Duration of illness is an important variable for untreated children with juvenile dermatomyositis

Duration of illness is an important variable for untreated children with juvenile dermatomyositis
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DOI:
10.1016/j.jpeds.2005.10.032
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发表时间:
2006-02-01
影响因子:
5.1
通讯作者:
Ramsey-Goldman, R
Ramsey-Goldman, R
中科院分区:
医学2区
文献类型:
--
作者:
Pachman, LM;Abbott, K;Ramsey-Goldman, R

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目的探讨幼年型皮肌炎(JDM)患儿未经治疗的症状持续时间对临床和实验室状况的影响。研究设计对166例未经治疗的JDM患儿首次就诊时的物理和实验室数据进行分析。疾病活动度评分(DASS)评估皮肤和肌肉受累情况。身高和体重与国家健康和营养调查III数据集进行了比较。结果未经治疗的JDM患儿身高和体重均低于全国常模(P>.0005),非白人儿童较白人儿童虚弱(P>.0005)。年龄较大的儿童有更多的吞咽困难(P=.017)和关节炎(P>.001)。未经治疗的JDM病程与DAS虚弱呈负相关(P>.0005),与DAS皮肤无关,与病理性钙化呈正相关(P=.006)。初诊时4种肌酶(醛缩酶、乳酸脱氢酶、肌酸激酶、谷草转氨酶/天冬氨酸氨基转移酶)水平趋于正常(P>均为0.01)。结论初诊症状持续时间是JDM的重要指标,应纳入诊断标准和治疗强度的决策。
Objective To evaluate the impact of duration of untreated symptoms in children with juvenile dermatomyositis (JDM) on clinical and laboratory status at diagnosis.Study design We examined physical and laboratory data from the first physician visit for 166 untreated children with JDM. Disease activity scores (DASs) assessed skin and muscle involvement. Height and weight were compared with the National Health and Nutrition Examination Survey III dataset. Duration of untreated illness was designated as the time from first sign of rash or weakness to diagnostic visit.Results Boys and girls with untreated JDM were shorter and lighter than national norms (P > .0005 for both), and nonwhite children were weaker than white children (P > .0005). Older children had more dysphagia, (P = .017) and arthritis (P > .001). Duration of untreated JDM was negatively associated with DAS weakness (P > .0005), unrelated to DAS skin, and positively associated with pathological calcifications (P = .006). With untreated disease >= 4.7 months, serum levels of 4 muscle enzymes (aldolase, lactic dehydrogenase, creatine kinase, serum glutamic-oxaloacetic transaminase/aspartate aminotransferase) tended toward normal (P > .01 for each).Conclusions Duration of untreated symptoms is an important variable and should be included in decisions concerning both diagnostic criteria and intensity of therapy for children with JDM.