Kartagener's Syndrome

  • Kartagener's Syndrome is an inherited disease that affects embryonic development. In this disorder, cilia and flagella lack dynein arms and are therefore nonmotile.
  • Individuals with this syndrome may have their heart on the right side of the body – a condition called situs inversus.
  • Affected individuals have serious respiratory problems, since the cilia of their respiratory epithelium are nonmotile.
  • Males are sterile because the flagella of their spermatozoa are nonmotile.
  • Left and right side defining mechanism results in transposition of viscera in thorax and abdomen.
  • Situs inversus totalis – rare
  • Approximately 20% of the cases with total situs inversus also suffer from bronchiactasis and chronic sinusitis – due to abnormal cilia
  • bronchiactasis is when the bronchioles are expanded b/c they've lost elasticity. Th emucous cannot be cleared out of the lungs.
  • Chronic sinusitis – wherever there is a syndrome that leads to the lack of clearing of the mucous, they are called chronic sinusitis.
  • Other conditions of abnormal sidedness are called laterality sequences.
  • Dominantly Left-sided – have polysplenia
  • Dominantly right sided – asplenia.
  • Kartagener's develops in embryo at 3rd week.

    - There is a group of genetic diseases in which cilia beat either ineffectively or not at all, like in Kartagener's immotile cilium syndrome.  Affected cilia exhibit various ultrastructural defects in their internal structure, such as a lack of dynein arms or missing spokes.  Patients with this syndrome suffer various respiratory problems caused by the accumulation of particles in the lungs; males are typically sterile because of the loss of sperm motility, and 50% have an alimentary tract that is a mirror image of the usual pattern (situs inversus) - i.e., it rotates in the opposite direction during early development.

    - Kartagener's Syndrome is due to dynein arm defect.  Results in immotile cilia.


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