Kartagener Syndrome
Kartagener Syndrome is a condition that means the combination of bronchiectasis, recurrent sinusitis and situs inversus, which means that the organs on the left side are on the right side.
The most important symptom is that the heart should be on the left, but on the right. In a person whose heart is on the right, bronchiectasis occurs due to lung damage. In addition, if there is recurrent sinusitis, the possibility of Kartagener’s syndrome is very high.
Is Kartagener Syndrome Genetic?
This disease shows autosomal recessive inheritance. In other words, the probability of the second child of a family having a child with Kartagener syndrome is 25%.
What is the Frequency of Kartagener Syndrome?
One out of every 30,000 children born is born with this disease.
Diagnosis
About half of patients with Kartagener syndrome have 3 symptoms, while half may not.
These findings;
Situs Inversus (Organs that should be on the left are on the right);
Approximately 15-20% of children with different locations of organs have Kartagener’s syndrome. In short, Kartagener may not develop in every child whose organs are different.
Sinusitis;
In cases with Kartagener’s Syndrome, frequent recurrent sinusitis is also associated as a result of the deterioration of the movement of the hairs that allow the cleaning of secretions called cilia.
Bronchiectasis
When the cilia deteriorate, the germs cannot be eliminated from the lungs and the germs settle in the lungs. Infections recur, bronchi are damaged and bronchiectasis develops.
What Are the Symptoms of Kartagener Syndrome?
- The heart is on the right
- Frequent cough, phlegm, fever due to bronchiectasis in the lungs
- Frequent sinusitis
- Cough with frequent phlegm
- Male infertility
Kartagener Syndrome Genetic Characteristics
Gene mutations occur due to cilia disorder and nearly 30 genetic mutations have been identified.
Therefore, it is important to make a genetic diagnosis when children with Kartagener syndrome are considering a second child. This is very important for prenatal diagnosis.
Kartagener Syndrome Treatment
- Children with bronchiectasis have sputum accumulation in the lungs due to excessive sputum.
- To prevent this, postural drainage should be done.
- For this, respiratory physiotherapy is performed and applied to people with microbial growth sputum culture.
What are the Vaccines to be Made?
Children with Kartagener syndrome should receive the flu vaccine in September or October of each year. A pneumococcal vaccine should also be given.
Microbes that reproduce frequently in sputum.
- influenza (58%)
- pneumoniae (21%)
- aureus (19%)
- aeruginosa (14%)
- coli (10%)
Surgery
Ear tubes can also be used in cases of frequent ear infections. In cases where bronchiectasis is severe and suitable for surgery, the damaged area can be surgically removed.
Which Doctors Should Be Consulted for Kartagener Syndrome?
- Children with Kartagener’s syndrome are followed up by pediatric lung diseases specialists.
- The lungs should be closely monitored and their function should be performed at regular intervals.
- For genetic diagnosis, follow-up is done in the genetics department.
