Article
Biochemistry & Molecular Biology
Federica Casiraghi, Marta Todeschini, Manuel Alfredo Podesta, Marilena Mister, Barbara Ruggiero, Matias Trillini, Camillo Carrara, Olimpia Diadei, Alessandro Villa, Ariela Benigni, Giuseppe Remuzzi
Summary: Immune dysregulation plays a key role in the pathogenesis of steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS). However, no major B- or T-cell alterations have been described for adults, unlike in pediatric cases. This study found that patients with SDNS/FRNS have expansion of memory B cells and reduced memory Tregs. The levels of CD45RO(+) Tregs at baseline may help predict which patients will achieve sustained remission following rituximab infusion.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Immunology
Yan Cheng, Tian-yi Wang, Hong-li Yuan, Wei Li, Jing-ping Shen, Zheng-xin He, Jing Chen, Jie-ying Gao, Fu-kun Wang, Jiang Gu
Summary: This article provides an updated understanding of nocardiosis in immunosuppressed nephrotic syndrome patients receiving long-term glucocorticoid and immunosuppressant treatment. The study includes a literature review and case studies, and offers clinical and microbiological data to assist physicians in managing nocardiosis patients.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2022)
Article
Urology & Nephrology
Ho Jun Chin, Dong-Wan Chae, Yong Chul Kim, Won Suk An, ChunGyoo Ihm, Dong-Chan Jin, Sung Gyun Kim, Yong-Lim Kim, Yong-Soo Kim, Yoon-Goo Kim, Ho Seok Koo, Jung Eun Lee, Kang Wook Lee, Jieun Oh, Jung Hwan Park, Hongsi Jiang, Hyuncheol Lee, Sang Koo Lee
Summary: This study showed that combined therapy with tacrolimus and low-dose steroid was noninferior to high-dose steroid for inducing complete remission in adults with minimal change nephrotic syndrome. Additionally, relapse rates were significantly lower in patients maintained on tacrolimus and steroid compared to those on steroid alone, with no clinically-relevant safety differences observed.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2021)
Article
Pediatrics
Cheryl L. Tran, David T. Selewski, Gia J. Oh, Jonathan P. Troost, Susan F. Massengill, Samhar Al-Akash, Shefali Mahesh, Rasheda Amin, Isa F. Ashoor, Rahul Chanchlani, Mahmoud Kallash, Robert P. Woroniecki, Debbie S. Gipson
Summary: This study investigated the vaccination practices among pediatric patients with nephrotic syndrome, as well as the knowledge of parents and providers regarding vaccine recommendations. The results indicate that vaccine recommendations by medical providers significantly impact vaccine adherence in these patients, highlighting the need for improved education opportunities in this area.
FRONTIERS IN PEDIATRICS
(2021)
Review
Biochemistry & Molecular Biology
Anna Kawalec, Katarzyna Kilis-Pstrusinska
Summary: This article summarizes the current evidence on the impact of microbiota alterations on immune-mediated primary glomerulonephritis in children. It focuses on the link between dysbiosis and the onset or recurrence of idiopathic nephrotic syndrome, immunoglobulin A nephropathy, and membranous nephropathy. The aim is to describe possible pathomechanisms, differences in gut microbiota composition between pediatric patients and healthy controls, and the potential usage of microbiota manipulations in supportive therapy. It also highlights the need for further research in this field.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, General & Internal
Liping Rong, Lizhi Chen, Jia Rao, Qian Shen, Guomin Li, Jialu Liu, Jianhua Mao, Chunyue Feng, Xiaowen Wang, Si Wang, Xinyu Kuang, Wenyan Huang, Qingshan Ma, Xiaorong Liu, Chen Ling, Rong Fu, Xiaojie Gao, Guixia Ding, Huandan Yang, Mei Han, Zhimin Huang, Qian Li, Qiuye Zhang, Yi Lin, Xiaoyun Jiang, Hong Xu
Summary: The study investigated the genetic spectrum of NPHS1 variants in Chinese children with nephrotic syndrome, finding that the variants mainly cause congenital nephrotic syndrome and early onset nephrotic syndrome. Steroids and immunosuppressants may be effective treatments for selected patients.
