Paz-Filho Gilberto Jorge, Delibasi Tuncay, Erol Halil Kutlu, Wong Ma-Li, Licinio Julio
John Curtin School of Medical Research, Australian National University, Canberra, Australia.
J Pediatr Endocrinol Metab. 2009 Nov;22(11):1069-74. doi: 10.1515/jpem.2009.22.11.1069.
The few identified leptin-deficient children have immune deficiency.
To evaluate whether a newly-identified leptin-deficient boy has immune defects; to assess the immune changes during leptin replacement.
A 5 year-old boy with congenital leptin deficiency was evaluated before, 2 weeks and 6 weeks after the initiation of recombinant methionyl human leptin. Thymic volume was measured by computed tomography. Humoral immunity was assessed by measuring levels of several immunoglobulins. Cellular immunity was evaluated by the analysis of lymphocyte proliferation in response to mitogens. Lymphocyte subsets were quantified by flow cytometry.
At baseline, thymic volume was increased. The lymphocyte subsets count and humoral/cellular immunities were normal. After treatment, proliferative response to mitogens increased by 1.5- to 3-fold, and lymphocyte count decreased by 17%.
Immune defects are not an obligatory feature of congenital leptin deficiency. Even in the absence of significant immune defects, leptin replacement therapy enhanced T-cell responsiveness.
少数已确诊的瘦素缺乏儿童存在免疫缺陷。
评估一名新确诊的瘦素缺乏男孩是否存在免疫缺陷;评估瘦素替代治疗期间的免疫变化。
对一名5岁先天性瘦素缺乏男孩在开始重组甲硫氨酰人瘦素治疗前、治疗2周和6周后进行评估。通过计算机断层扫描测量胸腺体积。通过检测几种免疫球蛋白水平评估体液免疫。通过分析淋巴细胞对有丝分裂原的增殖反应评估细胞免疫。通过流式细胞术对淋巴细胞亚群进行定量分析。
基线时,胸腺体积增大。淋巴细胞亚群计数及体液/细胞免疫正常。治疗后,对有丝分裂原的增殖反应增加了1.5至3倍,淋巴细胞计数下降了17%。
免疫缺陷并非先天性瘦素缺乏的必然特征。即使在无明显免疫缺陷的情况下,瘦素替代疗法也可增强T细胞反应性。