ABSTRACT
OBJECTIVES: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome. STUDY DESIGN: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected. Five age groups (<1, 1-4, 5-11, 12-16, >16 years) and four geographic macro-areas, Western Europe, Central-Eastern Europe, Latin America, Asian-African resource-limited countries (LRC), were identified. RESULTS: Time to referral was significantly higher in LRC. Intensive anti-inflammatory treatment, including biologics, respiratory support and mechanic ventilation were more frequently used in older children and in European countries. The mortality rate was higher in very young children (<1 year), in older patients (>16 years of age) and in LRC. Multivariate analysis identified the residence in LRC, presence of severe cardiac involvement, renal hypertension, lymphopenia and non-use of heparin prophylaxis, as the factors most strongly associated with unfavorable outcomes. CONCLUSIONS: The stratification of patients by age and geographic macro-area provided insights into the clinical presentation, treatment and outcome of MIS-C. The mortality and sequelae rates exhibited a correlation with the age and geographical areas. Patients admitted and treated in LRC displayed more severe outcomes, possibly due to delays in hospital admission and limited access to biologic drugs and to intensive care facilities.
Subject(s)
Age of Onset , COVID-19 , Registries , SARS-CoV-2 , Systemic Inflammatory Response Syndrome , Humans , Child , COVID-19/epidemiology , COVID-19/mortality , COVID-19/complications , Child, Preschool , Female , Male , Infant , Adolescent , Retrospective Studies , Systemic Inflammatory Response Syndrome/epidemiology , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/therapy , Europe/epidemiology , Infant, NewbornABSTRACT
Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome.
Subject(s)
Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/physiopathology , Adolescent , Churg-Strauss Syndrome/drug therapy , Diagnosis, Differential , Female , HumansABSTRACT
Wegener's granulomatosis (WG) is an uncommon systemic vasculitis that is rarely encountered in children. A 15-year old boy presented with a one-month history of nasal obstruction, hemorrhagic rhinorrhea, malaise, fever, anorexia and weight loss, together with high values of inflammatory markers, microscopic hematuria and progressive decrease of renal functions. Renal biopsy revealed rapidly progressive crescentic glomerulonephritis with rare findings of interstitial and periglomerular granulomas. The diagnosis of WG was established and intravenous methylprednisolone and cyclophosphamide therapy followed by oral application of prednisone and azathioprine led to a complete clinical and laboratory remission of the disease. The second renal biopsy performed after 28 months of treatment did not show any activity of the process. Currently, the boy is without any clinical or laboratory signs of active disease. Since untreated WG has a fatal prognosis, early diagnosis and appropriately aggressive immunosuppressive therapy are necessary for a favorable outcome.
