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Down Syndrome

Brian G. Skotko, MPP, MD Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Down syndrome (DS) is a congenital condition associated with intellectual disability and an increased chance of multisystem medical problems.

  • Synonym: trisomy 21

Pediatric Consideratio...

DIAGNOSIS

HISTORY

~85% of mothers of infants with DS learn of the diagnosis postnatally, although this is changing with the availability of prenatal screening such as NIPS. 

PHYSICAL EXAM

  • In November 201...

TREATMENT

ISSUES FOR REFERRAL

  • Infant stimulation programs (Early Intervention)

  • Lactation consultant

  • Physical/occupational/speech therapy

  • Pediatric cardiologist, if indicated

SURGERY/OTHER PROCEDURES

Repair ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

The American Academy of Pediatrics recommends ongoing assessment and review, at least annually, the following surveillance: 
  • Vision: Assess for s...

REFERENCES

1
Gregg  AR, Skotko  BG, Benkendorf  JL, et al. Noninvasive prenatal screening for fetal aneuploidy, 2016 update: a position statement of the American College of Medi...

ADDITIONAL READING

  • Antonarakis SE, Skotko BG, Rafii MS, et al. Down syndrome. Nat Rev Dis Primers. 2020 Feb 6;6(1):9.

  • Bull M, Committee on Genetics...

SEE ALSO

Algorithm: Intellectual Disability 

CODES

ICD10

  • Q90.0 Trisomy 21, nonmosaicism (meiotic nondisjunction)

  • Q90 Down syndrome

  • Q90.1 Trisomy 21, mosaicism (mitotic nondisjunction)

  • Q90.9 Down syndrome, unspecified

  • Q90.2 Trisomy 21, translocation

SNOMED

CLINICAL PEARLS

  • 99% of young adults/adults with DS report being happy with their lives.

  • DS specialty clinics, including virtual ones like dsc2u.org, may improve medical outcomes.

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