Survival, mortality, and complications in patients with β‐Thalassemia major in northern Taiwan

Chern, Jimmy P. S.; Su, Sheng‐Fang; Lin, Kai‐Hsin; Chang, S. L.; Lu, Meng-Yao; Jou, Shiann-Tarng; Lin, Dong‐Tsamn; Ho, Wan‐Ling; Lin, Kuo‐Sin

doi:10.1002/pbc.21028

Cited by 40 publications

(35 citation statements)

References 15 publications

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“…17 In another study, hypogonadism was detected in 54.3% of girls >15 years and in 63.6% of boys >17 years. 18 A statistically significant relationship could not be found between endocrine complications and mean ferritin levels in our study (p> 0.05). These types of complications can be seen more frequently after 1st decade of life and mean age of our patients was 10.3 years.…”

contrasting

confidence: 45%

Common Complications in Beta-Thalassemia Patients.

Yaman¹,

Işık²,

Yaralı³

et al. 2013

UHOD

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Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 ß-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with diagnosis of thalassemia major and 11 with thalassemia intermedia. Totally, 56.7% of patients were male and 43.3% were female. Ages varied between 2-20 years with the mean age of 10.3±4.8 years. Mean ferritin level was 2212±1370 ng/mL (41-6263 ng/mL) for 4.5 years. Complications were increased with increasing age. Complication rates were significantly higher among thalassemia major patients compared to thalassemia intermedia patients. There was no statistically significant relationship between complications and mean ferritin levels. The most common complications were endocrine complications (38.8%). Cardiac complications developed in 22.4% of the patients; gastroenterological complications in 19.4%; allergic complications in 9%; infectious complications in 1.5%; and thrombosis was detected in 1.5%. The endocrine complications were osteoporosis, growth retardation, developmental delay, short stature, hypothyroidism, delayed puberty, hypogonadism, and diabetes mellitus. The cardiac complications were left ventricular wall hypertrophy, diastolic dysfunction, systolic dysfunction, heart failure, pericardial effusion, dilated cardiomyopathy, left ventricular dilatation, left atrial dilatation, and fatal arrhythmias. Mortality occurred in one (1.5%) out of 67 ß-thalassemia patient due to dilated cardiomyopathy and fatal arrhythmia. Keywords: Thalassemia major, Thalassemia intermedia, Complications ÖZET Beta Talasemili Hastalarda S›k Görülen KomplikasyonlarTalasemi hastalar›n›n son 40 y›l içerisinde yaflam sürelerinde uzama olmakla birlikte yafl artt›kça görülen komplikasyonlar hastalar›n yaflam kalitelerini olumsuz yönde etkilemektedir. Çal›flmam›zda 1 Ocak 2004-31 May›s 2009 tarihleri aras›nda hastanemizde takipli 67 ß-talasemi hastas›nda görülen komplikasyonlar retrospektif olarak incelenmifltir. Çal›flmaya dahil edilen hastalar›n 56's› talasemi majör, 11'i talasemi intermedia tan›s›yla takip edilmektedir. Olgular›n %56.7'si erkek, %43.3'ü k›z idi. Yafllar› 2-20 y›l aras›nda olup, yafl ortalamalar› 10.3±4.8 y›l olarak hesapland›. Hastalar›n 4.5 y›l boyunca bak›lan ortalama ferritin düzeyleri 2212±1370 ng/mL (41-6263 ng/mL) olarak bulundu. Hastalar›n komplikasyonlar›nda yaflla birlikte art›fl oldu¤u saptand›. Komplikasyonlar talasemi majörlü hastalarda talasemi intermedial› hastalara göre istatistiksel olarak anlaml› derecede daha s›k görüldü. Ortalama ferritin de¤eri ile komplikasyon geliflme s›kl›¤› aras›nda istatistiksel olarak anlaml› bir iliflki bulunmad›. Hastalar›m›zda en s›k rastlanan komplikasyonlar %38.8 oran› ile endokrin komplikasyonlard›. Olgular›n %22.4'ünde kardiyak, %19.4'ünde gastroenterolojik, %9'unda all...

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contrasting

confidence: 45%

Common Complications in Beta-Thalassemia Patients.

