{"title":"Gastrointestinal complications of cystic fibrosis","authors":"Christabella Ng, Andrew Prayle","doi":"10.1016/j.paed.2024.07.002","DOIUrl":"10.1016/j.paed.2024.07.002","url":null,"abstract":"<div><div>Cystic fibrosis is a multisystem disorder, and gastrointestinal (GI) disease contributes significantly to its morbidity. This review outlines the major gastrointestinal manifestations of CF, and highlights areas of common misunderstanding. Areas particularly important to practice, such as impact upon malabsorption, bowel obstruction and gastro-oesophageal reflux are considered in detail. The impact of new CFTR modulator therapies on CF-related GI conditions is discussed and treatment options for individual pathologies is discussed.</div></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 10","pages":"Pages 363-367"},"PeriodicalIF":0.0,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312383","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cyclical vomiting syndrome","authors":"Ishaq Abu-Arafeh","doi":"10.1016/j.paed.2024.07.003","DOIUrl":"10.1016/j.paed.2024.07.003","url":null,"abstract":"<div><div>Cyclical vomiting syndrome (CVS) was described over 100 years ago, but it is often underdiagnosed and undertreated, even after a diagnosis is made. It is relatively common, affecting almost 2% of school-age children in some studies. Although it is traditionally seen as a childhood disease related to migraine, CVS does occur in adults. The main characteristic of CVS is the stereotypical recurrent nature of episodes of intense nausea and vomiting lasting from few hours to few days and followed by a complete resolution of symptoms. The diagnosis is predominantly a clinical one and there are internationally accepted criteria for diagnosis. The management of acute attacks of CVS aims to relieve symptoms, reduce the duration of attacks and prevent dehydration and hospital admission. Management also includes appropriate counselling on healthy lifestyle, provision of individual management plans and preventive medications. The aim of management is to reduce the number of attacks and improve quality of life. About half the children with CVS start to have migraine with or without aura in late adolescence and around 40% continue with CVS into early adult life. This article is aimed at healthcare professionals looking after children with CVS and describes the clinical presentation, the criteria required for diagnosis and outlines the different treatment options.</div></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 10","pages":"Pages 368-372"},"PeriodicalIF":0.0,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ammonia, lactate and blood gases: a user's guide","authors":"James E Davison","doi":"10.1016/j.paed.2024.07.006","DOIUrl":"10.1016/j.paed.2024.07.006","url":null,"abstract":"<div><div>Basic biochemical tests are frequently obtained in acutely unwell neonates and children, as well as in some elective situations. Correct interpretation is essential in identifying rare inherited primary metabolic disorders, but secondary causes of hyperammonaemia, elevated blood lactate or acid-base derangement are more common and require appropriate treatment of the underlying cause. Ammonia is the waste product of protein metabolism and is highly toxic. Ammonia should be measured in any sick neonate, and in children with unexplained encephalopathy. Further testing is needed to determine if it is secondary to other factors, or due to a primary metabolic disorder affecting urea cycle function. Specific treatment should be instigated urgently to avoid long term neurological sequelae. Lactate elevation indicates anaerobic respiration and is often secondary to hypoxia or poor tissue perfusion but can indicate a metabolic disorder affecting mitochondrial function or energy metabolism. Blood gas analysis to review acid-base status is a critical test in any sick neonate or child, and correct interpretation will indicate if there is a respiratory or metabolic basis. A metabolic acidosis with elevated anion gap may indicate a primary metabolic disorder. These tests can help identify patients who may have a primary metabolic disorder, and management should be discussed urgently with a specialist metabolic centre.</div></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 10","pages":"Pages 388-392"},"PeriodicalIF":0.0,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Chronic peritoneal dialysis in children","authors":"Iona Madden, Michelle Blaauw, Natasha Baugh, Lesley Rees, Rukshana Shroff","doi":"10.1016/j.paed.2024.06.010","DOIUrl":"10.1016/j.paed.2024.06.010","url":null,"abstract":"<div><p>End stage kidney disease is rare in children and adolescents. The availability and types of kidney replacement therapy (KRT) vary widely, with a global prevalence of 18–100 per million age-related population (pmarp). Chronic peritoneal dialysis (PD) is considered the dialysis modality of choice in younger children given its almost universal applicability, cost-effectiveness, and the possibility of a home-based treatment. This is most compatible with a child's schooling and social life. Advances in technology have improved outcomes even for the youngest children, but mortality remains high, and the morbidity and burden of care should not be underestimated. Optimal management of children on dialysis requires a multidisciplinary team and consideration of the child and family's expectations. With few high-quality randomised trials, clinicians are reliant on prospective cohort studies and international registries to inform and improve management strategies. Children on dialysis have a lifetime of KRT ahead of them. The selection of dialysis modality and management must consider the principles of dialysis access preservation. This review summarises current epidemiology, principles of dialysis, PD access, modalities, prescriptions, and complications of PD, as well as a brief discussion on PD in infants with their unique ethical and technical considerations.</p></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 9","pages":"Pages 323-331"},"PeriodicalIF":0.0,"publicationDate":"2024-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142087431","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Complications of short bowel syndrome","authors":"Gautham Pai, Theodoric Wong, Girish Gupte","doi":"10.1016/j.paed.2024.07.004","DOIUrl":"10.1016/j.paed.2024.07.004","url":null,"abstract":"<div><div>Short bowel syndrome is the most common reversible cause of intestinal failure. Most of the children are started on parenteral nutrition (PN) after surgery to enable growth and allow time for intestinal adaptation. This is a process whereby the shorter length of bowel is able to achieve complete function as if the entire length of bowel is present. With management advances, most children with short bowel syndrome can discontinue PN and establish full enteral feeds. This article mainly focuses on the complications of short bowel syndrome that need to be avoided for the child to achieve intestinal adaptation, establish on enteral feeds/oral diet and achieve enteral autonomy.