Kartagener Syndrome

Reviewed 06/2018
 


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 secondhand tobacco smoke exposure as well as other environmental pollutants.

  • Recommended for patients to be up-to-date with their childhood immunizatio...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Monitor for progression of lung disease is strongly recommended:

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

REFERENCES

Leigh MW, Zariwala MA, Knowles MR. Primary ciliary dyskinesia: improving the diagnostic approach. Curr Opin Pediatr.  2009;21(3):320–325.  [View Abstract]
Horani A, Brody SL, Ferkol T...

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.

  • Bush A, O'Callaghan C. Primary ciliary dyskin...

CODES

ICD10

Q89.3 Situs inversus 

ICD9

759.3 Situs inversus 

SNOMED

42402006 Kartagener syndrome (disorder) 

CLINICAL 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...

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