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What is the prevalence of Oculo-Dento-Digital Dysplasia?

How many people does Oculo-Dento-Digital Dysplasia affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Oculo-Dento-Digital Dysplasia

Oculo-Dento-Digital Dysplasia (ODDD) is a rare genetic disorder characterized by abnormalities in the eyes, teeth, and fingers. The prevalence of ODDD is estimated to be extremely low, with only a few hundred cases reported worldwide. This condition is inherited in an autosomal dominant manner, meaning that a person with ODDD has a 50% chance of passing it on to their children. ODDD can vary in severity and presentation, making diagnosis challenging. Individuals with ODDD may experience vision problems, dental abnormalities, and finger deformities. Early detection and appropriate management are crucial for optimizing the quality of life for those affected.



Oculo-Dento-Digital Dysplasia (ODDD) is a rare genetic disorder that affects multiple systems in the body. It is characterized by abnormalities in the eyes, teeth, and fingers. ODDD is inherited in an autosomal dominant manner, meaning that an affected individual has a 50% chance of passing the condition on to each of their children.



The prevalence of ODDD is difficult to determine due to its rarity and the wide range of symptoms it presents. However, it is estimated to affect approximately 1 in every 50,000 to 100,000 individuals worldwide. The condition has been reported in various ethnic groups and populations, suggesting that it is not specific to any particular region or ethnicity.



ODDD primarily affects the development of the eyes, causing vision problems such as cataracts, glaucoma, and microphthalmia (abnormally small eyes). Dental abnormalities are also common, including small or missing teeth, enamel defects, and delayed tooth eruption. Additionally, individuals with ODDD may have distinctive facial features and abnormalities in their fingers, such as syndactyly (fusion of fingers) or camptodactyly (permanent flexion of fingers).



Due to its rarity, ODDD often goes undiagnosed or misdiagnosed. Genetic testing can confirm the diagnosis, and management typically involves addressing the specific symptoms and complications associated with the condition.


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