</div></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 10","pages":"Pages 373-378"},"PeriodicalIF":0.0,"publicationDate":"2024-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Paediatric inflammatory bowel disease: an update on current practice","authors":"Zachary Green, James J Ashton, R Mark Beattie","doi":"10.1016/j.paed.2024.07.005","DOIUrl":"10.1016/j.paed.2024.07.005","url":null,"abstract":"<div><div>Paediatric inflammatory bowel disease (PIBD), consisting of Crohn's disease, ulcerative colitis and inflammatory bowel disease unclassified, is a spectrum of chronic inflammatory conditions associated with significant morbidity. The incidence of PIBD continues to increase and disease phenotype is more severe than adult-onset disease. Referral to specialist services and diagnosis of PIBD can be slow; and faecal calprotectin is an invaluable tool for the prioritization of further investigation. Greater understanding of pre-existing treatments (such as anti-TNF monoclonal antibody therapy) and a growing arsenal of biologic and small molecule drugs have brought about significant changes in disease management. Whilst important challenges remain in the longer-term treatment of PIBD, including growth, nutrition and management of refractory disease, there remains a strong research focus on understanding underlying disease pathogenesis and a move towards personalized medicine. This review describes investigations, diagnosis and management of PIBD and presents contemporary evidence on nutritional and medical management.</div></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 10","pages":"Pages 379-387"},"PeriodicalIF":0.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Paediatric major incident translational in-situ simulation: a how-to guide","authors":"Natalie Bee, Kirsty Kilpatrick","doi":"10.1016/j.paed.2024.06.006","DOIUrl":"10.1016/j.paed.2024.06.006","url":null,"abstract":"<div><p>Major incidents in paediatrics are rare in the western world but when they occur the morbidity and mortality can be devastating. We need to ensure our teams are prepared and trained to manage such events should they occur. Major incident planning has historically been through large group discussions away from the clinical environment. With the use of in-situ translational simulation we have the opportunity to strengthen major incident planning by testing organizational systems and responses whilst simultaneously training staff, developing both their technical and non-technical skills. This paper guides the multidisciplinary team through ten key steps to developing your own major incident simulation.</p></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 8","pages":"Pages 300-304"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141690608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Upper limb function in children with cerebral palsy: a structured approach to assessment and management","authors":"Jo Lloyd, May Yip, Jill Cadwgan","doi":"10.1016/j.paed.2024.06.004","DOIUrl":"10.1016/j.paed.2024.06.004","url":null,"abstract":"<div><p>The cerebral palsies<span><span> (CP) have a UK prevalence of 1–2/1000. 60–80% of children with CP have upper-limb involvement. Physical movement difficulties including tone abnormalities, weakness and secondary musculoskeletal function are associated with sensory, cognitive difficulties and medical co-morbidities. Understanding the underlying </span>pathophysiology of CP and structured multidisciplinary assessment in the context of the International Classification of Functioning, Disability and Health (ICF) can guide management to improve activity and participation for children with CP. A range of therapy, medical and surgical interventions may be offered to a child and their family. The selection of treatment options should be focused on individualized and realistic goals. Clear outcomes should be evaluated. This review aims to summarize a structured approach to assessment and management of upper limb function in CP; and is illustrated by case examples.</span></p></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 8","pages":"Pages 279-289"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141961962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Lower-limb botulinum toxin A treatment in ambulant children with cerebral palsy","authors":"Lesley R Katchburian, Marcos V Katchburian","doi":"10.1016/j.paed.2024.06.005","DOIUrl":"10.1016/j.paed.2024.06.005","url":null,"abstract":"<div><p>Cerebral palsy<span><span> (CP) is frequently characterized by hypertonia<span>, impairing motor function and affecting the quality of life of children and young people with CP (CYPwCP). </span></span>Botulinum Toxin A<span><span> (BoNT-A) has emerged as a widely utilized treatment modality to manage lower limb hypertonia<span> in ambulant CYPwCP. This paper explores the current evidence base and clinical practice surrounding the use of BoNT-A in this population. BoNT-A injections are indicated for the management of focal lower limb hypertonia, targeting specific muscles that contribute to functional limitations. Clinical assessment plays a pivotal role in determining the suitability of BoNT-A treatment, with emphasis placed on evaluating tone, functional impairments, and individualized treatment goals. Timing of treatment is crucial, with interventions typically initiated when dynamic tone significantly impacts motor function and mobility. Optimal treatment age remains a topic of debate, with studies indicating benefits across various age groups. Close monitoring and regular follow-up evaluations are essential components of BoNT-A treatment, ensuring timely adjustments to address changing needs. BoNT-A represents a valuable therapeutic option for managing lower limb hypertonia in ambulant CYPwCP. Clinical decision-making should be guided by comprehensive assessments, utilization of appropriate outcome measures, and consideration of individualized treatment goals. Further research is warranted to elucidate optimal treatment strategies, long-term efficacy, and potential </span></span>adverse effects to optimize outcomes in this patient population.</span></span></p></div>","PeriodicalId":38589,"journal":{"name":"Paediatrics and Child Health (United Kingdom)","volume":"34 8","pages":"Pages 290-299"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141691418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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