KS is a type of primary ciliary dyskinesia (PCD) with an inherited autosomal recessive pattern; result from defective cilia mobility due to a dynein arm defect.
Gene DNA H5 and DNA H 11 are know to cause primary ciliary dyskinesia, both genes encode for dynein.
Clinical triad: Recurrent Sinusitis, Bronchoectasis and Situs Inversus.
Other finding: male and female infertility, digital clubbing.
The KS is reported like 1 case per 32 000 life births in EUA, 000 in Spain Cuba
Situs Inversus occurs randomly in half of patient with PCD, therefore, for every patient with KS, another patient have PCD but no Situs Inversus.
Diagnosis test:
Sinus radiographs, chest radiographs, high CT scan.
Screening test include saccharin test and the measurement of nasal and exhale nitric oxide.
Audiologic test.
Pulmonary function study.
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