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Case presentation

Case presentation. Mariam Mukhtar. Case:. 24 year old male patient k/C of asthma and bronchiectasis. c/o: worsening SOB and C/P x 1m . History . Neonatal history: Situs inversus & dextrocardia repeated URTI : Recurrent OM & coryza Chronic ,thick , muciod rhinorrhea

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Case presentation

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  1. Case presentation Mariam Mukhtar

  2. Case: 24 year old male patient k/C of asthma and bronchiectasis. c/o: worsening SOB and C/P x 1m .

  3. History Neonatal history: • Situs inversus & dextrocardia • repeated URTI : Recurrent OM & coryza Chronic ,thick , muciod rhinorrhea • recurrent LRTI : Pneumonia ,bronchiolitis

  4. History At age of 3 years: Diagnosed to have asthma: • SOB , chronic cough • Nasal polyps • obstructive pattern of PFT • improvement on BD

  5. History: On asthma meds: • Multiple admissions • Symptoms of sinusitis • Worsening of productive cough • recurrent pleural effusions • Thoracotomy & decortication

  6. Imaging reports Early childhood: • Situs inversus totalis • Hyper-infilated lungs • Areas of consolidation Last few years: • Bronchiectatic changes ( progressive) • Opacified sinus cavities • pleural effusion

  7. Examination • Normal vitals • Finger clubbing • Chest: basal Coarse crackles, reduced breath sounds on left side • nose: Pale, edematous mucosa & polyps • Tenderness over sinuses

  8. Imaging

  9. imaging

  10. imaging

  11. imaging

  12. imaging

  13. Kartagener syndrome Triad of bronchiectasis , chronic sinusitis and situs inversus

  14. Kartagener syndrome • AR inheritance • defective ciliary motility • 1 /3200 live births • Situs inversus in 50%

  15. Clinical manifestations • Chronic URTI,LRTI • Bronchiectasis • Corneal abnormalities • poor sense of smell • Male Infertility • female sub-fertility

  16. diagnosis • 50 visits • Average age 10-14 at diagnosis • Clinical/ tests: nitric oxide test , mucociliary transport test , semen analysis & PFT • Imaging • Biopsy

  17. Other associations • transposition of great vessels • Trilocular /bilocular heart • Pyloric stenosis • Polysplenia • Hepatic statosis • Hydrocephalus

  18. Imaging Plain films: • Bronchial wall thickening • Bronchial dilatation + loss of normal tapering • Finger in glove sign ( mucus plug) • Situs inversus

  19. Kartagener syndrome

  20. CT Scans • Bronchiectasis Non specific tubular or cystic • Consolidation • Mucocele • Tree in bud or centrolobular nodules • Scarring • Situs inversus • Opacified sinus cavities , hypoplastic frontal sinuses

  21. Kartagener syndrome

  22. Differential diagnoses • Hereditary impaired muco-cilliary clearance : e.g. CF , Young syndrome • Impaired immunity: 1ry immunodeficiency syndrome, AIDS • Hyper-sensitvity and immune reaction: e.g. ABPA • Alpha 1 antitrypsin deficiency

  23. Management • Medical care: Immunizations , A/B ,inhaled BD , Steriod , PT • Surgical care: Tympanostomy tubes ,sinus surgeries • Consultations: ENT , chest ,OB/ GYNE and urology physicians , social workers

  24. Thank you References: Medscape Radiopaedia.org. Radiographics journal Wikipedia

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