Education And Debate

Grand Rounds - Hammersmith Hospital Late presentation of Kartagener's syndrome Consequences of ciliary dysfunction

BMJ 1994; 308 doi: (Published 19 February 1994) Cite this as: BMJ 1994;308:519
  1. M Perraudeau
  1. Royal Postgraduate Medical School, Hammersmith Hospital, London W12 0NN.

    Primary ciliary dyskinesia provides a unique insight into the role of cilia in the human body. It is usually diagnosed in childhood, when prompt treatment of respiratory infections can minimise irreversible damage to the lungs.

    Case history

    A 48 year old man presented with a four month history of cough productive of green sputum, a two month history of night sweats, and a two day history of left sided pleuritic chest pain. He had previously had recurrent chest infections, recurrent purulent rhinosinusitis, and had produced more than one cupful of sputum a day for many years. He smoked 20 cigarettes a day.

    On examination he had a fever (38°C), a regular pulse (100 beats/min), and blood pressure of 130/80 mm Hg. He was not clubbed. His apex beat was localised to the right fifth intercostal space and he had signs of consolidation in the left mid-zone. He had complete situs inversus viscerum.

    He had a haemoglobin concentration of 140 g/l and a peripheral leucocyte count of 11.8x109/l with 88% neutrophils. His erythrocyte sedimentation rate (34 mm in the first hour) and C reactive protein concentration (15 mg/l) were raised. Routine biochemistry tests gave normal results. Blood cultures were sterile but sputum culture grew Streptococcus pneumoniae. Culture was negative for acid fast bacilli. Chest radiography confirmed dextrocardia, with the aortic arch lying on the right side of the trachea (fig 1). The left lung had three lobes with consolidation in the middle lobe. A gastric air bubble was noted on the right side. The plain film showed no evidence of bronchiectasis but computed tomography of the chest after his pneumonia had resolved showed bronchiectasis in the left middle lobe and right lower lobe (fig 2).

    FIG 1

    Chest radiograph showing dextrocardia with the aortic arch lying on the right side of the …

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