This is a PA  Chest Xray of a  16 years old boy who was complaining  of having chronic cough associated with  foul  smelling copious amount of sputum who had  been treating  for recurrent  upper respiratory tract infections and recurrent otitis media in his childhood.



What are the abnormalities seen in this Xray?
What is the Diagnosis?


ANSWER

Kartagener’s syndrome
X-ray Chest PA view in Kartagener’s syndrome showing dextrocardia and features of bronchiectasis, more on the left side. Left dome of diaphragm is higher and the fundal gas bubble is seen on the right side, indicating situs inversus.


Kartagener Syndrome , also known as immotile ciliary syndrome or Primary ciliary dyskinesia, is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract , sinuses, Eustachian tube, middle ear and fallopian tube.
Kartagener Syndrome = combination of situs inversus, chronic sinusitis, and bronchiectasis


It is thought that the molecular defect is in the dynein component of cilial apparatus.


CLINICAL FREATURES
Chronic recurrent respiratory infections, including sinusitis, bronchitis, pneumonia and bronchiectasis
Chronic suppurative otitis media
Infertility is common, due to defective ciliary action in the fallopian tube in affected females or diminished sperm motility in affected males
Occationally hydrocephalus


In the ECG there are inverted P waves in lead I abd there is reversed R-wave progression.


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20 comments

  1. Anonymous Says:
  2. bronchectasis

     
  3. Anonymous Says:
  4. Bronkopneumonia?

     
  5. Situs inversus with multiple cystic areas of bronchiectasis. Kartagener Syndrome???

     
  6. Ashish Says:
  7. Kartagener syndrome--combination of situs inversus (reversal of the internal organs), chronic sinusitis, and bronchiectasis
    Dr.P.S.N.Raju

     
  8. Wasif Says:
  9. Brocheactasis.

     
  10. Anonymous Says:
  11. kartagener's syndrome

     
  12. Anonymous Says:
  13. kartagener's syndrome

    Based on the history of recurrent OM
    and the CXR finding of dextrocardia with situs inversus...

     
  14. Anonymous Says:
  15. dextrocardia + bronchiectasis

     
  16. Anonymous Says:
  17. arash from iran

    dextrocardia , brocnchiectasis and sinusitis can be seen in kartagerner sx

     
  18. hina khan Says:
  19. CXR:dextrocardia and homogenous hazziness in L lower lung.
    DIAGNOSISkartegner syndrome

     
  20. bronchiectasis

     
  21. Anonymous Says:
  22. DEXTROCARDIA....CYSTIC FIBROSIS.... KARTEGNER SYNDROME

     
  23. tareq daghma Says:
  24. KARTEGNER SYNDROME

     
  25. situs inversus, ateletasis, past Hx of otitis media and chest infections----Kartegners syndrome

     
  26. Anonymous Says:
  27. Kartegners syndrome

    dr.tareque anam

     
  28. Anonymous Says:
  29. clinical symptoms and x ray findings goes to kartegeners syndrom

     
  30. Anonymous Says:
  31. like can't tell for sure

     
  32. Anonymous Says:
  33. kartagener's syndrome
    the boy also has infertility

     
  34. Anonymous Says:
  35. primary ciliary dyskinesia (probably Kartagener syndrome)

     
  36. Anonymous Says:
  37. kartagener's syndrome

     

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