Öz
Anahtar Kelimeler
Kaynakça
- 1. Quirolo K, Vichinsky E. Hemoglobinopathies. In: Kliegman RM. Behrman RE, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. WB Saunders Co.Philadelphia;2003: Chap.454.
- 2. Beutler E, Ferlitti V, Ho NJ, et al. Relationship of body iron stores to levels of serum ferritin, serum iron, unsaturated iron binding capacity and transferrin saturation in patients withiron storage disease. Acta Haematol 2002;107:145-9.
- 3. Spirlto P, Maron BJ: Doppler echocardiography for assessing left ventricular diastolic function. Ann Int Med.1988;109:122.
- 4. Vogel M, Anderson LJ, Holden S, et al. Tissue Doppler echocardiography in patients with thalassaemia detects early myocardial dysfunction related to myocardial iron overload. Eur Heart J. 2003;24:113-119.
- 5. Spirito P, Lupl G, Melevendi C, Vecchio C: Restrictive diastolic abnormalities identified by Doppler echocarQiography in patients with thalassaemia major. Circulation. 1990;82:88.
- 6. Lewls BS, Lewls N, Dagarı I, Rachmllewitz EA,Gotsman MS, Sapoznikov D: Studies of left ventricular function in anemia due to B-thalassaemia. Isr J Med Sci 18:928, 1982
- 7. Nazan ÖZBARLAS, Arınan BİLGİÇ, Fatma GÜMRÜK, Çiğdem ALTAY. Talasemili Hastalarda Sol Ventrikül Diyastolik Fonksiyonunun Doppler Ekokardiyografi ile Değerlendirilmesi. Türk Kardiyol Dern Arş 1993;21:85-89.
- 8. Mustafa KIR, Nurettin ÜNAL, Gül Sağın SAYLAM, Ulaş KARADAŞ, Murat ŞAHİN. Ventriküler septal defektli çocuklarda sol ventrikül fonksiyonlarının miyokardiyal perfromans indksi (Tei indeksi) kullanılarak değerlendirilmesi. Dokuz Eylül tıpl fakültesi dergisi. 2008;S:113-119.
- 9. Italian Society of Hematology practice guidelines fort he management of iron overload in thalassemia majör and related disorders. Haematologica 2008;93:741-752.
- 10. Behzad A., Zahra B., Mahmoud M., Mahsa M. Evaluating the correlation between serum NT-proBNP level and diastolic dysfunction severity in beta-thalassemia majör patients. J Tehran Heart Cent. 2016;11:68-72.
- 11. Helen B., Kazım Ö., Erkan E.,Osen A. Assessment of left ventricular volüme and function in patients with beta thalassemia major. İKSST dergi. 2018;10:59-64.
Öz
Objective: Although there is increased survival and quality of life
for thalassemia patients, which results from a genetic defect in globin
synthesis, heart diseases are still important causes of morbidity and mortality
related to the iron burden. Therefore, we aimed to evaluate the cardiac
functions of thalassemia patients followed up in Hatay State Hospital using echocardiography.
Method: A total of 101 patients with 7 thalassemia intermedia
and 94 thalassemia major who were followed at Hatay state hospital were
examined retrospectively. Of patients, 47
were male and 54 were female. Their ages ranged between 1-17 years (mean
8.6±4.6 years). M-mode echocardiography was used to measure LVEDd (left
ventricular end-diastolic diameter) and EF (ejection fraction). The demographic
and laboratory values of the patients were obtained from their files.
Results: The mean of LVEDd (mm) and EF (%) were evaluated as 34.8±6.1,
and 66.36±1.4 with M-mode echocardiography, respectively. When the correlation between age, ferritin and LVEDd
and EF were evaluated; it was determined that there was a high positive
correlation (r=0.649) between age and LVEDd (p<0.01). A negative correlation was detected between ferritin
and EF (r=-0.271) (p<0.01). EF was found to be
significantly lower in the group with ferritin <2000. LVEDd was found to be
increased in the group with ferritin >2000, although it was not
statistically significant.
Conclusions: In our study, it was shown that EF was decreased with increasing
ferritin and LVEDS was increased with increasing age of patients with
thalassemia. The ideal ferritin level which was ensured after
suitable chelation therapy should be an important goal to reduce cardiac
involvement.
Anahtar Kelimeler
Kaynakça
- 1. Quirolo K, Vichinsky E. Hemoglobinopathies. In: Kliegman RM. Behrman RE, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. WB Saunders Co.Philadelphia;2003: Chap.454.
- 2. Beutler E, Ferlitti V, Ho NJ, et al. Relationship of body iron stores to levels of serum ferritin, serum iron, unsaturated iron binding capacity and transferrin saturation in patients withiron storage disease. Acta Haematol 2002;107:145-9.
- 3. Spirlto P, Maron BJ: Doppler echocardiography for assessing left ventricular diastolic function. Ann Int Med.1988;109:122.
- 4. Vogel M, Anderson LJ, Holden S, et al. Tissue Doppler echocardiography in patients with thalassaemia detects early myocardial dysfunction related to myocardial iron overload. Eur Heart J. 2003;24:113-119.
- 5. Spirito P, Lupl G, Melevendi C, Vecchio C: Restrictive diastolic abnormalities identified by Doppler echocarQiography in patients with thalassaemia major. Circulation. 1990;82:88.
- 6. Lewls BS, Lewls N, Dagarı I, Rachmllewitz EA,Gotsman MS, Sapoznikov D: Studies of left ventricular function in anemia due to B-thalassaemia. Isr J Med Sci 18:928, 1982
- 7. Nazan ÖZBARLAS, Arınan BİLGİÇ, Fatma GÜMRÜK, Çiğdem ALTAY. Talasemili Hastalarda Sol Ventrikül Diyastolik Fonksiyonunun Doppler Ekokardiyografi ile Değerlendirilmesi. Türk Kardiyol Dern Arş 1993;21:85-89.
- 8. Mustafa KIR, Nurettin ÜNAL, Gül Sağın SAYLAM, Ulaş KARADAŞ, Murat ŞAHİN. Ventriküler septal defektli çocuklarda sol ventrikül fonksiyonlarının miyokardiyal perfromans indksi (Tei indeksi) kullanılarak değerlendirilmesi. Dokuz Eylül tıpl fakültesi dergisi. 2008;S:113-119.
- 9. Italian Society of Hematology practice guidelines fort he management of iron overload in thalassemia majör and related disorders. Haematologica 2008;93:741-752.
- 10. Behzad A., Zahra B., Mahmoud M., Mahsa M. Evaluating the correlation between serum NT-proBNP level and diastolic dysfunction severity in beta-thalassemia majör patients. J Tehran Heart Cent. 2016;11:68-72.
- 11. Helen B., Kazım Ö., Erkan E.,Osen A. Assessment of left ventricular volüme and function in patients with beta thalassemia major. İKSST dergi. 2018;10:59-64.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Sağlık Kurumları Yönetimi |
| Bölüm | Medical Science Research Makaleler |
| Yazarlar | |
| Yayımlanma Tarihi | 31 Aralık 2019 |
| Kabul Tarihi | 31 Aralık 2019 |
| Yayımlandığı Sayı | Yıl 2019 |