P3.T390: Toddler with Hypoxemic Respiratory Failure

Introduction: Most viral pneumonias are treated with supportive care, but recommended therapy in the setting of severe disease with COVID-19/multisystemic inflammatory syndrome includes steroids because they have been shown to improve outcomes. Severe and atypical infections are often the first manifestation of a primary immunodeficiency among infants and young children. Steroids cause iatrogenic immunosuppression that can distort diagnostic and treatment efforts, potentially leading to complications.

Case Description: A 16-month-old otherwise healthy male presents to the Emergency Department with 48 hours of high fevers. His exam is notable for labored breathing and hypoxia. Lab work reveals an elevated absolute neutrophil count, elevated inflammatory markers, and a positive respiratory panel for seasonal coronavirus. The patient is transferred to an outside hospital for ongoing care. He is subsequently admitted to the pediatric ward where he received Remdesivir and high-dose methylprednisolone for a presumed COVID-19 pneumonia with features of multisystem inflammatory syndrome. His respiratory status declines rapidly, necessitating escalation of care to the pediatric intensive care unit and intubation. Broad spectrum antibiotics are initiated. Tracheal cultures return positive for Aspergillus fumigatus and antifungal coverage is added; all other testing to date is unrevealing for a source of pneumonia. He continues to worsen and within five days of admission he is placed on venovenous extracorporeal membrane oxygenation. Dihydrorhodamine testing reveals inadequate neutrophil superoxide production, consistent with chronic granulomatous disease. The patient develops secondary hemophagocytic lymphohistiocytosis and requires continuous renal replacement therapy as well as immunomodulator therapy. Despite aggressive treatment efforts, the patient’s disease progresses with poor prognosis and the family ultimately elects for comfort care. He dies shortly thereafter in his family’s arms.

Discussion: This case illustrates a bad outcome that resulted from Aspergillus- pneumonia as the presenting infection in a patient with undiagnosed chronic granulomatous disease. Time and again, transfers of care are a contributing factor in complications. In this case, mislabeling of seasonal coronavirus as COVID-19 led to initiation of treatments that likely accelerated the course of this infection. We highlight the importance of clear communication during handoffs as well as the need for judicious steroid use.

Conclusion: COVID-19 is managed differently from seasonal coronavirus and special effort must be made to avoid mislabeling of these viruses. Judicious use of steroids is always needed to avoid exacerbation of other infectious processes and minimize diagnostic confusion. Lastly, though chronic granulomatous disease is relatively rare, physicians should consider this diagnosis when evaluating patients with atypical and severe infections.