Management of immune thrombocytopenic purpura in children.

Management of immune thrombocytopenic purpura in children.
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儿童免疫性血小板减少性紫癜的治疗。

DOI:
10.1046/j.1468-0734.2001.00040.x
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发表时间:
2001
期刊:
Reviews in clinical and experimental hematology
影响因子:
--
通讯作者:
H. Gadner
H. Gadner
中科院分区:
--
文献类型:
--
作者:
H. Gadner

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免疫性血小板减少性紫癜(ITP)是最常见的获得性出血性疾病,发生在以前健康的儿童中,可分为两种主要形式。急性和慢性ITP是良性疾病,无论治疗与否,自发性恢复的可能性都很高。无论给予何种治疗,完全缓解率均可达到80-90%。只有10-20%的儿童血小板减少症持续6个月以上,表现为慢性病程,随着时间的推移,其缓解的可能性也很高(高达80%或更多)。临床病程的多变性,以及缺乏一致的临床特征,使得决定是否及如何治疗变得困难。尽管颅内出血(ICH)的风险仅为0.1-0.9%,但由于担心危及生命的出血,大多数医生被迫对所有有症状的儿童进行治疗。急性ITP常用的治疗方案是皮质类固醇、静脉注射免疫球蛋白(IVIgG)或静脉注射抗d免疫球蛋白(anti-D)。到目前为止,没有证据表明初始治疗可以预防脑出血或慢性病程。慢性ITP通常使用相同的药物,似乎类固醇脉冲可能与IVIgG一样有效。抗- d也被认为是治疗慢性疾病的一种可靠而廉价的替代品。慢性ITP治疗的一个主要问题是反复输注Ig (IVIgG或anti-D)和/或皮质类固醇是否可以推迟或最终排除脾切除术,这必须考虑到只有一小部分对治疗有抵抗力的患者。在这些情况下,应优先采用腹腔镜方法。脾切除术无效的儿童(< 20%的病例)需要使用其他药物进行二线治疗,如环磷酰胺或硫唑嘌呤,并应重新诊断以排除继发性ITP。
Immune thrombocytopenic purpura (ITP) is the most common acquired bleeding disorder occurring in previously healthy children and can be classified into two major forms. Acute and chronic ITP are benign conditions with a high probability of spontaneous recovery with or without therapy. Rates of 80-90% complete remission can be achieved irrespective of the treatment given. In only 10-20% of children thrombocytopenia persists for more than six months, showing a chronic course, which also has a high probability of remitting over time (up to 80% or more). The variability of the clinical course, and the lack of consistent clinical features, make the decision on whether and how to treat difficult. Most physicians are driven to treat all children with symptoms by concern over life-threatening hemorrhage, although the risk of intracranial hemorrhage (ICH) is only 0.1-0.9%. The commonly used treatment regimens for acute ITP are corticosteroids, intravenous immunoglobulins (IVIgG), or intravenous anti-D immunoglobulin (anti-D). So far, there is no evidence that initial therapy can prevent ICH or a chronic course of the disease. In chronic ITP the same drugs are generally used and it seems that pulses with steroids may be just as effective as IVIgG. Anti-D may also be considered a reliable and cheap alternative for chronic disease. A major problem in the management of chronic ITP is the question of whether repeated infusions of Ig (IVIgG or anti-D) and/or corticosteroids can postpone or ultimately preclude splenectomy, which must be considered only for a small proportion of patients resistant to therapy. In these cases, a laparoscopic approach should be preferred. Children who fail to respond to splenectomy (< 20% of cases) warrant second line treatment with other drugs, like cyclophosphamide or azathioprine and deserve a revisit of diagnosis for exclusion of secondary ITP.
特发性血小板减少性紫癜特定儿童抗核抗体检测的临床意义。
DOI: 10.1097/00043426-199707000-00006
发表时间: 1997
期刊: Journal of pediatric hematology/oncology
影响因子: --
作者:
Zimmerman,SA;Ware,RE
通讯作者: Ware,RE
大剂量口服地塞米松治疗慢性儿童特发性血小板减少性紫癜。
DOI: 10.1016/s0022-3476(96)70410-9
发表时间: 1996
期刊: The Journal of pediatrics
影响因子: --
作者:
Adams,DM;Kinney,TR;O'Branski-Rupp,E;Ware,RE
通讯作者: Ware,RE