IS IRON DEFICIENCY ANEMIA AN ADDITIONAL FEATURE OF JERVELL-LANGE NIELSEN SYNDROME?
Bu olguda ciddi demir eksikliği anemisi olan ve konjenital sağırlık ile uzun QT sendromu nedeniyle Jervell and Lange-Nielsen sendromu tanısı alan hasta sunulmuştur. Ciddi demir eksikliği anemisi olan hastalarda Jervell and Lange-Nielsen sendromunun akılda tutulması gerektiğini vurgulamak isteriz. Demir eksikliği anemisi bu sendromun ek bir bulgusu olabilir. Aynı zamanda Jervell and Lange-Nielsen sendromu tanılı hastalarda demir eksikliği anemisi açısından değerlendirilmesi gerekir
DEMİR EKSİKLİĞİ ANEMİSİ JERVELL-LANGE NIELSEN SENDROMUNUN EK BİR BULGUSU MUDUR?
In this case, a 5-years-old boy who admitted to our hospital with severe iron deficiency anemia and diagnosed as Jervell and Lange-Nielsen syndrome because of long QT and congenital deafness was presented. We would like to emphasize that Jervell and Lange- Nielsen syndrome should be in mind in patients who have severe iron deficiency anemia. We suggest that iron deficiency anemia may be an additional feature of this syndrome. We also suggest that all patients with Jervell and Lange-Nielsen syndrome should be examined for iron deficiency anemia as well
___
- 1)Adu-Gyamfi Y, Said A, Chowdhary UM, et al.: Anaesthetic-induced ventricular tachyarrhythmia in Jervell and Lange-Nielsen syndrome. Can J Anaesth 1991, 38:345-6.
- 2)Chiang CE, Roden DM: The long QT syndromes: ge- netic basis and clinical implications. J Am Coll Cardiol 2000; 36: 1-12.
- 3)Duggal P, Vesely MR, Wattanasirichaigoon D, et al.: Mutation of the gene for IsK associated with both Jervell and Lange-Nielsen and Romano-Ward forms of long-QT syndrome. Circulation 1998; 97: 142-6.
- 4)Fraser GR, Froggatt P, James TN: Congenital deafness associated with electrocardiographic abnormalities, fainting attacks and sudden death. Quart J Med 1964;33: 361.
- 5)Guntheroth WG: Long QT syndrome in children [editorial]. Circulation 1993; 87;2058-9.
- 6)Hiejima K, Suzuki F, Satake S, et al.: Electrophysiologic studies of Jervell, Lange-Nielsen syndrome. Chest 1981; 79: 446- 8.
- 7)Jervell, A, Lange-Nielsen, F: Congenital deaf-mutism, functional heart disease with prolongation of Q-T interval and sudden death. Am Heart J 54: 59-68, 1957.
- 8)Jervell A, Sivertssen E: Surdo-cardialt syndrome. Nordisk Medicine 1967; 78: 1443.
- 9)Khan IA: Clinical and therapeutic aspects of congenital and acquired long QT syndrome. Am J Med 2002; 112: 58–66.
- 10)Komsuoglu B, Goldeli O, Kulan K, et al.: The Jervell and Lange-Nielsen syndrome: Int J Cardiol 1994; 47: 189-92.
- 11)Langslet A and Sorland SJ: Surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval present at birth, and severe anaemia and syncopal attacks in childhood. Br Heart J 1975; 37: 830–2.
- 12)Mani A, Singh T, Calton R, et al.: Cardiovascular response in anemia. Indian J Pediatr 2005; 72: 297-300.
- 13)Neyroud N, Tesson F, Denjoy I, et al.: A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome. Nature Genet 1997; 15: 186-9.
- 14)Oreto G, Luzza F, Donato A, et al.: Electrocardiographic changes associated with haematocrit variations. Eur Heart J 1992; 13: 634-7.
- 15)Park MK, Troxler RG: Syncope. In Pediatric Cardiology for Practioners Park MK, Troxler RG Eds; 4th edition, Mosby Inc, St. Louis, Missouri: 2002; 449-59.
- 16)Pernot C: Le sendrome cardio-auditif de Jervell et Lange-Nielsen. Aspects electrocardiographiques Proceeding of The Association of European Pediatric Cardiologist 1972; 8: 28.
- 17)Roden DM and Woosley RL: QT prolongation and arrhythmia suppression, Am Heart J 1985; 2: 411–5.
- 18)Salen P, Nadkarni V: Congenital long-QT syndrome: a case report illustrating diagnostic pitfalls. J Emerg Med 1999; 17: 859-64.
- 19)Schulze-Bahr E, Haverkamp W, Wedekind H, et al.: Autosomal recessive long-QT syndrome (Jervell Lange-Nielsen syndrome) is genetically heterogeneous. Hum Genet 1997; 100: 573-6.
- 20)Singh NK, Trivedi RC, Singh SK, et al.: Electrocardiographic changes in anaemia. Indian Heart J 1986; 38: 67-70.
- 21)Splawski I, Timothy KW, Vincent GM, et al.: Molecular basis of the long-QT syndrome associated with deafness. New Eng J Med 1997; 336: 1562-7.
- 22)Stanojević M, Stankov S: Electrocardiographic changes in patients with chronic anemia. Srp Arh Celok Lek 1998; 126: 461-6(abstract).
- 23)Tyson J, Tranebjaerg L, Bellman S, et al.: IsK and KvLQT1: mutation in either of the two components of the delayed rectifier potassium channel can cause the Jervell and Lange-Nielsen syndrome. (Abstract) Am J Hum Genet 1997; 61: A349.
- 24)Coats AJ: Ethical authorship and publishing. Int J Cardiol 2009;131:149-50.