Managing Pediatric Asthma Exacerbations: The Role of Timely Systemic Corticosteroid Administration in Emergency Care Settings-A Multicentric Retrospective Study.

BACKGROUND: Asthma is the most prevalent chronic respiratory condition in children. An asthma exacerbation (AE) is a frequent reason for emergency department (ED) visits. An important step in the management of a moderate to severe AE is the administration of systemic corticosteroids (SCS) within 1 h after ED presentation. This study aimed to determine the timing of SCS administration and correlate this with the length of stay and oxygen therapy duration and to explore factors predicting timely administration. METHODS: This study used a retrospective multicenter observational design based on electronic medical records review. Children aged < 18 years, presenting to the ED with a moderate to severe AE were included. RESULTS: 205 patients were included. Only 28 patients received SCS within 60 min after ED arrival. The median time to SCS administration was 169 min (Q1 92-Q3 380). A correlation was found between timing and oxygen treatment duration (r = 0.363, p < 0.001) and length of stay (r = 0.368, p < 0.001). No patient characteristics predicted timely SCS administration. CONCLUSIONS: Three in four children who presented with a moderate to severe AE at the ED did not receive SCS within the first hour. A prolonged timing of SCS administration correlated with a prolonged length of stay and extended need for oxygen support.

Pseudomonas aeruginosa in children with cerebral palsy: a prospective study.

Introduction: Children with cerebral palsy (CP) often present with chronic respiratory symptoms. Pseudomonas aeruginosa (PA), is a known pathogen associated with more severe respiratory disease. Preventive actions to eradicate this bacterium and to improve the respiratory condition of children with CP could be very valuable. Therefore, we assessed the prevalence of PA and its association with respiratory disease. Methods: Throat swabs were taken in children with CP, aged 0-18 years. Data from patient records were extracted from the electronic medical records. Follow-up of respiratory symptoms was done by the Liverpool respiratory symptom questionnaire (LRSQ) after 3 months. Results: A throat swab and a completed LRSQ after 3 months were received from 79 children with CP. Twenty-eight patients (35.4%) were found to have at least one positive respiratory culture. Only 4 patients (5.1%) were contaminated with PA. Gram negative bacteria were isolated in 21.5% of the positive throat swabs, S. aureus was found in 13.9%. Most pathogens were found in patients with higher GMFCS score (GMFCS IV and V). Results of the LRSQ showed that 52.1% of these patients reported having 1 cold in the past 3 months. Discussion: The prevalence of PA in our population of children with CP is low, gram-negative bacteria were most commonly found. The respiratory consequences of being colonized with these bacteria were limited. These results may have been affected by the COVID-19 pandemic. Further research is recommended.

Successful use of fenfluramine as an add-on treatment for Dravet syndrome.

PURPOSE: Despite the development of new antiepileptic drugs, Dravet syndrome frequently remains therapy resistant and is a catastrophic epilepsy syndrome. Fenfluramine is an amphetamine-like drug that has been used in the past as a part of antiobesity treatments. Because of the possible cardiac adverse effects (valve thickening, pulmonary hypertension) associated with use of fenfluramine, it was withdrawn from the market in 2001. In Belgium, a Royal Decree permitted examination of the potential anticonvulsive effects of fenfluramine in a clinical trial consisting of a small group of patients diagnosed with Dravet syndrome. METHODS: Herein, we report 12 patients, 7 female and 5 male, with a genetically proven (11 of 12) diagnosis of Dravet syndrome who received fenfluramine as add-on therapy. KEY FINDINGS: Their ages at their last evaluation ranged from 3-35 years. The mean dosage of fenfluramine was 0.34 (0.12-0.90) mg/kg/day. Exposure duration to fenfluramine ranged from 1-19 years. Seven of the patients who were still receiving the fenfluramine treatment at the time of the last visit had been seizure-free for at least 1 year. In total, patients had been seizure-free for a mean of 6 (1-19) years. In seven patients, the fenfluramine treatment was interrupted once during the follow-up; seizures reappeared in three of the seizure-free patients. Subsequent reintroduction of fenfluramine controlled the seizures in these three patients again. Only two patients exhibited a mild thickening of one or two cardiac valves without clinical significance. SIGNIFICANCE: Compared with a recent long-term follow-up series in which a maximum of 16% of patients with Dravet syndrome were seizure-free, our result of 70% of patients with Dravet syndrome remaining seizure-free is noteworthy. Given the limitations of this observational study, a larger prospective study should be undertaken to confirm these promising results.

Sarcomatous degeneration in fibrous dysplasia of the rib cage.

Malignant degeneration in fibrous dysplasia is a rare occurrence. Most cases are reported in polyostotic fibrous dysplasia with predisposition of the femur, tibia, maxilla, and mandible. The most commonly observed malignant tumors are osteosarcoma, fibrosarcoma, and chondrosarcoma. We describe a case of a low-grade osteosarcoma occurring in polyostotic fibrous dysplasia of the rib cage in a 50-year-old man.