Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant: A case report

Kim, Danbi; Shin, Ju Ae; Han, Seung Beom; Chung, Nack-Gyun; Jeong, Dae Chul

In children, Pneumocystis pneumonia (PCP) was mostly reported as epidemics in premature and malnourished infants in the 1940s and 1950s. [1] After then, most cases of PCP developed in children with immunocompromised states, and patients infected with human immunodeficiency virus (HIV) have comprised a majority of patients with PCP since the 1980s. [2] Accordingly, the suspicion and diagnosis of PCP in children without any evidence of an underlying immunocompromised state are not easy. Primary immune deficiency (PID), as well as HIV infection, malignancy, and transplantation, are known as risk factors for PCP, [3] and PCP can be an initial clinical manifestation of PID. [4] [5] [6] We diagnosed a 5-month-old male infant presenting with cyanosis and interstitial pneumonia with PCP. Although he had no previous history consistent with PID, further evaluation revealed X-linked hyper-IgM (HIGM) syndrome, and he received hematopoietic cell transplantation for the underlying HIGM syndrome. This case report was approved by the Institutional Review Board of Seoul St. Mary's Hospital (Approval No.: KC18ZESI0177). Written informed consent for publication was obtained from the patient's parent.