Extrarenal Manifestations of Atypical Hemolytic Uremic Syndrome: A Systematic Review and Meta-Analysis
Bibliographic record
Abstract
Background: Atypical Hemolytic Uremic Syndrome (aHUS) is a chronic thrombotic microangiopathy (TMA) resulting from abnormal alternative complement pathway activation. Although renal vasculature is commonly targeted, aHUS can involve many organ systems. Our review investigates the incidence and clinical outcomes of aHUS patients with extrarenal manifestations. Methods: The literature search was conducted through PubMed/Medline, Embase, Web of Science Core Collection, and CINAHL. The search criteria comprised terms such as ‘aHUs', ‘extrarenal’, and various individual organ systems. Clinical features specific to the patient were collected, including extrarenal involvement, laboratory values, genetic abnormalities, and adverse outcomes. The meta-analysis was completed using SPSS version 22. Results: In total, 47 studies were included encompassing a sample size of 890 aHUS patients. The most widely reported genetic abnormalities were in factor H (CFH) mutations [12% (84/700 patients); n=19 studies] and anti-FH IgG antibodies [27.1% (102/376 patients); n=10 studies]. The most commonly occurring extrarenal manifestations across the included studies were related to the central nervous system [28% (240/858 patients), n=32 studies], with seizure being the most-common CNS symptom. This was followed by the gastrointestinal system [31% (230/741 patients); n=25 studies] and the cardiovascular system [16% (97/607); n=23]. The proportion of aHUS patients with end-stage renal disease was reported across 11 studies in 13.2% (61/463 patients) of this subpopulation, and the mortality rate was 8.9% (56/632 patients) across 27 studies. Conclusions: Extrarenal manifestations were present in approximately 20-30% of aHUS patients, depending on age and organ system. Our findings indicate neurologic and gastrointestinal involvement to be the most incident. Studies infrequently report genetic findings and are limited in population size, warranting larger multi-centered cohort investigations.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.011 | 0.009 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".