Iron overload in Chinese patients with hemoglobin H disease.

作者: Hui-Chi Hsu , Chung-King Lin , Shyh-Haw Tsay , Elizabeth Tse , Chao-Hung Ho

DOI: 10.1002/AJH.2830340410

关键词: HemochromatosisGastroenterologyHemosiderosisPathologyLiver biopsyInternal medicineTransferrin saturationMedicineHemoglobin H DiseaseHemoglobinopathyFerritinSerum iron

摘要: We examined the iron status of 23 adult patients with hemoglobin H (Hb H) disease. None them had received multiple blood transfusions or prolonged therapy. Studies included serum and ferritin concentrations, transferrin saturation, a desferrioxamine test, computed tomography (CT) scan liver, liver biopsy. Iron overload was found in 17 (73.9%), especially males splenomegaly (92.9% 100%, respectively). Four excessive alcohol consumption clinical manifestations severe overload. Idiopathic hemochromatosis associated HLA antigens, i.e., HLA-A3, -B7, -B14, were not any 15 tested. These findings indicate that is common Hb disease; such should abstain from be considered for treatment an chelating agent before irreversible organ damage occurs.

参考文章(25)
Martin J. Pippard, Sunday Stray, Simple assay for urinary iron after desferrioxamine therapy. American Journal of Clinical Pathology. ,vol. 77, pp. 324- 327 ,(1982) , 10.1093/AJCP/77.3.324
Silvia Fargion, Maria Domenica Cappellini, Alberto Piperno, Nikos Panajotopoulos, Guido Ronchi, Gemino Fiorelli, Association of hereditary spherocytosis and idiopathic hemochromatosis. A synergistic effect in determining iron overload. American Journal of Clinical Pathology. ,vol. 86, pp. 645- 649 ,(1986) , 10.1093/AJCP/86.5.645
Disler P, Lynch S, Jacobs P, Bothwell T, Charlton R, Brink B, Patterns of iron storage in dietary iron overload and idiopathic hemochromatosis. Journal of Laboratory and Clinical Medicine. ,vol. 88, pp. 725- ,(1976)
Bennett L. Blitzer, Gary B. Weiss, George W. Osbaldiston, Richard B. Markham, Roger Aamodt, Paul D. Berk, Sheldon M. Wolff, Anthony S. Fauci, Early idiopathic hemochromatosis with absent stainable bone marrow iron stores Gastroenterology. ,vol. 75, pp. 886- 888 ,(1978) , 10.1016/0016-5085(78)90475-4
P. Pootrakul, R. Rugkiatsakul, P. Wasi, INCREASED TRANSFERRIN IRON SATURATION IN SPLENECTOMIZED THALASSAEMIC PATIENTS British Journal of Haematology. ,vol. 46, pp. 143- 145 ,(2008) , 10.1111/J.1365-2141.1980.TB05946.X
D. A. RIGAS, R. D. KOLER, E. E. OSGOOD, New hemoglobin possessing a higher electrophoretic mobility than normal adult hemoglobin. Science. ,vol. 121, pp. 372- 372 ,(1955) , 10.1126/SCIENCE.121.3141.372
P. Pootrakul, V. Vongsmasa, P. La-ongpanich, P. Wasi, Serum ferritin levels in thalassemias and the effect of splenectomy. Acta Haematologica. ,vol. 66, pp. 244- 250 ,(1981) , 10.1159/000207129
Chung King Li, Hong Wen Peng, Chao Hung Ho, Chien Hui Yung, Iron Overload in Untransfused Patients with Hemoglobin H Disease Acta Haematologica. ,vol. 83, pp. 137- 139 ,(1990) , 10.1159/000205190
S. C. Tso, T. T. Loh, D. Todd, Iron overload in patients with haemoglobin H disease. Scandinavian Journal of Haematology. ,vol. 32, pp. 391- 394 ,(2009) , 10.1111/J.1600-0609.1984.TB00694.X
Robin M. Bannerman, Gerald Keusch, Martha Kreimer-Birnbaum, Vernon K. Vance, Stuart Vaughan, Thalassemia intermedia, with iron overload, cardiac failure, diabetes mellitus, hypopituitarism and porphyrinuria The American Journal of Medicine. ,vol. 42, pp. 476- 486 ,(1967) , 10.1016/0002-9343(67)90276-8