ULTRASTRUCTURAL DEFECTS OF THE CILIARY EPITHELIUM IN A CHILD WITH KARTAGENERS-SYNDROME

Kartagener's syndrome is a well known classical triad of presentations consisting of bronchiectasis, sinusitis and situs inversus. It is now recognized that the syndrome is an extreme presentation of primary ci liary dyskinesia, a large group of conditions with ultrastructural cil lary defects, leading to poor ciliary motility In various organ system s. A case of Kartagener's syndrome is presented in an eight year old T hai boy in whom the ultrastructural ciliary defects have been examined and in detail for the first time in Thailand. Incomplete lack of dyne in arms was recognized. In addition, disorientation of cillary axis wa s noticed. Due to severe bronchiectatic changes of the right lower lob e and right lingular lobe which did not improve despite adequate antib iotics, these lobes were surgically removed. The child has done well s ince, but still suffers occasional and recurrent bouts of sinusitis.