Differing Performance of the Warning Signs for Immunodeficiency in the Diagnosis of Pediatric Versus Adult Patients in a Two-Center Tertiary Referral Population
Differing Performance of the Warning Signs for Immunodeficiency in the Diagnosis of Pediatric Versus Adult Patients in a Two-Center Tertiary Referral Population
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DOI：
10.1007/s10875-018-0582-z
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发表时间：
2019-01-01
影响因子：
9.1
通讯作者：
Fernandez, James
中科院分区：
文献类型：
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作者：
Bjelac, Jaclyn A.;Yonkof, Jennifer R.;Fernandez, James
目的原发性免疫缺陷(PID)代表一系列临床表现的疾病。医学界寻求临床特征，以促进对免疫缺陷的评估，因为早期识别可以改善预后。我们希望确定临床特征，以提高广泛传播的杰弗里·莫代尔基金会免疫缺陷警告信号的诊断准确性。方法我们对北美两个中心的PID患者队列进行了回顾性图表回顾。对137例儿童和400例成人PID患者的病历进行了评估，并与生化免疫初步评估正常的对照组进行了比较。结果在成人PID患者中，不到45%的人出现了两个警告迹象，而在儿童人群中，诊断实用性得到了提高，因为在儿童人群中，警告迹象对的敏感度达到了%。与对照组相比，反复肺炎患儿的警示征象显著增加(OR 2.9，p
PurposePrimary immunodeficiency (PID) represents disorders with a spectrum of clinical presentations. The medical community seeks clinical features to prompt evaluation for immunodeficiency given improved prognosis with early identification. We hoped to identify clinical characteristics that would improve the diagnostic accuracy of the widely disseminated Jeffrey Modell Foundation warning signs for immunodeficiency.MethodsWe performed a retrospective chart review in a two-center North American cohort of patients with PID. Charts of 137 pediatric and 400 adult patients with PID were evaluated for the presence of these warning signs and compared to controls with normal preliminary biochemical immune evaluation.ResultsFewer than 45% of adults with PID presented with 2 warning signs, while diagnostic utility was improved in the pediatric population where the warning signs were found to be 64% sensitive. The warning signs found in a significantly increased proportion compared to controls differed for pediatric PID patients (recurrent pneumonia (OR 2.9, p