Once more the aHUS Alliance Global Action team presents a brief overview of new publications just added to our ‘Atypical HUS Virtual Library’.
As an international group of aHUS patients and family caregivers, we’re highly interested in advancements in the fields of disease diagnosis, management of aHUS, and advancements in knowledge. It’s the largest set of research and journal publications specific to this rare disease, and compiled independently to assure readers of an unbiased collection of current knowledge in this field.
Organized across 20 distinct topic categories to accommodate overlapping subjects and continuous research updates, this repository has grown since its inception five years ago and currently contains well over 1800 publications (see below for more details about our aHUS Virtual Library).
aHUS Remains Difficult to Diagnose
Physicians still have difficulty teasing out atyppical hus from other medical diagnoses which present with similar conditions. After all other medical diagnosis are ‘ruled out’, aHUS remains – explaining why this rare disease is often tagged with the notation that it’s a ‘diagnosis of exclusion’.
There are certain steps or analytic flow charts which physicians to take which can help to determine a diagnosis for patients exhibiting signs of thrombotic microangiopathy or TMA (see our article explaining aHUS/TMA Algorithms).
Since ‘regular HUS’ (STEC-HUS) is a much more common diagnosis, caused by is most commonly caused by an intestinal infection from Shiga toxin-producing strains of Escherichia coli bacteria, such as E. coli O157:H7.
The name of this recent publication says it all.
Ardissino G, Bernardi S, Dato L, Mancuso MC, Rossetti D, Tamburini G, Proverbio E, Porcaro L, Maragno C, Montini G. Typical or atypical hemolytic uremic syndrome? That is the question. Pediatr Nephrol. 2026 Jul 25. doi: 10.1007/s00467-026-07473-6. Epub ahead of print. PMID: 42501081.
Here are two more recent publications just added to our virtual library’s section on “Diagnosis” which illustrate the challenges in determining an aHUS diagnosis:
Okawa H, Wada Y, Kato N, Hanyuda M, Uchitubo R, Abe T, Takeuchi K, Nagaba Y, Maruyama S, Takeuchi Y. Atypical Hemolytic Uremic Syndrome Associated With Malignant Hypertension Presenting With Pulmonary-Renal Syndrome-like Symptoms. Kidney Med. 2026 Jun 4;8(8):101424. doi: 10.1016/j.xkme.2026.101424. PMID: 42471969; PMCID: PMC13380191.
Sharma, S., Goel, N., Jakhar, N. et al. CFI Mutation–Associated Atypical Hemolytic Uremic Syndrome Mimicking Autoimmune Hemolysis. Indian J Pediatr (2026). https://doi.org/10.1007/s12098-026-06358-8
aHUS affects Vital Organs
Thrombotic microangiopathies (TMAs) are a group of rare, life‐threatening disorders and thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are the main types of TMAs. The ‘atypical’ variety of HUS represents an estimated 5%–15% of HUS cases, which are most commonly caused by STEC-HUS is caused by bacterial infections (like E. coli, like shiga toxin STEC-HUS) while aHUS commonly is associated with by genetic mutations in the complement system
This first article has two-fold interest – the difficulty in teasing out a diagnosis between aHUS and regular HUS, and also the impact on organs other than the kidneys (extrarenal manifestations).
Ardissino, G., Bernardi, S., Dato, L. et al. Typical or atypical hemolytic uremic syndrome? That is the question. Pediatr Nephrol (2026). https://doi.org/10.1007/s00467-026-07473-6
Quite sobering, this publication’s Conclusion statement: Out of over 2,300 children included in this cohort, “…pediatric HUS was associated with significant short- and medium-term extrarenal morbidity. These findings highlight the need for vigilant long-term follow-up and refined treatment strategies targeting extrarenal disease burden in pediatric HUS”.
Raina R, Yerigeri K, Doshi K, Osagie-Ogbeide A, Haq I, Smith S, Khandelwal P, Licht C, Nester C, Boyer O. Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study. Pediatr Nephrol. 2026 Jun;41(6):1693-1704. doi: 10.1007/s00467-025-07079-4. Epub 2025 Dec 9. PMID: 41364181.
