In this article, patients with cerebrotendinous xanthomatosis (CTX) and caregivers detail their experience with lifelong symptoms, diagnosis, treatment and efficacy, and ongoing disease management. One patient and four caregivers describe the challenges associated with pursuing a correct diagnosis for years before testing confirmed a CTX diagnosis. They also detail their ongoing struggles and desire for greater access to physicians with CTX knowledge and to reliable online resources to continue their education about the disease and strategies for symptom management. The expert perspective is a direct response by three CTX researchers, including physicians who are treating patients with CTX in the United States and experts whose laboratories provide genetic and biochemical testing for CTX. They respond to many of the patient and caregiver concerns, including steps that are being taken to identify CTX earlier and provide access to confirmatory diagnostic testing sooner, and suggest the best online resources for CTX-related information and access to webinars and support groups. While the expert perspective is a direct response to the patient and caregiver authors' CTX journeys, it should be beneficial to any patient with CTX or their caregivers.
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http://dx.doi.org/10.1007/s12325-023-02687-8 | DOI Listing |
BMC Pediatr
December 2024
Department of Nursing and Midwifery, College of Health, Wellbeing and Life Sciences, Sheffield Hallam University, Sheffield, UK.
Background: Despite progress made towards SDG 3, sub-Saharan Africa lags behind the rest of the world, accounting for over 50% of global neonatal deaths. The increased number of hospital births in the region has not reciprocated the reduction in neonatal mortality rates. Sick newborns face uncertain journeys from peripheral facilities to specialized centres arriving in suboptimal conditions, which impacts their outcomes, due partly to the scarcity of dedicated neonatal transport services.
View Article and Find Full Text PDFBurns
December 2024
Children's Health Research Centre, The University of Queensland, Faculty of Medicine, Herston, Queensland, Australia; Australian Centre for Health Services Innovation (AusHI), Centre for Healthcare Transformation, School of Public Health and Social Work, Queensland University of Technology, Kelvin Grove, Queensland, Australia.
Introduction: The Brisbane Burn Scar Impact Profile (BBSIP) and the Patient and Observer Scar Assessment Scale (POSAS) are used in burn scar assessment to quantify patient health-related quality of life (HR-QoL). These questionnaires were developed using pen-and-paper delivery methods; however, there is a push towards electronic delivery of these questionnaires in both clinical practice and research. Equivalence testing is required to ensure that validity of these paper questionnaires is maintained electronically.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Department of Medical Genetics, Telemark Hospital Trust, Skien, Norway.
Background: Epidermolysis bullosa (EB) comprises a group of genetically and clinically heterogeneous diseases characterized by skin fragility and blistering. EB is incurable, and treatment consists of preventing blisters in addition to painful and time consuming skin care, often performed by the parents, in addition to monitoring other symptoms in cases of severe EB.
Results: The purpose of this study was to explore parental experiences of caring for a child with EB.
Sci Rep
December 2024
Department of Electronics and Telecommunications, Politecnico di Torino, 10123, Torino, Italy.
Chronic wounds are a syndrome that affects around 4% of the world population due to several pathologies. The COV-19 pandemic has enforced the need of developing new techniques and technologies that can help clinicians to monitor the affected patients easily and reliably. In this prospective observational study a new device, the Wound Viewer, that works through a memristor-based Discrete-Time Cellular Neural Network (DT-CNN) has been developed and tested through a clinical trial of 150 patients.
View Article and Find Full Text PDFTransfus Apher Sci
December 2024
Alexion, AstraZeneca Rare Disease, 121 Seaport Blvd, Boston, MA 02210, USA. Electronic address:
Plasma exchange (PE) outcomes in patients with trigger-associated thrombotic microangiopathy (TMA) have not been comprehensively reviewed. Embase and MEDLINE® were searched on 03/14/2022 for English language articles published after 2007, alongside a congress materials search (2019-2022; PROSPERO: CRD42022325170). Studies with patients with trigger-associated TMA (excluding thrombotic thrombocytopenic purpura, 'typical' hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli, post-partum TMA, and TMAs with known genetic cause) who received PE or plasma infusion (PI) and reported treatment response (including measures), safety, patient-/caregiver-reported outcomes, or economic burden data were examined.
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