Relationship Between Kartagener’s Syndrome and Internal Diseases: Coincidence or Coexistence

Abstract

Kartagener’s syndrome (KS) is included in the group of diseases arising from primary ciliary dyskinesia (PCD) and is characterized by the triad of situs inversus, chronic sinusitis and bronchiectasis.Patients with PCD, including KS, may present with various manifestations, such as male and female infertility, olfactory disorders, ophthalmopathy and hydrocephalus resulting from ciliary dysfunction.In our review of the literature, many rheumatic, renal, and malignant diseases and KS relationships have been reported. We think, as suggested by other authors , that chronic infectious processes, impaired mucociliary clearance and chronic hypoxia may play an important role in the development of KS coincidence with internal diseases.We write  this letter to draw attention to this issue.

Keywords

• Kartagener’s syndrome
• internal disease
• rheumatic disease

Supporting Institution

yok

Kartagener Sendromu ile dahili hastalıkların ilişkisi: Tesadüf mü?, Birliktelik mi?

Abstract

Kartagener sendromu,situs inversus, bronşektazi ve kronik sinüzit triadı ile karakterize bir siliyer diskinezi bozukluğudur. Kartagener sendromu, primer silier bozukluğuna bağlı olarak kadın ve erkek infertilitesi, koku alma ve görme kusuru, hidrosefali gibi klinik bulgular verebilir. Literatür araştırmamızda Kartagener sendromu ile birlikte romatizmal, renal ve malign hastalıkların birarada görüldüğü gözlenmektedir.Kronik enfeksiyöz süreçler, bozulmuş mukosilier klirensin Kartagener sendromu ve dahili hastalıkların gelişiminde rolu olduğu düşünülmektedir. Biz de dahili hastalıklar ve Kartagener sendromunun birlikte görülebileceğine dikkat çekmek için bu mektupu yazdık.

Keywords

• Kartagener Sendromu
• dahili hastalıklar

References

1. 1. Sleigh MA. Primary ciliary dyskinesia. Lancet. 1981;2(8244):476.
2. 2. Afzelius BA. A human syndrome caused by immotile cilia. Science. 1976;193(4250):317-9.
3. 3. Mastaglia GL, Goatcher P. Rare association of rheumatoid arthritis and Kartagener's syndrome: a common denominator?. Med J Aust. 1980;2(7):400.
4. 4. Rébora ME, Cuneo JA, Marcos J, Marcos JC. Kartagener syndrome and rheumatoid arthritis. J Clin Rheumatol. 2006;12(1):26-9.
5. 5. Martínez-Cordero E, Aracena-Taborga G, López-Zepeda J. Systemic lupus erythematosus and dextrocardia. Clin Exp Rheumatol. 1990;8(3):327-8.
6. 6. Limon M. Kartagener's syndrome and undifferentiated connective tissue disease. Int J Rheum Dis. 2024;27(5):e15179.
7. 7. Neffen H, Oehling A, Crisci CD. Kartageners syndrome: a report of six cases with special reference to humoral and cellular immunity. Respiration. 1980;40(3):161-7.
8. 8. Beutler A, Mackiewicz SH. Association of rheumatoid arthritis with Kartagener's syndrome in a patient with HLA-DR1-DR4-B27 haplotype. Z Rheumatol. 1992;51(5):253-5.

9. 9. Momeni A, Doroushi B, Taheri N. Kartagener syndrome with focal segmental glomerulosclerosis. Iran J Kidney Dis. 2013;7(6):499-501.
10. 10. El Houssni S, Laine M, Bziz A, Alhamany Z, Eddine Bourkadi J, Bayahia R. Amylose rénale révélant un syndrome de Kartagener [Renal amyloidosis revealing a Kartagener's syndrome]. Nephrol Ther. 2015;11(1):50-52.
11. 11. Flanagan MF, Hourihan M, Keane JF. Incidence of renal dysfunction in adults with cyanotic congenital heart disease. Am J Cardiol. 1991;68(4):403-6.
12. 12.Hida K, Wada J, Yamasaki H, et al. Cyanotic congenital heart disease associated with glomerulomegaly and focal segmental glomerulosclerosis: remission of nephrotic syndrome with angiotensin converting enzyme inhibitor. Nephrol Dial Transplant. 2002;17(1):144-7.
13. 13. Sayarlioglu H, Dagli CE, Dogan E, Sayarlioglu M, Koksal N. Kartagener's syndrome and polycystic kidney disease. NDT Plus. 2009;2(2):189-90.
14. 14. Kara B, Seher N, Eren-Karanis Mİ, et al. Nasopharyngeal carcinoma in a child with Kartagener`s syndrome. Turk J Pediatr. 2021;63(1):155-60.