Survival and disease complications in thalassemia major

被引:237
作者
Borgna-Pignatti, C
Rugolotto, S
De Stefano, P
Piga, A
Di Gregorio, F
Gamberini, MR
Sabato, V
Melevendi, C
Cappellini, MD
Verlato, G
机构
[1] Univ Ferrara, Dept Clin & Expt Med, I-44100 Ferrara, Italy
[2] Univ Verona, Dept Pediat, I-37100 Verona, Italy
[3] IRCCS San Matteo, Dept Pediat, Pavia, Italy
[4] Univ Turin, Dept Pediat, I-10124 Turin, Italy
[5] Univ Catania, Dept Pediat, I-95124 Catania, Italy
[6] Osped St Anna, Div Pediat, Annunziata Palma, Como, Italy
[7] Univ Bari, Dept Pediat, I-70121 Bari, Italy
[8] Osped Galliera, Div Pediat, Genova, Italy
[9] Univ Milan, Dept Internal Med, I-20122 Milan, Italy
[10] Univ Verona, Inst Med Stat, I-37100 Verona, Italy
来源
COOLEYS ANEMIA: SEVENTH SYMPOSIUM | 1998年 / 850卷
关键词
D O I
10.1111/j.1749-6632.1998.tb10479.x
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
We studied survival and disease complications in 1,146 patients with thalassemia major, born from January 1, 1960 to December 31, 1987, At last follow-up, in March 1997, probability of survival to age 20 years was 89% and to age 25 years was 82% for patients born in the years 1970-1974. Patients who died had a serum ferritin level, measured the year before death, significantly higher than those who survived. Diabetes was present in 5.4% of the patients; heart failure in 6,4%; arrhythmias in 5.0%, thrombosis in 1.1%, hypothyroidism in 11.6%, HIV infection in 1.8%. Hypogonadism was diagnosed in 55% of 578 patients who had reached pubertal age: 83.5% of hypogonadic females and 78.6% of males were receiving substitutive hormonal therapy, In conclusion, the survival of patients with thalassemia major is good and improving, but the prevalence of severe complications is still high.
引用
收藏
页码:227 / 231
页数:5
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