Kartagener's Syndrome
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چکیده
This is described as autosomal recessive inherited syndrome. Primary (genetic) defects in the structure and function of sensory and motile cilia result in multiple ciliopathies. A reduction in the number of arms which propel mucus (dynein arms) is a common abnormality but many other structural defects of the cilia have been found. Patients with normal cilia morphology but abnormal mucus propulsion have been detected. In diagnosing the syndrome, therefore, both ciliary structure and motility need to be assessed [1] .
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An unusual presentation of Kartagener's syndrome.
Kartagener's syndrome is a rare disorder which is seen in nearly half of the cases of primary ciliary dyskinesia. We report an unusual case of Kartagener's syndrome where the patient had associated ventricular septal defect, pectus excavatum and was fertile.
متن کاملA case of surgically resected lung cancer in a patient with Kartagener's syndrome.
Kartagener's syndrome is a rare inherited disorder with a triology of symptoms (bronchiectasis, sinusitis and situs inversus) and is also associated with abnormalities of the cilia of the respiratory epithelium. Lung cancer arising in Kartagener's syndrome is very rare and to date only 5 cases have been reported in the English and Japanese literature. We report on a case of a 65-year-old Japane...
متن کاملKartagener's syndrome and acute appendicitis.
Kartagener's syndrome is a combination of situs inversus totalis, sinusitis and bronchiectasis. Left sided appendicitis can present with problems in diagnosis and during surgery. We present here a case of Kartagener's syndrome and left sided appendicitis successfully managed in a peripheral hospital in Bahawalnagar, Pakistan.
متن کاملMultiple Lung Abscesses in a Patient with Kartagener's Syndrome
Kartagener's syndrome is a rare genetic disorder, which is mostly inherited as an autosomal recessive trait. There are 4 genes with a proven pathogenetic role in Kartagener's syndrome. It results from ciliary dysfunction and is commonly characterized by sinusitis, male infertility, hydrocephalus, and situs inversus. Since Kartagener's syndrome causes deficiency or even stasis of the transport o...
متن کاملPrimarily nasal origin of exhaled nitric oxide and absence in Kartagener's syndrome.
The exact origin of nitric oxide (NO) in exhaled air is not known. We wanted to further investigate at what site exhaled NO is produced and to determine whether children with Kartagener's syndrome exhibited altered levels of exhaled NO. NO was measured by chemiluminescence technique in air sampled directly from the nose and in normally exhaled air of four children (2.5-13 years old) with Kartag...
متن کاملKartagener's Syndrome: A Classical Case
BACKGROUND Recurrent lower respiratory tract infection (LRTI) is a very common problem we encounter in our clinical practice. Failure to recognize the specific cause of this condition may subject the patients to unnecessary and inappropriate treatment. CASE DETAILS among the various causes of recurrent LRTI, the most frequent causes are abnormalities of general or local impairment of immune m...
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