Takayasu arteritis in children

Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8–15). Three patients were Caucasians and one Asian. Arterial hypertension was the commonest mode of presentation followed by systemic symptoms. Other related symptoms were due to ischemia and consisted of abdomen, chest, and limb pain. An abdominal bruit was noted in only one patient. Inflammation markers were always abnormal. Angiography was performed in all cases; left subclavian artery and common carotid artery were more frequently involved. Renal artery stenosis was observed in two patients. One boy was diagnosed as having an associated immune deficiency (Wiskott-Aldrich syndrome). Treatment modalities included prednisone (n = 4), methotrexate (n = 3), and mycophenolate mofetil (MMF) (n = 1). Surgery was required in two patients. Follow-up ranged from 3 to 10 years since diagnosis. In three cases antihypertensive drugs and methotrexate were stopped, and prednisone was reduced to 7.5 mg/day

Preemptive treatment in the acute and early subacute phase of uncomplicated type B aortic dissections with poor prognosis factors

ObjectiveDue to its favorable outcome regarding late morbidity and mortality, thoracic endovascular repair (TEVAR) is becoming more popular for uncomplicated type B aortic dissection (TBAD). This study aimed to compare preemptive endovascular treatment and optimal medical treatment (OMT) and OMT alone in patients presenting uncomplicated TBAD with predictors of aortic progression.DesignRetrospective multicenter studyMethodsWe analyzed patients with uncomplicated TBAD and risk factors of progression in two French academic centers. Aortic events [defined as aortic-related (re)intervention or aortic-related death after initial hospitalization], postoperative complications, non-aortic events, and radiologic aortic progression and remodeling were recorded and analyzed. Analysis was performed on an intention-to-treat basis.ResultsBetween 2011 and 2021, preemptive endovascular procedures at the acute and early subacute phase (<30 days) were performed on 24 patients (group 1) and OMT alone on 26 patients (group 2). With a mean follow-up of 38.08 ± 24.53 months, aortic events occurred in 20.83% of patients from group 1 and 61.54% of patients from group 2 (p < .001). No patient presented aortic-related death during follow-up. There were no differences in postoperative events (p = 1.00) and non-aortic events (p = 1.00). OMT patients had significantly more aneurysmal progression of the thoracic aorta (p < .001) and maximal aortic diameter (p < .001). Aortic remodeling was found in 91.67% of patients in group 1 and 42.31% of patients in group 2 (p < .001). A subgroup analysis of patients in group 1 showed that patients treated with preemptive TEVAR and STABILISE had reduced maximum aortic diameters at the 1-year (p = .010) and last follow-up (p = .030) compared to those in patients treated with preemptive TEVAR alone.ConclusionPreemptive treatment of uncomplicated TBAD with risk factors of progression reduces the risk of long-term aortic events. Over 60% of medically treated patients will require intervention during follow-up, with no benefit in terms of postoperative events. Even after surgical treatment, patients in the OMT group had significantly more aneurysmal progression, along with poorer aortic remodeling

A922 Sequential measurement of 1 hour creatinine clearance (1-CRCL) in critically ill patients at risk of acute kidney injury (AKI)

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Relationship between molecular pathogen detection and clinical disease in febrile children across Europe: a multicentre, prospective observational study

BackgroundThe PERFORM study aimed to understand causes of febrile childhood illness by comparing molecular pathogen detection with current clinical practice.MethodsFebrile children and controls were recruited on presentation to hospital in 9 European countries 2016-2020. Each child was assigned a standardized diagnostic category based on retrospective review of local clinical and microbiological data. Subsequently, centralised molecular tests (CMTs) for 19 respiratory and 27 blood pathogens were performed.FindingsOf 4611 febrile children, 643 (14%) were classified as definite bacterial infection (DB), 491 (11%) as definite viral infection (DV), and 3477 (75%) had uncertain aetiology. 1061 controls without infection were recruited. CMTs detected blood bacteria more frequently in DB than DV cases for N. meningitidis (OR: 3.37, 95% CI: 1.92-5.99), S. pneumoniae (OR: 3.89, 95% CI: 2.07-7.59), Group A streptococcus (OR 2.73, 95% CI 1.13-6.09) and E. coli (OR 2.7, 95% CI 1.02-6.71). Respiratory viruses were more common in febrile children than controls, but only influenza A (OR 0.24, 95% CI 0.11-0.46), influenza B (OR 0.12, 95% CI 0.02-0.37) and RSV (OR 0.16, 95% CI: 0.06-0.36) were less common in DB than DV cases. Of 16 blood viruses, enterovirus (OR 0.43, 95% CI 0.23-0.72) and EBV (OR 0.71, 95% CI 0.56-0.90) were detected less often in DB than DV cases. Combined local diagnostics and CMTs respectively detected blood viruses and respiratory viruses in 360 (56%) and 161 (25%) of DB cases, and virus detection ruled-out bacterial infection poorly, with predictive values of 0.64 and 0.68 respectively.InterpretationMost febrile children cannot be conclusively defined as having bacterial or viral infection when molecular tests supplement conventional approaches. Viruses are detected in most patients with bacterial infections, and the clinical value of individual pathogen detection in determining treatment is low. New approaches are needed to help determine which febrile children require antibiotics.FundingEU Horizon 2020 grant 668303

Impact of infection on proteome-wide glycosylation revealed by distinct signatures for bacterial and viral pathogens

