Kartagener Syndrome

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 in Kartagener Syndrome

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;

Frequent recurrent sinusitis is also associated with 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 cleared from the lungs and the germs settle in the lungs. Infections recur, bronchi are damaged and bronchiectasis develops.

What are The Symptoms?

• The heart is on the right
• Frequent cough, phlegm, fever due to bronchiectasis in the lungs
• Frequent sinusitis
• Cough with frequent phlegm
• Male infertility

Genetic Traits

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. 65% of gene mutations can be identified.

Kartagener Syndrome Treatment

Children with bronchiectasis have sputum accumulation in the lungs due to excessive sputum. To prevent this, postural drainage is performed. For this, chest physiotherapy is performed and antibiotics are given to those who have microbe growth in sputum culture.

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 in Kartagener’s Syndrome

An ear tube can be inserted in those with 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 syndrome are followed by Pediatric Chest Diseases specialists.

Lungs are closely monitored and respiratory functions are monitored at regular intervals and followed up in the genetics department for genetic diagnosis.

 

Source : https://www.invitra.com/en/kartagener-syndrome/