Recurrent respiratory infections with severely damaged lung functions--Kartagener's syndrome--case report
Kartagener's (or Siewert) syndrome represents a clinical presentation of primary ciliary diskinesia (PCD) with 3 dominant symptoms: bronchiectasiae, chronic sinusitis and situs viscerum inversus totalis or dextrocardia, only. Siewet demonstrated the first case of this clinical phenomenon in 1904, which was recognized by Kartagener in 1933, as a congenital disorder. PCD is an autosomal recessive disease with extensive genetic heterogenity. Dyskinetic or completely absent motiliy of cilia predisposes to recurrent pulmonary and upper respiratory tract infections resulting in bronchiestasis. Also, infections of the middle ear are common due to lack of ciliary movement in the Eustachian tube. Men have reduced fertility due to spermatozoa with absent motility or abnormalities in the ductuli efferentes. Our case report represents a middle age woman, treated ambulatory for many years as a chronic obstructive pulmonary disease (COPD). The patient noted recurrent upper respiratory tract infections from her childhood, headaches and recurrent sinusitis. Also, she had extrauterine pregnancy, no children, in spite of several arteficial inseminations. Due to the clinical signs of the right heart failure and the described ECG changes, the echocardiographic examination was done, which showed the dextracardia and the ultrasonographic examination of the abdomen revealed the situs inversus, the finding being within the physiological limits. All data regarding situs viscerum invesrus, supported by CT verification of bronchiectasiae and chronic inflammation of maxillar sinus led us to conclude that the patient suffered from Kartagener's syndrome.