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June 1986

Kartagener's Syndrome: A Blinded, Controlled Study of Cilia Ultrastructure

Author Affiliations

From the Department of Otolaryngology, Massachusetts Eye and Ear Infirmary (Drs Eavey, Nadol, Joseph, and Strome); the Department of Pediatrics, Massachusetts General Hospital, Harvard Medical School (Drs Eavey, Holmes, and Lapey); and the Department of Biostatistics, Harvard School of Public Health (Dr Laird), Boston.

Arch Otolaryngol Head Neck Surg. 1986;112(6):646-650. doi:10.1001/archotol.1986.03780060058008

• We investigated respiratory mucosa cilia ultrastructure in patients homozygous for the gene for Kartagener's syndrome (KS) and patients apparently phenotypic for KS who had bronchiectasis and sinusitis but without situs inversus. Parents, as obligate carriers of the recessive KS gene, were also evaluated among other control groups. The four patients with KS had significantly fewer cilia outer dynein arms than normal subjects or parents of patients with KS. Two of five patients apparently phenotypic for KS demonstrated distinctive ultrastructural changes. No other subjects demonstrated explicit ultrastructural abnormalities. Internal control specimens showed that the number of outer dynein arms was consistent within a subject compared with variation between subjects. The outer dynein arm serves as a dependable ultrastructural marker. Carriers of KS do not demonstrate distinctive morphologic cilia abnormalities. Not every patient with chronic bronchiectasis and sinusitis demonstrates abnormal cilia ultrastructure.

(Arch Otolaryngol Head Neck Surg 1986;112:646-650)