanduri, Juan Carlos Q Velez
AKI is commonly encountered in patients with decompensated cirrhosis, and it is associated with unfavorable outcomes. Among factors specific to cirrhosis, hepatorenal syndrome type 1, also referred to as hepatorenal syndrome-AKI, is the most salient and unique etiology. Patients with cirrhosis are vulnerable to traditional causes of AKI, such as prerenal azotemia, acute tubular injury, and acute interstitial nephritis. In addition, other less common etiologies of AKI specifically related to chronic liver disease should be considered, including abdominal compartment syndrome, cardiorenal processes linked to cirrhotic cardiomyopathy and portopulmonary hypertension, and cholemic nephropathy...
November 2022: Clinical Journal of the American Society of Nephrology: CJASN
https://read.qxmd.com/read/35551744/renal-tlr7-expression-is-associated-with-renal-injury-in-female-mouse-model-of-systemic-lupus-erythematosus
#12
JOURNAL ARTICLE
Sarika Chaudhari, Bradley M D'Souza, Jessica Y Morales, Caroline Lima, Cassandra M Young-Stubbs, Rong Ma, Keisa W Mathis
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disorder associated with exaggerated immune activation, autoantibody production, and immune complex formation. SLE patients are predominantly women of reproductive age that often present with end organ damage, specifically in the kidneys, and hypertension. This detrimental sequelae is likely due to deposition of the immune complexes and the resulting inflammation, but the exact mechanisms are unknown. It is known however that immune complexes activate toll like receptors (TLRs) on immune cells and TLR7 particularly is known to promote the development and acceleration of the pathogenesis of SLE...
May 2022: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://read.qxmd.com/read/35529329/working-towards-a-treat-to-target-protocol-in-juvenile-proliferative-lupus-nephritis-a-survey-of-pediatric-rheumatologists-and-nephrologists-in-germany-and-austria
#13
JOURNAL ARTICLE
Kristina Vollbach, Catharina Schuetz, Christian M Hedrich, Fabian Speth, Kirsten Mönkemöller, Jürgen Brunner, Ulrich Neudorf, Christoph Rietschel, Anton Hospach, Tilmann Kallinich, Claas Hinze, Norbert Wagner, Burkhard Tönshoff, Lutz T Weber, Kay Latta, Julia Thumfart, Martin Bald, Dagobert Wiemann, Hildegard Zappel, Klaus Tenbrock, Dieter Haffner
Background: To describe treatment practices for juvenile proliferative lupus nephritis (LN) class III and IV of pediatric rheumatologists and nephrologists in Germany and Austria in preparation for a treat-to-target treatment protocol in LN. Methods: Survey study by members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Pediatric Nephrology (GPN) on diagnostics and (concomitant) therapy of LN. Results: Fifty-eight physicians completed the survey...
2022: Frontiers in Pediatrics
https://read.qxmd.com/read/35510378/the-impact-of-preeclampsia-in-lupus-nephritis
#14
JOURNAL ARTICLE
Gabriella Moroni, Marta Calatroni, Claudio Ponticelli
INTRODUCTION: Women with systemic lupus erythematosus (SLE), particularly those with lupus nephritis (LN), remain at high risk for adverse pregnancy outcome. Although in the last decades maternal and fetal outcomes have improved dramatically, preeclampsia remains a major cause of maternal and perinatal morbidity and mortality. AREAS COVERED: A narrative review of literature was conducted, underlying the importance of pre-conception counseling, and focusing on the correlation between preeclampsia and LN...
May 10, 2022: Expert Review of Clinical Immunology
https://read.qxmd.com/read/35407573/predictors-associated-with-adverse-pregnancy-outcomes-in-a-cohort-of-women-with-systematic-lupus-erythematosus-from-romania-an-observational-study-stage-2
#15
JOURNAL ARTICLE
Petronela Vicoveanu, Ingrid-Andrada Vasilache, Dragos Nemescu, Alexandru Carauleanu, Ioana-Sadiye Scripcariu, Dorina Rudisteanu, Alexandra Burlui, Elena Rezus, Demetra Socolov
BACKGROUND: Pregnancy in women with systemic lupus erythematosus (SLE) is accompanied by adverse pregnancy outcomes (APOs). We aimed to investigate the association between clinical, sonographic, and laboratory parameters and APOs (preeclampsia, intrauterine growth restriction, premature birth, and maternal mortality). METHODS: This observational retrospective study included all pregnancies in women with SLE who attended two tertiary maternity hospitals from Romania between January 2013 and December 2020...
