Atypical Hemolytic Uremic Syndrome With Complement Gene Abnormality C5680166
A rare condition causing damage to small blood vessels, affecting the kidneys and other organs. It leads to low red blood cells and platelet counts. Treatment suppresses the overactive immune response.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe anemia (hemoglobin <8 g/dL)
Acute kidney injury with oliguria
Confusion and seizures
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Thrombotic microangiopathy
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Complement C5 Inhibitor Therapy
Monoclonal antibody therapy targeting terminal complement to prevent endothelial damage. This represents the standard of care for aHUS with complement gene abnormalities and has dramatically improved outcomes in this patient population.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Therapeutic Plasma Exchange
Procedure to remove autoantibodies and replace deficient complement regulatory proteins. Typically performed daily or every other day until hematologic remission is achieved.
Additional treatment options
Immunosuppressive Agents
Corticosteroids and other immunosuppressants may be used to control complement dysregulation, particularly when antibody-mediated mechanisms are involved.
medicationComprehensive Laboratory Monitoring
Regular monitoring of hematologic parameters, renal function, and complement activity to assess treatment response and detect relapse early.
monitoringKidney-Supportive Nutritional Management
Dietary modifications including controlled protein intake, sodium restriction, and attention to electrolyte balance to support kidney function during recovery.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Atypical Hemolytic Uremic Syndrome With Complement Gene Abnormality C5680166?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Goodship TH, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2017.
Scully MA, Yadava M, Arshad SH. Complement-mediated atypical hemolytic uremic syndrome: a review of genetic abnormalities and current therapeutic options. Hematology. 2020.
Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013.
Fakhouri F, Zuber J, Frémeaux-Bacchi V. The kidney in complement-mediated renal diseases: focus on atypical hemolytic uremic syndrome. Nephrol Dial Transplant. 2019.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy