Hereditary sensory and autonomic…

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Hereditary sensory and autonomic neuropathy type 7

ORPHA:391397Disease

Also called CIP with hyperhidrosis and gastrointestinal dysfunction · Congenital insensitivity to pain with hyperhidrosis and gastrointestinal dysfunction · HSAN with hyperhidrosis and gastrointestinal dysfunction · HSAN7 · Hereditary sensory and autonomic neuropathy type VII · Hereditary sensory and autonomic neuropathy with hyperhidrosis and gastrointestinal dysfunction

What it is

A rare, genetic, periphery neuropathy characterized by a congenital insensitivity to pain, muscular hypotonia and gastrointestinal disturbances. Patients present with delayed motor milestones achievement, self-mutilations, skin ulcers, poor wound healing, painless fractures, hyperhidrosis, abdominal discomfort, diarrhea and/or constipation. Cognitive development is normal.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Infancy, Neonatal
Inheritance
Autosomal dominant
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

SCN11ADisease-causing germline mutation(s) (gain of function)

ICD-10 codes

G60.8filed under a broader ICD-10 category — shared with 25 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 12732MONDO 0014244OMIM 615548UMLS C3809882

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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