Attrv30m Amyloidosis C0268384
An inherited disorder causing abnormal protein deposits in the nervous system and heart. It often leads to nerve damage and heart problems. Medications can help slow progression in many patients.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Peripheral neuropathy
(neurologic)Autonomic dysfunction (orthostatic hypotension)
(neurologic)Carpal tunnel syndrome
(neurologic)Cardiac involvement (restrictive cardiomyopathy)
(neurologic)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
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
TTR Stabilizers (Tafamidis)
TTR stabilizers bind to the transthyretin protein and prevent its misfolding and aggregation into amyloid fibrils. This approach helps slow disease progression by stabilizing the mutant protein.
Additional treatment options
Liver Transplantation
Since the liver is the primary source of mutant transthyretin production, liver transplantation can reduce circulating mutant TTR levels. This is most effective in early-stage disease.
procedureGene Silencing Therapies
RNA-targeted therapies such as patisiran and inotersen reduce production of both mutant and wild-type TTR by inhibiting the TTR gene. This addresses the underlying genetic cause.
medicationCardiac Monitoring and Management
Regular cardiac assessment including echocardiography, ECG, and biomarkers to monitor disease progression and manage arrhythmias or heart failure symptoms appropriately.
monitoringAutonomic Symptom Management
Supportive lifestyle modifications including compression stockings for orthostatic hypotension, dietary adjustments for gastrointestinal symptoms, and temperature regulation for thermal dysregulation.
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 Attrv30m Amyloidosis C0268384?
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.
Adams D, Gonzalez-Duarte A, Suhr OB, et al. Patisiran, an RNAi therapeutic, for hereditary transthyretin amyloidosis. N Engl J Med. 2018;379(1):11-21.
Benson MD, Berk JL, Dispenzieri A, et al. Transthyretin amyloidosis: an update on disease manifestations and therapeutic strategies. J Am Coll Cardiol. 2021;78(8):842-857.
Gillmore JD, Gane E, Taubel J, et al. CRISPR-Cas9 In Vivo Gene Editing for Transthyretin Amyloidosis. N Engl J Med. 2021;385(6):493-502.
Sekijima Y, Ueda M, Koike H, Misawa S, Ishii T, Sobue G. Management of transthyretin amyloidosis: a systematic review and meta-analysis of current therapeutic options. Amyloid. 2022;29(4):233-244.
Medication Comparisons
Compare medications used to treat Attrv30m Amyloidosis C0268384. 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