FRONTIERS IN MEDICINE
(2021)
Article
Critical Care Medicine
Philip Alexander Efron, Dijoia B. Darden, Eric C. Li, Jennifer Munley, Lauren Kelly, Brittany Fenner, Dina C. Nacionales, Ricardo F. Ungaro, Marvin L. Dirain, Jaimar Rincon, Robert T. Mankowski, Christiaan Leeuwenburgh, Fredrick A. Moore, Scott C. Brakenridge, Thomas C. Foster, Orlando Laitano, Gemma Casadesus, Lyle L. Moldawer, Alicia M. Mohr, Ryan M. Thomas
Summary: This study found that male mice showed a decrease in gut microbiota diversity after chronic stress treatment, while female mice were able to recover their diversity after a certain period of time. This difference in recovery may explain the different outcomes between males and females after sepsis.
JOURNAL OF TRAUMA AND ACUTE CARE SURGERY
(2022)
Review
Biochemistry & Molecular Biology
Georgia Pitsava, Constantine A. Stratakis
Summary: The genetic basis of most types of adrenal adenomas has been studied, revealing associations with specific molecular defects. Genetic variants affecting the protein kinase A (PKA) signaling pathway have been linked to adrenal gland pathologies. Additionally, genes related to aldosterone production have been identified as causes of primary aldosteronism.
Article
Urology & Nephrology
Ana Maria Tato, Noa Carrera, Maria Garcia-Murias, Amir Shabaka, Ana Avila, Maria Teresa Mora Mora, Cristina Rabasco, Karina Soto, Francisco Jose de la Prada Alvarez, Loreto Fernandez-Lorente, Antolina Rodriguez-Moreno, Ana Huerta, Carmen Mon, Clara Garcia-Carro, Fayna Gonzalez Cabrera, Juan Antonio Martin Navarro, Ana Romera, Eduardo Gutierrez, Javier Villacorta, Alberto de Lorenzo, Beatriz Aviles, Miguel Angel Garca-Gonzalez, Gema Fernandez-Juarez, GLOSEN group
Summary: The frequency and distribution of genetic variants in patients with SRNS-FSGS and FSGS-UC were determined in this study. The results showed that genetic variants related to FSGS were detected regardless of the age of onset or family history of the disease. Therefore, genetic testing should be performed for patients with SRNS-FSGS or FSGS-UC, regardless of age or family history.
CLINICAL KIDNEY JOURNAL
(2023)
Article
Pediatrics
Zainab Arslan, Hazel Webb, Emma Ashton, Becky Foxler, Kjell Tullus, Aoife Waters, Detlef Bockenhauer
Summary: This study retrospectively analyzed children with non-syndromic primary SRNS followed at a center from 2005 to 2020. The results showed that 10% of the children had causative genetic variants, and they did not respond to immunosuppression and were more likely to progress to chronic kidney disease.
PEDIATRIC NEPHROLOGY
(2022)
Article
Medicine, General & Internal
Yunsi Li, Jin Chen, Yurong Zou, Wei Wang, Guisen Li
Summary: Lipoprotein glomerulopathy (LPG) is a rare glomerular disease caused by the deposition of lipoprotein thrombi in glomerular capillaries. Mutations in the APOE gene are primarily responsible for the disease. This case study demonstrates that compound heterogeneous mutations in the APOE gene inherited from both parents can lead to LPG. Intensive lipid-lowering therapy combined with RAS inhibitors is effective in treating LPG patients. Early renal biopsy and genetic mutation testing can help avoid unnecessary use of glucocorticoids and immunosuppressants.
