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Introduction: Simulation training offers an authentic team-based learning opportunity without risk to real patients. The Educational Corner at the annual congress of the European Branch of Extracorporeal Life Support Organisation (EuroELSO) provided an opportunity for multiple simulation training sessions facilitated by experts from all over the world. Aim: We aimed to review the educational impact of EuroELSO Educational Corner and whether it provides a quality ECLS training to a wide spectrum of multidisciplinary international attendees utilising high and low fidelity simulation, workshops and hands on sessions. Methods: During the congress, 43 sessions were conducted dedicated to ECLS education with identified educational objectives. The sessions focused on management of adults and children on V-V or V-A ECMO. Adult sessions covered emergencies on mechanical circulatory support with management of LVAD and Impella, managing refractory hypoxemia on V-V ECMO, emergencies on ECMO, renal replacement therapy on ECMO, V-V ECMO, ECPR cannulation and performing perfect simulation. Paediatric sessions covered ECPR neck and central cannulation, renal replacement on ECMO, troubleshooting, cannulation workshop, V-V recirculation, ECMO for single ventricle, PIMS-TS and CDH, ECMO transport and neurological injury. Results: The Educational Corner was attended by more than 400 participants over the two congress days. Majority of responders (88%) reported that training sessions met the set educational goals and objectives and that this would change their current practice. Almost all (94%) reported that they received useful information and 95% would recommend the session to their colleagues. Conclusion: The Educational Corner, as an integral component of the annual EuroELSO congress, achieved the set educational goals and provided quality education based on the recipient survey. Structured multidisciplinary ECLS education with standardised curriculum and feedback is an important key step in delivering quality training to an international audience. Standardisation of European ECLS education remains an important focus of the EuroELSO.

In this study we have reported the chemical composition of the essential oils obtained from the needles of twelve Pinus taxa, belonging to the subgenera Pinus (sections Pinus and Trifoliae) and Strobus (sect. Quinquefoliae). Monoterpenes dominated over sesquiterpenes in most of the investigated taxa of both subgenera, except in representatives of the subsection Pinaster, subgenus Pinus. α-Pinene was the most abundant terpene in eight pines, but other dominant terpenes were found in some taxa - trans-caryophyllene, germacrene D, abietadiene and β-pinene. In the subsect. Pinaster, the most dominant compounds varied from species to species. In addition, P. halepensis and P. pinaster had the highest percentage of diterpenes, while P. heldreichii was the richest in germacrene D. In the PCA and cluster analyses, three species from the subsect. Pinaster emerged as the most distant: P. halepensis, P. pinaster, and P. heldreichii. In addition, the possible taxonomic implications of the terpene profile in the analysed Pinus taxa were also discussed. U ovom proučavanju objavili smo hemijski sastav etarskih ulja dobijenih iz iglica 12 taksona roda Pinus, koji pripadaju podrodovima Pinus (sekcije Pinus i Trifoliae) i Strobus (sekcija Quinquefoliae). U većini istraživanih taksona monoterpeni dominiraju nad seskviterpenima kod oba podroda, osim kod uzoraka podsekcije Pinaster podroda Pinus. α-Pinen je najobilniji kod 8 borova, ali kod nekih taksona dominantni su trans-kariofilen, germakren D, abietadien i β-pinen. U subcekciji Pinaster dominantne komponente variraju od vrste do vrste. Nadalje, P. halepensis i P. pinaster imaju najveći procenat diterpena, dok je P. heldreichii najbogatiji u germakrenu D. U PCA i klaster analizama pokazale su se najudaljenije tri vrste subsekcije Pinaster: P. halepensis, P. pinaster i P. heldreichii. Nadalje, u analiziranim vrstama roda Pinus diskutovane su moguće taksonomske implikacije terpenskog profila.

