The role of the immune system in idiopathic nephrotic syndrome: a review of clinical and experimental studies

2013 ◽  
Vol 63 (1) ◽  
pp. 1-12 ◽  
Author(s):  
Wagner de Fátima Pereira ◽  
Gustavo Eustáquio Alvim Brito-Melo ◽  
Fábio Tadeu Lourenço Guimarães ◽  
Thiago Guimarães Rosa Carvalho ◽  
Elvis Cueva Mateo ◽  
...  
2004 ◽  
Vol 107 (2) ◽  
pp. 125-136 ◽  
Author(s):  
José G. van den BERG ◽  
Jan J. WEENING

Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins. Genetic defects that affect the function and the composition of the glomerular capillary wall, in particular of the visceral epithelial cells, have recently been recognized as the cause of familial forms of NS. MCNS (minimal change NS) and FSGS (focal and segmental glomerulosclerosis) are common non-familial forms of NS in which the causative defect has not yet been identified. Several studies have shown that non-familial NS is associated with the presence of circulating permeability factors and with complex disturbances in the immune system. Thus far, there is no direct evidence that these factors directly alter glomerular permeability to proteins, and some of these factors may be a consequence, rather than a cause, of NS. In this review, we will briefly highlight the mechanisms that underlie proteinuria in general and focus on the immunological disturbances associated with idiopathic NS, with attention to potential mechanisms whereby the immune system may directly act on the glomerular capillary filter.


2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Agnes Hackl ◽  
Seif El Din Abo Zed ◽  
Paul Diefenhardt ◽  
Julia Binz-Lotter ◽  
Rasmus Ehren ◽  
...  

AbstractIdiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia and usually responds well to steroids. However, relapses are frequent, which can require multi-drug therapy with deleterious long-term side effects. In the last decades, different hypotheses on molecular mechanisms underlying INS have been proposed and several lines of evidences strongly indicate a crucial role of the immune system in the pathogenesis of non-genetic INS. INS is traditionally considered a T-cell-mediated disorder triggered by a circulating factor, which causes the impairment of the glomerular filtration barrier and subsequent proteinuria. Additionally, the imbalance between Th17/Tregs as well as Th2/Th1 has been implicated in the pathomechanism of INS. Interestingly, B-cells have gained attention, since rituximab, an anti-CD20 antibody demonstrated a good therapeutic response in the treatment of INS. Finally, recent findings indicate that even podocytes can act as antigen-presenting cells under inflammatory stimuli and play a direct role in activating cellular pathways that cause proteinuria. Even though our knowledge on the underlying mechanisms of INS is still incomplete, it became clear that instead of a traditionally implicated cell subset or one particular molecule as a causative factor for INS, a multi-step control system including soluble factors, immune cells, and podocytes is necessary to prevent the occurrence of INS. This present review aims to provide an overview of the current knowledge on this topic, since advances in our understanding of the immunopathogenesis of INS may help drive new tailored therapeutic approaches forward.


2019 ◽  
Vol 39 (1) ◽  
Author(s):  
Roberta da Silva Filha ◽  
Sérgio Veloso Brant Pinheiro ◽  
Thiago Macedo e Cordeiro ◽  
Victor Feracin ◽  
Érica Leandro Marciano Vieira ◽  
...  

AbstractIntroduction: Renin angiotensin system (RAS) plays a role in idiopathic nephrotic syndrome (INS). Most studies investigated only the classical RAS axis. Therefore, the aims of the present study were to evaluate urinary levels of RAS molecules related to classical and to counter-regulatory axes in pediatric patients with INS, to compare the measurements with levels in healthy controls and to search for associations with inflammatory molecules, proteinuria and disease treatment. Subjects and methods: This cross-sectional study included 31 patients with INS and 19 healthy controls, matched for age and sex. Patients and controls were submitted to urine collection for measurement of RAS molecules [Ang II, Ang-(1-7), ACE and ACE2] by enzyme immunoassay and cytokines by Cytometric Bead Array. Findings in INS patients were compared according to proteinuria: absent (<150 mg/dl, n = 15) and present (≥150 mg/dl, n = 16). Results: In comparison to controls, INS patients had increased Ang II, Ang-(1-7) and ACE, levels while ACE2 was reduced. INS patients with proteinuria had lower levels of ACE2 than those without proteinuria. ACE2 levels were negatively correlated with 24-h-proteinuria. Urinary concentrations of MCP-1/CCL2 were significantly higher in INS patients, positively correlated with Ang II and negatively with Ang-(1-7). ACE2 concentrations were negatively correlated with IP-10/CXCL-10 levels, which, in turn, were positively correlated with 24-h-proteinuria. Conclusion: INS patients exhibited changes in RAS molecules and in chemokines. Proteinuria was associated with low levels of ACE2 and high levels of inflammatory molecules.


2020 ◽  
Vol 45 (8) ◽  
pp. 2508-2517
Author(s):  
Xue Yang ◽  
Fang-Lin Hou ◽  
Cheng Zhao ◽  
Cai-Yun Jiang ◽  
Xiu-Mei Li ◽  
...  

