Turner sendromu ve tamponada yol açan perikardiyal efüzyon birlikteliği: olgu sunumu

Year 2010, Volume: 17 Issue: 3, 25 - 27, 30.09.2010

Yasin Türker , Mehmet Özaydın

Abstract

Turner sendromu, tipik fenotipik özellikler ve X kromozomunun sayısal veya yapısal anormallikleri ile karakterize genetik bir hastalıktır. Turner sendromlu hastalarda konjenital kalp hastalığı sıktır. En sık aortanın konjenital malformasyonları, biküspit aorta, aort koarktasyonu ve aort dilatasyonu görülür. Bilgimize göre, Turner sendromunda perikardiyal efüzyon yalnızca bir kez bildirilmiştir. Perikardiyal efüzyon saptanan 50 yaşında Turner sendromlu bir hasta sunuldu.
Anahtar kelimeler: Turner sendromu, perikardiyal efüzyon, tamponat

Abstract

A patient with turner's syndrome associated with pericardial effusion causing cardiac tamponade: case report

Turner syndrome is a genetic disorder characterized by certain phenotypic features and structural or numerical abnormalities of X chromosome. There is high prevalence of congenital heart defects in patients with Turner's syndrome. Aortic malformations, bicuspid aortic valve, coarctation of the aorta and aortic dilation are the most common defects. To the best of our knowledge, only one case of pericardial effusion with Turner's syndrome has been reported. A case of pericardial effusion in a 50-year-old patient with Turner's syndrome is presented.
Key words: Turner's syndrome, pericardial effusion, tamponade

Keywords

Turner sendromu, perikardiyal efüzyon, tamponat

References

• Ranke MB, Saenger P. Turner's syndrome. Lancet 2001 Jul 28;358(9278):309-14.
• Sybert VP. Cardiovascular malformations and complications in Turner Syndrome. Pediatrics 1998; 101: e11-e18.
• Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with Turner`s syndrome. Pediatrics 1998; 102: e12.
• Ozaydin M, Varol E, Okutan H, Peker O, Dogan A, Altinbas A, Kahraman H. A patient with Turner's syndrome associated with unexplained left ventricular hypertrophy, severe left ventricular systolic dysfunction, atrial septal defect and pericardial effusion. Anadolu Kardiyol Derg 2007 Jun;7:237-8.
• Hall JG. Chromosomal clinical abnormalities. In: Behrman RE, Kliegman RM, Arvin AM (eds). Nelson Textbook of Pediatrics. 15th Ed. W.B Saunders Company, Philadelphia, 1996:137.
• Corey GR, Campbell PT, Von Tright P, et al: Etiology of large pericardial effusions. Am J Med 1993;95:209- 13.
• Tsang T, Oh J, Seward J: Diagnosis and management of cardiac tamponade in the era of echocardiography. Clin. Cardiol 1999; 22: 446-52.
• Sagrista-Sauleda J, Merce J, Permanyer-Miralda G, et al: Clinical clues to the causes of large pericardial effusion. Am J Med 2000;105:95-101
• Raynaud-Ravni C, Richard O, Freycon F. A familial case with generalized resistance to thyroid hormones. Arch Pediatr 1997;4:759-62.
• Punnose J, Agarwal MM, Premchandran JS. Transient diabetes insipidus and hypopituitarism after pituitary apoplexy: a rare association with pericardial effusion and painless thyroiditis. Am J Med Sci 2000;319(4):261-4.
• Parikh A, Ezzat S. Complete anterior pituitary failure and postpartum cardiomyopathy. Endocr Pract. 2006;12 (3): 284-7.