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Introduction An early diagnosis of necrotizing enterocolitis (NEC), a major gastrointestinal emergency in preterm newborns, is crucial to improve diagnostic approach and prognosis. We evaluated whether fecal high-mobility group box protein 1 (HMGB1) may early identify preterms at risk of developing NEC. Materials and Methods A case-control study including neonates admitted at the Neonatal Intensive Care Unit (NICU) of the Sapienza University Hospital "Umberto I" in Rome, from July 2015 to December 2016. Stool samples obtained from cases (preterm newborns with NEC) and controls (newborns without NEC) were collected at the enrolment (T0) and within 7-14 days after the first sample collection (T1). HMGB1, extracted and measured with western blot, was reported as densitometry units (DUS). Results HMGB1 levels in 30 cases (n = 28-Bell stage 1, n = 2 Bell stage 2) were higher [T0 21,462 DUS (95% CI, 16,370-26,553 DUS)-T1 17,533 DUS (95% CI, 13,052-22,014 DUS)] than in 30 preterm controls [T0 9,446 DUS (95% CI, 6,147-12,746 DUS)-T1 9,261 DUS (95% CI, 5,126-13,396 DUS), p 2,500 g [6,599 DUS (95% CI, 3,141-10,058 DUS), p = 0.003]. Multivariate analysis showed that the risk of NEC was significantly (p = 0.012) related to the HMGB1 fecal levels at T0. Conclusions We suggest fecal HMGB1 as a reliable marker of early NEC in preterm neonates. This study supports further investigation on the role of fecal HMGB1 assessment in managing preterm newborns at risk of NEC. Further studies are advocated to evaluate diagnostic accuracy of this marker in more severe forms of the disease.Objective Brain function monitoring technology for extracorporeal membrane oxygenation (ECMO) support has been developing quite slowly. Our objective was to explore the data distribution, variation trend, and variability of cerebral tissue regional oxygen saturation (CrSO2) in pediatric patients undergoing ECMO. Methods Eight patients who received venoarterial ECMO (V-A ECMO) were included in our study. All of them accepted continuous CrSO2 monitoring by near-infrared spectroscopy (NIRS) within 12 h of ECMO initiation until ECMO wean. Differences in the CrSO2 distribution characteristic, the variation trend of daily CrSO2, and the variability of CrSO2 for the first 5 days following ECMO initiation were compared between survivors and non-survivors according to pediatric intensive care unit (PICU) mortality. Results The percentage of time of CrSO2 less then 60% against the whole monitoring time was significantly lower in survivors in both hemispheres right 4.34% [interquartile range (IQR) = 0.39-8.55%] vs. 47.45% [IQR = 36.03-64.52%], p = 0.036; left 0.40% [IQR = 0.01-1.15%] vs. 30.9% [IQR = 26.92-49.62%], p = 0.036. Survivors had significantly higher CrSO2 on the first 4 days. Root mean of successive squared differences (RMSSD), the variability variable of CrSO2, was significantly lower in survivors (right 3.29 ± 0.79 vs. 6.16 ± 0.67, p = 0.002; left 3.56 ± 1.20 vs. 6.04 ± 1.44, p = 0.039). Conclusion Lower CrSO2, CrSO2 less then 60% over a longer period of time, and higher fluctuation of CrSO2 are likely associated with PICU mortality in pediatric patients undergoing V-A ECMO. Clinical Trial Registry URL http//www.chictr.org.cn/showproj.aspx?proj=46639, trial registry number ChiCTR1900028021.Introduction Sitosterolemia is a rare condition in children and is often misdiagnosed as familial hypercholesterolemia. Serious complications can result if not treated promptly and effectively. When pediatric patients are diagnosed with sitosterolemia, vascular, and cardiac studies are important to evaluate for the presence of atherosclerosis. Few cases of severe atherosclerotic heart disease in children with sitosterolemia have been reported, making this case worthy of presentation. Case Presentation Here, we report a case of sitosterolemia in an 8-year-old child. The patient presented with severe hypercholesterolemia and xanthoma. He was diagnosed two and a half years prior with familial hypercholesterolemia because his father had elevated cholesterol levels. Nor-NOHA solubility dmso After conventional treatment, the patient was dissatisfied with lipid level control and visited our hospital for further management. Genetic tests of the patient and parents found mutations in intron 7 (NM 022436.2, c.904+1G>A) and intron 9 (NM 022436.2, C. 1324+1de1G) of ABCG5. The 7 intron mutation was from his mother, and the 9 intron mutation was from his father. The patient was diagnosed with sitosterolemia. Results The child was treated with ezetimibe, a low plant sterol diet, and clopidogrel anticoagulant therapy. After 3 months of treatment, the blood lipid level was significantly lower. Conclusion Genetic testing should be completed as soon as possible to avoid misdiagnosis in children with abnormally elevated hypercholesterolemia who have a family history of elevated cholesterol. In addition, clinicians should rule out great arterial lesions and be vigilant in evaluating patients for systemic arterial disease and atherosclerosis.Background Ultrasonography (USG) has been described as an alternative diagnostic tool for malrotation that evaluates the orientation of the superior mesenteric vessels. However, literature concerning the management of patients who do not have abdominal symptoms is limited. We aimed to review the clinical course of infants showing abnormal orientation of the superior mesenteric vessels on USG who were asymptomatic at the time of diagnosis. Methods Seventy asymptomatic infants with abnormal orientation of the superior mesenteric vessels in a single center between 2014 and 2018 were retrospectively analyzed. Results The 70 patients, 21 underwent upper gastrointestinal series (UGIS) and 11 underwent abdominal surgery for other surgical conditions. Among the 32 (45.7%) patients who underwent UGIS or abdominal surgery, 11 were proven to have malrotation. Of the 38 (54.3%) patients who did not undergo UGIS or abdominal surgery, six patients were too unstable to undergo UGIS, five died due to cardiac