Introduction. Primary immune thrombocytopenia is a chronic acquired autoimmune disorder that is characterized by isolated thrombocytopenia (

The study aimed to discriminate renal allografts with impaired function by measuring cortical renal blood flow (cRBF) using magnetic resonance imaging arterial spin labelling (ASL-MRI) in paediatric and young adult patients. We included 18 subjects and performed ASL-MRI on 1.5 T MRI to calculate cRBF on parameter maps. cRBF was correlated to calculated glomerular filtration rate (GFR) and compared between patient groups with good (GFR ≥ 60 mL/min/1.73 m2) and impaired allograft function (GFR 2). Mean cRBF in patients with good allograft function was significantly higher than in patients with impaired allograft function (219.89 ± 57.24 mL/min/100 g vs. 146.22 ± 41.84 mL/min/100 g, p p p = 0.464), while PS indicated moderate correlation to GFR (r = 0.48, p p p

Introduction/Objective. Jeune syndrome (JS) is a rare hereditary ciliopathy characterized by asphyxiating thoracic dystrophy, shortened limbs and brachydactyly. Extraskeletal anomalies such as chronic renal failure (CRF), hepatic fibrosis, and retinitis pigmentosa may be a part of the JATD phenotype. The aim of this study is to present long-term follow-up of JS patients with early progressive kidney disease. Methods. This is a retrospective study of pediatric patients with JS and CRF who were treated at the University Children?s Hospital between January 1980 and December 2014. The patients? data were retrospectively reviewed from the medical records. Results. There were thirteen patients from 11 families, five girls and eight boys mean aged 4.3 years at the time of diagnosis. All of the patients had characteristic skeletal findings, retinal degeneration and an early onset of CRF at age range from 1.5 to 7 years. Five patients had neonatal respiratory distress and congenital liver fibrosis was diagnosed in five patients. One patient died due to complications of CRF, while others survived during follow-up of mean 11 years. IFT140 mutations were found in four genetically tested patients. Conclusion. The average incidence rate of JS with renal phenotype in Serbia was about 0.2 per one million of child population. Long-term survival of JS patients depends on renal replacement therapy, while skeletal dysplasia, growth failure, respiratory and eyes problems have impact on the patients? quality of life.

Objective: Comparison of early outcomes of normothermic cardiopulmonary bypass (N-CPB, ≥35°C) with hypothermic cardiopulmonary bypass (H-CPB, 28–34°C) for congenital heart defects.Methods: Data from 99 patients

Introduction: Approximately 90% of a million children worldwide born with congenital heart defect do not have an access to adequate pediatric cardiac care. The World Society for Pediatric and Congenital Heart Surgery, established in 2006 shifted the focus from providing individual pediatric cardiac care to developing global standards for the practice of pediatric cardiac surgery and professional education of the local teams. Materials and Methods: After recognizing the challenges of the local team regarding providing safe anesthesia and functioning as a broader team, we have focused our education on simplifying anesthetic procedures and advancing structured team approach. The appropriate selection of patients and simplifying anesthetic technique should be the standard of care. We introduced structured approach to daily education using just in time teaching, case based discussions and simple skill training simulation sessions. Furthermore, we enhanced team-training approach applying tools such as WHO surgical safety checklist and implementation manual, SAFE communication, introducing KDD with SMART aim, SCAMPs, advanced protocols of care and culture change tools. Results: Following a significant number of short missions to developing centers we have, within NGO, succeeded to support building and maintaining several local pediatric cardiac centers with structured approach to anesthesia and team building. Conclusion: The appropriate selection of patients is one of the most important contributing factors for decreasing morbidity and mortality rate in pediatric cardiac surgery patients. The anesthesia technique for pediatric cardiac procedures should be aimed at fast-track surgery, with early extubation as a goal. Regional blocks such as paravertebral and caudal should be considered for perioperative pain control. By introducing structured approach to daily education and by enhancing team-training approach we have contributed evolving sustainable pediatric cardiac centers in developing countries.

