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摘要： This article explores what an 'ideal' paediatric gastroenterology department in the future should look like and what it could potentially provide if given carte blanche by health funds.

关键词： PAPSA   Paediatric surgery   History  

摘要： The Pan African Paediatric Surgery Association (PAPSA) was formed in 1994. The need for an organisation in Africa to voice children's surgery and the trials and tribulations in forming this organisation was covered in this journal 2 years ago (Heinz R, Kyambi J, Lakhoo K. Surg Int 34(5):499-504, 2018). This article covers the history of the organisation post inception in 1994 to date. The near disbanding of the organisation due to political unrest and wars in Africa, to its success in the recent decade is highlighted in this manuscript.

关键词： antithrombotic prophylaxis   pediatric kidney transplantation   renal graft thrombosis   survey  

摘要： Background Renal graft thrombosis (RGT) is one of the main causes for early graft loss in pediatric kidney transplantation (KTx). Despite the lack of evidence-based recommendations, antithrombotic prophylaxis (aP) is used to prevent RGT. Methods An online survey supported by the European Society for Pediatric Nephrology was developed to investigate the current practice of aP in pediatric KTx recipients A total of 80 pediatric KTx centers from 37 countries participated in the survey. Antithrombotic prophylaxis was performed in 96% of the pediatric renal transplant centers (all/selected patients: 54%/42%). The main overall used drugs were as follows: low-molecular-weight heparin (89%), unfractionated heparin (UFH) (69%), and acetylsalicylic acid (ASS) (55%). Ten different aP management strategies were identified as follows: 51% used a single drug and 48% combined two drugs sequentially. The corresponding centers started aP predominantly within 24 hours after pediatric KTx;51% preferred UFH for starting aP. In centers switching to a second drug (51%), this change was performed after 10 +/- 6 days;of these 57% preferred ASS for maintenance aP. Reported median aP duration was 51 days (range 1-360). Conclusions Despite the use of aP in almost all responding pediatric KTx centers, there is no uniform management strategy. Notwithstanding, UFH seems to be the preferred drug for the early post-operative period of pediatric KTx, and ASS for maintenance prophylaxis following pediatric KTx. Prospective studies are needed to further evaluate the benefits and risks of aP, preferably resulting in guidelines for the management in pediatric KTx.

关键词： child   cystic fibrosis   exocrine pancreatic insufficiency   pancreatic diseases   pancreatitis   pancreatitis chronic  

摘要： Paediatric pancreatic diseases are often under-recognised and may be associated with severe diseases and significant clinical consequences. In recent years, advances have been made in key areas, particularly with the contributions from international societies and study groups focused on paediatric pancreatic disease research. This review focuses on the two key manifestations of pancreatic disorders in childhood, pancreatitis and exocrine pancreatic dysfunction.

关键词： extracorporeal membrane oxygenation   extracorporeal life support   risk-adjustment   benchmarking   paediatric extracorporeal membrane oxygenation prediction model   outcome prediction  

摘要： Introduction: There is a need for a universal risk-adjustment model that may be used regardless of the indication and nature of neonatal or paediatric extracorporeal membrane oxygenation support. The 'paediatric extracorporeal membrane oxygenation prediction' model appeared to be a promising candidate but required external validation. Methods: We performed a validation study using institutional database of extracorporeal membrane oxygenation patients (2008-2019). We used the published paediatric extracorporeal membrane oxygenation prediction score calculator to derive estimated mortality based on the model in this cohort of patients in our institutional database. We used standardized mortality ratio, area under the receiver operating characteristic curve and Hosmer-Lemeshow goodness-of-fit test in 10 deciles to assess model performance. Results: We analysed 154 extracorporeal membrane oxygenation episodes in 150 patients. About 53% of the patients were full term (age <= 30 days and gestation at birth > 37 weeks) neonates. The commonest category of extracorporeal membrane oxygenation support was cardiac (42%). The overall in-paediatric intensive care unit mortality was 37% (57/154) and the in-hospital mortality was 42% (64/154). Distribution of estimated mortality risk was similar to the derivation study. The calculated standardized mortality ratio was 0.81 based on the paediatric extracorporeal membrane oxygenation prediction model of risk-adjustment. The area under the receiver operating characteristic curve was 0.55 (0.45-0.64) and Hosmer-Lemeshow-test p value Conclusion: This small single-centre study with a small number of events was unable to validate the paediatric extracorporeal membrane oxygenation prediction-model of risk-adjustment. Although this remains the most promising of all the available models, further validation in larger data sets and/or refinement may be required before widespread use.

