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Congenital carved dystrophies: What exactly is brand-new?
Background ROTEM assay has gained increasing acceptance as a method for rapid and specific coagulation pathway assessment. However, its use in the neonatal population remains limited since reference ranges have not yet been established. Aims (1) to determine reference ranges for healthy term neonates of ROTEM parameters using non-activated assay (NATEM) in cord blood samples; (2) to assess whether delivery mode, gender, gestational age, birth weight and blood group (ABO and Rhesus) of the neonate, coagulation disorder and anticoagulant medication of the mother have an impact on NATEM parameters. Methods NATEM assay was conducted in cord blood samples of 189 term neonates without any medical history. Results Reference ranges (2.5th and 97.5th percentiles) are established for clotting time (CT), clot formation time (CFT), α-angle, clot amplitude at 5, 10 and 20 min (A5, A10, A20), maximum clot firmness (MCF), lysis index at 30 and 60 min (LI30, LI60, %) and maximum clot elasticity (MCE). Reference ranges for NATEM are CT 182-499 s, CFT 63-176 s, α-angle 58-78°, A5 28-52 mm, A10 37-61 mm, A20 42-66 mm, MCF 43-67 mm, LI30 97-100%, LI60 87-98% and MCE 75-203. Male neonates appear to be more hypocoagulable than females. Conclusions We demonstrate reference ranges for healthy term neonates in NATEM assay that could be used as a reference group for future studies of neonates with an underlying pathology.Staphylococcus lugdunensis is a rare cause of late-onset sepsis in preterm infants. To our best knowledge, we report the fourth case of a male preterm infant who developed fulminant late-onset sepsis due to Staphylococcus lugdunensis with persistent bacteremia secondary to an infected aortic thrombus confirmed with two positive blood cultures. Our patient was an extremely low birth weight growth-restricted infant born at 27 weeks gestation and initially required an umbilical arterial catheter for blood pressure and blood gas monitoring. The course of this neonate was complicated by severe respiratory distress syndrome that evolved into chronic lung disease along with multiple episodes of tracheitis. Hemodynamically, the infant had a significant patent ductus arteriosus, and an episode of medical necrotizing enterocolitis followed by Staphylococcus lugdunensis septicemia. He was diagnosed with an infected aortic thrombus, probably the occult focus responsible for the persistent bacteremia. After a 6-week course of intravenous antibiotics and 4-week course of anticoagulant therapy, the infant responded and recovered without complications.The term "severe neurologic impairment" (SNI) is used to describe a group of disorders of the central nervous system which arise in childhood, resulting in motor impairment, cognitive impairment and medical complexity. As a result, much assistance is required with activities of daily living. Since these patients are often unable to self-report pain, or they may exhibit uncommon behaviors when suffering, pain manifestations may go unrecognized. In this article, the basic principles of how to approach pain in children with SNI are discussed.High ability students have differential cognitive characteristics that require a specific educational response to develop their full potential. Cluster ability grouping is one of the available approaches to respond to their training. One of the main criticisms of this teaching method, also supported by the big-fish-little-pond effect (BFLPE), is that high ability students may show a decrease in their self-concept. The aim of this research is to present the evaluation carried out by parents of primary school high ability students on the effect of an educational cluster grouping program on their personal and social adjustment, comparing these variables before the beginning of the school year and at the end of it. Approximately 100 students' parents of the Educational Centre for Highly Ability Pupils participated in the evaluation. The instrument used for the evaluation was the Socialization Battery (BAS-2) for parents. The results showed that parents observed some improvements, either due to an increase in the scales measuring positive socialization variables or a decrease in the scales measuring negative socialization variables. The most substantial improvements are found in the second and third grades. We conclude that the results do not support the big-fish-little-pond effect (BFLPE).Breastfeeding is considered to be the cornerstone of child health. In Europe however, overall breastfeeding rates remain low. The present study aimed at estimating the frequency of breastfeeding in Greece during the COVID-19 pandemic period and comparing findings with the latest national study in order to identify a potential impact of the pandemic. Additionally, possible correlations of socio-cultural and demographic characteristics with breastfeeding indicators were investigated. This prospective cohort study included 847 women from five tertiary maternity hospitals, between January and December 2020. Data were collected by a structured questionnaire via interview during hospitalization and via telephone in the first, third and sixth month postpartum. Results showed that all breastfeeding indicators improved over the last three years. Full breastfeeding reached 7.2%, contrary to 0.78% of the latest national study at six months postpartum. Employment, marital status, educational level, mode of delivery, type of maternity hospital, body mass index before pregnancy, previous breastfeeding experience of the mother and infant's birth weight correlated significantly with breastfeeding indicators at different time periods. The COVID-19 pandemic seems to have favorably influenced breastfeeding initiation and duration in Greece due to lockdowns, home confinement and teleworking.