Divisions of Pediatric Hematology.
Pediatric Gastroenterology, University of Miami Miller School of Medicine, Miami, FL.
J Pediatr Hematol Oncol. 2022 May 1;44(4):155-158. doi: 10.1097/MPH.0000000000002433. Epub 2022 Feb 18.
Transfusion-associated iron overload may cause liver fibrosis. We compared transient elastography (TE) and aspartate aminotransferase-platelet ratio index (APRI), noninvasive markers for hepatic fibrosis, to liver histology in children and young adults with sickle cell disease (SCD) who were iron overloaded (cohort 1). Age-matched subjects with SCD but without iron overload (cohort 2) were enrolled for APRI and TE assessments. Nineteen subjects ages 10 to 21 years were transfused for a mean of 9.67 years, had a mean serum ferritin of 4899±2849 ng/mL, and a liver iron concentration of 15.56±10.12 mg/g dry liver weight by R2-magnetic resonance imaging. Mean APRI was 0.33±0.13 in cohort 1 and 0.27±0.10 in cohort 2. The mean liver stiffness measures (LSM) in cohort 1, assessed by TE, was 8.46±3.95 kPa, ranging from 3.5 to 14.6 kPa (expected normal <7 kPa). Cohort 2 had a mean LSM of 5.72±1.74 kPa (4.6 to 8.7 kPa). There was a good correlation between LSM and histologic fibrosis (t value 6.94, P<0.0001). There was no significant correlation between APRI and histologic fibrosis and between APRI and LSM. A high LSM suggests liver fibrosis in children and adults with SCD with iron overload and may merit histologic confirmation especially if persistent.
输血相关的铁过载可导致肝纤维化。我们比较了瞬态弹性成像（TE）和天门冬氨酸氨基转移酶-血小板比值指数（APRI）这两种非侵入性肝纤维化标志物与铁过载的镰状细胞病（SCD）儿童和年轻成人（队列 1）的肝组织学。为了进行 APRI 和 TE 评估，我们纳入了年龄匹配的无铁过载 SCD 对照（队列 2）。19 名 10 至 21 岁的患者接受输血治疗，平均 9.67 年，血清铁蛋白平均值为 4899±2849ng/mL，肝铁浓度通过 R2 磁共振成像测量为 15.56±10.12mg/g 干肝重。队列 1 的平均 APRI 为 0.33±0.13，队列 2 为 0.27±0.10。通过 TE 评估，队列 1 的平均肝硬度测量值（LSM）为 8.46±3.95kPa，范围为 3.5 至 14.6kPa（预期正常值<7kPa）。队列 2 的平均 LSM 为 5.72±1.74kPa（4.6 至 8.7kPa）。LSM 与组织学纤维化之间存在良好的相关性（t 值 6.94，P<0.0001）。APRI 与组织学纤维化之间以及 APRI 与 LSM 之间均无显著相关性。高 LSM 提示铁过载的 SCD 儿童和成人存在肝纤维化，尤其是持续存在时，可能需要组织学确认。