Article
Urology & Nephrology
Daojing Ying, Wangkai Liu, Lizhi Chen, Liping Rong, Zhilang Lin, Sijia Wen, Hongjie Zhuang, Jinhua Li, Xiaoyun Jiang
Summary: Secondary steroid-resistant nephrotic syndrome (SRNS) refers to the condition when patients with initial steroid-sensitive nephrotic syndrome develop steroid resistance in subsequent relapses. The study found that no response to intensified immunosuppression (IIS) was an independent predictor for end-stage renal disease (ESRD) in patients with secondary SRNS.
KIDNEY INTERNATIONAL REPORTS
(2021)
Article
Nutrition & Dietetics
Turolo Stefano, Edefonti Alberto, Morello William, Bolzan Giulia, Syren M. Louise, Tamburello Chiara, Agostoni Carlo, Montini Giovanni
Summary: Patients with idiopathic nephrotic syndrome (INS) show differences in fatty acid profiles compared to healthy controls, with an increase in the pro-inflammatory omega-6 series. Adherence to the Mediterranean diet was associated with lower levels of linoleic acid and total omega-6 in patients, and a negative correlation was found between proteinuria and the anti-inflammatory omega-3 series.
Article
Medicine, General & Internal
Judy Savige, B. Andre Weinstock
Summary: Previously, genetic kidney disease was often recognized through shared clinical features among family members. However, now it is diagnosed through genetic testing that demonstrates a pathogenic variant in a disease-associated gene. Genetic diagnosis not only identifies the mode of inheritance and potential at-risk family members but also provides valuable information about complications, clinical course, and management strategies. Informed consent is necessary for genetic testing as it has significant implications for patients' and their families' lives, including employment and insurance, and carries social, ethical, and financial consequences. Patients seek understandable genetic test results and want their at-risk family members to undergo testing as well. Sharing anonymized results in registries contributes to the advancement of disease understanding and facilitates diagnoses in other families. Patient support groups play a crucial role in normalizing the disease, educating patients, and keeping them informed about recent advances and treatments. Some registries encourage patients to contribute their genetic variants, clinical features, and treatment responses. Additionally, patients increasingly volunteer for clinical trials of novel therapies, including those dependent on genetic diagnosis or variant identification.
FRONTIERS IN MEDICINE
(2023)
Article
Multidisciplinary Sciences
Most. Nazma Parvin, Md. Abdul Aziz, Sikder Nahidul Islam Rabbi, Mir Md. Abdullah Al-Mamun, Mohammed Hanif, Md. Saiful Islam, Mohammad Safiqul Islam
Summary: The study identified a significant association of ABCB1 and NR3C1 gene polymorphisms with prednisolone-resistant pediatric nephrotic syndrome, while other gene polymorphisms showed no significant association with resistance risk.
JOURNAL OF ADVANCED RESEARCH
(2021)
Article
Pediatrics
Filippo Ghidini, Francesco Fascetti Leon, Federica De Corti, Davide Meneghesso, Germana Longo, Alberto Sgro, Stefania Michelon, Salvatore Metrangolo, Luisa Meneghini, Marco Castagnetti, Elisa Benetti, Piergiorgio Gamba, Patrizia Dall'Igna
Summary: This study investigated the impact of complex bench surgery on the outcomes of pediatric kidney transplantation. The results showed that complex bench surgery did not compromise graft survival or increase the risk of vascular complications.
