201 results match your criteria: "Bristol Children's Hospital[Affiliation]"

Background: The application of international recommendations for paediatric maintenance haemodialysis (HD) could be strengthened by national laws or written recommendations. Our aim was therefore to describe the national rules governing paediatric maintenance HD in European countries.

Methods: A national representative, approved by the president of each paediatric nephrology society, was contacted in all 42 European countries to complete two online questionnaires.

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Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients.

Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation.

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Aim: Some children require long-term drainage of the bladder but do not tolerate clean intermittent catheterisation (CIC) urethrally. We aimed to compare long term suprapubic catheter (SPC) drainage vs Mitrofanoff conduit (allowing CIC) by comparing the survival of the drainage methods and rates of urinary tract infection (UTI).

Method: Retrospective review of a single surgeon's experience (2007-2023).

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The first 8000 days of life, from birth to adulthood, encompasses critical phases that shape a child's health and development. While global health efforts have focused on the first 1000 days, the next 7000 days (ages 2-21) are equally vital, especially concerning the unmet burden of surgical conditions in low- and middle-income countries (LMICs). Approximately 1.

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Article Synopsis
  • The study focuses on evaluating long-term outcomes for congenital heart diseases (CHDs) rather than just short-term postoperative metrics, highlighting the need for improved monitoring practices.* -
  • Researchers identified nine common CHDs and established metrics for long-term survival and reinterventions, using a national cohort of over 29,000 children born with these conditions from 2000 to 2022.* -
  • The findings reveal varying survival rates and reintervention incidences at 10 years post-treatment for different CHDs, indicating significant differences in long-term health outcomes for affected children.*
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Article Synopsis
  • Congenital cardiac care involves many people like patients, families, doctors, and healthcare workers who all help make important decisions together.
  • Game theory studies how people make choices and how those choices affect each other, sort of like a strategy game.
  • Using game theory can help doctors make better decisions that focus on what’s best for the patient, which may lead to better health results.
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Background: Kidney failure at any age has a significant impact on quality of life (QoL) but the overall symptom burden for children and young people (CYP) is poorly described. Kidney failure has no cure and whilst transplantation is the preferred management option, it is not always possible, with patients requiring supportive care at the end of their lives.

Aim: To use the literature to understand the symptom burden for CYP with kidney failure who are approaching end-of-life.

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Purpose: Adequate preoperative information is known to improve patient outcomes. We aimed to evaluate perioperative education for paediatric patients and families undergoing intestinal stoma formation.

Methods: UK paediatric surgery centres were invited to recruit patients aged 6-16 years with a stoma in situ or reversed within the last 2 years.

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Background: Paediatric femoral shaft fractures can be managed with single- or double-leg hip spica casting between ages six-months and six-years. The aim of this review was to determine if single-leg hip spicas reduce the impact on family life without compromising fracture stability.

Methods: The study was registered on PROSPERO (CRD42023454309).

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The experiences of individuals with body dysmorphic disorder: A systematic review and thematic synthesis of qualitative research.

Body Image

September 2024

Clinical Psychology Training, School of Psychology, Cardiff University/Cardiff and Vale University Health Board, 11th Floor, Tower Building, 70 Park Place, Cardiff CF10 3AT, UK. Electronic address:

Body Dysmorphic Disorder (BDD) is a distressing psychological condition where an individual is preoccupied by a perceived issue with their appearance. Qualitative studies enable nuanced aspects of BDD phenomenology to be investigated. The current systematic review used thematic synthesis to integrate the findings from the extant qualitative studies.

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Article Synopsis
  • This study investigates the role of inversions—structural variants that involve the rearrangement of DNA—in genetic diseases, using data from 33,924 families involved in the 100,000 Genomes Project.
  • Researchers identified 47 ultra-rare rearrangements, including de novo inversions, in genes linked to disease, with analyses correlating genetic findings to clinical outcomes in some cases, including a specific diagnosis for three family members.
  • The findings suggest that while inversions are less common in genetic diseases compared to other structural variants, they can significantly contribute to the etiology in approximately 1 in 750 families with rare conditions.
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Background: Pediatric inflammatory bowel disease (pIBD) incidence has increased over the last 25 years. We aim to report contemporaneous trends across the South West United Kingdom.

Methods: Data were provided from centers covering the South West United Kingdom (Bristol, Oxford, Cardiff, Exeter, and Southampton), with a total area at-risk population (<18 years of age) of 2 947 534.

