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Congenital insensitivity to pain with anhidrosis (CIPA)

Related Terms

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Background

  • Congenital insensitivity to pain with anhidrosis (CIPA) is one of several ectodermal dysplasias, disorders that affect the outer layer of a developing embryo. This layer, called the ectoderm, develops into body parts such as the eyes, nails, hair, and skin, including pain receptors and sweat glands.
  • In CIPA, the nerves that sense heat and pain are abnormal or absent. As a result, those who suffer from CIPA are insensitive to heat and pain. People with CIPA are also unable to sweat, a condition called anhidrosis, which affects the ability to regulate body temperature and may lead to fevers in hot weather.
  • Because people with CIPA do not sense pain, they often injure themselves. Self-mutilating behavior is also common among CIPA patients, who may bite their own tongues, lips, and fingers. Numerous injuries can result in excessive scarring, infection, and deformities. Damaged limbs may even lose function or have to be amputated.
  • CIPA is caused by a mutation or defect in the NTRK1 gene. It is inherited, or passed down in families, as an autosomal recessive trait. A person must inherit two copies of the defective NTRK1 gene (one copy from each parent) for the disease to appear. People who inherit a mutated gene from only one parent are called "carriers" of the disease, and they can pass the mutation to their children.
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Risk Factors

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Causes

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Signs and Symptoms

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Diagnosis

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Complications

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Treatment

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Integrative Therapies

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Prevention

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Author Information

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References

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

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The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.