What is Kartangener’s syndrome?

Kartagener’s syndrome, also known as primary ciliary dyskinesia, is a rare genetic disorder that affects the structure and function of cilia. Cilia are hair-like structures found on the surface of cells throughout the body, particularly in the respiratory tract, reproductive system, and the brain

 

The chest x-ray PA view show dextrocardia with kartangener’s syndrome.

Bilateral lower lungs prominent interstitial making with both tubular track and ring like dilation of airway suggesting bronchiectasis.

Diagnosis:- Kartangener’s syndrome.

 

Kartangener's syndrome

 

What is kartangener’s syndrome.

Kartagener’s syndrome, also known as primary ciliary dyskinesia, is a rare genetic disorder that affects the structure and function of cilia. Cilia are hair-like structures found on the surface of cells throughout the body, particularly in the respiratory tract, reproductive system, and the brain. In individuals with Kartagener’s syndrome, the cilia are abnormally shaped and do not move properly, leading to a range of symptoms, including chronic respiratory infections, infertility, and problems with organ development.

 

The syndrome is caused by mutations in genes that are involved in the formation and function of cilia. Some of the common signs and symptoms of Kartagener’s syndrome include chronic sinusitis, bronchitis, and pneumonia, along with coughing, wheezing, and difficulty breathing. Infertility is also a common feature of the syndrome, as the abnormal cilia prevent sperm from moving properly in males and the movement of eggs in females is impaired. Kartagener’s syndrome is typically diagnosed through a combination of medical history, physical examination, and specialized tests such as electron microscopy, genetic testing, and measurement of ciliary function. Treatment typically involves management of symptoms and complications, such as antibiotics for respiratory infections, assisted reproductive technologies for infertility, and surgery for organ abnormalities.

 

Clinical history of kartangener’s syndrome.

 

The clinical history of Kartagener’s syndrome typically includes the following:-

Recurrent respiratory infections:- Individuals with Kartagener’s syndrome often suffer from recurrent respiratory infections, such as sinusitis, bronchitis, and pneumonia. These infections are usually chronic and can lead to long-term damage to the lungs if left untreated.

Chronic cough and congestion:- Chronic cough and congestion are common symptoms of Kartagener’s syndrome due to the impaired movement of cilia in the respiratory tract. The cough may be productive, with sputum or phlegm, and may worsen during or after a respiratory infection.

Infertility:- Impaired ciliary movement in the reproductive tract can lead to infertility in both males and females. In males, it can lead to low sperm count or abnormal sperm motility, while in females, it can cause difficulty in the movement of eggs from the ovaries to the uterus.

Situs inversus:- Situs inversus is a condition where the organs in the chest and abdomen are reversed from their normal positions. Individuals with Kartagener’s syndrome often have situs inversus, which means their heart and other organs are located on the opposite side of the body than usual.

Other organ abnormalities:- Kartagener’s syndrome can also lead to other organ abnormalities, such as abnormalities in the heart, liver, and spleen.

Family history:- Kartagener’s syndrome is a genetic disorder, so individuals with a family history of the condition may be at a higher risk of developing it themselves.

If you suspect you may have Kartagener’s syndrome, it is important to seek medical attention from a healthcare provider who can perform a thorough evaluation and provide appropriate treatment.

 

https://www.radiologystar.com/case-study-2/

 

BOOK LINK :- Brant and Helms’ Fundamentals of Diagnostic Radiology¬†

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