Improving quality of pediatric onco-critical care: A 2-year experience using PROACTIVE at a cancer center in Jordan.

PROACTIVE (PediatRic Oncology cApaCity Assessment Tool for IntensiVe CarE) is a consensus-derived tool that evaluates pediatric onco-critical care (POCC) services and identifies gaps amenable to improvement. King Hussein Cancer Center (KHCC), an oncology hospital in Jordan, completed PROACTIVE in 2021 and 2022. We evaluated PROACTIVE's ability to identify gaps and improve POCC services at KHCC by analyzing score changes and interviewing site leaders to understand mechanisms of improvement. Results identified three types of outcomes: direct (e.g., improved multidisciplinary communication), indirect (e.g., guidelines implementation), and other outcomes unrelated to PROACTIVE (e.g., funding mechanisms). PROACTIVE can assist institutions strengthen and monitor POCC services over time.

Case Report: Hypocomplementemic urticarial vasculitis syndrome in a pediatric patient with complement factor 1 deficiency.

Urticarial vasculitis (UV) is a type III hypersensitivity reaction, characterized by immune complex deposition in small vessels leading to complement activation. Hypocomplementemic urticarial vasculitis syndrome (HUVS) represents the most severe form of UV, manifesting as chronic and recurrent urticarial skin lesions with leukocytoclastic vasculitis on histology, hypocomplementemia, anti-C1q antibodies, and systemic organ involvement. This case study focuses on an adolescent who initially presented with invasive pneumococcal infection and was later diagnosed with two rare disorders: HUVS and coexisting complement factor 1 (CF1) deficiency by genotyping. The role of CF1 deficiency in the development of HUVS in this patient is uncertain but has not previously been described.

Case Report: Refractory macrophage activation syndrome requiring high-dose anakinra, emapalumab, and etoposide therapy in early-onset systemic juvenile idiopathic arthritis associated with adenoviremia.

Macrophage activation syndrome (MAS) is a life-threatening condition characterized by the excessive stimulation of macrophages and T lymphocytes, provoked by infections, malignancy, and autoimmune or autoinflammatory conditions such as systemic juvenile idiopathic arthritis (sJIA). Clinical signs of sJIA may include high-spiking, quotidian fevers, lymphadenopathy, hepatosplenomegaly, and a salmon-colored migratory, evanescent rash. By contrast, MAS is characterized by unremitting fevers and diffuse, fixed, maculopapular rashes. In addition to hepatosplenomegaly and lymphadenopathy, patients with MAS may also have clinical signs of coagulopathy, as well as cardiac, lung, renal, and central nervous system dysfunction. The empiric treatment for MAS is initially high-dose IV corticosteroids, but usually requires addition of immunomodulators such as tacrolimus or a biologic such as Anakinra to control. The addition of immunotherapies for MAS has improved patient outcomes. We present a 2-year-old male patient with a history of early-onset sJIA, who presented with MAS refractory to corticosteroids and anakinra triggered by adenoviremia that required addition of emapalumab to control. We believe this is the first reported case of a combination of immunosuppressive therapy of emapalumab, etoposide, anakinra, tacrolimus, and corticosteroids used in the successful treatment of infection-induced MAS in early-onset sJIA. Given the lack of treatment guidelines and approved therapies for MAS, alternative strategies should be considered for patients with an intractable course.