Kartagener Syndrome

Reviewed 06/2017
 


BASICS

DESCRIPTION

  • Kartagener syndrome (KS) is a rare disorder characterized by defects in the ultrastructure of cilia impairing their motility. It is also referred to as primary ciliary dyskinesia (PC...

DIAGNOSIS

HISTORY

  • Presentation typically includes cardinal features of upper and lower respiratory tract infections.

  • Respiratory distress manifests in neonatal period and persists year round (1).

  • Lower r...

TREATMENT

GENERAL MEASURES

  • Limit first-hand and/or second-hand tobacco smoke exposure, as well as other environmental pollutants.

  • Recommend patients to be up-to-date with their childhood immunizations a...

ONGOING CARE

FOLLOWUP RECOMMENDATIONS

MONITORING

  • Monitor for progression of lung disease is strongly recommended.

    • Including spirometry to determine pulmonary function level and sputum cultures on every...

REFERENCES

Leigh MW, Pittman JE, Carson JL et al. Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome. Genet Med.  2009;11(7):473–487. [View Abstract on OvidInsights]
...

ADDITIONAL READING

  • Bush A, Chodhari R, Collins N et al. Primary ciliary dyskinesia: current state of the art. Arch Dis Child.  2007;92(12):1136–1140. [View Abstract on OvidInsights]

  • Bush A, O’Call...

CODES

ICD10

Q89.3 Situs inversus 

ICD9

759.3 Situs inversus 

SNOMED

42402006 Kartagener syndrome (disorder) 

PEARLS

  • KS is characterized by defects in the ultrastructure of cilia impairing their motility.

  • Is also referred to as PCD

  • Impaired motility results in an array of abnormalities, including situs inversus...

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