TY - JOUR
T1 - The heart of the matter
T2 - Secondary hypogammaglobulinemia and constrictive pericarditis
AU - Schmitt, Erica G.
AU - Dalal, Aarti S.
AU - Kothari, Alok
AU - Kitcharoensakkul, Maleewan
N1 - Publisher Copyright:
Copyright © 2021 by the American Academy of Pediatrics
PY - 2021/6/1
Y1 - 2021/6/1
N2 - 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 a-1antitrypsin 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.
AB - 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 a-1antitrypsin 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.
UR - http://www.scopus.com/inward/record.url?scp=85107624332&partnerID=8YFLogxK
U2 - 10.1542/peds.2020-021808
DO - 10.1542/peds.2020-021808
M3 - Article
C2 - 34049957
AN - SCOPUS:85107624332
SN - 0031-4005
VL - 147
JO - Pediatrics
JF - Pediatrics
IS - 6
M1 - e2020021808
ER -