Introduction. Post-transplant lymphoproliferative disorder (PTLD) is a heterogeneous group of diseases, characterized by abnormal lymphoid proliferation following transplantation. It is a disease of the immunosuppressed state, and its occurrence is mostly associated with the use of T-cell depleting agents, and also intensification of immunosuppressive regimens. In the majority of cases, PTLD is a consequence of Epstein-Barr virus (EBV) infection and is a B-cell hyperplasia with CD-20 positive lymphocytes. The 2008 World Health Organization classification for lymphoid malignancies divides PTLD into four major categories: early lesions, polymorphic PTLD, monomorphic PTLD and Hodgkin PTLD. The treatment and prognosis depend on histology. The cornerstone of PTLD therapy includes reduction/withdrawal of immunosuppression, monoclonal anti CD-20 antibody (rituximab) and chemotherapy. Outline of Cases. We reported here our experiences with three patients, two girls aged 7.5 and 15 and a 16-year old boy. They had different organ involvement: brain, combined spleen-liver and intestines, respectively. Even though EBV was a trigger of lymphoid proliferation as it was confirmed by histopathology or in cerebrospinal fluid, qualitative EBV-PCR was positive only in one patient at disease presentation. Reduction of immunosuppression therapy was applied in treatment of all three patients, while two of them received rituximab and ganciclovir. They had an excellent outcome besides many difficulties in diagnosis and management of disease. Conclusion. Qualitative EBV-PCR is not useful marker in pediatric transplant recipients. Our suggestion is that patients with the risk factors like T-cell depleting agents, immunosuppressant protocol or increasing immunosuppressive therapy and EBV miss-match with donor must be more accurately monitored with quantitative EBV PCR. [Projekat Ministarstva nauke Republike Srbije, br. 175085]

Monitoring brain integrity and neurocognitive function is a new and important target for the management of a patient treated with extracorporeal membrane oxygenation (ECMO), in particular because of the increasing awareness of cerebral abnormalities that may potentially occur in this setting. Continuous regular monitoring, as well as repeated assessment for cerebral complications has become an essential element of the ECMO patient management. Besides well-known complications, like bleeding, ischemic stroke, seizures, and brain hypoperfusion, other less defined yet relevant injury and clinical manifestations are increasingly reported and impacting on ECMO patient prognosis at short term. Furthermore, it is becoming more evident that neurologic complication may not occur only in the early phase. Indeed, other potential adverse events related to the long-Term neurocognitive function have been also recently documented either in children or adult ECMO patients. Despite increasing awareness of these aspects, generally accepted protocols and clinical management strategies in this respect are still lacking. Current means to monitor brain perfusion or detecting ongoing cerebral tissue injury are rather limited, and most techniques provide indirect or post-insult recognition of irreversible tissue injury. Continuous monitoring of brain perfusion, serial assessment of brain-derived serum biomarkers, timely neuro-imaging, profesand post-discharge counselling for neurocognitive dysfunction, particularly in pediatric patients, are novel pathways focusing on neurologic assessment with important implications in daily practice to assess brain function and integrity not only during the ECMO-related hospitalization, but also at long-Term to re-evaluate the neuropsychological integrity, although well designed studies will be necessary to elucidate the cost-effectiveness of these management strategies.

Shock index (SI) (heart rate [HR]/systolic blood pressure [SBP]) has been used to predict outcome in both adult and pediatric sepsis within the intensive care unit (ICU). We aimed to evaluate the utility of SI before pediatric ICU (PICU) admission.We conducted a retrospective observational study of children referred to a pediatric intensive care transport service (PICTS) between 2005 and 2011. The predictive value of SI, HR, and blood pressure at three prespecified time points (at referral to PICTS, at PICTS arrival at the referring hospital, and at PICU admission) and changes in SI between the time points were evaluated. Death within the first 48 h of ICU admission (early death) was the primary outcome variable.Over the 7-year period, 633 children with sepsis were referred to the PICTS. Thirty-nine children died before transport to a PICU, whereas 474 were transported alive. Adjusting for age, time points, and time duration in a multilevel regression analysis, SI was significantly higher in those who died early. There was a significant improvement in SI with the transport team in survivors but not in nonsurvivors. However, the predictive value of a change in SI for mortality was no better than either a change in HR or blood pressure.The absolute or change in SI does not predict early death any more than HR and SBP individually in children with sepsis.

