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首页> 外文期刊>Pediatric Reports >Total absence of the pericardium associated with hypogammaglobulinemia and bronchiectasis in a girl
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Total absence of the pericardium associated with hypogammaglobulinemia and bronchiectasis in a girl

机译：在一个女孩中完全没有心血糖和支气管切除的心包

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We report the case of an 8-years-old girl with recurrent pulmonary infections and wheezing since infancy, in whom asthma and immunoglobulin-G deficiency were diagnosed at the age of 7 months. Since then, the patient was treated for asthma without any satisfactory control of the disease. Cardiomegaly was finally diagnosed radiologically that led to cardiac assessment. Echocardiography suggested left sided partial anomalous pulmonary venous return that was not confirmed at angio-computed tomography scan and cardiac magnetic resonance imaging. Instead, total absence of the pericardium with relative left lung hypoplasia and left-sided bronchiectasis was diagnosed. Immune defect was confirmed. Adequate treatment by immunoglobulin supplementation and observance of the recommended care of bronchiectasis allowed favorable evolution. This case of an unusual association between an exceptional pericardial malformation and immune deficiency causing lower respiratory tract infections complicated by leftsided bronchiectasis highlights the absolute necessity to explore further any child with insufficient asthma control.
机译：我们举报了一个8岁的女孩，以来的一个8岁的女孩经常发生肺部感染和血液喘息，其中哮喘和免疫球蛋白-G缺乏症在7个月的年龄诊断。从那时起，患者被治疗哮喘，没有任何令人满意的疾病。心脏肿大终于诊断出神经诊断，导致心脏评估。超声心动图表明左侧偏异常肺静脉返回，在血管计算机断层摄影扫描和心脏磁共振成像中未确认。相反，诊断出具有相对左肺发育性和左侧支气管切除术的心包。确认免疫缺陷。通过免疫球蛋白的充分处理补充和遵守支气管扩张的推荐护理允许的进化。这种情况与左侧支气管扩张复杂化的特殊心包畸形和免疫缺陷之间的异常关联，导致左侧支气管切除复杂的呼吸道感染突出了探索哮喘控制不足的任何孩子的绝对必要性。

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