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Hypodontia and nail dysgenesis

Related Terms

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Background

  • Hypodontia and nail dysgenesis (HND), also known as Witkop tooth and nail syndrome or TNS, is a form of ectodermal dysplasia, which affects the ectoderm, the outer layer of the developing fetus. Because the ectodermal layer develops into many parts of an infant's body, including the nails and teeth, these parts may not develop normally.
  • HND was first described by a dermatologist named Witkop in 1965 when he observed patients with tooth and nail problems. Patients with HND may also have symptoms that affect the hair. Unlike in many forms of ectodermal dysplasia, however, the sweat glands do not appear to be affected. Symptoms of HND have been found to vary widely among patients.
  • People with HND may have absent, widely spaced, or cone-shaped teeth with narrow crowns. Fingernails and toenails may be poorly developed, abnormally small, ridged, spoon shaped, brittle, and slow to grow. Rarely, the nails separate from the nail beds and fall off. In people with HND, the toenails are generally more severely affected than the fingernails. Occasionally, sparse scalp hair is observed in HND.
  • HND is an inherited genetic disorder that is passed down from parents to children. It is caused by a mutation or defect in the MSX1 gene. This gene is known to be involved in tooth development. The disorder follows an autosomal dominant inheritance pattern, meaning only one copy of the defective gene is needed for the disease to appear.
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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.