DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

22q11.2 Deletion Syndrome (DiGeorge Syndrome)

HIGH SEVERITY

The most common microdeletion syndrome caused by deletion of 22q11.2. Characterized by congenital heart defects, thymic hypoplasia, immunodeficiency, hypocalcemia, and characteristic facial features. Variable expressivity.

Global Affected

800.0K

Countries

15

Symptoms

Congenital heart defects
Thymic hypoplasia
Immunodeficiency
Hypocalcemia
Characteristic facial features
Speech delay
Learning disabilities
Schizophrenia risk

Treatment Options

Cardiac surgery for congenital heart defects
Calcium and vitamin D supplementation
Immune monitoring and prophylaxis
Early developmental intervention
Speech therapy
Educational support
Mental health services
Endocrine management

Risk Factors

1De novo microdeletion (90% of cases)
2Inherited deletion (10% of cases)
3Advanced maternal age
4Parental inversion polymorphism

Diagnostic Methods

  • 1Fluorescence in situ hybridization (FISH)
  • 2Chromosomal microarray (CMA)
  • 3MLPA (Multiplex Ligation-dependent Probe Amplification)
  • 4Echocardiography for cardiac defects
  • 5Immunologic evaluation
  • 6Calcium level monitoring

Prognosis

Good with comprehensive care. 85% survive to adulthood. Cardiac surgery outcomes excellent. Cognitive function ranges from normal to mild intellectual disability. Mental health conditions manageable with support. Life expectancy near normal with proper management.

Prevention

  • Genetic counseling for affected families
  • Prenatal diagnosis via CVS or amniocentesis
  • Preimplantation genetic diagnosis (PGD)
  • Carrier testing in parents

Research Status

Cardiac surgery for heart defects. Calcium and vitamin D supplementation. Immune monitoring. Early intervention services. Mental health support. Multidisciplinary care throughout life.

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK1116
  • https://medlineplus.gov/genetics
  • https://rarediseases.org

Medical Disclaimer

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