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一例普通可变免疫缺陷患者妊娠的病例报告,强调个性化免疫球蛋白替代治疗的必要性。

A case report of pregnancy in a patient with common variable immunodeficiency emphasizing the need for personalized immunoglobulin replacement.

作者信息

Więsik-Szewczyk Ewa, Jahnz-Różyk Karina

机构信息

Department of Internal Medicine, Pneumonology, Allergology, and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland.

出版信息

Medicine (Baltimore). 2018 Nov;97(44):e12804. doi: 10.1097/MD.0000000000012804.

Abstract

RATIONALE

Subcutaneous immunoglobulin administration facilitated by recombinant human hyaluronidase is a new mode of immunoglobulin replacement. It has been approved for treatment in primary and secondary antibody immunodeficiency. To date, it has not been reported in the literature as therapy of choice during pregnancy.

PATIENT CONCERNS

We report a 31-year-old woman with common variable immunodeficiency (CVID) followed during her first pregnancy.

DIAGNOSES

The patient had a history of increased susceptibility to infections and autoimmune phenomena. From diagnosis at the age 29, she received IVIg replacement with partial response to treatment. Due to medical indications and lack of venous access, we had to search for another mode of application. The patient refused traditional, weekly conventional subcutaneous immunoglobulin (SCIg) administration.

INTERVENTIONS

Immunoglobulin replacement therapy was successfully continued during pregnancy after the IV route was replaced with subcutaneous administration facilitated by recombinant human hyaluronidase. The frequency of infusions was every 3-4 weeks.

OUTCOMES

The treatment was effective and well tolerated by the patient who continued it after delivery. Dosage and the schedule of infusions provided sufficient immunoglobulin G (IgG) levels for the newborn baby.

LESSONS

The presented CVID case illustrates that the selection of the mode of immunoglobulin administration has to be a shared decision, which considers both patient preferences and medical needs. This approach is especially important for the pregnancy period. The case shows that the switch from IVIg to fSCIg can be a management option during pregnancy.

摘要

理论依据

重组人透明质酸酶辅助皮下注射免疫球蛋白是一种新的免疫球蛋白替代方式。它已被批准用于原发性和继发性抗体免疫缺陷的治疗。迄今为止,文献中尚未报道其为妊娠期的首选治疗方法。

患者情况

我们报告了一名31岁患有常见变异型免疫缺陷(CVID)的女性,在其首次怀孕期间接受随访。

诊断

该患者有感染易感性增加和自身免疫现象的病史。自29岁确诊以来,她接受静脉注射免疫球蛋白替代治疗,治疗有部分反应。由于医学指征和缺乏静脉通路,我们不得不寻找另一种应用方式。患者拒绝传统的每周常规皮下免疫球蛋白(SCIg)给药。

干预措施

在静脉注射途径被重组人透明质酸酶辅助皮下给药替代后,免疫球蛋白替代治疗在孕期成功继续。输注频率为每3 - 4周一次。

结果

治疗有效,患者耐受性良好,产后继续接受治疗。输注的剂量和时间表为新生儿提供了足够的免疫球蛋白G(IgG)水平。

经验教训

所呈现的CVID病例表明,免疫球蛋白给药方式的选择必须是一个共同的决定,要同时考虑患者的偏好和医疗需求。这种方法在孕期尤为重要。该病例表明,从静脉注射免疫球蛋白转换为重组人透明质酸酶辅助皮下免疫球蛋白(fSCIg)在孕期可以是一种管理选择。

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