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关键词： Paediatric cardiology   paediatric cardiac surgery   Nigeria   sub-Saharan Africa   COVID-19 pandemic  

摘要： The COVID-19 pandemic is currently ravaging the globe and the African continent is not left out. While the direct effects of the pandemic in regard to morbidity and mortality appear to be more significant in the developed world, the indirect harmful effects on already insufficient healthcare infrastructure on the African continent would in the long term be more detrimental to the populace. Women and children form a significant vulnerable population in underserved areas such as the sub-Saharan region, and expectedly will experience the disadvantages of limited healthcare coverage which is a major fall out of the pandemic. Paediatric cardiac services that are already sparse in various sub-Saharan countries are not left out of this downsizing. Restrictions on international travel for patients out of the continent to seek medical care and for international experts into the continent for regular mission programmes leave few options for children with cardiac defects to get the much-needed care. There is a need for a region-adapted guideline to scale-up services to cater for more children with congenital heart disease (CHD) while providing a safe environment for healthcare workers, patients, and their caregivers. This article outlines measures adapted to maintain paediatric cardiac care in a sub-Saharan tertiary centre in Nigeria during the COVID-19 pandemic and will serve as a guide for other institutions in the region who will inadvertently need to provide these services as the demand increases.

关键词： healthcare providers   neurodevelopmental disorders   paediatric rehabilitation   sleep education   sleep health practices   sleep knowledge  

摘要： Background Sleep disorders are highly prevalent in children with neurodisabilities, yet they seem under-recognized in paediatric rehabilitation settings. The aim of this study was to assess among two groups of healthcare professionals (HCPs) working in paediatric rehabilitation: (1) sleep health practices and (2) knowledge about sleep physiology, sleep disorders and sleep hygiene. Methods We performed a cross-sectional sleep survey among medical and non-medical HCPs and the general population. Participants (30 rehabilitation physicians [RPs], 54 allied health professionals [AHPs] and 63 controls) received an anonymous 30-item survey consisting of three domains: (1) general information, (2) application of sleep health practices and (3) sleep knowledge. Results RPs address sleep issues more frequently in clinical practice than AHPs. Sleep interventions mostly consist of giving advice about healthy sleep practices and are given by the majority of HCPs. While RPs demonstrated the highest scores on all knowledge domains, total sleep knowledge scores did not exceed 50% correct across groups, with AHPs and controls showing equal scores. Sleep hygiene rules closest to bedtime and related to the sleep environment were best known, whereas those related to daytime practices were rarely mentioned across all groups. A small minority of HCPs (RPs 20%;AHPs 15%) believed to possess sufficient sleep knowledge to address sleep in clinical practice. No association was found between self-perceived knowledge and sleep knowledge scores among HCPs. Conclusions Sleep should become a standard item for review during routine health assessments in paediatric rehabilitation settings. HCPs' limited exposure to sleep education may result in feelings of incompetence and inadequate sleep knowledge levels, affecting their sleep health practices. Appropriate sleep training programs should be implemented to empower HCPs with knowledge, skills and confidence, needed to recognize and treat sleep disorde

关键词： intestinal failure   intestinal rehabilitation   intestinal transplantation   parenteral nutrition   short bowel syndrome  

摘要： Intestinal failure is a complex and debilitating condition characterised by inadequate small intestinal function requiring parenteral or intravenous nutrition to maintain health and, for children, to enable growth and development. Although parenteral nutrition can be prescribed in many hospitals, children with chronic intestinal failure have improved outcomes when managed at a paediatric centre by a multidisciplinary team with specialised expertise in the comprehensive management of intestinal failure. Recent advances in the medical, surgical and nutritional approach have been effective at optimising intestinal rehabilitation and achieving enteral autonomy while limiting complications of intestinal failure. The role of intestinal transplantation in the management of the child with intestinal failure continues to evolve as an option for children with life-threatening complications of intestinal failure. The aim of this review is to highlight key advances in the care of children with intestinal failure.

关键词： Age-appropriate dosage forms   Dose flexibility   Dosing vehicles   Excipients for paediatric use   Off-patent drugs  

