kartagener syndrome

kartagener syndrome

Kartagener syndrome– transposition of internal organs in combination with bronchiectasis and chronic sinusitis. Defects of cilia and flagella are manifested in the syndrome of immovable cilia (nl), the development of recurrent chronic bronchitis and sinusitis is likely. More than half of patients with a similar syndrome have situs viscerus inversus – transposition of internal organs (heart on the right, liver on the left, etc.), which together constitute Kartagener’s syndrome (*244400, 14q32, DNECL gene [600112, dynein heavy chain], p ).Clinical characteristics. Bronchiectasis, chronic cough, nasal polyps, sinusitis, anosmia, corneal lesions, otitis media, persistent headaches, transposition of internal organs, hypogammaglobulinemia A, reduced leukocyte motility. In Kartagener syndrome and immobile cilia, spermatozoa do not move, although such men are potentially fertile. In these cases, artificial insemination is carried out with the subsequent introduction of the conceptus into the uterus. With Kartagener’s syndrome and immovable cilia syndrome, women are fertile. Treatment is symptomatic. Surgical restoration of the normal arrangement of the chest organs. Synonyms. Kartagener’s Triad, Sievert’s Syndrome See also Immovable cilia syndrome (nl), Asthenia (nl), Bronchiectasis

ICD

• • Q87 Other specified syndromes of congenital malformations involving multiple systems
  •  Q89.3 Situs inversus MSH. 244400 Kartagener syndrome

Note. Transposition of the internal organs (270100, I4q32, gene SIV, p) is also observed in Nemark’s syndrome (208530). Literature. Kartagener M, Horlacher A: Situs viscerum inversus und Polyposis nasi in einem Faile familiaerer Bronchiektasien. Beitr. Klin. Tuberk. 87: 331-333, 1936; Kartagener M, Stucki P: Bronchiectasis with situs inversus. Arch. Pediat. 79: 193-207, 1962; Siewert A.K. Uber einen Fall von Bronchiectasie bei einem Patienten mit Situs inversus viscerum. Berl. Klin. Wochr. 41:139-141, 1904