id author title date pages extension mime words sentences flesch summary cache txt cord-281259-1wptx49j Kim, Danbi Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant: A case report 2019-02-15 .txt text/plain 2308 139 48 title: Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant: A case report Human immunodeficiency virus infection, malignancy, solid organ or hematopoietic cell transplantation, and primary immune deficiency compose the risk factors for Pneumocystis pneumonia (PCP) in children, and PCP can be an initial clinical manifestation of primary immune deficiency. [9] [10] [11] [12] Especially, 43% of infants diagnosed with X-linked HIGM syndrome before 1 year of age initially presented with PCP. However, pediatricians should consider that PCP can be an initial clinical presentation of PID, [4] [5] [6] and therefore, PCP should be suspected in infants with severe interstitial pneumonia accompanying normal breathing sounds when common viral and bacterial pathogens are not identified, even though the infants may show no evidence of immune deficiency. In conclusion, we report an infant who presented with severe interstitial pneumonia and was diagnosed with PCP as the initial manifestation of underlying HIGM syndrome. Pneumocystis jiroveci pneumonia revealing de novo mutation causing X-linked hyper-IgM syndrome in an infant male. ./cache/cord-281259-1wptx49j.txt ./txt/cord-281259-1wptx49j.txt