关键词： Myelin-oligodendrocyte glycoprotein   Acute disseminated encephalomyelitis   Optic neuritis   Transverse myelitis   Vision   Cognition  

摘要： There is increasing knowledge on the role of antibodies against myelin oligodendrocyte glycoprotein (MOG-abs) in acquired demyelinating syndromes and autoimmune encephalitis in children. Better understanding and prediction of outcome is essential to guide treatment protocol decisions. Therefore, this part of the Paediatric European Collaborative Consensus provides an oversight of existing knowledge of clinical outcome assessment in paediatric MOG-ab-associated disorders (MOGAD). The large heterogeneity in disease phenotype, disease course, treatment and follow-up protocols is a major obstacle for reliable prediction of outcome. However, the clinical phenotype of MOGAD appears to be the main determinant of outcome. Patients with a transverse myelitis phenotype in particular are at high risk of accruing neurological disability (motor and autonomic), which is frequently severe. In contrast, having a single episode of optic neuritis any time during disease course is broadly associated with a lower risk of persistent disability. Furthermore, MOG-ab-associated optic neuritis often results in good functional visual recovery, although retinal axonal loss may be severe. The field of cognitive and behavioural outcome and epilepsy following demyelinating episodes has not been extensively explored, but in recent studies acute disseminated encephalomyelitis (-like) phenotype in the young children was associated with cognitive problems and epilepsy in long-term follow-up. In conclusion, main domains of importance in determining clinical outcome in paediatric MOGAD are visual, motor, autonomic and cognitive function. A standardised evaluation of these outcome domains in all children is of importance to allow adequate rehabilitation and follow-up. (C) 2020 The Authors. Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.

关键词： Ultrasonography   Displacement   Stability   Lateral humeral condyle fractures   Paediatric   Treatment  

摘要： Introduction: Lateral humeral condyle fracture is one of the most common fractures in children. However, the prediction of the stability of the fracture with a cutoffdisplacement of 2 mm remains controversial. The aim of this study was to evaluate the reliability of the routine cutoffdisplacement of 2 mm in predicting the stability of paediatric lateral humeral condyle fractures. Methods: A cohort of 79 children with imaging results for lateral humeral condyle fractures from 2013 to 2019 was evaluated. The displacement on the radiographs was measured by three surgeons at different levels, and ultrasound images were obtained by two senior surgeons. The interobserver and intraobserver reliability was assessed by the intraclass correlation coefficient (ICC). A binary logistic regression model and receiver operating characteristic (ROC) curves were used to evaluate the association between the measurement and the integrity of cartilage hinges. Results: The ICC for the interobserver reliability was 0.85, and the intraobserver reliability was 0.93. For each additional millimetre of displacement, the odds of cartilage hinge disruption increased by 70%. The ROC curve determined that the Youden index was only 0.07 (sensitivity, 97.8%;specificity, 8.8%) with a cutoffdisplacement of 2 mm. Conclusions: The routine cutoffdisplacement of 2 mm may not reliably reflect the stability of paediatric lateral humeral condyle fractures. The cutoffvalue is sensitive but not specific for predicting whether the cartilage hinge is intact. (C) 2020 Elsevier Ltd. All rights reserved.

