Güngör T, Rohrbach E, Solem E, Kaltwasser J P, Kornhuber B
Department of Paediatric Haematology and Oncology, Johann Wolfgang Goethe University Hospital, Frankfurt/Main, Germany.
Arch Dis Child. 1996 Apr;74(4):323-7. doi: 10.1136/adc.74.4.323.
Nineteen children and adolescents receiving repeated transfusions and subcutaneous desferrioxamine treatment were investigated in an attempt to quantitate iron overload non-invasively. Before patients were started on desferrioxamine individual relationships were correlated for 12 to 36 months between transfused iron, absorbed iron estimated gastrointestinally, and increasing serum ferritin concentrations. Patients with inflammation, increased liver enzymes, or haemolysis were excluded from analysis. The relationship between the variables could be described by a logarithmic regression curve (y = transfused iron [plus eventually gastrointestinally absorbed iron] = iron overload = a+b log [x = serum ferritin]) for each individual patient. All patients showed close correlation (R2) between x and y (median R2 of 0.909, 0.98, and 0.92 in thalassaemia, aplastic anaemia, and sickle cell anaemia patients, respectively). When started on desferrioxamine, current serum ferritin concentrations were used to derive the iron overload from each individual regression curve. The derived estimated iron overload ranged from 0.6 g to 31 g. Left ventricular dilatation was observed in three patients with beta thalassaemia and in one patient with aplastic anaemia with median iron overload of 20.7 (14.1-31.3) g and 24.0 g respectively. Hypothyroidism was found in four patients with beta thalassaemia and one patient with aplastic anaemia with iron overload between 14.7 (6.8 and 26.1) g and 15.1 g respectively. Human growth hormone deficiency was detected in three patients with beta thalassaemia with an iron overload of 4.2 (3.5-6.8) g; all three patients had excellent desferrioxamine compliance.
对19名接受反复输血和皮下注射去铁胺治疗的儿童和青少年进行了研究,试图非侵入性地定量铁过载情况。在患者开始接受去铁胺治疗之前,对输注铁、胃肠道估计吸收的铁以及血清铁蛋白浓度升高之间的个体关系进行了12至36个月的相关性分析。排除有炎症、肝酶升高或溶血的患者进行分析。每个患者的变量之间的关系可用对数回归曲线(y = 输注铁[最终可能加上胃肠道吸收的铁] = 铁过载 = a + b log[x = 血清铁蛋白])来描述。所有患者的x和y之间均显示出密切相关性(R2)(地中海贫血、再生障碍性贫血和镰状细胞贫血患者的R2中位数分别为0.909、0.98和0.92)。开始使用去铁胺时,利用当前血清铁蛋白浓度从每条个体回归曲线得出铁过载情况。得出的估计铁过载范围为0.6克至31克。在3名β地中海贫血患者和1名再生障碍性贫血患者中观察到左心室扩张,其中位铁过载分别为20.7(14.1 - 31.3)克和24.0克。在4名β地中海贫血患者和1名再生障碍性贫血患者中发现甲状腺功能减退,其铁过载分别在14.7(6.8和26.1)克至15.1克之间。在3名铁过载为4.2(3.5 - 6.8)克的β地中海贫血患者中检测到人生长激素缺乏;所有3名患者对去铁胺的依从性都很好。