Department of Radiological Sciences, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
J Pediatr. 2012 Feb;160(2):281-285.e1. doi: 10.1016/j.jpeds.2011.07.050. Epub 2011 Sep 9.
To assess the effects of chronic erythrocyte transfusions on prevalence of sonographic incidence of organ damage in children with sickle cell anemia (SCA).
Children (N=148; mean age, 13.0 years) with SCA, receiving chronic transfusions (average, 7 years), underwent abdominal sonography at 25 institutions. After central imaging review, spleen, liver, and kidney measurements were compared with published normal values. Potential relations between ultrasound, clinical, and laboratory data were explored via analysis of variance, Student t test, and Cochran-Mantel-Haenzel tests of non-zero correlation.
Average spleen length was similar to normal children, but over one-third had spleen volumes >300 mL, 15 had previous splenectomy for splenomegaly, and 24 had abnormal splenic echotexture. Two-thirds had hepatobiliary disease; 37 had prior cholecystectomy, 46 had gallstones, and 16 had gallbladder sludge. Gallbladder disease correlated with older age (P=.002), longer liver length (P<.001), longer duration of transfusions (P=.034), and higher total bilirubin (P<.001). Liver (P<.001) and renal lengths (P≤.005) were larger than published norms.
In children with SCA, long-term transfusion therapy may not prevent development or progression of abdominal organ dysfunction.
评估慢性红细胞输注对镰状细胞贫血（SCA）患儿超声器官损伤发生率的影响。
148 名儿童（平均年龄 13.0 岁）患有 SCA，接受慢性输血（平均 7 年），在 25 家机构进行腹部超声检查。在中央成像审查后，将脾、肝和肾的测量值与已发表的正常值进行比较。通过方差分析、学生 t 检验和 Cochran-Mantel-Haenzel 检验探索超声、临床和实验室数据之间的潜在关系，用于检验非零相关。
平均脾脏长度与正常儿童相似，但超过三分之一的脾脏体积>300 mL，15 例因脾肿大而行脾切除术，24 例脾脏回声异常。三分之二的患儿有肝胆疾病；37 例有胆囊切除术史，46 例有胆结石，16 例有胆囊泥沙样结石。胆囊疾病与年龄较大（P=.002）、肝长较长（P<.001）、输血时间较长（P=.034）和总胆红素较高（P<.001）相关。肝脏（P<.001）和肾脏长度（P≤.005）大于已发表的正常值。
在 SCA 患儿中，长期输血治疗可能无法预防腹部器官功能障碍的发生或进展。
