Cardiac Disorders i n Children with B - Thalassemia Major

作者: Arsang Sh , Eshaqhosseni K , Miri R

DOI:

关键词:

摘要: C ardiac failure due to transfusional iron overload is the maj or cause of death in patients with β - thalassemia major. Also ineffective erythropoiesis and increased gastrointestinal absorption lead body this induced heart failure. For reason, regular evaluation cardiac function recommended for all as integral part their management identify early changes left ventricular function.The study was conducted Qom Children’s Hospital, Iran, from 1st March 2008 30 th October 2012; 140 confirmed cases o f major andcontrol group consisted 50 healthy children. were diagnosed on basis hemoglobin electrophoresis. 2 D, M mode Doppler echocardiography performed cases. Statistical comparis controls by using unpaired t test. The age ranged 3 year 22years mean 14.25 years. There 86 (61%) males 54 (39%) females. All irregular chelation therapy 42 (33%) had LV dysfunction; global dysfunction. Left dimensions, stroke volume E/A ratio considerably affected group. A significant percentage have This ma inly chronic anemia, .Regular monitoring may reduce complication a great extent.

参考文章(20)
Z. Fadoo, M. Khurshid, U. S. Ahmed, M. Yousuf, S. Bano, M. Bana, Mehnaz Atiq, Cardiac disease in beta-thalassaemia major: Is it reversible? Singapore Medical Journal. ,vol. 47, pp. 693- 696 ,(2006)
Lisa J. Anderson, Mark A. Westwood, Sally Holden, Bernard Davis, Emma Prescott, Beatrix Wonke, John B. Porter, J. Malcolm Walker, Dudley J. Pennell, Myocardial iron clearance during reversal of siderotic cardiomyopathy with intravenous desferrioxamine: a prospective study using T2* cardiovascular magnetic resonance. British Journal of Haematology. ,vol. 127, pp. 348- 355 ,(2004) , 10.1111/J.1365-2141.2004.05202.X
Patrick W. O'Leary, Kritvikrom Durongpisitkul, Timothy M. Cordes, Kent R. Bailey, Donald J. Hagler, A. Jamil Tajik, James B. Seward, Diastolic Ventricular Function in Children: A Doppler Echocardiographic Study Establishing Normal Values and Predictors of Increased Ventricular End-Diastolic Pressure Mayo Clinic Proceedings. ,vol. 73, pp. 616- 628 ,(1998) , 10.1016/S0025-6196(11)64884-2
Ishwar C. Verma, Ved P Choudhry, Pawan K. Jain, Prevention of thalassemia: a necessity in India. Indian Journal of Pediatrics. ,vol. 59, pp. 649- 654 ,(1992) , 10.1007/BF02859390
Athanassios Aessopos, Spyros Deftereos, Maria Tsironi, Fotios Karabatsos, Jacqueline Yousef, Christina Fragodimitri, Antonia Hatziliami, Markissia Karagiorga, Predictive echo-Doppler indices of left ventricular impairment in B-thalassemic patients Annals of Hematology. ,vol. 86, pp. 429- 434 ,(2007) , 10.1007/S00277-007-0257-Y
A Hoffband, A sensitive test for early myocardial iron loading. European Heart Journal. ,vol. 24, pp. 26- 27 ,(2003) , 10.1016/S0195-668X(02)00464-5
Marco Vaccari, Roberto Crepaz, Monica Fortini, Maria R. Gamberini, Sabrina Scarcia, Walter Pitscheider, Giuliano Bosi, Left Ventricular Remodeling, Systolic Function, and Diastolic Function in Young Adults With β-Thalassemia Intermedia Chest. ,vol. 121, pp. 506- 512 ,(2002) , 10.1378/CHEST.121.2.506
Athanasios Aessopos, Maria Kati, Dimitrios Farmakis, Heart disease in thalassemia intermedia: a review of the underlying pathophysiology Haematologica. ,vol. 92, pp. 658- 665 ,(2007) , 10.3324/HAEMATOL.10915
Suhaib Ahmed, Mohammed Saleem, Bernadette Modell, Mary Petrou, Screening extended families for genetic hemoglobin disorders in Pakistan. The New England Journal of Medicine. ,vol. 347, pp. 1162- 1168 ,(2002) , 10.1056/NEJMSA013234
Athanasios Aessopos, Dimitrios Farmakis, Antonia Hatziliami, Christina Fragodimitri, Fotis Karabatsos, Jacqueline Joussef, Eftichia Mitilineou, Evanthia Diamanti‐Kandaraki, John Meletis, Markisia Karagiorga, None, Cardiac status in well‐treated patients with thalassemia major European Journal of Haematology. ,vol. 73, pp. 359- 366 ,(2004) , 10.1111/J.1600-0609.2004.00304.X