Article
Hematology
Xueqing Dou, Wenhui Zhang, Man-Chiu Poon, Xinsheng Zhang, Runhui Wu, Xiaoqin Feng, Linhua Yang, Peng Cheng, Shu Chen, Ying Wang, Hu Zhou, Meijuan Huang, Yanping Song, Chenghao Jin, Donglei Zhang, Lingling Chen, Wei Liu, Lei Zhang, Feng Xue, Renchi Yang
Summary: This study investigates the risk factors and management experience of factor FIX inhibitors in Chinese patients with hemophilia B. The researchers found that large deletions in the F9 gene were significantly associated with inhibitor development. Additionally, low-dose immune tolerance induction therapy may be a feasible approach for eradicating FIX inhibitors.
Article
Hematology
Jan Astermark, Katharina Holstein, Yasmina L. Abajas, Susan Kearney, Stacy E. Croteau, Riana Liesner, Eva Funding, Christine L. Kempton, Suchitra Acharya, Stefan Lethagen, Petra LeBeau, Joel Bowen, Erik Berntorp, Amy D. Shapiro
Summary: The study evaluates the use and outcome of ITI in patients with HB and inhibitors. The success of tolerization is lower in HB patients with inhibitors, with complications possibly correlated to mutation type and ITI outcomes.
Article
Urology & Nephrology
Pietro Ravani, Manuela Colucci, Maurizio Bruschi, Marina Vivarelli, Michela Cioni, Armando DiDonato, Paolo Cravedi, Francesca Lugani, Francesca Antonini, Marco Prunotto, Francesco Emma, Andrea Angeletti, Gian Marco Ghiggeri
Summary: In children with nephrotic syndrome maintained in remission with prednisone and calcineurin inhibitors, a single dose of ofatumumab was not superior to a single dose of rituximab in preventing relapse at 1 year or 24 months. B cell subpopulation reconstitution and adverse events were comparable between the two groups.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2021)
Article
Immunology
Miguel Fribourg, Michela Cioni, GianMarco Ghiggeri, Chiara Cantarelli, Jeremy S. Leventhal, Kelly Budge, Sofia Bin, Leonardo Riella, Manuela Colucci, Marina Vivarelli, Andrea Angeletti, Laura Perin, Paolo Cravedi
Summary: CyTOF analysis of immune cells from children with SDNS receiving B cell depleting therapy identified an expansion of class-switched B cell subsets in relapsing patients, while T cell subsets and other major immune compartments were largely unaffected by B cell depletion and similar between relapsing and stable children.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Medicine, General & Internal
Erik Berntorp, Kathelijn Fischer, Daniel P. Hart, Maria Elisa Mancuso, David Stephensen, Amy D. Shapiro, Victor Blanchette
Summary: Haemophilia A and B are rare congenital disorders caused by deficiency of clotting factors, leading to symptoms such as bleeding and joint disease. With advancements in treatment, individuals with haemophilia can now expect almost normal life expectancy and quality of life, although the development of inhibitory antibodies remains a major challenge for some patients. Gene therapy for haemophilia is progressing and may soon become a reality.
NATURE REVIEWS DISEASE PRIMERS
(2021)
Review
Immunology
Biswanath Basu, Andrea Angeletti, Bilkish Islam, Gian Marco Ghiggeri
Summary: Nephrotic proteinuria is a common characteristic of various glomerulonephritis conditions with different pathogenetic mechanisms. Monoclonal antibodies targeting CD20 have shown success in treating proteinuric conditions, but some cases are resistant. New human and humanized monoclonal antibodies offer advantages and may be effective in rituximab-resistant cases. Dose remains a question and further research is needed to determine the optimal dosage for different proteinuric renal conditions.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Multidisciplinary Sciences
Qing Ye, Chao Zhou, Sisi Li, Jingjing Wang, Fei Liu, Zhixia Liu, Jianhua Mao, Haidong Fu
Summary: This study analyzed the immune cell characteristics of patients with nephrotic syndrome, finding immune cell imbalances including T cells, B cells, Monocytes, and NK cells, confirming the involvement of immune factors in the pathogenesis of nephrotic syndrome.