OBJECTIVE: Early deaths in pediatric sepsis may limit the impact of therapies that can only be provided on PICUs. By introducing selection and survivorship biases, these very early deaths may also undermine the results of trials that employ standard consent procedures. We hypothesized that: 1) the majority of deaths in children with severe sepsis occur very early, within 24 hours of referral to PICU; and 2) a significant proportion of deaths occur before PICU admission. DESIGN, SETTING, AND PATIENTS: We studied consecutive referrals of newborns through to 16 years of age, between 2005 and 2011 to the Children's Acute Transport Service, the North Thames regional pediatric intensive care transport service, with a working diagnosis of "sepsis," "severe sepsis," "meningococcal sepsis," or "septic shock." INTERVENTIONS: The primary outcome measure was the proportion of deaths within 24 hours of referral. Survival distributions of previously healthy children were compared with those with significant comorbidities. MEASUREMENTS AND MAIN RESULTS: Thirteen thousand four hundred and nine referrals were made to Children's Acute Transport Service, of whom 703 (5%) met inclusion criteria. Data on survival to 1 year were available in 627 of 703 patients (89%). One hundred thirty children (130/627; 21%; 95% CI, 18-24%) died in the first year. A higher proportion of children with comorbidity cases (46/85, 54%, 44-64) died compared with previously healthy cases (84/542; 16%; 13-19; p < 0.0005, Fisher exact test). Seventy-one deaths occurred within 24 hours of PICU referral (71/130, 55%, 46-63). The timing of death differed with comorbidity. Similar proportions of children survived to 24 hours (previously healthy children 90% vs children with comorbidity 83%, p = 0.06). However, deaths after 24 hours were infrequent among previously healthy cases (28/84 deaths, 33%, 24-44%) compared with children with comorbidity cases (31/46 deaths, 66%, 53-79%) (p < 0.001, Fisher exact test). CONCLUSIONS: This majority of deaths among children referred for pediatric intensive care with for severe sepsis occur within 24 hours. This has important implications for future clinical trials and quality improvement initiatives aimed at improving sepsis outcomes.

Congenital heart disease (CHD) severity is associated with a lower quality of life (QOL). Given the lack of data, we sought to identify surgical and ICU factors associated with QOL scores. Patients from 8 centers who underwent Fontan procedure, TOF or TGA repair who participated in the Pediatric

Introduction. The association between celiac disease and eating disorders has been very rarely reported. This is the first report on celiac disease associated with bulimia in this part of Europe. Case report. An adult female patient with history of bulimia and one uncomplicated pregnancy was admitted to the Gastroenterology Department, due to long lasting dyspeptic symptoms, constipation, major weight loss and fatigue. After positive serological screening, the diagnosis of celiac disease was confirmed with histopathology examination of duodenal biopsy specimen. Conclusion. Complicated interactions between celiac disease and bulimia can make them difficult to diagnose and treat. It is important to consider the presence of celiac disease in patients with bulimia and gastrointestinal symptoms.

Purpose. CYP3A and P-gp both function to reduce the intracellular concentration of drug substrates, one by metabolism and the other by transmembrane efflux. Moreover, it has been serendipitously noted that the two proteins have many common substrates and inhibitors. In order to test this notion more fully, systematic studies were undertaken to determine the P-gp-mediated transport and inhibitory characteristics of prototypical CYP substrates.

