Iron overload in chinese patients with hemoglobin H disease

Hsu, Hui‐Chi; Lin, Chiun‐Ku; Tsay, Shyh‐Haw; Tse, Elizabeth; Ho, Chao‐Hung; Chow, Mo‐Ping; Yung, Chien‐Hui; Peng, Hong-Wen

doi:10.1002/ajh.2830340410

Cited by 22 publications

(14 citation statements)

References 22 publications

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“…[61][62][63] Raised serum ferritin levels are correlated with increasing age but are unrelated to previous history of transfusions, iron supplement, or herbal medicine treatment. 22,31 It is likely that iron absorption is increased in Hb H disease, secondary to enhanced erythropoiesis as a result of hemolysis and anemia.…”

Section: Iron Overloadmentioning

confidence: 95%

“…However, it is very important to ensure that these women do not continue iron supplement therapy unnecessarily once the pregnancy is over, because as patients with Hb H disease, they are prone to develop iron overload with time. 22,31,[61][62][63] In patients with marked splenomegaly and hypersplenism, splenectomy can result in highly significant hematologic and clinical improvements. 127 Postoperative complications include septicemia, deep venous thrombosis, and pulmonary embolism.…”

Section: Treatmentmentioning

confidence: 99%

“…64 Excessive alcohol consumption is an additional risk factor. 62 In Hong Kong, 60 patients underwent computed tomographic (CT) scan of liver, and 51 of them (85%) had a signal-intensity ratio of less than 1, indicative of iron overload. 31,65 In others, liver biopsy revealed increased liver iron content and hepatic fibrosis and/or cirrhosis, without serologic evidence of either hepatitis B or C. 22,31 In addition, 25 asymptomatic adult patients had cardiac echocardiography done, and all showed abnormal left ventricular diastolic function.…”

Section: Iron Overloadmentioning

confidence: 99%

See 2 more Smart Citations

Hemoglobin H disease: not necessarily a benign disorder

Chui

Fucharoen

Chan

2003

Blood

284

235

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Section: Iron Overloadmentioning

confidence: 95%

Section: Treatmentmentioning

confidence: 99%

Section: Iron Overloadmentioning

confidence: 99%

See 1 more Smart Citation

Hemoglobin H disease: not necessarily a benign disorder

Chui

Fucharoen

Chan

2003

Blood

284

235

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“…In nontransfused patients with severe thalassemia, abnormal dietary iron absorption results in an increased body iron burden between 2 and 5 g per year depending on the severity of erythroid expansion [25]. Analysis of nontransfused adult patients with HbH disease also indicates that iron overload is common in this disorder [26]. If regular transfusions are required, as in beta-thalassemia major patients, this doubles the rate of iron accumulation.…”

Section: Introductionmentioning

confidence: 99%

Ineffective erythropoiesis and thalassemias

Rivella

2009

Current Opinion in Hematology

148

144

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Purpose of review In thalassemia, ineffective erythropoiesis is characterized by apoptosis of the maturing nucleated erythroid cells. New studies also suggest that limited erythroid cell differentiation plays a role in the development of ineffective erythropoiesis. This would further exacerbate anemia and increase iron absorption. Recent findings During erythroid differentiation and maturation, it is critical that the components of hemoglobin are made in stoichiometric amounts. It is, therefore, conceivable that factors that modify this process intrinsically or extrinsically will also affect erythropoiesis. Several proteins have the potential to alter erythroid replication and differentiation in conditions of ineffective erythropoiesis. Elevated erythropoietin levels increase the number of erythroid precursors bearing a phosphorylated form of Jak2. This, in a pathological condition, may contribute to limited erythroid differentiation. Unbalanced synthesis of globins and heme modifies the activity of the heme-regulated inhibitor kinase, affecting proliferation and differentiation of the erythroid precursors. In addition, inefficient elimination of reactive oxygen species, which are increased under conditions of iron overload, may also hamper erythropoiesis. Summary Use of Jak2 inhibitors may limit the overproduction of immature erythroid cells in thalassemia, with the potential of reversing extramedullary hematopoiesis and preventing splenectomy. In addition, preventing iron overload and formation of reactive oxygen species may also be beneficial in limiting tissue damage and ineffective erythropoiesis.

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“…We did not check hepatitis C because its prevalence was low among young generation in Taiwan [40], [41]. We didn't investigate hemochromatosis because it is not frequent in Taiwan, only noted in some particular diseases like hemoglobin H disease [42], chronic hepatitis B or C [43]. Sixth, the use of WBC as a proxy indicator for inflammation might be not very accurate, better indicator like C-reactive protein should be considered in future study.…”

Section: Discussionmentioning

confidence: 99%

Hyperferritinemia and Hyperuricemia May Be Associated with Liver Function Abnormality in Obese Adolescents

2012

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BackgroundThe iron status in human body and its association with liver function in adolescents was rarely studied. The objective was to investigate the association among the levels of serum ferritin, uric acid and alanine aminotransferase (ALT) in adolescents.Methods and ResultsA total of 2090 adolescents negative for hepatitis B surface antigen from one junior high school (786, 12–13 years), three senior high schools (973, 15–16 years) and one college (331, 18–19 years) participated in this survey. Anthropometric and biochemical measurements, including complete blood count, ALT, serum ferritin and uric acid were performed. An ALT>42 U/L was defined as elevated, a ferritin level >200 µg/L was defined as hyperferritinemia. A uric acid level >460 µmol/L in males and >340 µmol/L in females was defined as hyperuricemia. The chi-squared test, linear regression and multivariate logistic regression were used for the data analysis. Elevated ALT levels were detected in 76 (3.6%) students and were more prevalent in males than females (6.4% vs. 2.0%, p<0.001). The univariate analysis found gender, age group, body mass index, ferritin level, uric acid level and white blood cell count all to be significantly associated with elevated ALT. Linear regression showed a positive correlation among log(ferritin), uric acid level and ALT level. Elevated ALT occurred more frequently at ferritin level >100 µg/L. The logistic regression analysis found that body mass index, hyperferritinemia and hyperuricemia were significant factors associated with the ALT elevation, but gender, age, and white blood cell count were not.ConclusionsHyperferritinemia and hyperuricemia are two independently significant factors associated with ALT elevation among obese adolescents. More studies are needed to corroborate any hypothesis related to these phenomena.

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Iron overload in chinese patients with hemoglobin H disease

Cited by 22 publications

References 22 publications

Hemoglobin H disease: not necessarily a benign disorder

Hemoglobin H disease: not necessarily a benign disorder

Ineffective erythropoiesis and thalassemias

Hyperferritinemia and Hyperuricemia May Be Associated with Liver Function Abnormality in Obese Adolescents

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