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婴幼儿期短暂性低丙种球蛋白血症:30例患儿的转归

Transient hypogammaglobulinemia of infancy and early childhood: outcome of 30 cases.

作者信息

Doğu Figen, Ikincioğullari Aydan, Babacan Emel

机构信息

Department of Pediatric Immunology and Allergy, Ankara University, Faculty of Medicine, Ankara, Turkey.

出版信息

Turk J Pediatr. 2004 Apr-Jun;46(2):120-4.

PMID:15214739
Abstract

Tansient hypogammaglobulinemia of infancy (THI) is characterized by an abnormal delay in the onset of immunoglobulin synthesis. In the present study, clinical and immunological data and outcome of 30 patients with THI are presented. Between 1995-2001, 464 patients with frequent upper and lower airway infections admitted to the Pediatric Immunology and Allergy outpatient clinic; 30 of them (aged between 6-46 months) were diagnosed as THI. Patients had periodic evaluations at 3-6 month intervals until their condition had improved clinically and immunoglobulins had normalized. In 21 patients immunoglobulin levels reached normal age-matched levels at a median age of 27 months. In nine patients (aged 25-57 months) immunoglobulin levels were still under 2SD for age and various IgG subclass deficiencies were detected in five of them. The results of the present study indicated that THI is not a rare disease. Long-term follow-up with detailed clinical and laboratory evaluations is needed in these patients.

摘要

婴儿暂时性低丙种球蛋白血症(THI)的特征是免疫球蛋白合成开始出现异常延迟。在本研究中,呈现了30例THI患者的临床、免疫学数据及转归情况。1995年至2001年间,464例因频繁上、下呼吸道感染而入住儿科免疫学与过敏门诊的患者;其中30例(年龄在6至46个月之间)被诊断为THI。患者每隔3至6个月进行定期评估,直至临床病情改善且免疫球蛋白恢复正常。21例患者的免疫球蛋白水平在中位年龄27个月时达到与年龄匹配的正常水平。9例患者(年龄在25至57个月之间)的免疫球蛋白水平仍低于年龄标准差的2倍,其中5例检测到各种IgG亚类缺陷。本研究结果表明,THI并非罕见疾病。这些患者需要进行详细的临床和实验室评估的长期随访。

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