Amylin is normally secreted in a regulated fashion by the pancreatic β-cells in parallel with insulin and has been reported to have bone-conserving properties. Type I diabetes mellitus results in a low-turnover osteopenia in the presence of decreased amylin, which is in contrast to type II diabetes where less bone loss, in the presence of high amylin levels, occurs. We investigated the effects of amylin on bone mineral metabolism in normal and dibetic (streptozotocin-induced) rats, in order to ascertain whether amylin would modify the streptozotocin-induced diabetic osteopenia. Tenweek-old male Sprague-Dawley rats were randomized as follows: group A (n=18) received normal saline; group B (n=18) received amylin; group C, diabetic rats (n=23), received normal saline; and group D, diabetic rats (n=23), received amylin. Amylin (100 pmol/100 g b. w.) was administered by a daily subcutaneus injection. Double calceinlabeled tibiae were removed for histomorphometric analysis followed sacrifice on day 19. Results showed no difference in blood ionized calcium between groups. Blood glucose remained above 600 mg/dl in the diabetic animals and was not affected by the administration of amylin. Serum osteocalcin, insulin-like growth factor-1 (IGF-1), parathyroid hormone (PTH), and 1,25 dihydroxyvitamin D [1,25(OH)2D] were significantly lower in the diabetic rats compared with control group A by day 19. Amylin produced higher levels of serum osteocalcin in group B on day 9 (P

Immunosuppressant therpay is associated with osteoporosis both clinically, post-transplantation, and experimentally. In rats, cyclosporin A (CsA) and FK506 induce a state of high turnover rapid bone loss. After 14 days of administration in immunosuppressive doses, the more recently discovered immunosuppressant, rapamycin, resulted in no change of cancellous bone volume. A longer study over 28 days has now been carried out; contrasting the new drug with CsA and FK506. Sixty, 10-week-old Sprague-Dawley rats were randomly divided into five groups of 12 rats each. The first group served as an aging control. The remaining four groups received, by daily gavage, a combined vehicle placebo, CsA 15 mg/kg, FK506 5 mg/kg, and rapamycin 2.5 mg/kg, respectively. CsA- and FK506-treated rats, but not those treated with rapamycin, demonstrated high turnover osteoporosis with raised serum 1,25(OH)2D (p0.05) and elevated serum osteocalcin (p0.05). The trabecular bone area was decreased by 66% (p0.01) in the CsA group and 56% (p0.05) in the FK506-treated group compared with the control animals. The CsA- and the rapamycin-treated groups failed to gain weight and developed severe hyperglycemia (20 mmol/l, p0.001) by day 14 but which largely resolved by day 28. Unlike the groups treated with CsA and FK506, rapamycin-treated rats had no loss of trabecular bone volume but there was increased modeling and remodeling and a decreased longitudinal growth rate. Rapamycin may thus confer a distinct advantage over the established immunosuppressants in not reducing bone volume in the short term.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration of cyclosporine A to male and female rats accelerates bone remodeling and causes bone loss, among other side-effects. The newer immunosuppressant drugs, FK506 and CsG, have been synthesized to counteract the toxic effects of CsA, yet maintain clinical efficacy. We investigated the in vivo effects of long-term administration of these drugs on bone mineral metabolism in the rat. Five groups of Sprague-Dawley rats, 15 per group, were allocated to receive by daily gavage for a period of 28 days: (1) Cs-vehicle; (2) CsA 15 mg/kg b.w.; (3) CsG 15 mg/kg b.w.; (4) FK506 vehicle; (5) FK506 5 mg/kg b.w. Blood was sampled on days 0, 14, and 28 for measurement of ionized calcium (Ca2+), parathyroid hormone (PTH), 1,25-(OH)2-vitamin D, and bone gla protein (BGP). Tibiae were removed on day 28 after double calcein labeling for histomorphometric analysis. Immunosuppressant groups were compared with the respective vehicle groups. Neither CsA or CsG affected the levels of Ca2+ or PTH, whereas by day 28 FK506 caused a decrease in Ca2+ and a corresponding rise in PTH (P < 0.05). The 1,25-(OH)2-vitamin D and BGP levels in both the CsA and CsG groups were increased on days 14 and 28 (P < 0.05), while FK506 had no effect on these serum levels. Tibial bone histomorphometry revealed that all 3 immunosuppressants increased measures of bone formation and bone resorption, accompanied by a significant reduction in percent trabecular area, most marked with FK506. This report demonstrates that all three immunosuppressants have adverse effects on bone--most deleterious with FK506.