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KMID : 0387820130200010055
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Clinical Pediatric Hematology-Oncology
2013 Volume.20 No. 1 p.55 ~ p.58
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Long-term Follow-up of Hb Madrid Patient
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Koo Dong-Hoe
Oh Suk-Joong
Kim Hong-Joo
Jung Hye-Lim
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Abstract
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We report a long-term follow-up of unstable hemoglobin (Hb) patient. He was diagnosed as Hb Madrid [b115(G17)Ala→Pro] by direct DNA sequencing and restriction enzyme analysis. Hydroxyurea had been given for b-chain hemoglobinopathies through activation of gamma(g)-chain synthesis. Nowadays he still needs transfusion three or four times per year, but he had been free of hemolytic crisis after hydroxyurea. Although he has been treated for hemochromatosis with parenteral and oral iron-chelating agents, liver cirrhosis complicated by esophageal varix was developed and treated with endoscopic ligation. In addition, he is on warfarin maintenance for anticoagulation therapy for extensive portal vein and superior mesenteric vein thrombosis which presented with abdominal pain and diagnosed by CT scan. In management of unstable Hb patients, physician should monitor and control the serum ferritin level with iron-chelating agents, and be aware of possible long-term complication including hemochromatosis, cirrhosis or thromboembolism.
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KEYWORD
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Hb madrid, Follow-up study, Hemochromatosis, Thrombosis, Complications
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