Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies
Atypical hemolytic uremic syndrome (aHUS) is a rare and potentially life-threatening thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Without timely intervention, it may progress to end-stage kidney disease. The condition is p...
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| Language: | English |
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Korean Society of Pediatric Nephrology
2025-06-01
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| Series: | Childhood Kidney Diseases |
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| Online Access: | http://chikd.org/upload/ckd-25-017.pdf |
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| author | Ji Yeon Song |
| author_facet | Ji Yeon Song |
| author_sort | Ji Yeon Song |
| collection | DOAJ |
| description | Atypical hemolytic uremic syndrome (aHUS) is a rare and potentially life-threatening thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Without timely intervention, it may progress to end-stage kidney disease. The condition is primarily attributed to dysregulation of the alternative complement pathway, with up to 60% of patients exhibiting genetic mutations in complement regulatory proteins such as complement factor H, complement factor I, membrane cofactor protein, and thrombomodulin. The introduction of complement inhibitors, such as eculizumab and ravulizumab, has significantly improved clinical outcomes by reducing recurrence and preserving renal function. These advances have redefined treatment approaches, particularly in pediatric patients and those undergoing kidney transplantation. The prophylactic use of complement inhibitors is now recommended for transplant recipients with moderate to high genetic risk. This review aimed to examine the pathophysiology, clinical manifestations, and treatment strategies of aHUS. Early and targeted complement inhibition is essential for preventing irreversible kidney damage and optimizing outcomes, particularly in children and transplant recipients. |
| format | Article |
| id | doaj-art-41d9ed7f9c934e93bf1d4f804a18cbb9 |
| institution | Matheson Library |
| issn | 2384-0242 2384-0250 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Korean Society of Pediatric Nephrology |
| record_format | Article |
| series | Childhood Kidney Diseases |
| spelling | doaj-art-41d9ed7f9c934e93bf1d4f804a18cbb92025-08-01T04:43:27ZengKorean Society of Pediatric NephrologyChildhood Kidney Diseases2384-02422384-02502025-06-01292525910.3339/ckd.25.017830Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategiesJi Yeon Song0 Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University School of Medicine, Yangsan, Republic of KoreaAtypical hemolytic uremic syndrome (aHUS) is a rare and potentially life-threatening thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Without timely intervention, it may progress to end-stage kidney disease. The condition is primarily attributed to dysregulation of the alternative complement pathway, with up to 60% of patients exhibiting genetic mutations in complement regulatory proteins such as complement factor H, complement factor I, membrane cofactor protein, and thrombomodulin. The introduction of complement inhibitors, such as eculizumab and ravulizumab, has significantly improved clinical outcomes by reducing recurrence and preserving renal function. These advances have redefined treatment approaches, particularly in pediatric patients and those undergoing kidney transplantation. The prophylactic use of complement inhibitors is now recommended for transplant recipients with moderate to high genetic risk. This review aimed to examine the pathophysiology, clinical manifestations, and treatment strategies of aHUS. Early and targeted complement inhibition is essential for preventing irreversible kidney damage and optimizing outcomes, particularly in children and transplant recipients.http://chikd.org/upload/ckd-25-017.pdfalternative complement pathwayatypical hemolytic uremic syndromecomplement inactivating agentskidney transplantationpediatrics |
| spellingShingle | Ji Yeon Song Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies Childhood Kidney Diseases alternative complement pathway atypical hemolytic uremic syndrome complement inactivating agents kidney transplantation pediatrics |
| title | Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies |
| title_full | Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies |
| title_fullStr | Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies |
| title_full_unstemmed | Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies |
| title_short | Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies |
| title_sort | atypical hemolytic uremic syndrome pathophysiology clinical presentation and treatment strategies |
| topic | alternative complement pathway atypical hemolytic uremic syndrome complement inactivating agents kidney transplantation pediatrics |
| url | http://chikd.org/upload/ckd-25-017.pdf |
| work_keys_str_mv | AT jiyeonsong atypicalhemolyticuremicsyndromepathophysiologyclinicalpresentationandtreatmentstrategies |