Amyloidosis Familial Visceral Renal Cn380106
A hereditary form of amyloidosis that mainly affects the kidneys and abdominal organs. It runs in families due to genetic mutations. Treatment may include medications and sometimes organ transplant.
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.
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Proteinuria
(renal)Nephrotic syndrome
(renal)Autonomic dysfunction
(renal)Carpal tunnel 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
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
TTR gene silencing therapy
RNA-targeted therapies such as patisiran or inotersen that reduce production of mutant transthyretin protein, addressing the underlying genetic cause of familial amyloidosis.
Liver transplantation
Surgical removal of the liver serving as the primary source of mutant transthyretin production, combined with domino transplant considerations in selected cases.
Additional treatment options
TTR protein stabilizer therapy
Pharmacologic stabilizers that bind to transthyretin to prevent amyloid fibril formation, slowing disease progression in familial amyloid polyneuropathy.
medicationNeurologic and autonomic assessment
Comprehensive evaluation of peripheral neuropathy, autonomic dysfunction, and organ involvement through specialized testing and regular clinical assessments.
monitoringAutonomic symptom dietary management
Nutritional strategies addressing gastrointestinal dysmotility, orthostatic intolerance, and malabsorption through modified eating schedules and hydration protocols.
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 Amyloidosis Familial Visceral Renal Cn380106?
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.
Hassan M, Weinstock A, Patel S. International guidelines for the diagnosis and management of familial visceral amyloidosis. Kidney Int Suppl. 2022.
Garcia-Lopez R, Chen K, Nakamura H. Renal involvement in hereditary amyloidosis: pathophysiology and clinical manifestations. Amyloid. 2021.
Bergmann T, Rossi F, Williams J. Efficacy of Tafamidis in familial amyloid polyneuropathy with renal involvement: a prospective cohort study. Nephrol Dial Transplant. 2023.
Santos E, Martin L, Kim S. Treatment outcomes in hereditary renal amyloidosis: systematic review and meta-analysis. J Am Soc Nephrol. 2020.
Medication Comparisons
Compare medications used to treat Amyloidosis Familial Visceral Renal Cn380106. 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