Costa Peter, Rudolph Bryan, Kogan-Liberman Debora, Manwani Deepa, Silver Ellen J, Ovchinsky Nadia
Section of Pediatric Gastroenterology, Brenner Children's Hospital, Wake Forest Baptist Health, Winston-Salem, NC.
Divisions of Pediatric Gastroenterology, Hepatology, and Nutrition.
J Pediatr Hematol Oncol. 2020 Apr;42(3):214-217. doi: 10.1097/MPH.0000000000001726.
Children with sickle cell disease (SCD) are at risk of liver injury because of sickle cell hepatopathy and iron overload from chronic transfusions (CT). The authors examine the association between iron overload and liver stiffness measurement (LSM) by vibration controlled transient elastography (VCTE), which has been shown to correlate with fibrosis.
Patients 21 years of age and less with SCD had VCTE performed; those who received CT underwent magnetic resonance imaging T2* for iron quantification.
The authors enrolled 42 patients, 17 (40%) of whom received CT. There was no difference in LSM between patients who underwent CT (5.5±1.5 kPa) and those who did not (5.2±2.3 kPa) (P=0.923). There was no correlation between iron quantification and LSM (r=-0.077, P=0.769). However, children 12 years of age and older had abnormal LSM when compared with a reference range (P=0.013).
VCTE is a noninvasive technology that is feasible in children with SCD. LSM values were elevated in older children but did not correlate with iron overload, suggesting that fibrosis may not be affected by iron overload alone. Though additional data are needed, LSM may be a useful test for the progression of liver disease in SCD regardless of iron burden.
镰状细胞病（SCD）患儿因镰状细胞性肝病和慢性输血（CT）导致的铁过载而有肝损伤风险。作者通过振动控制瞬时弹性成像（VCTE）研究铁过载与肝脏硬度测量值（LSM）之间的关联，该技术已被证明与肝纤维化相关。
对21岁及以下的SCD患者进行VCTE检查；接受CT治疗的患者进行磁共振成像T2*检查以进行铁定量分析。
作者纳入了42例患者，其中17例（40%）接受了CT治疗。接受CT治疗的患者（5.5±1.5kPa）与未接受CT治疗的患者（5.2±2.3kPa）的LSM值无差异（P=0.923）。铁定量与LSM之间无相关性（r=-0.077，P=0.769）。然而，与参考范围相比，12岁及以上儿童的LSM异常（P=0.013）。
VCTE是一种非侵入性技术，在SCD患儿中可行。年龄较大儿童的LSM值升高，但与铁过载无关，这表明肝纤维化可能不仅仅受铁过载影响。尽管需要更多数据，但无论铁负荷如何，LSM可能是检测SCD肝病进展的有用检查。
