Kartageners Syndrome-Versatile Presentations
Belaldavar B P, Ankale N R, Tubachi J and Khadabadi S N
KLE university' s Jawaharlal Nehru Medical College with Dr. Prabhakar Kore Hospital & Medical research Centre, Belgaum, Karnataka, India. Pin code: 590010
Abstract—Primary ciliary dyskinesia (PCD) is a rare genetic disorder with an autosomal recessive mode of inheritance. About 50% of PCD patients develop situs inversus and Kartagener's syndrome, which has been classically described as a triad of dextrocardia, sinusitis and bronchiectasis, and male infertility; its incidence is estimated to be around 1:15000. Patient presents with recurrent attacks of upper and lower respiratory tract infections since childhood, sinusitis, mucopurulent nasal discharge, anosmia, otitis media, bronchiectasis and recurrent episodes of wheezing. In addition, as sperm motility and fallopian tube transport of ova are dependent on normal cilia motility, PCD patients may also suffer from infertility. Physical examination may show dextrocardia secondary to situs inversus. Because of the unusual constellation of clinical features crossing various organ systems, the diagnosis is often delayed even though the first signs may present in infancy. The differential diagnosis includes neonatal respiratory distress, asthma and allergic rhinitis or sinusitis, cystic fibrosis, or primary immunodeficiencies. Hence confusing the treating doctor and putting the patient in distress. We present series of such cases.
Index Terms—Kartagener's syndrome, Sinusitis, Bronchiectasis, Situs inversus
Cite: Belaldavar B P, Ankale N R, Tubachi J and Khadabadi S N, "Kartageners Syndrome-Versatile Presentations," International Journal of Pharma Medicine and Biological Sciences, Vol. 2, No. 1, pp. 32-37, January 2013.