Thursday, September 6, 2012

Constituent infections of Kartagener’s syndrome

A syndrome is a combination of symptoms of different diseases, which collectively characterize a particular mental or physical disorder. Rare hereditary Kartagener’s syndrome, a subgroup of primary ciliary dyskinesia (P.C.D.), involves infections of the genitals and respiratory tract: male infertility, severe sinus infection symptoms(sinusitis), situs inversus and bronchiectasis.

Kartagener’s syndrome triad
Sinusitis, situs inversus and bronchiectasis are in fact known as Kartagener’s syndrome triad in medical terminology. Although the Russian medical expert Siewert discussed the triad for the first time in 1904, the triad was named after his German counterpart Manes Kartagener for explaining the triad as a unique congenital syndrome in detail in 1933. The Swedish ultrastructuralist Bjorn Afzelius established a link between the syndrome and male infertility, and the fertility disorder was included in the triad in 1976.
The syndrome, an autosomal recessive disease, is ascribed to irregular structure and malfunctioning of cilia. The structural irregularities include absence of the dynein arms of the ciliary microtubule. Principal feature of the syndrome is incorrect (dysmotile) or no (immotile) movement of the ciliated hair cells occupying the mucosa, affecting the mucus drainage in the paranasal sinuses, ears and lungs. The syndrome fiddles with proteins required for the cilia movement in the spermatozoa and respiratory tract.
Bronchiectasis means the irreversible dilation and distortion of the airway passages in the respiratory tract (bronchi). Obstruction of mucocillary clearance in the tracheobronchial tree and paranasal sinuses and nasal passages dilates the passages and triggers sinusitis respectively. Bronchiectasis affects the tissues of the airway, whereas sinusitis involves the sinus tissues.
Situs inversus
The word situs inversus means “position” (situs) and “inverted” (inversus) in Latin. Situs inversus implies reversal of all visceral (large organs of the body) organs, for instance, the liver placed on the left, heart placed on the right… The disorder affects the embryonic node tissues. Males suffering from the syndrome may have infertility problem due to impaired movement of the sperms.
Symptoms of the syndrome
  • Chronic cough throughout the year
  • Chronic bronchitis
  • Disturbed smell sense
  • Nasal polyps
  • Nasal congestion
  • Several episodes of sinus infections occur every year. These episodes improve with antibiotics but may reappear.
  • Recurrent severe sinus infection symptoms generally trigger sinus pressure headache in the eye region and over the maxillary or frontal sinuses.
  • Repeated instances of pneumonia
  • Persistent ear infections
  • Thick mucus discharge

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