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