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目的 :通过对 2 5例低丙球血症患儿的临床及实验室指标的检查 ,探查其病因 ,提出早期诊断及治疗的方法 ,以期挽救病儿生命并提高患儿的生命质量。方法 :利用IgG、IgA、IgM单抗 ,通过放射比浊法测定患儿血清IgG、A、M水平。利用CD3 FITC、CD4 E、CD8 FITC和CD19 PE ,通过流式细胞仪 (FACS)检测病人淋巴细胞亚群 ,临床观察患儿临床表现及IVIG替补治疗效果。结果 :1.全部患儿均有各系统反复感染史 ,以呼吸道感染最多见 ,其次为腹泻等。 2 .全部病例均有血清IgG水平的显著下降 ,特别是IgG1,2 ,3亚型的降低 ,10例B细胞降低 ,仅 1例T细胞降低。故根据上述病史及上述指标检测即可确诊本病。 3 经基因及其产物蛋白检测 ,明确诊断 :CVID 14例 ,高IgM血症 1例 ,THG 4例 ,XLA 6例。结论 :1 低丙球血症是反复感染的原因之一。 2 需要应用多种高技术的检测方法来明确其病因。 3 低丙球血症可应用长期静脉替补丙球进行治疗。
OBJECTIVE: To investigate the etiological factors of 25 children with hypo-cysteinemia and to provide an early diagnosis and treatment method for them to save their lives and improve their quality of life. Methods: Serum IgG, A and M levels were measured by nephelometry using IgG, IgA and IgM monoclonal antibodies. The lymphocyte subsets of patients were detected by flow cytometry (FACS) with CD3 FITC, CD4 E, CD8 FITC and CD19 PE. The clinical manifestations and the effect of IVIG replacement therapy were observed clinically. Results: 1.All of the infants had the history of repeated infections of each system, with the most common respiratory infections, followed by diarrhea. Serum IgG levels decreased significantly in all cases, especially in the reduction of IgG1, 2 and 3 subtypes, 10 cases of B cells decreased, and only 1 case of T cells decreased. Therefore, according to the above history and the above indicators to detect the disease can be diagnosed. 3 gene and its product protein detection, a clear diagnosis: CVID 14 cases, 1 case of hyper IgMmia, THG 4 cases, 6 cases of XLA. Conclusions: 1 Hypovolemia is one of the causes of recurrent infections. 2 Need to apply a variety of high-tech detection methods to determine the etiology. 3 low prolactin can be applied to long-term intravenous replacement of ciliary ball for treatment.