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小儿选择性IgM缺乏症与IgG4缺乏症:一种极其罕见的关联。

Paediatric selective IgM deficiency and IgG4 deficiency: an extremely unusual association.

作者信息

Bolia Rishi, Misra Durga Prasanna, Aggarwal Amita, Srivastava Anshu

机构信息

Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

BMJ Case Rep. 2014 Sep 1;2014:bcr2014204769. doi: 10.1136/bcr-2014-204769.

Abstract

Humoral immunodeficiency disorders present in children after 6 months of age with recurrent respiratory and gastrointestinal infections. These may be due to the absence of B cells causing panhypogammaglobulinaemia, or due to selective deficiencies in immunoglobulin subfractions. We present the case of a child with selective deficiency of IgM and IgG4 resulting in chronic diarrhoea and recurrent lower respiratory infections. With appropriate treatment of infections, the child had good symptom relief. Such an unusual combination of antibody deficiency has not been previously described in children. This case serves to illustrate the need for awareness to institute timely therapy for infection along with appropriate prophylactic measures like vaccination for these children.

摘要

体液免疫缺陷障碍在6个月龄后的儿童中出现,表现为反复的呼吸道和胃肠道感染。这些可能是由于B细胞缺乏导致全低丙种球蛋白血症,或者是由于免疫球蛋白亚组分的选择性缺乏。我们报告一例患有IgM和IgG4选择性缺乏的儿童病例,该患儿出现慢性腹泻和反复的下呼吸道感染。通过对感染进行适当治疗,患儿症状得到了很好的缓解。这种不寻常的抗体缺乏组合此前在儿童中尚未有过描述。该病例说明需要提高认识,以便为这些儿童及时开展感染治疗以及采取适当的预防措施,如接种疫苗。

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