April 1, 2022: Journal of Clinical Medicine
https://read.qxmd.com/read/35368572/beyond-isn-rps-lupus-nephritis-classification-adding-chronicity-index-to-clinical-variables-predicts-kidney-survival
#16
JOURNAL ARTICLE
Gabriella Moroni, Giulia Porata, Francesca Raffiotta, Silvana Quaglini, Giulia Frontini, Lucia Sacchi, Valentina Binda, Marta Calatroni, Francesco Reggiani, Giovanni Banfi, Claudio Ponticelli
Background: A renewed interest for activity and chronicity indices as predictors of lupus nephritis (LN) outcome has emerged. Revised National Institutes of Health activity and chronicity indices have been proposed to classify LN lesions, but they should be validated by future studies. The aims of this study were ( 1 ) to detect the histologic features associated with the development of kidney function impairment (KFI), and ( 2 ) to identify the best clinical-histologic model to predict KFI at time of kidney biopsy...
January 27, 2022: Kidney360
https://read.qxmd.com/read/34779387/serum-levels-of-plasminogen-activator-urokinase-receptor-and-cardiotrophin-like-cytokine-factor-1-in-patients-with-nephrotic-syndrome
#17
JOURNAL ARTICLE
Natalia Chebotareva, Anatoliy Vinogradov, Venzsin Cao, Alla Gindis, Angelina Berns, Igor Alentov, Natalia Sergeeva
The pathogenesis of primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) remains unknown to date. Some circulating permeability factors are being discussed. This work assessed molecule candidates for permeability in serum samples of patients with nephrotic syndrome (NS). MATERIALS AND METHODS: 41 patients with chronic glomerulonephritis (CGN) were included in our study. 17 patients had FSGS, 7 patients had MCD, 5 patients had membranoproliferative glomerulonephritis (MPGN), 6 patients had IgA nephropathy, and 6 patients had membranous nephropathy (MN)...
February 2022: Clinical Nephrology
https://read.qxmd.com/read/34727096/glucocorticoids-induce-partial-remission-of-focal-segmental-glomerulosclerosis-but-not-interstitial-nephritis-in-covid-19-acute-kidney-injury-in-an-apol1-low-risk-genotype-white-patient
#18
JOURNAL ARTICLE
Piotr J Nowak, Joanna Forycka, Natalia Cegielska, Karolina Harendarz, Małgorzata Wągrowska-Danilewicz, Marian Danilewicz, Tomasz Płoszaj, Maciej Borowiec, Rafał Wlazeł, Michał Nowicki
BACKGROUND COVID-19 can be complicated by kidney disease, including focal segmental glomerulosclerosis (FSGS), interstitial nephritis, and acute kidney injury (AKI). Almost all known cases of COVID-19-associated glomerulonephritis have been in patients of African descent, with G1 or G2 apolipoprotein L1 (APOL1) risk alleles, and they presented collapsing type of FSGS. CASE REPORT We report a case of biopsy-confirmed non-collapsing FSGS with secondary acute interstitial nephritis and AKI in a young White man with APOL1 low-risk genotype, who had COVID-19 pneumonia...
November 2, 2021: American Journal of Case Reports
https://read.qxmd.com/read/34697868/variations-in-clinical-presentation-and-biomarkers-amongst-biopsy-proven-lupus-nephritis-patients-a-top-end-retrospective-cohort-study
#19
JOURNAL ARTICLE
Chi Xu, Catherine Clarke, Kim Ling Goh, Asanga Abeyaratne, Manohar Mogulla, William Majoni, Kelum Priyadarshana
BACKGROUND: Lupus nephritis (LN) is common feature of Systemic Lupus Erythematosus (SLE) and affects 50% of patients with SLE. Racial differences in incidence and prevalence have been well documented worldwide. In Australia, higher incidence and prevalence of SLE had been previously reported in Aboriginal and Torres Strait Australians compared to non-Indigenous Australians. AIM: to describe the differences in clinical features and lupus biomarkers between Aboriginal and Torres Strait Islander Australian and non-Indigenous Australian LN patients...
October 25, 2021: Internal Medicine Journal
https://read.qxmd.com/read/34599036/renal-denervation-exacerbates-lps-and-antibody-induced-acute-kidney-injury-but-protects-from-pyelonephritis-in-mice
#20
JOURNAL ARTICLE
Alexander M C Böhner, Alice M Jacob, Christoph Heuser, Natascha E Stumpf, Alexander Effland, Zeinab Abdullah, Catherine Meyer-Schwesiger, Sibylle von Vietinghoff, Christian Kurts
BACKGROUND: Renal denervation (RDN) is an invasive intervention to treat drug-resistant arterial hypertension. Its therapeutic value is contentious. Here we examined the effects of RDN on inflammatory and infectious kidney disease models in mice. METHODS: Mice were unilaterally or bilaterally denervated, or sham operated, then three disease models were induced: nephrotoxic nephritis (NTN, a model for crescentic GN), pyelonephritis, and acute endotoxemic kidney injury (as a model for septic kidney injury)...
October 2021: Journal of the American Society of Nephrology: JASN
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