Hereditary Sensory And Autonomic Neuropathy Type 6 C3539003
A rare inherited nerve disorder causing loss of pain sensation and autonomic dysfunction. It primarily affects children and leads to injuries from unnoticed trauma. Treatment involves wound care, infection prevention, and supportive measures.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Seizures
Autonomic crisis (hyperthermia)
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe hypotonia
(neurologic)Developmental delay
(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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive Multidisciplinary Care
Coordinated care involving neurologists, gastroenterologists, pulmonologists, and developmental specialists to address the complex needs of early-onset neuropathy with autonomic dysfunction.
Nutritional Support and Feeding Management
Management of feeding difficulties and potential dysphagia through modified food textures, positioning, and supplemental nutrition when oral intake is insufficient.
Additional treatment options
Autonomic Symptom Management
Targeted pharmacological management of autonomic symptoms such as temperature dysregulation, blood pressure instability, and excessive secretions.
medicationDevelopmental Physical Therapy
Age-appropriate exercises and positioning to support motor development, maintain joint mobility, and prevent contractures in children with developmental delays.
physical_therapyFamily Counseling and Support
Psychological support for families managing the stress of caring for a child with severe disability, including coping strategies and connection to support networks.
psychologicalWhen 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 Hereditary Sensory And Autonomic Neuropathy Type 6 C3539003?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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 25, 2026. · Read our editorial policy