Don’t Miss this High Interest Topic: Therapeutic Drugs
While it might appear that the focus varies among these publications, a common link among them is therapeutic drugs. They range from infection risks for people treated with complement inhibitors, stopping treatment when using a complement inhibitors, insights into new drug targets and current use, and the rise of biosimilars.
Bettoni S, Qviberg E, Chaloner E, Lavender H, Laabei M. Complement Inhibition in the Clinic: Are We Doing Enough to Protect Patients From Infection? Eur J Immunol. 2026 Jul;56(7):e70233. doi: 10.1002/eji.70233. PMID: 42387937; PMCID: PMC13324233.
Hockman A, Anuskiewicz S, Brennan E, Chowdhury SR, Coltoff A, Poston JN, Greenberg C, Djulbegovic B. Efficacy of eculizumab discontinuation in atypical hemolytic uremic syndrome: a systematic review and meta-analysis. Blood Adv. 2026 Jun 23;10(12):4183. doi: 10.1182/bloodadvances.2026020871. PMID: 40795230; PMCID: PMC12719173.
Kotenko O, Kozlovskaya N, Emirova K, Muzurov A, Moiseev S, Konakova I, Shavkin A, Makarova T, Gazizulina M, Vinogradov V, Ivanova E, Demyanova K, Korotchaeva Y, Proshina T, Melnikova Y, Рoladova L, Mazur Y, Markova O, Borozinets A. Long-Term Therapy with Eculizumab Biosimilar in Patients with Atypical Haemolytic Uraemic Syndrome: Outcomes of a Prospective Observational Study. Nephron. 2026 Jun 12:1-10. doi: 10.1159/000552914. Epub ahead of print. PMID: 42284286; PMCID: PMC13436925.
Mathew GG, Lakshmikanthan RR, Jenishbabu A, Pragatheeswaran S, Shamini A, Varadharajan J. Complement System Inhibitors in Nephrology: A Comprehensive Review. G Ital Nefrol. 2026 Jun 30;43(3):2026-vol3. doi: 10.69097/43-03-2026-08. PMID: 42423060.
Wu Q, Yu Z, Wu Z, Yang L, Lin X. Complement-targeted therapies for C3 glomerulopathy and atypical hemolytic uremic syndrome: a time-limited rapid systematic review with narrative synthesis. Front Med (Lausanne). 2026 Aug 4;13:1867718. doi: 10.3389/fmed.2026.1867718. PMID: 42621905; PMCID: PMC13487681.
There are dozens more that we’ve added to our ‘Virtual Library, such as 18 recent publications regarding advancements, case studies, and reviews on aHUS “Treatment”. You’ll find all of these and more within the ‘New Research’ scroll, and divided into categories so that you can jump to the group for a topic of special interest. Read on to see how our ‘Virtual Library’ works.
A ‘Virtual Library’ of aHUS-specific Research – What is that?
Often publications with advancements and insights into this very rare disease are scattered across medical journals related to more than nephrology and hematology – atypical HUS information can be found under varied terms and across multiple specialties. That’s where the aHUS Alliance Global Action team comes in, since as aHUS patients and family caregivers ourselves we have a vested interest in staying up-to-date with current research. It’s likely that other families affected by aHUS also wish to focus on a particular topic, so to aid navigation of our ‘Research and Publications’ page we’ve not only categorized entries but also have added links to jump to each Scroll (topic section) to speed your search.
Research articles about atypical HUS are listed within our website on topic-specific lists within our Info Centre, where you can see a list for “New Research” which are then also listed by category. Use the blue ‘jump links’ at the top of the page to go directly to a specific topic category, which are: New Research, Critical Care, Diagnosis, Treatment, Discontinuing Treatment/Relapse, Thrombotic Microangiopathy (TMA), Extra Renal (Effects on Organs other than Kidneys), Pregnancy, Transplants, Genetics, Complement, Secondary aHUS, Triggers, Research in Specific Nations, Case Studies, Patient Registries: Publications, Drug Discovery/Research, Summary Articles/Literature Reviews, Consensus Documents/Guidelines, and Varied Topics.
Visit our ‘virtual library’ of over 1,800 publications specific to aHUS
by clicking this link: Atypical HUS Research & Publications
