DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

CHARGE Syndrome

HIGH SEVERITY

A rare genetic disorder characterized by a specific set of birth defects: Coloboma, Heart defects, Atresia of the choanae, Growth retardation, Genital abnormalities, and Ear abnormalities. Caused by mutations in the CHD7 gene in most cases. Affects approximately 1 in 8,500-15,000 births.

Global Affected

800.0K

Countries

27

Symptoms

Coloboma (eye defect)
Heart defects
Choanal atresia
Growth deficiency
Genital hypoplasia
Ear abnormalities/deafness
Facial palsy
Swallowing difficulties
Developmental delays
Balance problems
Cleft lip/palate
Tracheoesophageal fistula
Characteristic facial features

Treatment Options

Surgical repair of defects
Cochlear implants/hearing aids
Growth hormone therapy
Hormone replacement therapy
Speech therapy
Occupational therapy
Physical therapy
Feeding therapy
Educational support
Vision services
Cardiac monitoring
Behavioral therapy

Risk Factors

1CHD7 gene mutation
2Sema3E gene mutation (rare)
3Spontaneous mutation in most cases
4Rare inherited cases
5No gender predilection

Diagnostic Methods

  • 1CHD7 gene testing
  • 2Clinical criteria evaluation
  • 3Imaging studies (heart, brain)
  • 4Hearing evaluation
  • 5Vision examination
  • 6Endoscopy for choanal atresia
  • 7Genetic counseling

Prognosis

Survival depends on severity of heart defects and choanal atresia. With modern medical care, many survive into adulthood. Intellectual disability ranges from mild to severe. Most require lifelong medical care and support. Independence varies widely. Early intervention significantly improves outcomes.

Prevention

  • Genetic counseling for families
  • Prenatal testing available
  • Early diagnosis at birth
  • Multidisciplinary team approach
  • Family support programs

Research Status

Multidisciplinary care from birth. Surgical repair of heart defects and choanal atresia. Cochlear implants for hearing loss. Early intervention services. Feeding therapy and possible G-tube. Hormone replacement therapy. Lifelong management required.

Sources

  • https://www.mayoclinic.org/diseases-conditions/charge-syndrome
  • https://www.hematology.org/education/patients
  • https://www.ncbi.nlm.nih.gov/books
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.