(1) Background and aims Obesity and high body max index (BMI) have been linked to elevated levels of inflammation serum markers such as C-reactive protein (CRP), interleukin-6 (IL-6), tumor necrosis factor alpha (TNF-alpha), adiponectin, and resistin. It has been described that adipose tissue presents a high production and secretion of these diverse pro-inflammatory molecules, which may have local effects on the physiology of the fat cell and also systemic effects on other organs. Our aim was to evaluate the impact that lifestyle modifications, following a Mediterranean Diet (MedDiet) program and physical activity (PA) training, would have on inflammatory biomarkers in a metabolically healthy prepubertal population with obesity (MHOPp) from Malaga (Andalusia, Spain). (2) Methods 144 MHOPp subjects (aged 5-9 years) were included in this study as they met ≤1 of the following criteria waist circumference and blood pressure ≥ 90 percentile, triglycerides > 90 mg/dL, high-density lipoprotein cholesterol (HDL-c) lsignificant correlation with anthropometric parameters, body composition, and physical activity. (4) Conclusions After 24 months of lifestyle modification, our MHOPp reduced their Z-score of BMI, leading to an improvement of inflammatory biomarkers but inducing deterioration in the adipokine profile, which does not improve with MedDiet and physical activity intervention. An adequate education within the family about healthier habits is necessary to prevent and reduce an excessive increase in obesity in childhood.The strategy for the selection of patients with a suspected SARS-CoV-2 infection is relevant for the organization of a children's hospital to provide optimal separation into COVID-19 and non-COVID-19 areas and pathways. We analyzed the proportion of children with COVID-19 presenting with gastrointestinal (GI) symptoms in 137 consecutive patients admitted between January 2020 and August 2021. GI symptoms were present as follows diarrhea in 35 patients (26%), vomiting in 16 (12%), and both of them in five (3%); the combination of fever, respiratory symptoms, and diarrhea was observed in 16 patients (12%). Of the 676 adult patients with COVID-19 admitted to our hospital in the same time interval, 62 (9.2%) had diarrhea, 30 (4.4%) had vomiting, and 11 (1.6%) had nausea; only one patient, a 38-year-old male, presented with isolated GI symptoms at the diagnosis. Although diarrhea was observed in one quarter of cases, one-half of them had the complete triad of fever, respiratory syndrome, and diarrhea, and only five had isolated diarrhea, of which two were diagnosed with a Campylobacter infection. The occurrence of either respiratory symptoms or gastrointestinal symptoms in our patients was not related to the patient age, while younger children were more likely to have a fever. Of the 137 patients, 73 (53%) could be tested for their serum level of SARS-CoV-2 specific IgG antibodies. The observed titer ranged between 0 (n = 3) and 1729 BAU/mL (median, 425 BAU/mL). Of 137 consecutive patients with COVID-19 admitted to our referral children's hospital, only three presented with an isolated GI manifestation. It is interesting to note that this finding turned out to be fully in keeping with what was observed on adult patients with COVID-19 in our hospital. selleck chemicals The additive diagnostic impact of gastrointestinal involvement for the triage of children with suspected COVID-19 appears limited.The study aimed to (1) investigate the reliability and usefulness of a proposed angular analysis during a modified sit-and-reach (MSR) test, and (2) compare the proposed MSR angular analysis and the commonly used MSR distance to verify the influence of the anthropometric characteristics in preschoolers. 194 preschoolers participated in the study. Before testing, the anthropometric characteristics were collected. Each participant performed the MSR test twice. The MSR distance score was obtained from the starting point to the reaching point, while the MSR angle score was calculated according to the approximate hip flexion angle. Both the relative and absolute reliability were good for the angular analysis during an MSR test in preschoolers (ICC ranging from 0.82 to 0.91, CV% ranging from 8.21 to 9.40). The angular analysis demonstrated good usefulness, with a lower typical error than the smallest worthwhile change in 3- and 5-year-old groups. The MSR angle scores could eliminate the concern of the influence of anthropometric characteristics, while MSR distance and anthropometric characteristics (i.e., sitting height and arm length) were found to be weakly correlated. In conclusion, the angular analysis when performing the MSR test is reliable and appears to eliminate the concern regarding the limb length bias.The three types of neurofibromatosis, namely type 1, type 2, and schwannomatosis, are generally associated with various benign tumors affecting the skin and the nervous system. On rare occasions, especially in patients with neurofibromatosis type 1 (NF1), malignant neoplasms may also be present, several of them possessing a more aggressive course than in individuals without this syndrome. As such, a clear delineation between the three variants of neurofibromatosis is crucial to establish the correct diagnosis and management, as well as predict the neoplasm-related outcomes. Neurofibromin, the principal product of the NF1 gene, is a potent inhibitor of cellular proliferation, having been linked to several key signaling pathways involved in tumor growth. Therefore, it may provide a useful therapeutic target for tumor management in these patients. In this article, we want to present the association between deficiency of neurofibromin and the consequences of the lack of this protein leading to different kinds of malignant tumors.
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