EUROPEAN JOURNAL OF PEDIATRIC SURGERY
(2023)
Article
Transplantation
Lyuben Lyubenov, Chongxu Shi, Danyang Zhao, Luying Yang, Yutian Lei, Elmina Mammadova-Bach, Letizia de Chiara, Roberto Semeraro, Samuela Landini, Paola Romagnani, Elena Voerg, Satish K. Devarapu, Ricarda Welz, Stephan T. Kiessig, Hans-Joachim Anders
Summary: Cholesterol crystal (CC) embolism leads to acute kidney injury (AKI) and ischaemic cortical necrosis with high mortality. Injection of Glu-plasminogen (Glu-Plg) showed a dose-dependent attenuation of thrombotic angiopathy, GFR loss, and ischaemic necrosis in mice with CC embolism. Intermediate dose of Glu-Plg had transient protective effect, while high dose provided full protection without bleeding complications.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Article
Endocrinology & Metabolism
F. Magri, C. Bellingeri, I. De Maggio, L. Croce, F. Coperchini, M. Rotondi, L. Chiovato, A. Spinillo, F. Beneventi
Summary: The purpose of this study was to explore the impact of mild subclinical hypothyroidism on pregnancy outcomes in TPOAb-negative women. The results showed that a first-trimester TSH level ranging from 4 to 10 mIU/L was significantly associated with an increased uterine artery pulsatility index and an increased risk of pre-eclampsia, small for gestational age, and gestational diabetes.
JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION
(2023)
Review
Biochemistry & Molecular Biology
Carolina Conte, Giulia Antonelli, Maria Elena Melica, Mirko Tarocchi, Paola Romagnani, Anna Julie Peired
Summary: Chronic kidney disease (CKD) is a global health burden affecting over 840 million people worldwide. It presents sex disparities in its pathophysiology, epidemiology, clinical manifestations, and disease progression. While CKD is more frequent in females, males have a higher risk of progressing to end-stage kidney disease. Recent studies have highlighted the role of sex hormones in the development and progression of CKD.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Transplantation
Paola Romagnani, A. Richard Kitching, Nelson Leung, Hans-Joachim Anders
Summary: Glomerulonephritis (GN) is a diverse group of immune-mediated disorders, which cannot be properly classified or treated based on histological patterns alone. To overcome this problem, a new classification system is proposed that considers disease category, immunological activity, and chronicity, which will guide the therapeutic approach.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Review
Endocrinology & Metabolism
S. Pinto, L. Croce, L. Carlier, E. Cosson, M. Rotondi
Summary: This review provides an overview of the peculiar aspects of gestational diabetes mellitus (GDM) and gestational thyroid dysfunction (GTD), highlighting the potential interactions and clinical consequences of these two frequent clinical conditions.
JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION
(2023)
Article
Medicine, General & Internal
Laura Croce, Spyridon Chytiris, Francesca Coperchini, Giovanni Ferraro, Linda Minelli, Antonella Navarra, Flavia Magri, Luca Chiovato, Pierpaolo Trimboli, Mario Rotondi
Summary: This study aimed to evaluate potential strategies for clinical and biochemical characterization of UH patients. The results showed that laboratory interferences are not more frequent in UH patients, suggesting that they should be managed in the same way as CAT patients until proven otherwise.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Marco Allinovi, Francesco Sessa, Gianluca Villa, Andrea Cocci, Samantha Innocenti, Maria Zanazzi, Lorenzo Tofani, Laura Paparella, Dritan Curi, Calogero Lino Cirami, Riccardo Campi, Andrea Mari, Agostino Ognibene, Maria Lorubbio, Alessandra Fanelli, Stefano Romagnoli, Paola Romagnani, Andrea Minervini
Summary: The study aimed to identify and compare serum and urinary predictors for predicting long-term decline in glomerular filtration rate (GFR) after robotic Nephron-Spearing Surgery (rNSS). The results showed that patients who developed clinical acute kidney injury (AKI) had a more pronounced decline in eGFR at 24 months. KineticGFR at 4 hours and NephroCheck at 10 hours were found to be efficient predictors of post-operative AKI and long-term eGFR decline. Combining these biomarkers could help identify high-risk patients as early as 10 hours after surgery.
