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[儿童自身免疫性特发性血小板减少性紫癜。附100例报告]

[Auto-immune idiopathic thrombocytopenic purpura in children. Apropos of 100 cases].

作者信息

Pladys P, Bergeron C, Bétrémieux P, Goasguen J, Fremond B, Le Gall E

机构信息

Service de pédiatrie-génétique médicale, hôpital Pontchaillou, Rennes, France.

出版信息

Pediatrie. 1993;48(3):181-8.

PMID:8393973
Abstract

The authors report the results of a retrospective study concerning 100 children with immune thrombocytopenic purpura who were referred between January 1981 and January 1991. Twenty-seven children had a chronic evolution with a significantly high risk: girls aged 9 years or more, hospitalization longer than 10 days, or patients with a history of frequent ecchymoses. The risk of haemorrhage was highest at the early phase of the disease and when the platelet count was below 25 G/l. The need for laboratory tests has been controversial and the authors suggest a simplified list of tests after having demonstrated the limited value of tests attempting to document the prepurpuric infectious episode or associated disease. The number of responders was comparable in the group receiving corticosteroids (78%) and in that receiving intravenous immunoglobulins (76.5%), as was the number of complete remissions in both groups (40.4% and 50%, respectively). 20.5% of the patients receiving intravenous immunoglobulins experienced a chronic evolution versus 29.8% in the group receiving corticosteroids (no significant difference). Seventeen patients underwent splenectomy with excellent result, particularly in one child who underwent urgent splenectomy because of intracranial haemorrhage.

摘要

作者报告了一项回顾性研究的结果,该研究涉及1981年1月至1991年1月期间转诊的100例免疫性血小板减少性紫癜患儿。27例患儿病情呈慢性进展,风险显著较高:9岁及以上女童、住院时间超过10天或有频繁瘀斑病史的患者。出血风险在疾病早期以及血小板计数低于25G/l时最高。实验室检查的必要性一直存在争议,作者在证明试图记录紫癜前期感染发作或相关疾病的检查价值有限后,提出了一份简化的检查清单。接受皮质类固醇治疗的组(78%)和接受静脉注射免疫球蛋白治疗的组(76.5%)的缓解者数量相当,两组的完全缓解者数量也相当(分别为40.4%和50%)。接受静脉注射免疫球蛋白治疗的患者中有20.5%病情呈慢性进展,而接受皮质类固醇治疗的组为29.8%(无显著差异)。17例患者接受了脾切除术,效果良好,尤其是一名因颅内出血接受紧急脾切除术的患儿。

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