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关键词： automated fragmented red blood cell percentage   fragmented red blood cell   haemolytic uraemic syndrome   microangiopathic haemolytic anaemia   schistocytes  

摘要： Introduction Presence of schistocytes in peripheral blood smear supporting haemolysis is important for diagnosis and decision-making in paediatric haemolytic uraemic syndrome (HUS). High observer dependency and requirement of expertise for peripheral smear evaluation propels us to think of other modalities to overcome these issues. We envisage that newer techniques like automated fragmented red blood cell percentage (FRC %), whose role has been described in transplant associated HUS and thrombotic thrombocytopenic purpura, can serve the purpose. Methods Twenty-eight children with HUS after excluding secondary causes were enrolled in this study. Blood samples were analysed for FRC% at admission, using SYSMEX XN-1000 (Japan) haematology analyser, and simultaneously, schistocytes in peripheral smear were reported by a single expert haematopathologist. Results Median age was 56 months ranging from 2 to 140 months. FRC% was elevated in 85.8% (n-24/28) with a mean of 4.56 +/- 3.1%. FRC% had a sensitivity of 95.4% (C.I: 77.16% to 99.88%) in children who had FRC% >1.49% with an accuracy of 85.71% (C.I: 67.33% to 95.97%). However, specificity was only 50% (C.I: 11.81% to 88.19%) with a positive likelihood ratio of 1.91. Receiver-operator curve showed an AUC value of 0.841. Conclusion We suggest automated FRC% as a rapid and highly sensitive index for screening of paediatric HUS;however, a peripheral blood film examination is a must in cases with count >2% to avoid false positives as the index has low specificity.

关键词： Intermittent scanning continual   glucose monitoring (isCGM)   ambulatory glucose profile (AGP)   time in range   Ramadan   G5 CGM SYSTEM   TREND ARROWS   FLASH   RECOMMENDATIONS   CONSENSUS   TYPE-1   ASSOCIATION   ADOLESCENTS   TECHNOLOGY   ADULTS  

摘要： Background and aim: Diabetes mellitus imposes a significant burden around the world generally and in the Middle East and North Africa specifically. Glucose monitoring is a cornerstone of diabetes management. Methods: Glycated haemoglobin has always been the main metric for assessing glycaemic control, but its use is linked with multiple pitfalls. As an alternative, continuous glucose monitoring is becoming a standard of care in many countries. Intermittent scanning glucose monitoring (isCGM) has acquired a worldwide popularity and has been proven to improve glycaemic control, hypoglycaemia detection and prevention, and quality of life. Results: The most recent International Society of Paediatric and Adolescent Diabetes practice. guidelines recommended its use in young people with diabetes observing Ramadan to ensure safe fasting. At a meeting in Abu Dhabi in November 2019, the Arab Society for Paediatric.& nbsp; Endocrinology and Diabetes brought together a number of regional diabetes experts, patient. representatives and international expert advisors to review the evidence for isCGM and propose. guidelines for its use in the Middle East and North Africa region. Conclusion: In this paper, the authors strongly recommend the use of isCGM for patients in MENA and present general recommendations and compressive specific guidance for physicians and patients, which they believe will also have wider resonance. (c) 2021 Elsevier B.V. All rights reserved.

关键词： Paediatric radiology   Optimization   Dosimetry  

摘要： Optimization of imaging examinations is a key requirement of both the International and European Basic Safety Standards, and the focus of much international activity. Although methodologies are well established in principle, there continues to be a variety of practical issues both in collecting and interpreting dose and image quality data and in making successful interventions to optimize exposures. A Coordinated Research Project, involving institutes from ten different countries, was established by the IAEA to assess the efficacy of recommended optimization methodologies in the field of paediatric radiology and to derive practical guidance on their implementation. The steps followed in this process were identification of the imaging process to be investigated (abdomen and chest x-rays, micturating cysto-urethrograms, and brain & thorax CT scans);collection of dose and image quality data;evaluation and comparison of the data between institutes and to standards;identification and implementation of interventions for optimization;and reevaluation of dose and image quality parameters. The project succeeded both in achieving effective interventions for optimization of specific imaging tasks in individual institutes and in identifying key issues with potential to handicap this process. The main area in which problems were encountered was in the collation of reliable dose and image quality data. The reasons for this were explored and a series of recommendations have been made, summarized into ?ten practical tips? for optimization to assist institutes, particularly those in the early stages of addressing optimization issues.

关键词： advanced scope of practice   audiology   ear nose and throat   middle ear disease   waiting list  

摘要： Aim Children with middle ear disease often experience lengthy delays waiting for outpatient paediatric ear nose and throat (ENT) services. This study aimed to investigate whether an alternative service delivery model using audiologists working in an expanded scope of practice reduced waiting times for children to access such services. Methods A total of 131 children consecutively referred to a large ENT outpatient service in Queensland, Australia, for middle ear and hearing concerns were prospectively allocated to either a standard ENT service or an advanced audiology (AA)-led service. Waiting times and attendance rates were collected and compared between the two patient groups. Results The median waiting time from referral to first offered appointment was 96 days for children in the AA-led service versus 417.5 days for children in the standard ENT service. Seventy-nine percent of children in the AA-led service attended their first offered appointment versus 61% in the standard ENT service. For children receiving grommets, the median waiting time from initial referral to grommet insertion was 226 days for children in the AA-led service versus 627 days for children in the standard ENT service. Conclusion The AA-led service was an effective alternative pathway to reduce waiting times for children referred to ENT services with middle ear and hearing concerns.

