Browse Items (17 total)

In children with severe generalized recessive dystrophic epidermolysis bullosa (RDEB), esophageal scarring leads to esophageal strictures with dysphagia, followed by malnutrition and delayed development. We describe a two-step multidisciplinary…

Recessive dystrophic epidermolysis bullosa (RDEB) is associated with high nutritional demands, esophageal strictures and dysphagia. About one quarter of the patients require gastrostomy tube placement to maintain adequate nutritional status. To…

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is a simple and reliable method, but there are few data about its use in pediatric forms of neuromuscular diseases (NMD). The aim of this study was to evaluate the nutritional effects and…

AIM: To develop a core outcome set (COS) for evaluating gastrostomy/gastrojejunostomy tube impact in children with neurological impairment. METHOD: Healthcare providers/researchers and caregivers rated the importance of candidate outcomes on a…

Introduction: Many paediatric hospitals are treating increasing numbers of children with medical complexity (CMC), diagnosed with chronic life-limiting illnesses and requiring life-sustaining home medical technology. These medically fragile children…

Introduction: This National Hospital Clinical Research Program (PHRC) was conducted in France between 2012 and 2016 to depict palliative practices in spinal muscular atrophy type 1 (SMA-1). New drugs (Nusinersen) have been developed and may modify…

Introduction: Patients may be admitted to hospital by paediatric palliative care units (PPCU) for different reasons, due to their different needs and clinical problems. The objective of this study is to present the data of patients admitted to the…

OBJECTIVES: To document the clinical and neurodevelopmental profiles of a cohort of patients with neonatal-onset propionic acidemia and to determine the efficacy of current therapy with respect to outcome. METHOD: The clinical, neurologic, and…

Following gastrostomy tube placement some children develop gagging, retching, vomiting, pain, or irritability during feedings. Conventional medical management is not always successful. It is possible that intolerance of gastrostomy tube feedings…

This is a report of two children who had severe recurrent gastric distension and vomiting, and five who experienced severe gastric distension without vomiting. Two of the five died during an episode of acute gastric distension. All had marked…

Feeding difficulties are common in neurologically impaired children, often leading to great distress and frustration in the child and family. A gastrostomy may be advocated if oral intake is inadequate causing poor weight gain or when there is…

Percutaneous endoscopic gastrostomy techniques are becoming more widely available and will be considered increasingly for patients with head and neck or upper gastrointestinal malignancies, and for neurological dysphagia, as occurs in motor neurone…

Pediatric surgeons can play an important role in offering procedures that may improve the quality of life for terminally ill children. As with all palliative interventions, surgical therapies should be evaluated in the context of explicitly defined…

INTRODUCTION: The creation of paediatric palliative care units (PPCU) could optimise the management of children with palliative focus after admission to a paediatric intensive care unit (PICU). This study describes the clinical and epidemiological…
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