Volume. 35, No. 2 july. 2011 - The Chest and Heart Association of ...

Chest & Heart JournalVol. 35, No. 2, July 2011CASE REPORTA Man with Chronic Cough – Evaluated AsKartagenar’s SyndromeMK Uddin, S Ahmed,Qayyum MA, MM Attar, Z AlanAbstract:Bronchiectasis in young patient always requires a through evaluation to find outthe aetiology, Patient from tuberculosis prevalent countries many a times failedto achieve due attention regarding the aetiology of bronchiectasis. The numbersof rare diseases which cause bronchiectasis outnumber our prediction on accountof vast population (around 150 million). So, it is not infrequent to see the severebronchiectasis along with complications which probably may be delayed if notable to avoid as most of congenital causes are orphan diseases.Key Words: cilia • primary ciliary dyskinesia • bronchiectasis • ciliary beatpattern • Kartagener’s syndrome.[Chest & Heart Journal 2011; 35(2) : 135-139]Case report:Sohel Ahmed (Figure-1), aged 20 years, tailor, nondiabetic,normotensive, has been suffering frompersistent cough since childhood and shortness ofbreath for last five years, Cough is more markedat morning. Cough is associated with mucoidsputum but there is history of infection 2-3 timesper year as evidenced by copious amount ofyellowish sputum along with fever. He developedgradual progressive shortness of breath over last5 years. Now dyspnoea occurs on minimal exertion.On enquiry he stated that there is frequent runnynose along with headache. There is no history ofchildhood pneumonia, measles, and whoopingcough. There are no features suggestive ofhemoptysis, arthritis, deafness, chronic diarrhea,visual impairment.Examination showed stunted growth, clubbing,cyanosis, pitting oedema and raised JVP. Chest isbarrel shaped, auscultation reveals shower ofcoarse crackles altered with coughing. Apex beatis found at right 5 th intercostal space medial tomid clavicular line. Pulmonary component of 2 ndheart sound (P2) is loud. There is nohepatosplenomegaly and features of ascites.Clinical impression is severe bronchiectasis, corpulmonale, dextocardia and respiratory failure dueto? primary ciliary dyskinesia (PCD). X-ray of thepatient confirmed dextocardia along with multiplering shadows (figure-2). HRCT scan of chestrevealed extensive bronchiectasis all over lungmore marked in lower lobes (figure -3).USG of abdomen revealed that the patient has situsinversus. Semen analysis showed that 100% spermare immotile. Sweat test are within normal limitbut saccharine test become positive. X-ray PNSare in favour of chronic sinusitis with absence offrontal sinuses while CT scan of para nasal sinuses1. Medical specialist, AFMI2. Assistant Professor, Respiratory Medicine, BSMMU3. Assistant Professor, Respiratory Medicine, NIDCH4. Lt. Co Associate Prof, MPH, AFMI5. Associate Professor & Director of NIDCH6. Register NIDCHCorrespondence to: Dr. (Lt. Col.) Md. Kabir Uddin, FCPS(Med), MCPS(Med), Armed Forces Medicine Istitute.E-mail: mzikabir772@yahoo.com