Article
Engineering, Biomedical
Vincenzo Tarzia, Nicola Pradegan, Matteo Ponzoni, Mattia Parolin, Paola Gaio, Davide Meneghesso, Mara Cananzi, Elisa Benetti, Michele Battistel, Fabio Zanella, Gino Gerosa
Summary: This study reported the successful use of the AngioVac System combined with extracorporeal membrane oxygenation to treat and remove clots and infection material in pediatric and adolescent patients. Follow-up results showed no recurrence of pathological material in both cases.
Article
Urology & Nephrology
Zhihui Zhu, Karoline A. T. Rosenkranz, Yoshihiro Kusunoki, Chenyu Li, Martin Klaus, Oliver Gross, Maria-Lucia Angelotti, Giulia Antonelli, Luigi Cirillo, Paola Romagnani, Nassim Bouteldja, Alireza Vafaei Sadr, Roman D. Buelow, Peter Boor, Hans-Joachim Anders
Summary: A preclinical study in mice suggests that triple blockade of RAS/SGLT2/MR may significantly improve renal outcomes in Alport syndrome and other progressive CKDs due to synergistic effects on the glomerular and tubulointerstitial compartments.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2023)
Article
Endocrinology & Metabolism
L. Croce, R. M. Ruggeri, C. Cappelli, C. Virili, F. Coperchini, M. Lagana, P. Costa, M. Dal Molin, S. Chytiris, F. Magri, L. Chiovato, M. Centanni, S. Cannavo, M. Rotondi
Summary: The aim of this study was to investigate the reason leading to the diagnosis of thyroid nodules (TN) and to compare the clinical characteristics of patients diagnosed incidentally with those of patients diagnosed for thyroid-related reasons.
JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION
(2023)
Article
Health Care Sciences & Services
Isabella Chiardi, Mario Rotondi, Marco Cantu, Franco Keller, Pierpaolo Trimboli
Summary: Macro-thyroid-stimulating hormone (macro-TSH) is a rare phenomenon that deserves attention in the field of thyroid. This case report describes a patient with consistently elevated TSH levels, but normal thyroid hormone and ultrasound results. Laboratory studies confirmed the diagnosis of macro-TSH. This case highlights the importance of considering macro-TSH as a potential cause in cases of unexplained TSH elevation, and expands our understanding of thyroid function anomalies.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Article
Endocrinology & Metabolism
Laura Croce, Cristina Pallavicini, Noemi Busca, Benedetto Cali, Giuseppe Bellastella, Francesca Coperchini, Flavia Magri, Luca Chiovato, Hellas Cena, Mario Rotondi
Summary: A prospective study was conducted to assess the efficacy of dietetic intervention in preventing post-thyroidectomy weight gain. The results showed that personalized pre-surgery diet counseling was effective in preventing weight gain after thyroidectomy.
Article
Environmental Sciences
Francesca Coperchini, Giuseppina De Marco, Laura Croce, Marco Denegri, Alessia Greco, Flavia Magri, Massimo Tonacchera, Marcello Imbriani, Mario Rotondi, Luca Chiovato
Summary: Industrial chemical PFAS are persistent pollutants. The effects of different PFAS on cell viability, proliferation, and secretion of CXCL8 in normal and cancer thyroid cells were evaluated. The specific cell type and target analyte showed strikingly different responses to individual PFAS. The results indicate that in vitro exposure to PFAS can modulate both mRNA and secreted protein levels of CXCL8.
ENVIRONMENTAL SCIENCE AND POLLUTION RESEARCH
(2023)
Article
Endocrinology & Metabolism
Rosa di Fraia, Daniela Esposito, Lucia Digitale Selvaggio, Francesca Allosso, Roberto Alfano, Mario Rotondi, Giancarlo Balercia, Giacomo Accardo, Daniela Pasquali
Summary: Compared to the control group, there is a significantly higher prevalence of nodular thyroid disease in patients with Klinefelter syndrome (KS). This increase is likely linked to low levels of FT4, inappropriate TSH secretion, and/or genetic instability.