Publications by authors named "Hill Susan"

Background: Severe acute malnutrition (SAM) is the most life-threatening form of undernutrition, and children hospitalised with complications have unacceptably high mortality. Complicated SAM is a multisystem disease characterised pathophysiologically by muscle wasting, systemic inflammation, metabolic dysfunction, and malnutrition enteropathy including epithelial barrier dysfunction. There is a clear need for novel interventions to address the underlying pathogenic perturbations of complicated SAM.

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Objectives: Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks.

Methods: This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF.

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Functional gastrointestinal disorders (FGID), such as infant regurgitation, infant colic, and functional constipation, are common and typically physiological phenomena during the early months of an infant's life and account for frequent consultations with pediatricians. Various infant formulas are marketed for their management and are frequently given by parents to infants before a medical consultation. However, the evidence supporting their effectiveness is limited and some have altered nutritional compositions when compared to standard formulas.

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Article Synopsis
  • Malnutrition is a major cause of child deaths globally, with Severe Acute Malnutrition (SAM) leading to high mortality rates, especially when compounded by infections or medical complications.
  • A clinical trial conducted in Zambia and Zimbabwe evaluated four potential treatments for malnutrition-related enteropathy in 125 hospitalized children aged 6-59 months with SAM, comparing bovine colostrum, N-acetyl glucosamine, teduglutide, budesonide, and standard care over 14 days.
  • Results indicated that teduglutide significantly reduced mucosal damage biomarkers, suggesting it may be a beneficial treatment for enteropathy in children with complicated malnutrition, with all interventions deemed safe.
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Background: Body composition could help identify malnutrition in pediatric patients, but there is uncertainty over which techniques are most suitable and prevailing opinion that measurements are difficult to obtain in practice. This study examined the acceptability, practicality, reliability, and validity of different anthropometric and body composition measurements in patients with complex diagnoses in a tertiary pediatric hospital.

Methods: A total of 152 children aged 5-18 years had weight, height, body mass index (BMI), mid-upper arm circumference (MUAC), 4-site skinfold thicknesses (SFT), bioelectrical impedance analysis (BIA), and dual-energy x-ray absorptiometry (DXA) assessed on admission and discharge.

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Background & Aims: Catheter-related bloodstream infection (CRBSI) is the most common, potentially life-threatening complication of long-term parenteral nutrition (PN). We prospectively assessed the incidence and risk factors for CRBSI in children receiving long-term home PN (HPN) for intestinal failure (IF) in a single IF rehabilitation center.

Methods: Data regarding episodes and potential risk factors for CRBSI in children on HPN were prospectively recorded.

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Objectives: Children requiring a central venous catheter (CVC) for long-term parenteral nutrition (PN) are at risk of CVC breakage. Modern intestinal failure (IF) management aims to preserve vascular access sites. CVC repair rather than removal is hence attempted for broken catheters.

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Background: Continuous glucose monitoring systems have been validated for eu- and hyperglycemic cats. The FreeStyle Libre 2 (FSL2) is sufficiently accurate in people during hypoglycemia to guide critical treatment decisions without confirmation of blood glucose concentration (BG).

Objectives: Assess FSL2 accuracy in cats with hypoglycemia.

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Objectives: Our objective was to evaluate the short- and long-term safety and efficacy of teduglutide treatment in infants and children with short bowel syndrome with intestinal failure (SBS-IF).

Methods: Two open-label phase 3 studies and 1 extension study investigated the short- and long-term safety and efficacy of teduglutide (0.05 mg/kg/day) in infants and children with SBS-IF: NCT03571516, 24-week study of infants who were randomized to receive teduglutide or standard of care (SoC); NCT02980666, 24-week study of infants and children who all received teduglutide; and NCT03268811, 24-week extension study of patients who completed NCT02980666 (patients could receive up to 48 weeks of total treatment).

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Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The mainstay of treatment for IF is parenteral nutrition (PN). The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition.

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Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The preferred treatment for IF is parenteral nutrition which may be required until adulthood. The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition.