关键词： Japan LCH Study Group   Langerhans cell histiocytosis   long‐   term complications   permanent consequence  

摘要： Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia derived from immature myeloid dendritic cells with the mitogen-activated protein kinase (MAPK) pathway gene mutation. LCH is rarely fatal, but patients develop various permanent consequences (PCs). We report the frequencies of LCH-related PCs in paediatric patients (n = 317) treated by the JLSG-96/02 AraC-containing regimens. One-third of LCH patients had at least one PC at a median follow-up of 12 years. Central nervous system (CNS)-related PCs (neurological and endocrinological) accounted for 21 center dot 5%, non-CNS-related 16 center dot 7%. We require novel therapeutic measures to further reduce the frequency of LCH-related PCs.

关键词： children   infectious diseases   microbiota   microbiome   pre‐   and probiotics  

摘要： In recent years, the field of infectious diseases has been hit by the overwhelming amount of information generated while the human microbiome is being disentangled. Based on the interaction between the microbiota and the immune system, the implications regarding infectious diseases are probably major and remain a challenge. Aims This review was conceived as a comprehensive tool to provide an overview of the available evidence regarding the influence of the microbiome on infectious diseases in children. Methods We present the main findings aroused from microbiome research in prevention, diagnosis and treatment of infectious disease under a paediatric perspective, to inform clinicians of the potential relevance of microbiome-related knowledge for translation to clinical practice. Results and Conclusion The evidence shown in this review highlights the numerous research gaps ahead and supports the need to move forward to integrating the so-called microbiome thinking into our routine clinical practice.

关键词： orbital abscess   orbital cellulitis   periorbital cellulitis   periorbital infection   pre-septal cellulitis   subperiosteal abscess  

摘要： Aim To identify the predictors of poor outcome and need for surgical management in paediatric patients with periorbital cellulitis. To assess the adherence to local guidelines in the management of periorbital cellulitis. Methods Retrospective descriptive analysis of clinical, laboratory and radiological characteristics of 175 paediatric periorbital cellulitis presentations at a UK teaching hospital over a 10-year period. Regression investigated correlations for continuous and categorical variables. Results A total of 175 paediatric presentations were diagnosed as periorbital infections over the 10-year period. Of these, 139 had pre-septal cellulitis, 27 had a subperiosteal abscess, 6 had an orbital cellulitis, 1 had an orbital abscess, 1 a cavernous sinus thrombosis and 1 an extradural abscess. Median age at presentation was 5 years (range: 1 month-17 years). In total, 169 (97%) cases received systemic antimicrobial treatment. Cross-sectional imaging occurred in 30% of cases and 18% required surgical intervention. Increasing C-reactive protein was associated with greater risk of post-septal disease and requiring surgery. The best predictors of post-septal disease in the multivariate analysis (R-2= 0.49,P= <= 0.001) were ophthalmoplegia (P= 0.009), proptosis (P= 0.016) and pain on eye movement (P= 0.046). Proptosis was the single most significant predictor of surgical management (R-2= 0.53,P= <0.001). Conclusion Multidisciplinary involvement and early medical management can improve outcomes for most patients. Those who deteriorate despite medical management should be considered for prompt imaging and surgical management to avoid serious life-threatening or sight-threatening complications.

摘要： Background Laparoscopic surgeries are associated with less postoperative pain and adverse events compared to open procedures. But, it still reduces the quality of life in children. Transversus abdominis plane (TAP) block is used to reduce pain. We hypothesized that dexmedetomidine or clonidine could improve the analgesic profile of levobupivacaine to the same extent during TAP block in children. Methods Ninety children were randomly allocated in a randomized double-blind trial to receive bilateral TAP block with levobupivacaine plus normal saline (group B, n = 30), or dexmedetomidine (group D, n = 30) or clonidine (group C, n = 30). Primary outcome was the modified Children's Hospital of Eastern Ontario Pain Scale score. Secondary outcomes included time to initial analgesic request, number of analgesic claims, total analgesic consumption, parents' satisfaction, sedation score and complications. Results Children of group D showed reduced pain scores compared to other groups. They represented the longest period of analgesia (565.00 +/- 71.5 min) with p < 0.001, and fewer patients required two doses of analgesia during the first postoperative day. The cumulative amount of backup analgesia was significantly different between these groups (p = 0.026). Higher parents' satisfaction scores were recorded in groups D and C compared to group B. Sedation among the study groups revealed significant differences (p = 0.035), but no severe complications were recorded. Conclusions Adding dexmedetomidine to levobupivacaine can extend the time of analgesia and reduce the use of postoperative backup analgesics with minimal sedation effects when used in TAP block in paediatrics undergoing laparoscopic orchiopexy. Clonidine can be used as an alternative adjuvant to local anaesthetics with good postoperative analgesic profiles. Significance Clonidine can alternate dexmedetomidine during TAP block with local anesthetics for pediatrics laparoscopies. Both can lead to better postoperative an