SCIENTIFIC REPORTS
(2021)
Article
Hematology
Zekun Li, Guoqing Liu, Wanru Yao, Zhenping Chen, Gang Li, Xiaoling Cheng, Yingzi Zhen, Di Ai, Kun Huang, Jie Sun, Man-Chiu Poon, Runhui Wu
Summary: This study uses a low-dose ITI protocol combined with immunosuppressive therapy to treat HBI. Results show a higher risk of allergic reactions and complications during treatment, but effective eradication of inhibitors can be achieved.
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
Biotechnology & Applied Microbiology
Aparajitha Srinivasan, Roland W. Herzog, Imran Khan, Alexandra Sherman, Thais Bertolini, Tung Wynn, Henry Daniell
Summary: The study successfully created the first transplastomic lettuce lines expressing a coagulation factor without antibiotic resistance gene. Oral administration of plant cells expressing FIX fused to CTB suppressed ADA in animals with haemophilia B, suggesting a potential new approach for treatment.
PLANT BIOTECHNOLOGY JOURNAL
(2021)
Review
Urology & Nephrology
Ruth E. E. Campbell, Joshua M. M. Thurman
Summary: Idiopathic nephrotic syndrome often responds to immunosuppressive treatment, but remains a significant cause of patient morbidity. Minimal change disease and FSGS are the usual causes of the syndrome, primarily affecting podocytes. Although the underlying causes of both diseases are not completely understood, there is evidence linking the immune system to both diseases. Immunomodulatory drugs, including biologic agents, have shown efficacy in treating idiopathic nephrotic syndrome and have provided insights into the diseases' pathogenesis.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Review
Immunology
Manuela Colucci, Julie Oniszczuk, Marina Vivarelli, Vincent Audard
Summary: B cells play a crucial pathogenic role in idiopathic nephrotic syndrome by altering immune homeostasis and affecting podocyte structure and functions through the production of antibodies, expression of activating surface molecules, and release of specific cytokines. Despite their ability to protect against infections, dysregulated B cells are associated with various autoimmune diseases.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Chemistry, Multidisciplinary
Nan Zhang, Mengru Li, Ziye Hou, Lan Ma, Ayesha Younas, Ziyi Wang, Xinchi Jiang, Jianqing Gao
Summary: Rheumatoid arthritis (RA) is a difficult-to-cure joint-related autoimmune disease, and novel strategies targeting the root causes are needed. Nanotechnology is closely linked to vaccine development, offering potential for revolutionizing RA treatment.
JOURNAL OF CONTROLLED RELEASE
(2022)
Editorial Material
Urology & Nephrology
Tilman B. Drueke, Ziad A. Massy
Summary: Key contributors to anemia in patients with chronic kidney disease include insufficient erythropoietin production, decreased red blood cell half-life, iron deficiency, and inflammation. Proteinuria may alter erythrocyte metabolism and increase erythrocyte death in these patients.
KIDNEY INTERNATIONAL
(2021)
Article
Endocrinology & Metabolism
Agnes Sola-Gazagnes, Catherine Pecquet, Stefano Berre, Peter Achenbach, Laure-Anne Pierson, Isabelle Virmoux-Buisson, Jocelyne M'Bemba, Fabienne Elgrably, Philippe Moguelet, Christian Boitard, Sophie Caillat-Zucman, Moussa Laanani, Joel Coste, Etienne Larger, Roberto Mallone
Summary: The study aimed to develop a management workflow for diagnosing insulin allergy and evaluate the diagnostic performance of confirmatory tests. Clinical criteria were validated as effective diagnostic guides, and the IDR test showed the best diagnostic performance.