CASE COMMUNICATIONS
IMAJ | volume 28
Journal 8, August 2026
pages: 514-516
Alternative Pathway Complement Dysregulation in Atypical HUS: A Case Communication
1 Ambulatory Pediatric Center, Saban Children’s Hospital, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
2 Allergy and Immunology Clinic, Saban Children’s Hospital, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
3 Pediatric Nephrology Clinic, Saban Children’s Hospital, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
4 Pediatric Infectious Disease Unit, Saban Children’s Hospital, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
5 Goldman Medical School, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
Summary
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In contrast to typical HUS, which is triggered by Shiga toxin–producing bacteria, aHUS results from genetic or acquired dysregulation of the alternative pathway (AP) of the complement system and has an estimated incidence of 1–2 cases per million per year [1]. Early recognition is critical, as terminal complement inhibition with C5 blockers significantly improves renal and overall outcomes [2,3].