The role of human rhinovirus/enterovirus (HRV/HEV) in severe lower respiratory tract infections remains unclear. We characterized the respiratory status of children admitted to a large academic pediatric intensive care unit (PICU) who tested positive for only HRV/HEV. One hundred and fifty-five children met inclusion criteria with 62% requiring positive pressure respiratory support of 5 cm of water pressure or more within the first 24 hours of admission. Among them, 34% had SaO2 to FiO2 ratios of 264 or less with 22 patients (14%) meeting criteria for pediatric acute respiratory distress syndrome. HRV/HEV is associated with significant respiratory disease in children admitted to the PICU.
Chronic granulomatous disease (CGD) is a primary immune deficiency due to defects in phagocyte respiratory burst leading to severe and life-threatening infections. Patients with CGD also suffer from disorders of inflammation and immune dysregulation including colitis and granulomatous lung disease, among others. Additionally, patients with CGD may be at increased risk of systemic inflammatory disorders such as hemophagocytic lymphohistiocytosis (HLH). The presentation of HLH often overlaps with symptoms of systemic inflammatory response syndrome (SIRS) or sepsis and therefore can be difficult to identify, especially in patients with a primary immune deficiency in which incidence of infection is increased. Thorough evaluation and empiric treatment for bacterial and fungal infections is necessary as HLH in CGD is almost always secondary to infection. Simultaneous treatment of infection with anti-microbials and inflammation with immunosuppression may be needed to blunt the hyperinflammatory response in secondary HLH. Herein, we present a series of X-linked CGD patients who developed HLH secondary to or with concurrent disseminated CGD-related infection. In two patients, CGD was a known diagnosis prior to development of HLH and in the other two CGD was diagnosed as part of the evaluation for HLH. Concurrent infection and HLH were fatal in three; one case was successfully treated, ultimately receiving hematopoietic stem cell transplantation. The current literature on presentation, diagnosis, and treatment of HLH in CGD is reviewed.
Continuous positive airway pressure (CPAP) is a form of non-invasive ventilation used to support pediatric patients with acute respiratory infections. Traditional CPAP interfaces have been associated with inadequate seal, mucocutaneous injury, and aerosolization of infectious particles. The helmet interface may be advantageous given its ability to create a complete seal, avoid skin breakdown, and decrease aerosolization of viruses. We aim to measure tolerability and safety in a pediatric population in the United States and ascertain feedback from parents and health care providers. We performed a prospective, open-label, single-armed feasibility study to assess tolerability and safety of helmet CPAP. Pediatric patients 1 month to 5 years of age admitted to the pediatric intensive care unit with pulmonary infections who were on CPAP for at least 2 hours were eligible. The primary outcome was percentage of patients tolerating helmet CPAP for 4 hours. Secondary measures included the rate of adverse events and change in vital signs. Qualitative feedback was obtained from families, nurses, and respiratory therapists. Five patients were enrolled and 100% tolerated helmet CPAP the full 4-hour study period. No adverse events or significant vital sign changes were observed. All family members preferred to continue the helmet interface, nursing staff noted it made cares easier, and respiratory therapists felt the set up was easy. Helmet CPAP in pediatric patients is well-tolerated, safe, and accepted by medical staff and families in the United States future randomized controlled trials measuring its effectiveness compared with traditional CPAP interfaces are needed.
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