Long-term assessment of T-cell populations in DiGeorge syndrome
Long-term assessment of T-cell populations in DiGeorge syndrome
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DOI：
10.1067/mai.2003.165
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发表时间：
2003-03-01
影响因子：
14.2
通讯作者：
Noroski, LM
中科院分区：
文献类型：
--
作者：
Chinen, J;Rosenblatt, HM;Noroski, LM
DiGeorge综合征患者存在广泛的T细胞缺乏症。部分DiGeorge综合征（pDGS）是具有可检测T细胞功能的患者的首选名称。在免疫学专家中，对于pDGS患者T细胞预防的必要性没有统一的意见。很少有研究已经解决了他们的免疫功能随着时间的推移的自然过程。Objective：本研究的目的是描述免疫参数在pDGS.Methods：我们回顾了45 pDGS患者的病历。在1、6、12、18、24、30、48、60、72、96和120月龄时记录外周血T细胞亚群计数和百分比，并计算每个个体在随访期间T细胞测量值的变化率（斜率）。通过免疫球蛋白的定量和抗体滴度回忆antigens.Results：T细胞亚群计数从pDGS患者普遍低于年龄匹配的正常人群，但没有严重抑郁（即，CD 4（+）T细胞百分比小于15%）的体液免疫进行了评估。CD 3（+）、CD 4+和CD 8（+）T细胞百分比的中位斜率分别为-0.7%、-0.8%和-0.1%/月（1岁），12 - 120月龄各亚群为0.1%/月。所有年龄段的人对植物血凝素的淋巴增殖反应都足够。免疫球蛋白缺陷或生产不足的特定antibodies were not detected.Conclusions：在我们的pDGS患者队列，T细胞数量或功能的显着恶化没有发生随着时间的推移。这一发现的临床意义包括停止T细胞缺乏预防措施的可能性，以及对具有稳定和足够免疫功能的pDGS患者进行重新评估的频率。
Patients with DiGeorge syndrome present with a broad range of T-cell deficiency. Partial DiGeorge syndrome (pDGS) is a preferred designation for patients with detectable T-cell function. Among immunology experts, there is no uniform opinion on the necessity of T-cell precautions for pDGS patients. Few studies have addressed the natural course of their immune function over time.Objective: The objective of this study was to describe the natural history of immune parameters in pDGS.Methods: We reviewed the medical records of 45 pDGS patients. Peripheral blood T-cell subsets counts and percentages were recorded at 1, 6, 12, 18, 24, 30, 48, 60, 72, 96, and 120 months of age, and the rates of change of T-cell measurements over the follow-up period (slopes) were calculated for each individual. Humoral immunity was evaluated by quantification of immunoglobulins and by testing antibody titers to recall antigens.Results: T-cell subsets counts from pDGS patients were generally lower than those of age-matched normal populations but were not severely depressed (ie, CD4(+) T-cell percentage less than 15%). The median of the slopes for CD3(+), CD4+, and CD8(+) T-cell percentages were -0.7%, -0.8%, and -0.1%/month, respectively, in the first year of age and 0.1%/month for each subpopulation from 12 to 120 months of age. Lymphoproliferative responses to phytohemagglutinin were adequate at all ages. Immunoglobulin deficiencies or inadequate production of specific antibodies were not detected.Conclusions: In our pDGS patient cohort, a significant deterioration of T-cell number or function did not occur over time. Clinical implications of this finding include the possibility of discontinuing T-cell deficiency precautions and frequency of reevaluations of pDGS patients with stable and adequate immune function.