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1.
Article | IMSEAR | ID: sea-225551

Résumé

Primary ciliary dyskinesia (PCD) is an autosomal recessive hereditary disease that includes various forms of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. Kartagener?s syndrome is a rare disorder and the prevalence is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. The defective movement of cilia leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. The diagnosis is made clinically and confirmed through electron microscopy, which reveals abnormalities of structural organization of the axoneme in cilia from respiratory epithelia and in spermatozoa. Underlying structural defects include 1) absent inner and/or outer dynein arms, 2) tubular defects, and 3) radial spoke defects. We hereby report a rare case of Kartagener?s syndrome, in an infertile male with immotile sperms. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in making the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.

2.
Article | IMSEAR | ID: sea-225491

Résumé

Kartagener?s syndrome is a rare disorder. The estimated prevalence of Kartagener?s syndrome is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. There is a defective movement of cilia, which leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. We hereby report a rare case of Kartagener?s syndrome, an infertile male with azoospermia. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in such patients so that the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.

3.
Article | IMSEAR | ID: sea-204466

Résumé

Kartagener`s syndrome, a rare autosomal recessive disorder is a type of Primary Ciliary Dyskinesia (PCD) associated situs inversus, bronchiectasis, sinusitis and male infertility. We present a case of a 5-year-old girl who came with features of bilateral glue ear, recurrent sinusitis, recurrent hemoptysis and dextrocardia. She was diagnosed to have Kartagener`s syndrome and was evaluated for recurrent hemoptysis.

4.
Article Dans Anglais | IMSEAR | ID: sea-182356

Résumé

Kartagener`s syndrome, a rare autosomal recessive disorder is a type of primary ciliary dyskinesia (PCD) associated situs inversus, bronchiectasis, sinusitis and male infertility. We present a case of a 12-year-old boy who came with features of bilateral glue ear, recurrent sinusitis, adenotonsillitis, obstructive sleep apnea and situ inversus. He was diagnosed to have Kartagener`s syndrome and was treated with bilateral grommet insertion, bilateral antral lavage and adenotonsillectomy to alleviate his symptoms.

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