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Clinical characteristics and management of plexiform neurofibromas in children with neurofibromatosis 1: A Japanese nationwide survey.
Brain Dev
November 2024
Department of Pediatrics, Faculty of Medicine, Saga University, Saga, Japan.
Article Synopsis
- The study aims to analyze the characteristics and treatment of plexiform neurofibromas (PNs) in Japanese children with neurofibromatosis 1 (NF1), particularly with the introduction of the MEK inhibitor selumetinib.
- Primary and secondary surveys were conducted across numerous pediatric and dermatology departments in Japan, tracking 49 patients aged 3.3 to 18.8 years with PNs, commonly found on the face, neck, and head.
- The findings highlight early onset and significant morbidities like cosmetic disfigurement and pain, showing that selumetinib use is linked to these symptoms, stressing the importance of early diagnosis and the need for a multicenter registry to improve management strategies.
Spatial Lipidomics Reveals Myelin Defects and Protumor Macrophage Infiltration in Malignant Peripheral Nerve Sheath Tumor Adjacent Nerves.
Lab Invest
January 2025
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands. Electronic address:
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas arising from peripheral nerves, accounting for 3% to 5% of soft tissue sarcomas. MPNSTs often recur locally, leading to poor survival. Achieving tumor-free surgical margins is essential to prevent recurrence, but current methods for determining tumor margins are limited, highlighting the need for improved biomarkers.
View Article and Find Full Text PDFQuality of life of patients with neurofibromatosis 1-Physical disability does not necessarily result in poor mental health.
Front Neurol
October 2024
Department of Neurosurgery, University of Ulm, Universitätsklinikum Ulm, Ulm, Germany.
Introduction: Neurofibromatosis 1 (NF1) is a chronic neurocutaneous disease known to profoundly affect quality of life (QoL). We have performed an analysis of disease severity, mental and physical QoL and compare the different subclasses among patients with neurofibromatosis 1 (NF1).
Patients And Methods: We conducted a prospective analysis of 89 NF1 patients between January 2016 and March 2018.
Altered body as a source of interactional problems in the family of individuals with neurofibromatosis type 1 - A polish study.
PLoS One
November 2024
DOCENTMED Individual Specialist Medical Practice in Lublin, Lublin, Poland.
Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, whose clinical picture is dominated by visible body changes as well as numerous somatic and behavioural abnormalities.
Aim: The aim of the study was to explore the ways in which the individual experiences NF1 in everyday life, with particular emphasis on the impact of the altered body on family interactions, in addition to the personal and social identity of individuals with NF1.
Methods: A qualitative study was performed using individual in-depth interviews with 93 individuals with NF1 (median age: 36.
[Nursing care for patients with rare dermatological diseases].
Soins
November 2024
Service de dermatologie pédiatrique, CHU de Toulouse, hôpital Larrey, 24 chemin de Pouvourville - TSA 30030, 31059 Toulouse cedex, France.
Rare dermatological diseases cause great difficulties for sufferers. They impact their lives through visibility, pain, restrictive care and sometimes serious complications. This article describes the management of three rare dermatological diseases by caregivers trained and sensitized to these pathologies in centers of reference for rare dermatological diseases.
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