Yaman¹,

Işık²,

Yaralı³

et al. 2013

UHOD

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“…The mean age observed in the present study was close to that of Chern et al [4] (14.8 ± 6.9 years) and comparable with the other study from Tehran (15.20 ± 3.1 years) and a study by Khalifa et al [5] (15.9 ± 3.1 years). In a study by Najafipour F et al [6] in Iran reported mean age was 15.62 ± 4.44 with youngest patient being 10 years and oldest being 27 years.…”

Section: Resultssupporting

confidence: 92%

Blood transfusion profile of Beta-Thalassemia Major patients attending a tertiary care hospital

Pattanashetti

Pilli²,

Pattanashetti

2017

APALM

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Background: Beta (β) thalassemia major is most common monogenic disorder in the world. Around 1,00,000 children are born each year with the severe homozygous state of the disease in India. The objectives of this study was to assess clinical data and the blood transfusion profile of Beta thalassemia major patients attending tertiary care hospital. This was undertaken as very few studies have been done in this region of the vast country. Methods:The study was done at a tertiary care teaching hospital from January 2014 to December 2014. Universal sampling method was used and 35 β thalassemia major patients who received blood transfusions at 2 to 4 weeks interval in the hospital were included in this study. Clinical details and blood transfusion record was collected on proforma for all patients and data interpreted.Result: Males (80%) outnumbered females (20%) with male to female ratio of 4:1. Nearly half of the study population was aged between 10 to 12 years (51.43). Majority of the patients had one blood transfusion per month (91.43%) and were on chelation therapy (71.43%). Majority of the patients received 100 to 125 blood transfusions during their lifetime (28.57 %). Least number of blood transfusions of more than 250 transfusions was taken by one patient (2.86 %). Conclusion:The present study describes the transfusion profile of β thalassemia major patients attending the tertiary care hospital and emphasizes on maintenance of transfusion record of β thalassemia major patients for better management of these patients.

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“…6 Most patient series outside Italy have observed comparatively worse outcomes after HLA-matched sibling BMT for thalassemia major. [12][13][14][15][16] Although the reasons for this disparity are unknown, there have been differences in recipient ethnicity, in the ability to assign a pretransplantation risk status, in how patients were prepared for transplantation, and in graft-versus-host disease (GVHD) prophylaxis, which might account for the disparate outcomes. There is also the possibility of a center effect, which might have contributed to an apparent poorer outcome in the smaller patient series outside Italy.…”

Section: Introductionmentioning

confidence: 99%

HLA-matched sibling bone marrow transplantation for β-thalassemia major

Sabloff

Chandy

Wang

et al. 2011

Blood

118

113

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We describe outcomes after human leukocyte antigen-matched sibling bone marrow transplantation (BMT) for 179 patients with ␤-thalassemia major. The median age at transplantation was 7 years and the median follow-up was 6 years. The distribution of Pesaro risk class I, II, and III categories was 2%, 42%, and 36%, respectively. The day 30 cumulative incidence of neutrophil recovery and day 100 platelet recovery were 90% and 86%, respectively. Seventeen patients had graft failure, which was fatal in 11. Six of 9 patients with graft failure are alive after a second transplantation. The day 100 probability of acute graft-versus-host disease and 5-year probability of chronic graftversus-host disease was 38% and 13%, respectively. The 5-year probabilities of overall-and disease-free survival were 91% and 88%, respectively, for patients with Pesaro risk class II, and 64% and 62%, respectively, for Pesaro risk class III. In multivariate analysis, mortality risks were higher in patients 7 years of age and older and those with hepatomegaly before BMT. The leading causes of death were interstitial pneumonitis (n ‫؍‬ 7), hemorrhage (n ‫؍‬ 8), and veno-occlusive disease (n ‫؍‬ 6). Proceeding to BMT in children younger than 7 years before development of end-organ damage, particularly in the liver, should improve results after BMT for ␤-thalassemia major. (Blood. 2011;117(5):1745-1750)

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Survival, mortality, and complications in patients with β‐Thalassemia major in northern Taiwan

Cited by 40 publications

References 15 publications

Common Complications in Beta-Thalassemia Patients.

Common Complications in Beta-Thalassemia Patients.

Blood transfusion profile of Beta-Thalassemia Major patients attending a tertiary care hospital

HLA-matched sibling bone marrow transplantation for β-thalassemia major

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