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Background: Primary, secondary and tertiary healthcare services in Europe create complex networks covering pediatric subspecialties, sociology, economics and politics. Two surveys of the European Society for Paediatric Nephrology (ESPN) in 1998 and 2017 revealed substantial disparities of kidney care among European countries. The purpose of the third ESPN survey is to further identify national differences in the conceptualization and organization of European pediatric kidney health care pathways during and outside normal working hours.

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Apixaban for Prevention of Thromboembolism in Pediatric Heart Disease.

J Am Coll Cardiol

December 2023

Department of Paediatrics, University of Melbourne, Parkville, Victoria, Australia; Haematology Research, Murdoch Children's Research Institute, Parkville, Victoria, Australia; Department of Haematology, Royal Children's Hospital, Melbourne, Victoria, Australia; Kids Cancer Centre, Sydney Children's Hospital, Randwick, NSW, Australia.

Background: Children with heart disease frequently require anticoagulation for thromboprophylaxis. Current standard of care (SOC), vitamin K antagonists or low-molecular-weight heparin, has significant disadvantages.

Objectives: The authors sought to describe safety, pharmacokinetics (PK), pharmacodynamics, and efficacy of apixaban, an oral, direct factor Xa inhibitor, for prevention of thromboembolism in children with congenital or acquired heart disease.

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Recurrent de novo variant causes childhood-onset amyotrophic lateral sclerosis (ALS) by excess sphingolipid synthesis.

J Neurol Neurosurg Psychiatry

January 2024

Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of the upper and lower motor neurons with varying ages of onset, progression and pathomechanisms. Monogenic childhood-onset ALS, although rare, forms an important subgroup of ALS. We recently reported specific variants resulting in sphingolipid overproduction as a cause for juvenile ALS.

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Purpose: We tested whether blinatumomab (Blina) is effective as a toxicity-sparing alternative to first-line intensive chemotherapy in children and young persons (CYP) with B-ALL who were chemotherapy-intolerant or chemotherapy-resistant.

Methods: Data were collected for consecutive CYP (age 1-24 years) with Philadelphia chromosome-positive or Philadelphia chromosome-negative B-ALL who received Blina as first-line therapy. Blina was given as replacement for postremission intensive chemotherapy to patients with chemotherapy intolerance or resistance.

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Acute electrolyte and acid-base imbalance is experienced by many children following kidney transplant. This is partly because doctors give very large volumes of artificial fluids to keep the new kidney working. When severe, fluid imbalance can lead to seizures, cerebral edema and death.

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Purpose Of Review: The pelvis and hip account for 0.3--4% of fractures of the immature skeleton, and generally result from high energy trauma. These fractures range in severity from apophyseal avulsions to complete disruptions of the pelvic ring.

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Aim: To investigate how magnetic resonance imaging (MRI) examinations are protocolled in tertiary paediatric neuroradiology centres around the UK for some of the more common presentations encountered in paediatric neuroradiology, and to identify any variations of note.

Materials And Methods: All 19 UK tertiary paediatric neuroradiology centres registered with the British Society of Neuroradiologists-Paediatric Group were contacted and asked if they could provide a copy of their standard MRI protocols. Twelve responded (63%) and 10 of the more common presentations were selected and the standard acquired sequences obtained at each participating centre were compared.

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Background: The UK kidney offering scheme introduced a kidney donor risk index (UK-KDRI) to improve the utility of deceased-donor kidney allocations. The UK-KDRI was derived using adult donor and recipient data. We assessed this in a paediatric cohort from the UK transplant registry.

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Background: Congenital anomalies are a leading cause of morbidity and mortality worldwide. We aimed to review the common surgically correctable congenital anomalies with recent updates on the global disease burden and identify the factors affecting morbidity and mortality.

Method: A literature review was done to assess the burden of surgical congenital anomalies with emphasis on those that present within the first 8000 days of life.

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Vagus nerve stimulation is a well-established treatment option for patients with drug-resistant epilepsy and has an expanding range of other clinical indications. Side effects of vagus nerve stimulation therapy include: cough; voice changes; vocal cord adduction; rarely, obstructive sleep apnoea; and arrhythmia. Patients with implanted vagus nerve stimulation devices may present for unrelated surgery and critical care to clinicians who are unfamiliar with their function and safe management.

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