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Children
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Clinical and Molecular Aspects of Current Therapies of the Immune-Mediated...
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Clinical and Molecular Aspects of Current Therapies of the Immune-Mediated Kidney Diseases in Children and Adolescents

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Nephrology".

Deadline for manuscript submissions: closed (30 September 2022) | Viewed by 2197

Special Issue Editors

Prof. Dr. Ryszard Grenda

E-Mail Website
Guest Editor
Department of Nephrology, Kidney Transplantation & Hypertension, Children's Memorial Health Institute, Aleja Dzieci Polskich 20, 04-730 Warsaw, Poland
Interests: pediatric nephrology; kidney transplantation; immunosuppression; extracorporeal therapy in nephrology; ethics in pediatric nephrology
Dr. Łukasz Obrycki

E-Mail Website
Guest Editor
Department of Nephrology, Kidney Transplantation and Hypertension Childrens`s Memorial Health Institute, 04-730 Warsaw, Poland
Interests: adolescent medicine; pathogenesis of primary hypertension in children and adolescents; target organ damage and complications of hypertension in pediatric patients; assessment of the central pressure and importance of the spurious hypertension; cardiovascular complications in patients with chronic kidney disease (Escape Network member); patients undergoing renal replacement therapy (3H Study Coordinator)

Special Issue Information

Dear Colleagues,

The wide spectrum of pediatric immune-mediated kidney diseases involves a variety of specific therapies. The classic therapeutic approach is focused on specific targets in the immune system, including cell receptors or intracellular enzymes. Blocking and/or depleting the activities of specific agents is used as an underlying therapeutic mechanism and a final clinical effect—the remission of the immunologic disease and relevant improvement, or at least the stabilization of kidney function to determine the duration and timing of the therapy. Depending on the drug specificity, therapeutic drug monitoring (TDM) or pre-dose evaluation of the target cell number serves as a cornerstone for dose adjustment. More recently, the local effect of several relevant drugs, including cyclosporine, dexamethasone, rituximab and abatacept on specific structures in podocytes is considered as another significant therapeutic mechanism. This may be important in a chronic setting, when the disease does not respond to the systemic mechanism of the drugs; however, it may be partially controlled by unlimited maintenance therapy. Balancing between the efficacy and safety of long-term therapy, selecting less invasive combined drug protocols and developing more precise diagnostic and monitoring tools are ongoing challenges in clinical practice and research in pediatric nephrology.

Prof. Dr. Ryszard Grenda
Dr. Łukasz Obrycki
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • immune-mediated kidney diseases
  • classic and biologic therapy
  • general and local drug effects
  • monitoring

Published Papers (1 paper)

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Research

11 pages, 635 KiB  
Article
A Multicenter Study Evaluating the Discontinuation of Eculizumab Therapy in Children with Atypical Hemolytic Uremic Syndrome
by Saeed AlZabali, Sawsan AlBatati, Khawla Rahim, Hassan Faqeehi, Abubaker Osman, Abdulaziz Bamhraz, Mohammed A. Saleh, Jameela A. Kari, Majed Aloufi, Loai Eid, Haydar Nasser, Abubakr Imam, Entesar AlHammadi, Omar Alkandari, Mohammed Al Riyami, Sidharth Sethi, Christoph Licht, Khalid A. Alhasan and Abdulkarim AlAnazi
Children 2022, 9(11), 1734; https://doi.org/10.3390/children9111734 - 11 Nov 2022
Viewed by 1764
Abstract
Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA), which has been treated successfully with eculizumab. The optimal duration of eculizumab in treating patients with aHUS remains poorly defined. Methods: We conducted a multicenter retrospective study in the Arabian [...] Read more.
Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA), which has been treated successfully with eculizumab. The optimal duration of eculizumab in treating patients with aHUS remains poorly defined. Methods: We conducted a multicenter retrospective study in the Arabian Gulf region for children of less than 18 years of age who were diagnosed with aHUS and who discontinued eculizumab between June 2013 and June 2021 to assess the rate and risk factors of aHUS recurrence. Results: We analyzed 28 patients with a clinical diagnosis of aHUS who had discontinued eculizumab. The most common reason for the discontinuation of eculizumab was renal and hematological remission (71.4%), followed by negative genetic testing (28.6%). During a median follow-up period of 24 months after discontinuation, 8 patients (28.5%) experienced HUS relapse. The risk factors of recurrence were positive genetic mutations (p = 0.020). On the other hand, there was no significant relationship between the relapse and age of presentation, the need for acute dialysis, the duration of eculizumab therapy before discontinuation, or the timing of eculizumab after the presentation. Regarding the renal outcomes after discontinuation, 23 patients were in remission with normal renal function, while 4 patients had chronic kidney disease (CKD) (three of them had pre-existing chronic kidney disease (CKD) before discontinuation, and one case developed a new CKD after discontinuation) and one patient underwent transplantation. Conclusions: The discontinuation of eculizumab in patients with aHUS is not without risk; it can result in HUS recurrence. Eculizumab discontinuation can be performed with close monitoring of the patients. It is essential to assess risk the factors for relapse before eculizumab discontinuation, in particular in children with a positive complement variant and any degree of residual CKD, as HUS relapse may lead to additional loss of kidney function. Resuming eculizumab promptly after relapse is effective in most patients. Full article
(This article belongs to the Special Issue Clinical and Molecular Aspects of Current Therapies of the Immune-Mediated Kidney Diseases in Children and Adolescents)
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