The Heart of the Matter: Secondary Hypogammaglobulinemia and Constrictive Pericarditis.
The Heart of the Matter: Secondary Hypogammaglobulinemia and Constrictive Pericarditis.
复制标题
问题的核心：继发性低丙种球蛋白血症和缩窄性心包炎。
DOI：
10.1542/peds.2020-021808
复制
发表时间：
2021-06
期刊：
影响因子：
8
通讯作者：
Kitcharoensakkul M
中科院分区：
文献类型：
--
作者：
Schmitt EG;Dalal AS;Kothari A;Kitcharoensakkul M
缩窄性心包炎是一系列影响心包的过程的最终共同结果。确定缩窄性心包炎的诊断和潜在病因通常是诊断性会合。在这里，我们描述了一位患者，他在全科医生面前表现为水肿、腹水和体重增加，并被发现继发于纵隔炎性肌纤维母细胞瘤的缩窄性心包炎。有趣的是，她的心肺症状相对较少，除了浮肿和轻微的颈静脉压升高外，她还有一次平淡无奇的心肺检查。在缩窄性心包炎的诊断评估中，发现她有低丙种球蛋白血症和严重的淋巴细胞减少。粪便中α-1-抗胰蛋白酶水平升高，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。本病例强调了重要的诊断考虑和管理的这些并发症的全科医生。我们回顾了一例因炎症性肌纤维母细胞瘤继发缩窄性心包炎的患者的蛋白丢失性肠病和继发性低丙种球蛋白血症。
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 that 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 exam. During the diagnostic evaluation for constrictive pericarditis, she was found to have hypogammaglobulinemia and profound lymphocytopenia. A stool alpha-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. We review a case of protein-losing enteropathy and secondary hypogammaglobulinemia in a patient that developed constrictive pericarditis secondary to an inflammatory myofibroblastic tumor.
登录
查看更多内容
影响因子：
2.4
作者：
Welch, Terrence D.;Oh, Jae K.
通讯作者：
Oh, Jae K.
影响因子：
168.9
作者：
BERNIER, JJ;FLORENT, C;LHIRONDEL, C
通讯作者：
LHIRONDEL, C
影响因子：
1.7
作者：
Mehta, Bhakti;Mascarenhas, Leo;Venkatramani, Rajkumar
通讯作者：
Venkatramani, Rajkumar
影响因子：
4.4
作者：
Wasserman, Richard L.
通讯作者：
Wasserman, Richard L.
影响因子：
4.6
作者：
Murashita, Takashi;Schaff, Hartzell V.;Greason, Kevin L.
通讯作者：
Greason, Kevin L.