Amyloidosis Hereditary Systemic 6 C5935573
A rare inherited condition where abnormal protein deposits build up in organs like kidneys and liver, potentially causing organ failure. Treatment focuses on symptom management and supportive care.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Cardiomyopathy
Autonomic dysfunction
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Peripheral neuropathy
(neurological)Nephrotic syndrome
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Tafamidis or related amyloid-stabilizing agents
TTR tetramer stabilizers that prevent dissociation of transthyretin into amyloid-forming monomers. These disease-modifying agents slow progression of systemic involvement.
Liver transplant evaluation for TTR reduction
Since the liver produces most circulating TTR protein, liver transplantation can significantly reduce amyloid production in selected patients with hereditary ATTR amyloidosis.
Additional treatment options
Organ-specific surveillance and progression monitoring
Regular cardiac, renal, and neurological monitoring including echocardiography, nerve conduction studies, and biomarker tracking to assess disease progression and treatment response.
monitoringHeart-healthy low-sodium dietary modifications
Dietary adjustments emphasizing reduced sodium intake and heart-healthy food choices to support cardiovascular function and manage fluid retention associated with cardiac involvement.
dietaryActivity modification and energy conservation
Activity planning with energy conservation techniques to manage fatigue while maintaining appropriate physical activity levels and independence.
lifestyleWhen 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 Amyloidosis Hereditary Systemic 6 C5935573?
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.
Benson MD, Kincaid JC. The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve. 2007;36(4):411-423.
Medication Comparisons
Compare medications used to treat Amyloidosis Hereditary Systemic 6 C5935573. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy