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What is Kartagener's Syndrome
Kartagener's Syndrome is an inherited condition that affects the entire respiratory tract. The respiratory passages - the nose, sinuses, middle ear, eustachian tubes (the passages that link the throat with the ears), throat, and the trachea, bronchi, and bronchioles - are lined with hair-like cilia. In a healthy individual, these cilia sweep dust, smoke and other particles out of the lungs. They also help clear mucus. For those with Kartagener's Syndrome, these cilia either do not work properly or not at all, resulting in recurring ear and lung infections.
Since the ventricles of the brain and the reproductive system also have cilia, Kartagener's Syndrome patients may also experience headaches and fertility difficulties.
Kartagener's Syndrome is known by several other names including: primary ciliary dyskinesia, immotile cilia Syndrome, Afzelius' Syndrome, Kartagener's Triad, Zivert's Syndrome, Siewert's Syndrome, dextrocardiobronchiectasis-sinusitis syndrome and Ziver-Kartagener Triad.
Manes Kartagener (a physician from Switzerland) was the one who "first recognized this clinical triad as a distinct congenital syndrome in 1933" (http://emedicine.medscape.com) although Dr. Siewert was the first to describe the clinical relationship between chronic sinusitis, male infertility and situs inversus - where a person's organs are located symmetrically opposite to their normal position (eg: the heart is found on the right side instead of the left) in the early 1900s.
"Situs inversus is what sets Kartagener Syndrome apart from primary ciliary dyskinesia. Complete situs inversus involves reversal of both the abdominal and thoracic organs so that they form a mirror image of normal. In partial situs inversus, the thoracic organs may be reversed while the abdominal organs are normally positioned, or vice versa" (www.healthline.com). Complete situs inversus happens approximately one in every 10,000 adults, and of those only 20 percent are diagnosed with Kartagener's Syndrome.
It occurs approximately one in every 32,000 live births in the United States.
A person can only develop Kartagener's Syndrome if they inherit the genes from both parents.
Symptoms and Treatments of Kartagener's Syndrome
People with suspected and confirmed KS may experience:
- chronic rhinitis
- recurrent or chronic sinusitis
- recurrent or chronic bronchitis or pneumonia
- bronchiectasis
- hearing impairments
- fertility problems
There is no cure for Kartagener's Syndrome or any of the ancillary conditions. Treatment focuses on preventing and treating the associated sinus, lung and ear infections. Treatment includes antibiotics to address sinus infections, bronchodilators and inhaled steroids to relieve chronic lung disease symptoms, and the insertion of tubes in the ears to allow fluid to drain.
Men who experience problems with fertility may find consultation with a fertility specialist helpful.
For many, respiratory infections recur less frequently after the age of 20 and so are able to live pretty normal lives.
Sources: http://emedicine.medscape.com; www.healthline.com; http://rarediseases.about.com; www.mamashealth.com; www.patient.co.uk
Add a Comment2 Comments
It is MOST IMPORTANT to emphasise that, in contradiction with this article, grommets (or, 'tubes in the ears') have generally been found to be ineffective in PCD/Kartegeners patients. Misguided repeat insertions of grommets in PCD patients (based on the eardrum presenting identiacally to a patient who simply has glue ear, which is solveable with grommets) may exacerbate existing hearing loss problems. For further information please feel free to contact the UK charity: PCD Family Support Group at www.pcdsupport.org.uk
May 5, 2010 - 5:55amThis Comment
Thank you for the clarification.
May 5, 2010 - 6:17amThis Comment