Mechanisms of infection and pathogenesis have predominantly been studied based on differential gene or protein expression. Less is known about posttranslational modifications, which are essential for protein functional diversity. We applied an innovative glycoproteomics method to study the systemic proteome-wide glycosylation in response to infection. The protein site-specific glycosylation was characterized in plasma derived from well-defined controls and patients. We found 3862 unique features, of which we identified 463 distinct intact glycopeptides, that could be mapped to more than 30 different proteins. Statistical analyses were used to derive a glycopeptide signature that enabled significant differentiation between patients with a bacterial or viral infection. Furthermore, supported by a machine learning algorithm, we demonstrated the ability to identify the causative pathogens based on the distinctive host blood plasma glycopeptide signatures. These results illustrate that glycoproteomics holds enormous potential as an innovative approach to improve the interpretation of relevant biological changes in response to infection

Genomic investigations of unexplained acute hepatitis in children

Since its first identification in Scotland, over 1,000 cases of unexplained paediatric hepatitis in children have been reported worldwide, including 278 cases in the UK1. Here we report an investigation of 38 cases, 66 age-matched immunocompetent controls and 21 immunocompromised comparator participants, using a combination of genomic, transcriptomic, proteomic and immunohistochemical methods. We detected high levels of adeno-associated virus 2 (AAV2) DNA in the liver, blood, plasma or stool from 27 of 28 cases. We found low levels of adenovirus (HAdV) and human herpesvirus 6B (HHV-6B) in 23 of 31 and 16 of 23, respectively, of the cases tested. By contrast, AAV2 was infrequently detected and at low titre in the blood or the liver from control children with HAdV, even when profoundly immunosuppressed. AAV2, HAdV and HHV-6 phylogeny excluded the emergence of novel strains in cases. Histological analyses of explanted livers showed enrichment for T cells and B lineage cells. Proteomic comparison of liver tissue from cases and healthy controls identified increased expression of HLA class 2, immunoglobulin variable regions and complement proteins. HAdV and AAV2 proteins were not detected in the livers. Instead, we identified AAV2 DNA complexes reflecting both HAdV-mediated and HHV-6B-mediated replication. We hypothesize that high levels of abnormal AAV2 replication products aided by HAdV and, in severe cases, HHV-6B may have triggered immune-mediated hepatic disease in genetically and immunologically predisposed children

The Optical Response of a Mediterranean Shrubland to Climate Change: Hyperspectral Reflectance Measurements during Spring

Remote sensing techniques in terms of monitoring plants’ responses to environmental constraints have gained much attention during recent decades. Among these constraints, climate change appears to be one of the major challenges in the Mediterranean region. In this study, the main goal was to determine how field spectrometry could improve remote sensing study of a Mediterranean shrubland submitted to climate aridification. We provided the spectral signature of three common plants of the Mediterranean garrigue: Cistus albidus, Quercus coccifera, and Rosmarinus officinalis. The pattern of these spectra changed depending on the presence of a neighboring plant species and water availability. Indeed, the normalized water absorption reflectance (R975/R900) tended to decrease for each species in trispecific associations (11–26%). This clearly indicates that multispecific plant communities will better resist climate aridification compared to monospecific stands. While Q. coccifera seemed to be more sensible to competition for water resources, C. albidus exhibited a facilitation effect on R. officinalis in trispecific assemblage. Among the 17 vegetation indices tested, we found that the pigment pheophytinization index (NPQI) was a relevant parameter to characterize plant–plant coexistence. This work also showed that some vegetation indices known as indicators of water and pigment contents could also discriminate plant associations, namely RGR (Red Green Ratio), WI (Water Index), Red Edge Model, NDWI1240 (Normalized Difference Water Index), and PRI (Photochemical Reflectance Index). The latter was shown to be linearly and negatively correlated to the ratio of R975/R900, an indicator of water status

Type B Aortic Dissection Treated With a Branched Aortic Arch Stent Graft and the STABILISE Technique

We report the case of a 57-year-old woman diagnosed with an asymptomatic chronic type B aortic dissection. The maximum aortic diameter was 70 mm in the proximal descending thoracic aorta. The entry tear was located at the aortic isthmus, and the proximal neck included all of the supra-aortic trunks. The targeted proximal neck was ≥q 25 mm. The dissection extended to the infrarenal aorta. The patient was treated with a custom branched aortic graft with two branches, one for the innominate trunk and one for the left common carotid artery, combined with the stent-assisted balloon-induced intimal disruption and relamination technique. This combined technique seemed to provide a proximal seal zone in the arch and allow remodeling of the distal aorta in this patient with aneurysmal type B aortic dissection

Takayasu arteritis in children

Abstract Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8–15). Three patients were Caucasians and one Asian. Arterial hypertension was the commonest mode of presentation followed by systemic symptoms. Other related symptoms were due to ischemia and consisted of abdomen, chest, and limb pain. An abdominal bruit was noted in only one patient. Inflammation markers were always abnormal. Angiography was performed in all cases; left subclavian artery and common carotid artery were more frequently involved. Renal artery stenosis was observed in two patients. One boy was diagnosed as having an associated immune deficiency (Wiskott-Aldrich syndrome). Treatment modalities included prednisone (n = 4), methotrexate (n = 3), and mycophenolate mofetil (MMF) (n = 1). Surgery was required in two patients. Follow-up ranged from 3 to 10 years since diagnosis. In three cases antihypertensive drugs and methotrexate were stopped, and prednisone was reduced to 7.5 mg/day.</p