Kartagener Syndrome Health Article

Treatment and management

Treatment for Kartagener syndrome involves treatment of the symptoms. Treatment for sinusitis includes the use of antibiotics to treat and prevent recurrent infection. Occasionally, surgery to relieve the sinusitis and remove nasal polyps that may be present is necessary. Daily chest physiotherapy to loosen mucus secretions is a common therapy as well, and if started early in life can help to prevent or delay development of bronchiectasis. Tympanoplasty in children with recurrent ear infections is often necessary.

Advances in reproductive technology allow for men who have Kartagener syndrome to have the opportunity to have children. A procedure called intracytoplasmic sperm injection or ICSI, now allow immotile or dysmotile sperm to fertilize an egg. ICSI involves injection of a single sperm into single eggs in order for fertilization to occur. This procedure first involves ovulation induction and egg retrieval to obtain eggs for attempt at fertilization by ICSI. In Vitro Fertilization (ICSI) pregnancy rates vary from center to center. Overall pregnancy rates of 10%–40% have been quoted worldwide, utilizing these procedures.

The chance for an affected male and his unaffected partner to have a child who has Kartagener syndrome is small. If the disease incidence is one in 32,000, then the chance for the unaffected woman to be a carrier of Kartagener syndrome is approximately one in 100 and the chance for having an affected child would be expected to be approximately one in 200 (0.5%). However, all children of affected males or females will be carriers for Kartagener syndrome.

Prognosis

The severity of Kartagener syndrome is variable. With the advent of antibiotic use for infection control, the life expectancy of a patient with Kartagener syndrome is close to or within the normal range, if there are no immediate problems in the newborn period.

BOOKS

Jones, Kenneth Lyons. Smith's Recognizable Patterns of Human Malformation. Philadelphia: W. B. Saunders Company, 1997.

PERIODICALS

Guichard, Cècile, et al. "Axonemal Dynein Intermediate-Chain Gene (DNAI1) Mutations Result in Situs Inversus and Primary Ciliary Dyskinesia (Kartagener Syndrome)." American Journal of Human Genetics (April 2001): 1030.

ORGANIZATIONS

American Lung Association. 1740 Broadway, New York, NY 10019-4374. (212) 315-8700 or (800) 586-4872. <http://www.lungusa.org>.

National Organization for Rare Disorders (NORD). PO Box 8923, New Fairfield, CT 06812-8923. (203) 746-6518 or (800) 999-6673. Fax: (203) 746-6481. <http://www.rarediseases.org>.

WEBSITES

OMIM Online Mendelian Inheritance in Man. Entries 244400 and 242650. <http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM>.

Tucker, Michael. "Clinical In Vitro Fertilization and Culture." IVF.com. <http://www.ivf.com/insem.html>.

Renee A. Laux, MS