Best Practice & Standards Committee Update (Sept 2020)

The committee is pleased to announce the publication of the first IPNA guideline on « steroid-resistant nephrotic syndrome in children ». Since its release in May 2020 in Pediatric Nephrology, the committee as well as many dedicated IPNA colleagues have been busy translating the document into different languages, some of which are already available on the IPNA website: Arabic, Chinese, Korean, Japanese, Portuguese, Russian, Slovenian and others.  

The committee is currently working on an adaptation of the guideline document for patients. Based on this success, the committee has embarked on a second guideline project on steroid-sensitive nephrotic syndrome in children. The core group has been set up and has met virtually.  The review of the literature is in progress, and the manuscript is expected to be submitted in a year’s time.

The committee has also conducted an online survey among colleagues from IPNA and regional Societies to establish the needs and expectations in terms of guideline development.  We would like to thank all the 765 colleagues who responded to this survey, the results of which are currently being analysed.

Finally, the committee is in the process of organizing the first IPNA guideline workshop that was originally planned in Taipei in September 2020 and is now rescheduled on March 30-31, 2021. Depending on the Covid situation, this meeting will take place on site, virtually, or both.

Olivia Boyer
Best Practice and Standards Committee Co-Chair

IPNA aHUS Guideline in Development

Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-driven thrombotic microangiopathy (TMA). With the advent of the complement C5 antibody eculizumab, outcomes of aHUS patients have significantly improved including outcomes of patients with kidney transplants. Given the rarity of aHUS and the resulting lack of randomized controlled trials, international treatment guidelines are currently not available and treatment recommendations are typically based on expert opinion. In addition, access to the prohibitively expensive eculizumab is limited to economically privileged countries thus creating an inacceptable and unjust global imbalance with respect to the management of aHUS patients. On this background and given its focus on the management of rare kidney diseases, IPNA initiated the development of clinical practice recommendations (CPRs) for aHUS. As for other IPNA CPRs, the new aHUS CPR will be developed applying the GRADE approach using PICO (population-intervention-comparator-outcome) questions on diagnosis and treatment of aHUS, will be based on information extracted from the available literature graded by evidence (“evidence tables”), and will eventually be published.

Christoph Licht

aHUS Guidelines Working Group Chair

Steroid-Sensitive Nephrotic Syndrome Clinical Guidelines

Meeting of the group on April 14, 2021: Finalizing the manuscript