关键词： colonoscopy   colorectal cancer   familial adenomatous polyposis   paediatric patients   surveillance  

摘要： Objectives: Prophylactic colectomy at a premalignant stage is the cornerstone of management of familial adenomatous polyposis (FAP). Before surgery, colonoscopy surveillance is recommended in children with FAP. This study aimed to examine the natural history of FAP in children by evaluating adenoma progression and factors influencing timing of colectomy. Method: Patients with FAP younger than 18 years at first surveillance colonoscopy and who had undergone more than 1 colonoscopy were identified. Demographic, endoscopic, genetic, and surgical data were retrieved. Cumulative adenoma (polyp) counts were obtained while accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing the timing of colectomy were evaluated. Results: Eighty-four patients (50% boys;mean age at first colonoscopy 13 years [standard deviation 1.97]) were identified, of which 83 had a family history of FAP. At first colonoscopy, 67 (79%) had <100 adenomas and 29 (35%) had colonic polyps identified despite rectal sparing. The median rate of polyp progression per patient was 12.5 polyps/year (range 0-145). Of the 45 (54%) patients who had undergone surgery, 41 (91%) underwent colectomy with ileorectal or ileodistal sigmoid anastomosis. Polyp progression did not alter the choice of surgical intervention in any patient. Conclusion: Our results suggest that adenoma number remains relatively stable in the majority of children under surveillance. Tailored surveillance intervals according to phenotype are a more appropriate strategy as recommended by recently published guidelines.

关键词： acute lymphocytic leukaemia (ALL)   CAR T-cell therapy   neurotoxicity  

摘要： Background The advent of chimeric antigen receptor (CAR) T-cell therapy has created a paradigm shift in the management of patients with refractory B-cell acute lymphocytic leukaemia (ALL). The aim of this study is to correlate imaging findings of CAR T-cell therapy related neurotoxicity with clinical course and eventual clinical outcome, with the hope that it will bring us a step closer to the identification of potential imaging biomarkers that may allow more accurate prognostication and risk stratification of patients. Procedure Our imaging database was queried from January 2018 to April 2020 to identify paediatric patients who fulfil the following criteria: (a) diagnosed with ALL, (b) underwent CAR T-cell therapy, and (c) had magnetic resonance imaging (MRI) brain studies performed before and after CAR T-cell therapy. A total of seven patients were included and all MRI studies were analysed by a paediatric neuroradiologist for the presence of acute neuroimaging findings post CAR T-cell infusion. Acute neuroimaging findings are defined as new imaging findings detected within 28 days of CAR T-cell infusion. Results Three out of four patients with acute neuroimaging findings had sustained complete remission for more than 6 months, while all three patients without acute neuroimaging findings had positive minimal residual disease (MRD) within 1 month. Both patients with acute diffuse leptomeningeal enhancement showed clinical improvement within 1-2 days. Conclusions Acute neuroimaging findings may be a potential imaging biomarker for peak neurotoxicity and treatment response, and it is not necessarily associated with poor outcome, as previously reported.

关键词： bacterial invasion   Crohn disease   metabolites   succinate   ulcerative colitis  

摘要： Objectives: Host-microbial relationship is disrupted in inflammatory bowel diseases (IBD). We hypothesized that altered gut luminal microenvironment can impact microbial virulence in IBD, leading to disruption of homeostasis and disease. We investigated the relationship between gut microenvironment and microbial virulence. Methods: Intestinal aspirates were collected from 10 non-IBD controls, 9 Crohn disease, and 10 ulcerative colitis paediatric patients during endoscopy. In vitro invasion of bacteria isolated from the duodenum and terminal ileum (TI) was quantified using gentamicin protection assays. Intestinal epithelial cells were infected in vitro by known Escherichia coli strains with patient intestinal aspirates added. Nuclear magnetic resonance spectroscopy (NMR) analysis was conducted on intestinal aspirates to identify metabolites associated with invasion;these metabolites were then introduced to the infection model. Results: There was no difference in in vitro invasion of bacteria obtained from intestinal aspirates of non-IBD and IBD patients. Incubation of laboratory E coli strains with TI aspirates from IBD patients increased their invasion into epithelial cells in vitro. NMR analysis revealed intestinal metabolites that correlated with bacterial invasion;succinate present in the intestinal aspirates correlated positively, whereas acetate and formate related negatively with invasion. Addition of exogenous succinate increased invasion of E coli in vitro. Conclusions: Alterations in the gut microenvironment in IBD can affect bacterial invasion. Succinate is associated with increased bacterial invasion and can alter bacterial virulence in IBD. This highlights the interaction between specific metabolites and bacteria that could be instrumental in propagating or suppressing inflammation in paediatric IBD patients.