To compare the clinical effectiveness of total (TS) or partial (PS) splenectomy in pediatric hereditary spherocytosis, a systematic review and meta-analysis was performed (PROSPERO registration CRD42015030056). There were 14 observational studies comparing pre- and post- operative hematologic parameters. Secondary outcomes include in-hospital infections, surgical complications, symptomatic recurrence and biliary disease. TS is more effective than PS to increase hemoglobin (3.6 g/dL vs 2.2 g/dL) and reduce reticulocytes (12.5% vs 6.5%) after one year; outcomes following PS are stable for at least 6 years. There were no cases of overwhelming post-splenectomy sepsis. A population-based patient registry is needed for long-term follow-up.
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机译：为了比较全（TS）或部分（PS）脾切除术在小儿遗传性球囊细胞增多症中的临床效果，进行了系统的综述和荟萃分析（PROSPERO注册CRD42015030056）。有14项观察性研究比较了术前和术后的血液学参数。次要结局包括医院内感染，外科手术并发症，有症状的复发和胆道疾病。一年后，TS比PS更有效地增加血红蛋白（3.6 g / dL对2.2 g / dL）并减少网状细胞（12.5％对6.5％）； PS后的结局稳定至少6年。脾切除术后没有败血症的病例。需要进行基于人群的患者登记，以便进行长期随访。
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