complications, and the remaining patient developed midgut volvulus and died 3 days after emergency surgery. The remaining 32 patients who did not undergo UGIS or abdominal surgery were discharged without additional tests, and all were asymptomatic until their last follow-up. In the multivariate analysis, history of heart surgery and the presence of more than three anomalies were significantly associated with malrotation. Conclusion A significant number of malrotation were diagnosed in asymptomatic infants with abnormal orientation of the superior mesenteric vessels on USG. Infants with major cardiac or multiple anomalies need special attention and should undergo UGIS in a promptly manner to confirm malrotation.Objective To identify risk and protective factors for mental health symptoms associated with lifestyle changes caused by home confinement in pediatric subjects and in children and adolescents with a neuropsychiatric disorder. Study design This was a prospective, cross-sectional study conducted from May 10 to May 31, 2020. Two online anonymous surveys were developed population-based and clinical-based (children with neuropsychiatric disorders). Outcomes included emotional and behavioral symptoms, as assessed by psychometric scales (BPSC, PPSC, PSC, CES-DC and SCARED, respectively), and lifestyle changes during home confinement (i.e., physical activity, screen time, home schooling, reading). Results The sample included 9,688 pediatric subjects, and 289 children and adolescents with a neuropsychiatric disorder. The presence of siblings was a protective factor in all ages. In pre- and school children male sex, a diagnosis of autism, residency in highly affected areas, high parental educational level or job loss, and screen time (>2 h/day) were risk factors. Physical activity, home-schooling, reading, talking with other people were protective factors. Residency in highly affected areas, a diagnosis of mood disorder, parental job loss, and screen time, were associated with a worsening of the depressive symptoms, whereas physical activity, talking with other people, playing with parents were protective activities. Screen time was also a risk factor for anxiety symptoms, while physical activity, reading and talking with other people were protective factors. Conclusions This study identified risk and protective factors for mental health symptoms associated with lifestyle changes caused by COVID-19 home confinement to promote mental well-being in pediatrics during pandemic times.Introduction Safety incidents preceding manifest adverse events are barely evaluated in neonatal intensive care units (NICUs). This study aimed at identifying frequency and patterns of safety incidents in our NICU. Methods A 6-month prospective clinical study was performed from May to October 2019 in a German 10-bed level III NICU. A voluntary, anonymous reporting system was introduced, and all neonatal team members were invited to complete paper-based questionnaires following each particular safety incident. Safety incidents were defined as safety-related events that were considered by the reporting team member as a "threat to the patient's well-being" which "should ideally not occur again." Results In total, 198 safety incidents were analyzed. With 179 patients admitted, the incident/admission ratio was 1.11. Medication errors (n = 94, 47%) and equipment problems (n = 54, 27%) were most commonly reported. Diagnostic errors (n = 19, 10%), communication problems (n = 12, 6%), errors in documentation (n = 9, 5%) and hygiene problems (n = 10, 5%) were less frequent. Most safety incidents were noticed after 4-12 (n = 52, 26%) and 12-24 h (n = 47, 24%), respectively. Actual harm to the patient was reported in 17 cases (9%) but no life-threatening or serious events occurred. Of all safety incidents, 184 (93%) were considered to have been preventable or likely preventable. Suggestions for improvement were made in 132 cases (67%). Most often, implementation of computer-assisted tools and processes were proposed. Conclusion This study confirms the occurrence of various safety incidents in the NICU. To improve quality of care, a graduated approach tailored to the specific problems appears to be prudent.MIS-C is a novel clinical syndrome in children and adolescents, was first encountered in the spring of 2020 as a post COVID-19 multisystem inflammatory syndrome. The highest number of SARS-CoV-2 infections in Austria were registered in November 2020. In December 2020, eight patients with MIS-C were hospitalized at the pediatric department of Klinik Donaustadt, a large municipal hospital in Vienna. Six of the patients needed pediatric intensive care. As we lack any international consensus on the diagnosis and treatment of this severe complication, we used a multidisciplinary approach. The purpose was to establish an internal standard for maximizing the efficacy and safety of treatment, and standardizing the clinical management of this disease.Objectives The use of telemedicine has grown exponentially as an alternative to providing care to patients with epilepsy during the pandemic. We investigated the impact of the current pandemic among children with epilepsy from two distinct pediatric epilepsy centers. We also compared perceptions among those who received telemedicine against those who did not. Methods We developed a questionnaire and invited families followed in Freiburg, Germany, and Calgary, Alberta, Canada, to participate during the initial 9 months of the pandemic. The survey contained 32 questions, 10 of which were stratified according to telemedicine exposure. Results One hundred twenty-six families (80 in Freiburg, 46 in Calgary) participated, and 40.3% received telemedicine care. Most children (mean age 10.4 years, SD 5.1) had chronic epilepsy but poorly controlled seizures. Negative impacts were reported by 36 and 65% of families who had to reschedule appointments for visits and diagnostics, respectively. Nearly two-thirds of families reported no change in seizure frequency, while 18.
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