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Background: Pediatric intestinal pseudo-obstruction (PIPO) encompasses a variety of rare, heterogeneous, and disabling disorders that severely affect gastrointestinal motility and are associated with high morbidity and mortality. PIPO management is complex and focuses on maintaining an optimal nutritional status, improving gut function, relieving symptoms, and treating complications. Nutritional issues prevail, and PIPO patients often experience severe undernutrition and faltering growth.

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Texting-based programs are increasingly used to support parents as their child's first teacher and create links between home and school. However, there is scant evidence about the influence of program implementation on parent uptake and attrition-a key component of such programs. This article describes the design and delivery of Kindytxt, a literacy-based text-messaging program for parents with a child at Kindergarten in Western Australia, and examines the influence of recruitment method, area socioeconomic status, and teacher participation on parent uptake and attrition.

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Objectives: Children receiving home parenteral nutrition (HPN) are at risk of iron deficiency anaemia. Our aim was to determine the incidence of iron deficiency anaemia in paediatric HPN and efficacy of each therapeutic approach.

Methods: Retrospective data collection from children receiving HPN at a tertiary referral centre over a 12-month period (2015).

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Purpose: To undertake a pilot study estimating patient-level costs of care for paediatric short bowel syndrome (SBS) from the healthcare provider perspective.

Methods: A pilot group of patients with anatomical SBS was selected at a single specialist tertiary centre in the United Kingdom. The Patient Level Information and Costing System (PLICS) was used to extract costing data for all hospital-based activities related to SBS, from the implementation of PLICS in 2016 to April 2021.

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Objective: To review the outcome of children with severe neurological impairment (NI) and intestinal failure (IF) referred to our specialist multidisciplinary IF rehabilitation service and to discuss implications.

Design: Case report series, descriptive analysis.

Setting: IF rehabilitation programme at a tertiary children's hospital in the UK.

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Purpose: Genome sequencing (GS) for diagnosis of rare genetic disease is being introduced into the clinic, but the complexity of the data poses challenges for developing pipelines with high diagnostic sensitivity. We evaluated the performance of the Genomics England 100,000 Genomes Project (100kGP) panel-based pipelines, using craniosynostosis as a test disease.

Methods: GS data from 114 probands with craniosynostosis and their relatives (314 samples), negative on routine genetic testing, were scrutinized by a specialized research team, and diagnoses compared with those made by 100kGP.

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Objectives: Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/100,000 live births) that requires a multidisciplinary team approach to management. In January 2019, the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with SBS were devised.

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There are significant concerns regarding prescription and misuse of prescription opioids in the perioperative period. The Faculty of Pain Medicine at the Royal College of Anaesthetists have produced this evidence-based expert consensus guideline on surgery and opioids along with the Royal College of Surgery, Royal College of Psychiatry, Royal College of Nursing, and the British Pain Society. This expert consensus practice advisory reproduces the Faculty of Pain Medicine guidance.

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Background: This analysis assessed combined safety data from 4 clinical studies of teduglutide in pediatric patients with short-bowel syndrome-associated intestinal failure (SBS-IF).

Methods: Safety data from teduglutide-treated patients in 4 clinical trials were pooled. The completed 12-week and 24-week phase 3 core studies (NCT01952080/EudraCT 2013-004588-30 and NCT02682381/EudraCT 2015-002252-27) enrolled children aged 1-17 years with SBS-IF.

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Congenital chloride diarrhea (CCD) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a transmembrane chloride/bicarbonate ion exchanger mainly expressed in the apical brush border of the ileal and colonic epithelium. Lifelong, secretory, chloride-rich diarrhea and hypochloremic, hypokalemic metabolic alkalosis are characteristic. Histological evidence of bowel inflammation is not typically described in CCD and has only been reported in a few patients.

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Background: Better tools are needed to diagnose and identify children at risk of clinical malnutrition.

Objectives: We aimed to compare body composition (BC) and malnutrition screening tools (MSTs) for detecting malnutrition on admission; and examine their ability to predict adverse clinical outcomes [increased length of stay (LOS) and complications] in complex pediatric patients.

Methods: This was a prospective study in children 5-18 y old admitted to a tertiary pediatric hospital (n = 152).

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