An "On Demand" canakinumab regimen for treating children with Colchicine-Resistant familial Mediterranean fever - A multicentre study.

OBJECTIVES: Canakinumab, a human monoclonal antibody targeted at interleukin-1 beta, has demonstrated safety and efficacy in preventing familial Mediterranean fever (FMF) attacks among individuals with colchicine-resistant (crFMF). The manufacturer orders prescribe monthly subcutaneous injections. However, a subset of our patients is treated with an "canakinumab on demand " (COD) strategy, with wider intervals between drug administrations. Therefore, we aimed to compare disease activity and drug safety between COD and "canakinumab fixed frequency" (CFF) policies. METHODS: This retrospective study collected data from three Israeli paediatric rheumatology centres, of children with crFMF who were treated with canakinumab. Epidemiological and clinical parameters, cumulative drug dosages, and adverse events were compared between children treated by both policies. RESULTS: Twenty-five (49 %) children were treated according to COD policy and 26 according to CFF policy. Demographic parameters and most of the disease features did not differ significantly between the groups. Both groups showed significant reduction in attacks after canakinumab introduction. The median number (interquartile range) of attacks per month did not differ significantly between the COD and CFF groups (0.33 (0.08, 0.58) and 0.13 (0, 0.5), respectively, p = 0.485 (even though, per definition, COD patients presumably had an attack before receiving the second canakinumab dose). The mean monthly dose was lower for the COD than the CFF group (1.13 ± 1.13 vs. 3.16 ± 1.46 mg/kg, p < 0.001). Adverse events were similar between the groups. CONCLUSION: For individuals with crFMF, COD compared to CFF policy can achieve similar efficacy and safety, with a lower accumulated canakinumab dose, rendering it less immunosuppressive and less expensive.

Clinical implications of extremely elevated C-reactive protein among febrile immunocompetent children.

AIM: To identify the various diagnoses associated with extremely elevated C-reactive protein (CRP) (>30 mg/dL) among immunocompetent children and to evaluate its clinical implications during emergency department (ED) workup and hospital management. METHODS: Children (3 months-18 years) with fever in ED were included, retrospectively. The cohort was divided into two groups-'extremely elevated CRP' (>30 mg/dL) and 'highly elevated CRP' (15-30 mg/dL). RESULTS: Included were 1173 patients with CRP 15-30 mg/dL and 221 with CRP > 30 mg/dL. Bacterial infection was more prevalent among the extremely elevated CRP group (94.1% vs. 78.5%, respectively, p = 0.002). Specifically, bacterial pneumonia (52%), cellulitis (7.2%) and sepsis (4.1%) were more prevalent among this group. More of these patients were reported as 'Ill appearing' [78 (35.3%) vs. 166 (17.4%), p < 0.001]. They were more often treated with fluids [33 (14.9%) vs. 50 (5.3%), p < 0.001] and a higher portion of them required admission to an intensive care unit [11 (5.0%) vs. 16 (1.7%), p = 0.007]. CONCLUSION: Febrile children with extremely elevated CRP showed greater illness severity (haemodynamic instability, PICU admissions), thus careful clinical attention is desirable in these cases. More than half of them had bacterial pneumonia, which reinforces the importance of relevant investigation when diagnosis is unclear.

The effect of COVID-19 pandemic on hospitalizations and disease characteristics of adolescents with anorexia nervosa.

Social distancing and quarantines have major negative psychological implications. Our aim was to study the rate of pediatric hospitalizations due to anorexia nervosa (AN) during the first year of coronavirus disease 2019 (COVID-19) pandemic as compared to previous years, with regard to clinical and laboratory parameters. This is a retrospective study in a tertiary pediatric hospital in Israel. Data regarding inpatient hospitalizations due to AN were retrieved, then epidemiological, clinical, and laboratory parameters compared. During the entire study period, 275 hospitalizations were due to AN: 94 patients were admitted during the 12 months of the pandemic as compared to a yearly mean of 45.25 during 2015-2019, resulting in a 2.4-fold increase (p < 0.001). The mean admission age and female predominance were similar in the two study groups. The weight of the patients at admission was higher during the COVID-19 period (44.5 kg vs. 41.2 kg, p = 0.004), and fewer patients had clinical signs typical of AN upon physical examination (p = 0.022). There was a 35% reduction in median hospitalization duration (9 days [IQR 8.21] in 2020-2021 and 14 [IQR 6, 16.75] days in 2015-2019, p = 0.01). No other differences were found. CONCLUSIONS: During the first year of the COVID-19 pandemic, there was a significant increase in the number of adolescents hospitalized with AN. Nevertheless, disease characteristics were not more severe as compared to the preceding 5 years. WHAT IS KNOWN: • Social distancing and quarantines were announced during the COVID-19 pandemic in numerous countries. These measures have potential negative psychological effects on adolescents. WHAT IS NEW: • During the COVID-19 pandemic period, there was an increase in the number of hospitalizations of adolescents with AN, although disease characteristics were not more severe as compared to the preceding 5 years.