Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction

Kawasaki disease is an acute vasculitis of childhood. Its clinical presentation is well known, and coronary artery aneurysms are classical complications. Shock and pleural or pericardiac effusion are rare presentations of the disease. In intensive care units, the disease may be mistaken for septic shock or toxic shock syndrome. Owing to the fact that immunoglobulin therapy improves the course of the disease, especially if given early, and thus the diagnosis should not be delaye

Severe neonatal hyporegenerative anemia due to anti-Vw (anti-MNS9) alloantibody

No abstract availabl

Point-of-care hemostasis in children with congenital heart disease, the POCHEMO study : baseline reference values of thromboelastometry and impedance aggregometry

: Viscoelastic tests and impedance aggregometry allow coagulation evaluation at the bedside, but reference values are scarce in pediatrics. The aim of this study was to establish reference values of thromboelastometry and impedance aggregometry for this population and compare it between age groups. This prospective, single-center, observational study evaluates viscoelastic tests and impedance aggregometry in children with congenital heart disease. A total of 204 children were included with a median age of 3.6 years old. We provide references values for this population with median, percentile 2.5 and percentile 97.5. Infants demonstrate for extrinsic activity a shorter coagulation time (52 [49-55] vs. 56 [51-62] s, P = 0.007) and clot formation time (90 [71-118] vs. 113 [93-146] s, P < 0.0001) so as for intrinsic activity a shorter clot formation time (53 [44-69] vs. 75 [59-92] s, P < 0.0001). The maximal clot firmness was significantly stronger in infants for extrinsic (65 [61-69] vs. 59 [54-63] mm, P < 0.0001), intrinsic (68 [64-70] vs. 61 [57-65] mm, P < 0.0001), and fibrinogen (12 [9-16] vs. 10 [8-13] mm, P = 0.02) activities. Platelet aggregation was significantly higher in infants with an amplitude at 6 min of 28 [23-34] vs. 22 [15-27] Ω, P less than 0.0001, a maximum speed of 11 [9-13] vs. 7 [5-10] Ω/min, P less than 0.0001, and an area under the curve of 120 [92-135] vs. 86 [59-112] Ω min, P less than 0.0001. We provided the first reference values for impedance aggregometry and thromboelastometry in children with congenital heart disease. We showed that these infants tend to have accelerated coagulation and stronger clot firmness compared with older children, but this finding may have only minimal relevance when treating a bleeding child. Trial registration number: ClinicalTrials.gov (clinicaltrials.gov/ct2/show/NCT02387944)

ORCHID (Outcome Registry for CHIldren with severe congenital heart Disease) a Swiss, nationwide, prospective, population-based, neurodevelopmental paediatric patient registry: framework, regulations and implementation

Introduction: Congenital heart disease (CHD) is the most frequent birth defect. As survival has significantly improved, attention has turned to neurodevelopmental outcomes of children undergoing heart surgery in early infancy. Since multiple risk factors contribute to neurodevelopmental alterations, a nationwide registry collecting data on medical characteristics, interventions, clinical course and neurodevelopment until school-age is needed to improve the quality of management, identify risk- and protective factors affecting neurodevelopment, and facilitate multicentre trials. Methods and analysis: The Swiss Outcome Registry for CHIldren with severe congenital heart Disease (ORCHID) is a nationwide, prospective, population-based patient registry developed (1) to collect baseline characteristics and clinical data of CHD patients operated with bypass-surgery or hybrid procedures in the first 6 weeks of life in Switzerland, (2) to monitor long-term neurodevelopment, and (3) to relate clinical characteristics and neurodevelopment to identify risk and protective factors in these children. This registry started data collection relating to pregnancy, birth, preoperative course, catheter-based and surgical treatment, postoperative course and reinterventions in 2019. The primary outcome includes standardised neurodevelopmental assessments at 9 to 12 months, 18 to 24 months and 5.5 to 6 years. We expect to include 80 to 100 children per year. Correlation and regression analyses will be used to investigate risk- and protective factors influencing neurodevelopment. Ethics and dissemination of results: Swiss ORCHID received support by the Accentus Charitable Foundation, the Anna Mueller Grocholoski Stiftung, the Swiss Society of Paediatric Cardiology, the Verein Kinderherzforschung, and the Corelina - Stiftung für das Kinderherz, and was approved by the cantonal ethics committees. Findings will be presented at national and international scientific meetings, and published in peer-reviewed journals. Results will also be shared with patient organizations, primary health care providers, and public health stakeholders to ensure a widespread dissemination of the results

Weak-signal extraction enabled by deep-neural-network denoising of diffraction data

Removal or cancellation of noise has wide-spread applications for imaging and acoustics. In every-day-life applications, denoising may even include generative aspects which are unfaithful to the ground truth. For scientific applications, however, denoising must reproduce the ground truth accurately. Here, we show how data can be denoised via a deep convolutional neural network such that weak signals appear with quantitative accuracy. In particular, we study X-ray diffraction on crystalline materials. We demonstrate that weak signals stemming from charge ordering, insignificant in the noisy data, become visible and accurate in the denoised data. This success is enabled by supervised training of a deep neural network with pairs of measured low- and high-noise data. This way, the neural network learns about the statistical properties of the noise. We demonstrate that using artificial noise (such as Poisson and Gaussian) does not yield such quantitatively accurate results. Our approach thus illustrates a practical strategy for noise filtering that can be applied to challenging acquisition problems.Comment: 8 pages, 4 figure

Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction.

Kawasaki disease is an acute vasculitis of childhood. Its clinical presentation is well known, and coronary artery aneurysms are classical complications. Shock and pleural or pericardiac effusion are rare presentations of the disease. In intensive care units, the disease may be mistaken for septic shock or toxic shock syndrome. Owing to the fact that immunoglobulin therapy improves the course of the disease, especially if given early, and thus the diagnosis should not be delayed

Suspected Case of Drug-Induced Acute Respiratory Distress Syndrome following Trimethoprim-Sulfamethoxazole Treatment

In this article, we reported the case of a child patient who was admitted to our PICU for severe acute respiratory distress syndrome (ARDS) while being treated with trimethoprim-sulfamethoxazole (TMP-SMX) for osteomyelitis. Based on the timing of exposure, lack of alternative explanations, and clinical course similar to previously described cases, we suspect that TMP-SMX may have triggered ARDS. Despite meeting criteria for extracorporeal membrane oxygenation cannulation, conservative management and lung recruitment with high-frequency percussive ventilation could avoid the latter