การเจริญเติบโต ขนาดตับและม้ามในผู้ป่วยฮีโมโกลบิน อี/เบต้าธาลัสซีเมียชนิดพึ่งพาเลือด Growth, liver and spleen of Hb E/beta-thal

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Ampaiwan Chuansumrit
Nongnuch Sirachainan
Pimpun Kitpoka
Praguywan Kadegasem
Duantida Songdej
Werasak Sasanakul
Pakawan Wongwerawattanakoon

Abstract

A retrospective evaluation of the growth of the liver and spleen among 113 patients (66 males, 47 females) with transfusion dependent hemoglobin E/beta-thalassemia (Hb E/β thal) was reported. They received regular transfusions of leucocyte poor red blood cells 10-15 mL/kg every 4 weeks to maintain their pretransfusion Hb at 7-8 g/dL in group 1 (n = 25) and at least 9 g/dL in group 2 (n = 88). A healthy lifestyle involving diet, water intake, sleep, exercise and personal hygiene was advised. Iron chelation was provided when the serum ferritin level approached > 1,000 ng/mL. The results revealed that thalassemia facies were found in group 1 only. The Z-scores of weight and height and the final height of patients in group 1 did not significantly differ from those of group 2. However, the percentage of liver and spleen growth ≥ 5 cm below the costal margin in group 1 was significantly higher than those observed in group 2. Therefore, regular transfusion with pretransfusion Hb at least 9.0 g/dL and subsequent iron chelation should be adequately provided to transfusion dependent Hb E/β thal to achieve the optimal growth and the size of the liver and spleen, less than 5 cm below the costal margin.

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นิพนธ์ต้นฉบับ (Original article)

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