Atypical Hemolytic Uremic Syndrome With I Factor Anomaly C2752039
A type of aHUS involving genetic changes in Factor I, needed to regulate complement. Usually affects children. Managed with complement inhibitors and plasma therapy.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Microangiopathic hemolytic anemia
Thrombocytopenia
Visual disturbances
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Renal impairment
(renal)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
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Complement C5 inhibitor therapy
Blockade of terminal complement complex formation to prevent complement-mediated endothelial damage and thrombotic microangiopathy in i-factor (factor I) deficiency.
Fresh frozen plasma infusion
Replacement of deficient factor I through plasma transfusion to restore complement regulatory function and prevent uncontrolled C3 activation.
Additional treatment options
Prophylactic anticoagulation
Anticoagulant therapy in patients with recurrent thrombotic events to prevent venous and arterial thrombosis associated with complement-mediated endothelial injury.
medicationRenal-heart protective nutrition
Dietary modifications including reduced sodium, controlled potassium and phosphorus intake, and adequate but not excessive protein to support both cardiovascular and renal health.
dietaryComplement genetic and biochemical monitoring
Periodic evaluation of complement levels, genetic testing for factor I mutations, and family screening to guide treatment decisions and prognosis.
monitoringWhen 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 I Factor Anomaly C2752039?
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.
Loirat C, Fakhouri F, Ariceta G, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016.
Fakhouri F, Hourmant M, Campistol JM, et al. Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial. Am J Kidney Dis. 2016.
Kvinge KM, Ghirardo J, Smith-Jackson K. Complement factor I mutations in atypical hemolytic uremic syndrome: systematic review and meta-analysis of genetic variants. Blood Rev. 2021.
Medication Comparisons
Compare medications used to treat Atypical Hemolytic Uremic Syndrome With I Factor Anomaly C2752039. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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