Olynyk J K, Luxon B A, Britton R S, Bacon B R
Department of Internal Medicine, Saint Louis University School of Medicine, Missouri 63110-0250, USA.
Am J Gastroenterol. 1998 Mar;93(3):346-50. doi: 10.1111/j.1572-0241.1998.00346.x.
Biochemical measurement of the hepatic iron concentration (HIC) is essential for the diagnosis of hereditary hemochromatosis (HH). The aim of this study was to determine whether the HIC at the time of diagnosis could predict the subsequent phlebotomy requirements and to determine whether saturation of HIC occurred in HH.
Fifty-four patients (32 male, 22 female) with homozygous HH were evaluated, and HIC was measured in liver biopsies. Patients were subjected to weekly phlebotomy (500 ml) until the transferrin saturation was <50% and/or the serum ferritin concentration was <50 microg/L. The relationship between HIC and total body iron stores (as measured by phlebotomy requirements) was determined using both linear and nonlinear (sigmoidal model) least squares regression.
The HIC ranged from 3,742 to 41,040 microg/g dry wt. A linear relationship between HIC and total body iron stores (iron removed, IR, g) best described the data both in male (HIC = 1986 IR - 3494; r = 0.83; p < 0.001) and female HH patients (HIC = 1251 IR + 2690; r = 0.75; p < 0.001). Men required eight more phlebotomies (2 g iron) on average, compared with women, to reach normal iron stores. There was no evidence of saturation of hepatic iron levels at higher total body iron stores. However, accurate prediction of individual phlebotomy requirements based on the HIC or serum ferritin concentration at the time of diagnosis was not possible.
The phlebotomy requirement for treatment of HH cannot be accurately predicted from the initial HIC or serum ferritin level. Within the range examined, hepatic iron deposition did not saturate in HH.
肝脏铁浓度（HIC）的生化检测对于遗传性血色素沉着症（HH）的诊断至关重要。本研究旨在确定诊断时的HIC是否能够预测后续放血治疗的需求，并确定HH患者的HIC是否会饱和。
对54例纯合子HH患者（32例男性，22例女性）进行评估，并在肝活检中测量HIC。患者每周进行一次放血（500毫升），直到转铁蛋白饱和度<50%和/或血清铁蛋白浓度<50微克/升。使用线性和非线性（S形模型）最小二乘法回归确定HIC与全身铁储存（通过放血需求测量）之间的关系。
HIC范围为3742至41040微克/克干重。HIC与全身铁储存（去除的铁，IR，克）之间的线性关系最能描述男性（HIC = 1986 IR - 3494；r = 0.83；p < 0.001）和女性HH患者（HIC = 1251 IR + 2690；r = 0.75；p < 0.001）的数据。与女性相比，男性平均需要多进行8次放血（2克铁）才能达到正常铁储存。在全身铁储存较高时，没有证据表明肝脏铁水平会饱和。然而，根据诊断时的HIC或血清铁蛋白浓度无法准确预测个体放血需求。
无法根据初始HIC或血清铁蛋白水平准确预测HH治疗的放血需求。在所研究的范围内，HH患者的肝脏铁沉积不会饱和。
