A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong

Au, Wing‐Yan; Lam, William W. Y.; Chu, Winnie C.W.; Yuen, HL; Ling, Alvin Siu-Cheung; Li, Rever Chak-Ho; Chan, Hoi Ming Herman; Lee, Harold Kwok-Kuen; Law, Monica; Liu, Herman Sung Yu; Liang, Raymond; Ha, Shau Yin

doi:10.3324/haematol.12367

Cited by 38 publications

(46 citation statements)

References 9 publications

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“…Despite these limitations, our data suggested that those children who were on deferiprone‐based therapy (mono or combination) had a significantly reduced risk of developing IFG. This finding is novel and very encouraging, as other investigators have also shown that reversal or correction of abnormal glycaemia was feasible in adults using higher doses of DFP‐based therapy . Our study is the first to suggest that a Deferiprone‐based regime could actually prevent the risk of IFG in children.…”

Section: Discussionsupporting

confidence: 71%

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Prevalence of diabetes mellitus in Chinese children with thalassaemia major

Liang

Bajoria

Jiang

et al. 2017

Tropical Med Int Health

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Abstractobjective Diabetes mellitus is a common endocrinopathy in patients with b-thalassaemia major (b-TM), which is high prevalent in southern China. This study aimed to determine the cause and prevalence of glycaemic disorders in Chinese children with b-TM.methods In this prospective study, fasting glucose and insulin (FINS) levels were assessed in 267 b-TM and 80 non-TM control children. Homeostatic model assessment (HOMA) and the quantitative insulin sensitivity check index (QUICKI) were evaluated. Iron overload was assessed by serum ferritin (SF), total units of blood transfused and cardiac T2*.results b-TM had higher FPG (P < 0.001), FINS (P < 0.001) and HOMA-IR (P < 0.05), but lower QUICKI (P < 0.01) than those of controls. The impaired fasting glucose (IFG) was present in 30% of children, whereas 2% had diabetes. The prevalence of IFG in b-TM group was higher in children aged >10 years (OR 6.5; 95% CI 3.7-11.4; P < 0.001), SF of >2500 lg/l (OR 4.8; 95% CI 2.1-11.1; P < 0.01), serum ALT levels of >50 IU/l (OR 2.1; 95% CI 1.2-3.7; P < 0.05) and cardiac T2* of <20 ms (OR 3.3; 95% CI 1.7-6.6; P < 0. 01). The children on deferiprone (DFP) had a reduced incidence of glycaemic aberrations than those on other chelating agents (OR 0.4; 95% CI 0.23-0.8; P < 0.05).conclusions Our data suggest that IFG occurred in 30% of b TM children, perhaps due to insulin resistance secondary to iron overload. Deferiprone-containing chelating agent may have a protective effect.keywords thalassaemia major, iron overload, insulin resistance diabetes, cardiac load, short stature

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Section: Discussionsupporting

confidence: 71%

“…It is well established that Cardiac T2* is a better marker of iron load than serum ferritin . Recently Ang et al .…”

Section: Discussionmentioning

confidence: 99%

Prevalence of diabetes mellitus in Chinese children with thalassaemia major

Liang

Bajoria

Jiang

et al. 2017

Tropical Med Int Health

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“…Myocardial iron loading (cardiac T2* <20 ms) has been reported in a cross‐sectional study by Au et al . as being strongly associated with DM, hypogonadism, hypothyroidism and hypoparathyroidism in β thalassemia major. The results of our decade‐long longitudinal study corroborate the findings of this study with respect to DM and hypogonadism .…”

Section: Discussionmentioning

confidence: 97%

History of myocardial iron loading is a strong risk factor for diabetes mellitus and hypogonadism in adults with β thalassemia major

Ang

Tzoulis

Prescott

et al. 2014

European J of Haematology

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Endocrinopathies are common complications of transfusional hemosiderosis among patients with β thalassemia major (TM). Previous studies had shown associations between some endocrinopathies and iron overload of the myocardium, liver and/or endocrine organs as assessed by MRI techniques. This retrospective analysis of 92 patients with TM (median age 36 yr) from a tertiary adult thalassemia unit in UK aimed to determine independent risk factors associated with endocrinopathies among these patients. Unlike previous studies, longitudinal data on routine measurements of iron load [worst myocardial and liver T2* values since 1999, worst LIC by MRI-R2 since 2008 and average 10-yr serum ferritin (SF)] up to April 2010 together with demographic features and age of initiating chelation were analyzed for associations with endocrinopathies. The most common endocrinopathies in this cohort were hypogonadism (67%) and diabetes mellitus (DM) (41%), and these were independently associated with myocardial T2* <20 ms (P < 0.001 and P = 0.008, respectively) and increased age (P = 0.002 and P = 0.016, respectively). DM and hypogonadism were independently associated with average SF >1250 μg/L (P = 0.003) and >2000 μg/L (P = 0.047), respectively. DM was also associated with initial detection of abnormal myocardial T2* at an older age (30 yr vs. 24 yr, P = 0.039). An abnormal myocardial T2* may therefore portend the development of DM and hypogonadism in patients with TM.

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“…However, improvements in MRI T2* measurements has become a surrogate measure to gauge the merit of intervention in some of these diseases. Finally, it was observed that cardiac T2* measurements is predictive of transfusion associated endocrine failure, and may obviate the need for imaging individual endocrine organs (47, 67).…”

Section: Imaging Protocol and Data Analysis For Assessment Of Myocardmentioning

confidence: 99%

MRI of cardiac iron overload

Chu

Lam³

2012

Magnetic Resonance Imaging

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Transfusion therapy has greatly improved the survival of transfusion dependent thalassemia major (TM) patients; however, the resultant iron load damages tissues including the heart, liver and endocrine organs. Among these, heart complication still remains the leading cause of mortality. Myocardial iron deposition can occur independently of other solid organ involvement; conversely, the heart may be spared despite heavy siderosis in other tissues. Iron chelation treatment diminishes the risk of hemosiderosis; however, the chelation treatment has its own toxicities and might not be available to all patients due to costs. Close monitoring of individual organ iron concentration and function is thus important for optimization of individual patient care. This review outlines the importance and clinical significance of recently available MRI techniques for monitoring cardiac iron load.

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A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong

Cited by 38 publications

References 9 publications

Prevalence of diabetes mellitus in Chinese children with thalassaemia major

Prevalence of diabetes mellitus in Chinese children with thalassaemia major

History of myocardial iron loading is a strong risk factor for diabetes mellitus and hypogonadism in adults with β thalassemia major

MRI of cardiac iron overload

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