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Dekaban-Arima syndrome

Related Terms

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Background

  • Dekaban-Arima syndrome (DAS), also known as chorioretinal coloboma with cerebellar vermis aplasia, is a rare medical condition, often recognized at birth, that primarily affects the eyes and brain. Eye problems associated with this disorder include coloboma, or small holes in the retina and in the tissue between the retina and the lens of the eye. Brain abnormalities include the underdevelopment or absence of the cerebellar vermis, a part of the brain. The cerebellar vermis, which lies between the right and left halves of the brain, is responsible for balance and coordination. Other symptoms may include cysts in the kidneys and the development of fibrous tissue in the liver.
  • Many researchers believe that DAS is related to a condition called Joubert syndrome, which is also characterized by underdevelopment or absence of the cerebellar vermis. Some researchers believe that people with Joubert syndrome can be divided into two groups: those with retinal problems (DAS) and those without.
  • DAS is inherited, or passed down from parents to children, as an autosomal recessive trait. This means that two copies of the defective gene must be inherited for the disease to appear. The exact genetic mutation or defect that causes this condition is unknown.
  • DAS is extremely rare, but its exact prevalence is not known. While there is little information regarding the lifespan of people with DAS, the scientific literature does suggest that many individuals with DAS die from complications associated with kidney disease, such as kidney failure.
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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.