Paediatrics and Child Health (United Kingdom)最新文献

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.

The cerebral palsies (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 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.

Cerebral palsy (CP) is frequently characterized by hypertonia, impairing motor function and affecting the quality of life of children and young people with CP (CYPwCP). Botulinum Toxin A (BoNT-A) has emerged as a widely utilized treatment modality to manage lower limb hypertonia 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 adverse effects to optimize outcomes in this patient population.

Cerebral palsy is a disorder of posture and movement. Multiple postural management interventions exist to prevent complications and improve outcomes. In this short review we followed a systematic approach to appraise the quality of evidence supporting each intervention and assessed the effectiveness across the International Classification of Functioning, Disability and Health (ICF) outcomes. Evidence-based recommendations are essential for assisting clinicians in selecting and implementing interventions aligned with the individual's goals. This review aims to summarize and evaluate the evidence supporting postural interventions for CP published after Gough's review in 2009. Additionally, it aims to provide clinical bottom lines according to GMFCS or MACS levels. We conducted our search for systematic reviews or population register studies evaluating postural management interventions in people with cerebral palsy using an umbrella systematic review design. We assessed the quality of evidence for each intervention and made recommendations using GRADE (Grading of Recommendation, Assessment, Development, and Evaluation) and the Evidence Alert Traffic Light approaches. From a total of 955 citations identified 33 unique studies met our inclusion criteria. The 33 studies included 41 interventions, measuring 22,835 participants, across 207 RCTs and 1 population register study. Of the 41 interventions, 14 were postural control interventions to improve movement; 11 were tone management interventions to improve movement or prevent deformity; 2 were postural management interventions to support upright posture; and 14 were postural management interventions to prevent deformity. Several postural control interventions are effective for improving movement, and several postural management interventions may prevent deformity, with hip surveillance having the best evidence.

Spasticity and dystonia are the commonest tonal abnormalities presenting in children and young people with Cerebral Palsy (CP). Hypertonia can cause difficulties in a wide variety of areas and impact on quality of life. Secondary changes in muscle and bone can result in further disability. Management of hypertonia is facilitated by appropriate identification of goals, muscles involved and type of hypertonia present. Stretches, exercise programmes, orthotic and equipment interventions are important mainstays in management of hypertonia. Identifying and addressing possible triggers can avert need for escalation of treatments. Medication choice and suitability of surgical interventions are influenced by other individual factors such as acceptability, route of administration and perceived benefits versus risks of intervention. Multidisciplinary working is an important cornerstone of hypertonia management. There are several medications available for treatment of hypertonia, although evidence is limited in their use in childhood. Where children have not tolerated or benefitted from medical treatments, surgical interventions may be appropriate. This short article is aimed at everyone involved in the care of children with hypertonia as a result of CP. It discusses how to select the right treatment for right patient and offers practical advice to clinicians.

Healthcare practitioners will encounter children who use orthoses to improve or maintain body structures and functions, activities and participation. Orthotic interventions involve complex science and the benefits need to outweigh the burden. Interdisciplinary collaborative family centred goal setting, from birth to adulthood is essential. An understanding of why and when orthoses are helpful allows a deeper discussion with families and can improve adherence. This article presents a systematic ‘Inside-Out Approach’ incorporating the International Classification of Functioning, Disability and Health and the International Organization for Standardization objectives for orthotic interventions. Pictorial and Table Tools identify potential goals for bones and joints, muscles, motor control of standing and walking, activities and participation, and prevention of pain which is an indicator of quality of life. Achieving short and long term goals requires early intervention. The design, alignments and dosage, the duration and frequency the child needs to wear the orthosis, required to achieve goals will be determined by an understanding of natural history and prognosis, the agreed goals and the child's schedule of activities. A Dosage Tool is presented. Recent improved understanding of the contribution of the footwear, individualized and optimized joint and segment alignments and segment proportion is discussed.

Haematuria and proteinuria are common findings on urinalysis in childhood. They usually occur in either children with specific symptoms e.g. macroscopic haematuria or nephrotic syndrome, or in those who have non-specific symptoms (usually when looking to exclude urinary tract infection) and sometimes in asymptomatic children. The majority of children fall into the latter two groups, the finding is temporary and not associated with long term renal disease. If the finding is persistent or patients have specific clinical features then renal abnormalities are more frequent and appropriate investigation is required. This review provides a rationale for an approach that allows reassurance to be given to children and their families in whom the finding is transient and benign, whilst identifying those in whom renal abnormalities are present and treatment required. It describes algorithms for macroscopic haematuria (MaH), asymptomatic microscopic haematuria (MiH) and proteinuria.

Cystic kidney diseases encompass a range of genetic disorders in which the primary cilia of the cells are affected and thereby cysts form as a result. There are an increasing range of cystic renal diseases recognized due to the advances in genomics. The most common genetic kidney condition is autosomal dominant polycystic kidney disease (ADPKD). ADPKD leads to renal failure in adulthood. In children, hypertension is common and if treated, may slow down renal decline. The most common cystic kidney disease causing renal failure in children is autosomal recessive polycystic kidney disease (ARPKD). ARPKD also affects the liver. These conditions often have extra-renal features which also need to be addressed. Until recently, treatments were mainly supportive but now it is possible to slow down development of cyst formation and renal decline in ADPKD. This raises hope for treatment for other cystic renal conditions as more genes are identified and underlying mechanisms defined.

Urinary tract infections (UTI) are a common problem in childhood. The clinical presentation is variable depending on age and whether there is pyelonephritis or cystitis. UTI are diagnosed by culturing an appropriately collected urine sample. Most children with UTI can be managed safely with oral antibiotics irrespective of whether they have cystitis or pyelonephritis. Following a single UTI a significant proportion of children will develop recurrent UTI and many of these children will have identifiable risk factors. Treating children with recurrent UTI with repeated courses of antibiotics or long term prophylactic antibiotics puts patients at risk of infections with multi-resistant organisms. Furthermore recurrent UTI are also associated with the risk of renal parenchymal damage with long term health implications. It is therefore essential that management of children with UTI focuses not only on early diagnosis and treatment but also on UTI prevention.