Lymphoid interstitial pneumonitis and hypogammaglobulinemia in children.
Lymphoid interstitial pneumonitis and hypogammaglobulinemia in children.
复制标题
儿童淋巴样间质性肺炎和低丙种球蛋白血症。
DOI：
10.1164/arrd.1981.124.4.491
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
Richards,W
中科院分区：
文献类型：
--
作者：
Church,JA;Isaacs,H;Saxon,A;Keens,TG;Richards,W
淋巴样间质性肺炎是一种罕见的过程，与儿童低γ -球蛋白血症的关联是罕见的。3名患者，一名10岁男孩（患者1），一名17岁女孩（患者2），一名13岁男孩（患者3）被评估为进行性间质性肺炎和低γ球蛋白血症。在就诊时，肺部疾病的症状或体征已经存在了9年、6年和1.5年，分别包括严重的劳累性呼吸困难、x线片上的无症状肺炎和轻度劳累性呼吸困难。血清免疫球蛋白缺乏是可变的，所有患者的IgG和IgA均下降，患者2的IgM在正常范围内。中性粒细胞功能、T淋巴细胞数量和丝裂原反应均正常。体外免疫球蛋白产生的测量提示原发性B淋巴细胞功能障碍。肺功能检查显示患者1为限制性肺疾病，患者1和患者2为动脉低氧血症，所有患者肺泡-动脉氧差均增加。每位患者的肺标本镜检显示弥漫性淋巴实质浸润，形成滤泡，偶有生发中心，不同程度的纤维化，病毒、细菌和真菌检查均阴性。在这些患者中，导致抗体停止产生和显著淋巴细胞增生过程的因素仍有待确定。
Lymphoid interstitial pneumonitis is an uncommon process, and an association with hypogammaglobulinemia in children is rare. Three patients, a 10-yr-old boy (Patient 1), a 17-yr-old girl (Patient 2), and a 13-yr-old boy (Patient 3) were evaluated for progressive interstitial pneumonitis and hypogammaglobulinemia. At presentation, symptoms or signs of lung disease had been present for 9, 6, and 1.5 yr and consisted of severe exertional dyspnea, asymptomatic pneumonitis on roentgenogram, and mild exertional dyspnea, respectively. Serum immunoglobulin deficiencies were variable with IgG and IgA being depressed in all patients and IgM being within normal limits in Patient 2.In vitroneutrophil function and T lymphocyte number and mitogen responses were normal. Measurement ofin vitroimmunoglobulin production suggested a primary B lymphocyte dysfunction. Pulmonary function testing revealed restrictive lung disease in Patient 1, arterial hypoxemia in Patients 1 and 2, and increased alveolar-arterial oxygen differences in all patients. Microscopic examination of lung specimens from each patient revealed diffuse lymphoid parenchymal infiltration with formation of follicles with occasional germinal centers, varying degrees of fibrosis, and negative examinations for viruses, bacteria, and fungi.The factors responsible for the cessation of antibody production and for the striking lymphoproliferative process seen in these patients remain to be identified.