Liver disease in transfusion dependent thalassaemia major

Li, Chi Kong; Chik, Ki Wai; Lam, Christopher Wai‐Kei; To, KF; Yu, Simon C.H.; Lee, V; Shing, Mmk; Cheung, A Y K; Yuen, Patrick Man Pan

doi:10.1136/adc.86.5.344

Cited by 56 publications

(56 citation statements)

References 19 publications

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“…A poor correlation between liver iron stores and serum ferritin level has been shown in patients receiving iron chelator. 34 The correlation is especially poor when serum ferritin exceeds 5500 pmol/L, 35 a level that is exceeded in 30% (9 of 30) of our patients. It remains to be determined whether by using a more robust indicator of tissue iron load, such as liver iron content, 36 a positive correlation between iron load and indexes of vascular function would emerge.…”

Section: Discussionmentioning

confidence: 75%

Arterial Stiffness and Endothelial Function in Patients With β-Thalassemia Major

2002

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Background-Increased iron store has been linked to risk of cardiovascular disease. Structural alterations of arteries in ␤-thalassemia major patients and in vitro functional disturbance of vascular endothelial cells by thalassemic serum have been described. We sought to determine whether arterial stiffness and endothelial function are altered in vivo. Methods and Results-Thirty thalassemia patients (16 male) aged 22.2Ϯ7.4 years were recruited. Left ventricular (LV) mass and function were assessed echocardiographically. Carotid and brachioradial artery stiffness was assessed by stiffness index and pulse-wave velocity (PWV), respectively. FMD (rϭϪ0.40, Pϭ0.03; rϭϪ0.41, Pϭ0.03) and positively with indexed LV mass (rϭ0. 50, Pϭ0.005; rϭ0.40, Pϭ0.027). Nonetheless, no significant correlation existed between ferritin level and carotid stiffness, PWV, or FMD. Conclusions-Increased arterial stiffness, endothelial dysfunction, and LV hypertrophy occur in patients with ␤-thalassemia major, which may result in reduction of mechanical efficiency of the heart.

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Section: Discussionmentioning

confidence: 75%

Arterial Stiffness and Endothelial Function in Patients With β-Thalassemia Major

2002

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“…Figure 3 documents the poor association of ferritin and HIC and total body iron previously reported. 2,6 Organ dysfunction due to iron overload was common in young adults with TM (Table 2). Twenty-three percent of patients 25 years or older had heart disease requiring medication.…”

Section: Complications Of ␤-Thalassemia Major 35mentioning

confidence: 99%

“…31 Among 73 TM patients from Hong Kong aged 1.5 to 22.5 years (mean, 11.7 years) and undergoing biopsy from 1992 to 1999, none had evidence of cirrhosis and 30% had liver fibrosis. 6 …”

Section: Iron Overloadmentioning

confidence: 99%

Complications of β-thalassemia major in North America

Cunningham

Macklin

Neufeld

et al. 2004

Blood

413

309

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Treatment of patients with ␤-thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the National Institutes of Health-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11 010 ng/mL (median, 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry weight and 23% of patients had values of 15 mg/g dry weight or higher. No patients 15 years or younger and 5% of patients aged 16 to 24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVADs) placed. Among 80 episodes of bacteremia in 38 patients, 90%were attributable to the CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with ␤-thalassemia.

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“…2 Multicenter cross-sectional studies have reported that the development and the severity of liver fibrosis are strongly related to the extent of liver iron overload and to the presence of chronic HCV infection. [3][4][5][6][7] Long-term observation of thalassemics who had undergone bone marrow transplantation showed that severe iron overload and chronic HCV infection were independent risk factors for liver fibrosis progression. 8 In the last 20 years, intensive and adequate iron chelation therapy has reduced the iron overload in transfusion-dependent thalassemics, 9 and the screening of blood donors for HCV markers has minimized the risk of de novo HCV infection.…”

Section: Introductionmentioning

confidence: 99%

Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C

Marco¹,

Capra²,

Gagliardotto³

et al. 2008

Haematologica

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Iron overload and hepatitis virus C infection cause liver fibrosis in thalassemics. In a monocentric retrospective analysis of liver disease in a cohort of 191 transfusion-dependent thalassemics, in 126 patients who had undergone liver biopsy (mean age 17.2 years; 58 hepatitis virus C-RNA positive and 68 hepatitis virus C-RNA negative) the liver iron concentration (median 2.4 mg/gr dry liver weight) was closely related to serum ferritin levels (R = 0.58; p<0.0001). Male gender (OR 4.12) and serum hepatitis virus C-RNA positivity (OR 11.04) were independent risk factors for advanced liver fibrosis. The majority of hepatitis virus C-RNA negative patients with low iron load did not develop liver fibrosis, while hepatitis virus C-RNA positive patients infected with genotype 1 or 4 and iron overload more frequently developed advanced fibrosis. Hepatitis virus C infection is the main risk factor for liver fibrosis in transfusion-dependent thalassemics. Adequate chelation therapy usually prevents the development of liver fibrosis in thalassemics free of hepatitis virus C-infection and reduces the risk of developing severe fibrosis in thalassemics with chronic hepatitis C.

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Liver disease in transfusion dependent thalassaemia major

Cited by 56 publications

References 19 publications

Arterial Stiffness and Endothelial Function in Patients With β-Thalassemia Major

Arterial Stiffness and Endothelial Function in Patients With β-Thalassemia Major

Complications of β-thalassemia major in North America

Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C

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