摘要： More than 10 years after the Paediatric Regulation came into place there is still a strong need for paediatric medicines for off-patent drug substances. Numerous compounds for which a paediatric formulation does not exist can be found on the WHO Model List of Essential Medicines for Children and in the EMA Inventory of the Needs for Paediatric Medicines. Many of these compounds are off patent, which offers the opportunity for obtaining marketing authorisations for paediatric use. The present study focused on the development of paediatric immediate-release mini-tablet formulations for furosemide. Essential formulation criteria included the use of excipients that are regarded as safe for children, the ease of manufacturing, a high dose flexibility, fast disintegration, a robust drug release and a good acceptability. Only excipients regarded as safe for use in children were used in formulation screening. Compressibility, tablet hardness, disintegration and palatability were the main screening parameters. Formulations with a hardness of > 20 N, a disintegration time < 3 min (fast disintegration) and a good palatability were selected for mini-tablet production. Based on this pre-assessment two mini-tablet formulations with a furosemide drug load of 2.5 mg were developed. Both were easy to manufacture, had an appropriate hardness, a short disintegration time and met pharmacopoeial requirements with regard to content uniformity and physical testing. Biorelevant in vitro dissolution experiments mimicking different modes (with water or dosing vehicles) of administering age-appropriate furosemide doses to children of different age groups indicated a fast and robust drug release. Overall, the novel mini-tablet formulations present with an increased dose flexibility, excipient safety, swallowability and palatability and are thus a promising starting point for the development of solid oral dosage forms for drugs with paediatric therapeutic needs.

关键词： Myelin-oligodendrocyte glycoprotein   Children   Acute disseminated encephalomyelitis   Optic neuritis   Transverse myelitis   Encephalitis  

摘要： Over the past few years, increasing interest in the role of autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs) as a new candidate biomarker in demyelinating central nervous system diseases has arisen. MOG-abs have now consistently been identified in a variety of demyelinating syndromes, with a predominance in paediatric patients. The clinical spectrum of these MOG-ab-associated disorders (MOGAD) is still expanding and differs between paediatric and adult patients. This first part of the Paediatric European Collaborative Consensus emphasises the diversity in clinical phenotypes associated with MOG-abs in paediatric patients and discusses these associated clinical phenotypes in detail. Typical MOGAD presentations consist of demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM) in younger, and optic neuritis (ON) and/or transverse myelitis (TM) in older children. A proportion of patients experience a relapsing disease course, presenting as ADEM followed by one or multiple episode(s) of ON (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), relapsing ON (RON) or relapsing neuromyelitis optica spectrum disorders (NMOSD)-like syndromes. More recently, the disease spectrum has been expanded with clinical and radiological phenotypes including encephalitis-like, leukodystrophy-like, and other non-classifiable presentations. This review concludes with recommendations following expert consensus on serologic testing for MOG-abs in paediatric patients, the presence of which has consequences for long-term monitoring, relapse risk, treatments, and for counselling of patient and families. Furthermore, we propose a clinical classification of paediatric MOGAD with clinical definitions and key features. These are operational and need to be tested, however essential for future paediatric MOGAD studies. (C) 2020 The Authors. Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.

摘要： The article offers information about styled artistic portrait photograph shows open ether anaesthesia of a child around 1952–1953. Topics include the child has a metal frame Bellamy–Gardner anaesthesia mask; and the anaesthetic induction agent has ether in an 8 oz bottle with a Bellamy–Gardner dropper.

关键词： Tuberculosis   Chest imaging   Children   Computerised tomography   Medical resonance imaging  

摘要： Tuberculosis (TB) remains a significant cause of death from an infectious disease worldwide. The diagnosis of pulmonary TB in children is often challenging as children present with non-specific clinical symptoms, have difficulties providing specimens and have a low bacillary load. Radiological imaging supports a clinical diagnosis of pulmonary TB in children, can assess response to treatment and evaluate complications of TB. However, radiological signs on plain radiographs are often non-specific and inter-observer variability in the interpretation contribute to the difficulties in radiological interpretation and diagnosis. The goal of this review is to discuss the advantages and features of cross-sectional imaging such as ultrasound, Computed tomography (CT) and Magnetic resonance imaging (MRI) in diagnosing pulmonary TB (PTB) and its complications in children. (C) 2020 Published by Elsevier Ltd.

关键词： ante situm   auto-transplantation   ex situ   hypothermic perfusion  

摘要： Background Evolving surgical technology and medical treatment have led to an expansion of indications to enable resection of large hepatic tumours with involvement of other abdominal structures. Methods Twelve extended liver and abdominal resections, either ex situ with auto-transplantation of the liver remnant or ante situm with veno-venous bypass (VVBP) were performed between 2016 and 2018. We describe our preoperative assessment, compare surgical strategies and assess outcomes. Results The median age of the 10 adult patients was 50.5 years with a majority suffering from sarcoma-like tumours. The two paediatric patients were 3 and 8 years of age, both with hepatoblastoma. Two patients underwent ex situ resections with auto-transplantation of the liver remnant, and nine patients had ante situm tumour removal with the use of VVBP in four. All patients achieved a good immediate liver function. Local infection and acute kidney injury were found in two patients. One patient underwent biliary reconstruction for bile leak. Tumour recurrence was seen in seven patients (58.3%), with four lung metastases. Five patients died from tumour recurrence (41.7%) during the follow-up. Conclusion Extreme liver resections should be performed in experienced centres, where surgical subspecialties are available with access to cardiovascular support. Additionally, experience in split and living-donor liver transplantation is beneficial.