2020 ◽  
Vol 11 (5) ◽  
pp. 121-127
Author(s):  
Ajay Kushwaha ◽  
Nisha Kumari Ojha

Children are more susceptible to various infections because of underdeveloped immune system as compared to adults. Strengthening the immune system is a natural way to help the body fight against the disease-causing pathogens and immunomodulators can play a major role in this context. Various Ayurveda classics and studies published in journals related to Ayurveda drugs for improving immunity are reviewed and analysed. In Ayurveda, the objective of immune enhancement is achieved through the use of the Amalakyadi Rasayana (an Immunomodulators), as it increases longevity of life, memory, intellect, luxture, complexion, voice, strength of the body functions, strength of all senses and provides the resistance to disease, improves glow and power. Analysis of classical references and various experimental studies show that Amalakyadi Rasayana posse immuno-modulatory, Antioxidant, Anti-inflammatory, Antimicrobial, Anthelmintic activity. Present paper is a review to update knowledge on pharmacological properties, therapeutic actions and possible mode of action of the selected formulation, Amalakyadi rasayana from Yogaratnakara (Rasayanadhikara/17) to enhance the immunity in children. Rasayana is an important part of Ayurvedic therapeutics used to improve the quality of life by strengthening the tissue quality and by reducing the age-related tissue degeneration. This study reveals that Amalakyadi Rasayana have potential to improve or strengthen the immune system in children and thereby can lower down the morbidity rate in children.


2020 ◽  
Vol 11 (SPL4) ◽  
pp. 992-996
Author(s):  
Prasad Umakant Wallalwar ◽  
Pradnya Dandekar ◽  
Shweta Dadarao Parwe

The concept of Immunity is mentioned as ‘Vyadhikshamatwa’ in Ayurveda. In Ayurveda numbers of medicinal herbs are described under immunity-enhancing drugs category. The concept of immunity enhancer is explained under the category of Rasayan chikitsa in Ayurveda. These herbs are used in various diseases, especially by modulating the immune system. These herbs are called as Rasayan Dravyas. Ayurveda medical science comprises the herbs which are Rasayana and also which are non-Rasayana in nature. In Samhitas entirely enriched with enormous data of Rasayana medicinal herbs acting as immune modulators. The data of available Samhitas and researches show that these Rasayana herbs have immense potential as immune modulators. Advanced researches have proven that drugs like Guduchi, Haritaki, Ashwagandha, Brahmi, Jyotishmati act as a good immune enhancer. Theses herbs are mentioned in Ayurveda as Rasayana drugs. Experimental studies also have proven the role of Rasayana drugs as antioxidant, adoptogenic and having anti-stress activities. These Rasayana drugs as having immunity enhancer protect the health of a healthy person and protects them from any type of diseased condition. Charakacharya had mentioned Rasayanchikitsa adhyay as the first addhya of their chikitsasthan considering his aim as ‘Swasthasya Swasthya Rakshanam’. This means to protect the health of a healthy person as in priority by using immunity enhancer drugs as Rasayanas. This review article empowers such reviewed data on Rasayan medicinal herbs as a promising future target for immunotherapy.


1984 ◽  
Vol 25 (1) ◽  
pp. 100-106 ◽  
Author(s):  
Yang Ji-Yun ◽  
Tyrone Melvin ◽  
Richard Sibley ◽  
Alfred F. Michael

2021 ◽  
Vol 10 (14) ◽  
pp. 3160
Author(s):  
Agnieszka Pukajło-Marczyk ◽  
Danuta Zwolińska

Hemopexin (Hpx) is considered a factor in the pathogenesis of idiopathic nephrotic syndrome (INS). The aim of the study was to evaluate the serum and urine values of Hpx (sHpx and uHpx) in children with INS, analyze the role of Hpx, and assess its usefulness as a marker of the disease course. 51 children with INS and 18 age-matched controls were examined. Patients were divided into subgroups depending on the number of relapses (group IA—the first episode of INS, group IB—with relapses) and according to method of treatment (group IIA treated with gluco-corticosteroids (GCS), group IIB treated with GCS and other immunosuppressants). Hpx concentrations were determined by enzyme-linked immunosorbent assay (ELISA). sHpx and uHpx values in relapse were elevated in the whole INS group versus controls (p < 0.000). In remission their levels decreased, but still remained higher than in the control group (p < 0.000). In group IB uHpx levels were increased during remission as compared to group IA (p < 0.006). No significant impact of immuno-suppressants on sHpx was observed, but uHpx excretion in group IIA was higher in relapse (p < 0.026) and lower in remission (p < 0.0017) as compared to group IIB. The results suggest the role of Hpx in the pathogenesis of INS. Hpx may be a useful indicator for continuation of treatment, but it requires confirmation by further controlled studies.


2021 ◽  
Vol 9 (4) ◽  
pp. 96-100
Author(s):  
D. B. Nikityuk ◽  
S. V. Klochkova ◽  
N. T. Alexeeva ◽  
A. G. Kvaratskheliya

The article provides data on scientific research of the Moscow scientific school of academicians D.A. Zhdanov and M.R. Sapin in the field of morphofunctional organization of the lymphatic and immune systems. The historical materials and scientific research of their students and followers were studied. The biographical milestones in the life and professional activities of Academician of the USSR Academy of Medical Sciences D.A. Zhdanov and his student, academician of the RAS M.R. Sapin within the walls of the I.M. Sechenov First Moscow State Medical Institute (now – Sechenov University). The main scientific direction of D.A. Zhdanov taught about the lymphatic system. As a result of his research, the important role of the organs of the immune system in the formation of the body's response to antigenic effects was shown. Studying the professional and creative activities of M.R. Sapin showed that he was a fairly bright personality in the morphological community. A large number of his students and followers allows the scientific direction of academician M.R. Sapin live and develop today. New scientists-morphologists, using modern research methods, will continue and develop the teachings of M.R. Sapin, as in his time he continued and deepened the scientific direction of his teacher.


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