Divisions of Rheumatology and Immunology.
Cardiology.
Pediatrics. 2021 Jun;147(6). doi: 10.1542/peds.2020-021808.
Constrictive pericarditis is the final common result of a number of processes that affect the pericardium. Establishing the diagnosis and determining the underlying etiology of constrictive pericarditis are often a diagnostic rendezvous. Here, we describe a patient who presented to the general practitioner with edema, ascites, and weight gain and was found to have constrictive pericarditis secondary to an inflammatory myofibroblastic tumor of the mediastinum. Interestingly, she had a relative lack of cardiorespiratory complaints, and, aside from the edema and mildly elevated jugular venous pressure, she had an unremarkable cardiac and pulmonary examination. During the diagnostic evaluation for constrictive pericarditis, she was found to have hypogammaglobulinemia and profound lymphocytopenia. A stool α-1-antitrypsin level was sent and was elevated, which confirmed the diagnosis of protein-losing enteropathy, a rare but important complication of constrictive pericarditis. This case highlights important diagnostic considerations and management of these complications for the general practitioner.
缩窄性心包炎是许多影响心包的过程的最终共同结果。确立缩窄性心包炎的诊断并确定其潜在病因通常是一个诊断上的挑战。在这里，我们描述了一位患者，她因纵隔炎性肌纤维母细胞瘤而出现水肿、腹水和体重增加，到全科医生处就诊，被诊断为缩窄性心包炎。有趣的是，她相对缺乏心肺呼吸投诉，除了水肿和颈静脉压轻度升高外，她的心肺检查无明显异常。在诊断缩窄性心包炎时，她被发现存在低丙种球蛋白血症和严重的淋巴细胞减少症。还进行了粪便α-1-抗胰蛋白酶水平检测，结果升高，这证实了蛋白丢失性肠病的诊断，这是缩窄性心包炎的一种罕见但重要的并发症。本病例强调了全科医生在这些并发症的诊断和处理方面的重要考虑因素。
