PCD (PRIMARY CILIARY DYSKINESIA/IMMOTILE CILIA SYNDROME)
AUTO R
1/15,000 -1/20,000
ABSENT DYNEINE ARMS
½ OF KARTAGENER’S SYNDROME (1932)
SITUS INVERSUS(DEXTROCARDIA), NASAL POLYPOSIS, BRONCHTIECTASIS
MALE INFERTILITY
CHRONIC OME
SINUSITIS, POLYPOSIS
BRONCHIECTASIS
DIGITAL CLUBBING
Dx: TRACHEAL BIOPSY WITH INTRA OP LIGHT MICROSCOPY THEN2 % GLUTERALDEHYDE WITH E.M.
IF THEY HAVE SITUS INVERSUS—BY DEFINITION THEY HAVE THIS Dx
MAY REPRESENT UP TO 5 % OF PED CHR0NIC RHINOSINISITIS—–BUT SO WHAT!
Rx: SUPPORTIVE