This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To assess the safety and efficacy of intravenous immunoglobulin as add‐on treatment for children with encephalitis.
Purpose of review Studies indicating that non-coding RNAs (ncRNAs) play a regulatory role in sepsis are increasing rapidly. This present review summarizes recent publications on the role of microRNAs and long non-coding RNAs (lncRNAs) in sepsis. Recent findings MicroRNAs (miRNAs) and lncRNAs are being identified as potential sepsis biomarkers and therapeutic targets. Experimental studies have examined the biological mechanisms that might underpin the regulatory role of these ncRNAs in sepsis. Summary Clinical applications of miRNAs and lncRNAs in sepsis are on the horizon. These data could lead to the identification of novel treatments or indeed support the repurposing of existing drugs for sepsis. Validation of the findings from these preliminary studies and crucially integration of multiomics datasets will undoubtedly revolutionize the clinical management of sepsis.
The pterygium syndrome consists of the neck, the antecubital fossae and the popliteal regions together with flexion deformities of the limb joints and anomalies of the vertebrae. A family, three offspring of which appear to be affected with the same disorder, is presented. All three are female; there is also a normal female child of the same union.
Encephalitis is a serious neurologic condition that can result in admission to intensive care. Yet, there are no studies on pediatric intensive care unit (PICU) admission rates and usage of intensive care resources by children with encephalitis in England and Wales. The objectives of this study were to (1) define the PICU incidence and mortality rates for childhood encephalitis, (2) describe the usage of intensive care resources by children with encephalitis admitted to PICU and (3) explore the associated cost from PICU encephalitis admissions.Retrospective analysis of anonymized data for 1031 children (0-17 years) with encephalitis admitted (January 2003 to December 2013) to PICU in England and Wales.The PICU encephalitis incidence was 0.79/100,000 population/yr (95% confidence interval [CI]: 0.74-0.84), which gives an annual total of 214 bed days of intensive care occupancy for children admitted with encephalitis and an estimated annual PICU bed cost of £414,230 (interquartile range: 198,111-882,495) for this cohort. PICU encephalitis admissions increased during the study period (annual percentage change = 4.5%, 95% CI: 2.43%-6.50%, P ≤ 0.0001). In total, 808/1024 (78.9%) cases received invasive ventilation while 216/983 (22.0%) and 50/890 (5.6%) cases received vasoactive treatment and renal support, respectively. There were 87 deaths (8.4%), giving a PICU encephalitis mortality rate of 0.07/100,000 population (0-17 years)/yr (95% CI: 0.05-0.08).These data suggest that encephalitis places a significant burden to the healthcare service. More work is needed to improve outcomes for children with encephalitis.
A 15-year-old boy with trisomy 21, moderate left atrioventricular (AV) valve regurgitation and a previously repaired AV septal defect developed Aerococcus urinae infective endocarditis. The infected left AV valve and surrounding tissue were surgically removed and replaced with a prosthetic mitral valve; this was followed by 6 weeks of intravenous antibiotics. Towards the end of his antibiotic course, his C reactive protein level began to rise, and he subsequently developed worsening hip pain and severe anaemia …
Background: 4CMenB is immunogenic in infants and toddlers.We assessed persistence of human complement serum bactericidal activity (hSBA) following a fourth dose administered at 12, 18 or 24 months and characterised the antibody response to a fifth dose administered at 4 years of age.Methods: A phase 3, open label, multi-centre extension to a randomised controlled trial conducted in four countries (number of centres): Czech Republic (nineteen), Italy (four), Spain (four) and the United Kingdom (four).Four-year-old children who were either 4CMenB-naïve or had previously received a variety of 3-dose infant priming schedules and a booster vaccine as toddlers (follow-on group) were recruited.Venous blood samples were obtained to determine hSBA against four reference strains; acting as targets to assess immunity to each of the vaccine antigens, NadA (5/99), fHbp (H44/76), PorA (NZ98/254), and NHBA (M10713) at baseline (prior to vaccination, all participants) and one month following a dose of 4CMenB for all vaccine-naïve and follow-on participants primed with the 2, 3, 4 schedule, and a third of follow-on participants primed with a 2, 4, 6 month schedule.Results: At baseline (prior to vaccination), the proportion of participants (n = 468) with hSBA titers P 5 was similar across all followon groups: 89-100% against 5/99; 12-35% for H44/76; 8-12% for NZ98/254 and 53-80% for M10713 compared with 5%, 0%, 0%; and 60% respectively, for the vaccinenaïve controls (n = 206).Following a dose of 4CMenB at 4 years of age, this increased to 100% (5/99), 97-100% (H44/76), 80-95 % (NZ98/254) and 84-100% (M10713) (n = 210), compared with 89%, 70%, 24%, and 76% respectively for vaccine-naïve controls (n = 192).Conclusion: Waning of protective antibodies occurred 12-36 months after toddler booster regardless of age at boost.This was least marked against target strains 5/99 and M10713.A robust memory response occurred after a booster dose given at 4 years of age.
The importance of calcium (Ca2+) as a second messenger in T cell signaling is exemplified by genetic deficiencies of STIM1 and ORAI1, which abolish store-operated Ca2+ entry (SOCE) resulting in combined immunodeficiency (CID). We report five unrelated patients with de novo missense variants in ITPR3, encoding a subunit of the inositol 1,4,5-trisphosphate receptor (IP3R), which forms a Ca2+ channel in the endoplasmic reticulum (ER) membrane responsible for the release of ER Ca2+ required to trigger SOCE, and for Ca2+ transfer to other organelles. The patients presented with CID, abnormal T cell Ca2+ homeostasis, incompletely penetrant ectodermal dysplasia, and multisystem disease. Their predominant T cell immunodeficiency is characterized by significant T cell lymphopenia, defects in late stages of thymic T cell development, and impaired function of peripheral T cells, including inadequate NF-κB- and NFAT-mediated, proliferative, and metabolic responses to activation. Pathogenicity is not due to haploinsufficiency, rather ITPR3 protein variants interfere with IP3R channel function leading to depletion of ER Ca2+ stores and blunted SOCE in T cells.
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