Tso S C, Loh T T, Chen W W, Wang C C, Todd D
Ann Acad Med Singap. 1984 Jul;13(3):487-90.
Body iron status, as measured by serum ferritin, was studied in 101 adult Chinese thalassaemic patients, 46 males and 55 females. Thirty of them had mild disease (beta thalassaemia trait), 56 disease of intermediate severity (haemoglobin H disease) and 15 severe disease (homozygous beta thalassaemia, Hb E-beta thalassaemia and delta beta-beta thalassaemia). The extent of iron overload correlated with the severity of disease. In severe thalassaemia, iron overload occurred early in life and was independent of multiple transfusions. While significant impairment of left ventricular function was present in only one of six patients studied, evidence of decreased pituitary (especially gonadotrophic) function was observed in six out of seven. Hypocalcaemia, probably due to hypoparathyroidism, was seen in one.
通过血清铁蛋白测定的机体铁状态,在101例成年中国地中海贫血患者中进行了研究,其中男性46例,女性55例。他们中有30例患有轻度疾病(β地中海贫血特征),56例患有中度严重程度的疾病(血红蛋白H病),15例患有重度疾病(纯合子β地中海贫血、Hb E-β地中海贫血和δβ-β地中海贫血)。铁过载的程度与疾病的严重程度相关。在重度地中海贫血中,铁过载在生命早期就会出现,且与多次输血无关。虽然在研究的6例患者中只有1例存在左心室功能的显著损害,但在7例中有6例观察到垂体(尤其是促性腺激素)功能减退的证据。1例出现低钙血症,可能是由于甲状旁腺功能减退所致。