Association of chromhidrosis and segmental neurofibromatosis.

[Association of chromhidrosis and segmental neurofibromatosis]. Presse Med. 2018 Mar 09;: Authors: Velter C, Thubert S, Wolkenstein P, Le Cleach L PMID: 29530298 [PubMed - as supplied by publisher]
Source: Presse Medicale - Category: General Medicine Authors: Tags: Presse Med Source Type: research

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Background: Early definitive spinal fusion (EF) has been widely recommended to avoid spinal deformity progression for early-onset scoliosis (EOS) with neurofibromatosis type 1 (NF-1). In contrast, growing rod (GR) procedure has recently been recommended for EOS associated with the dystrophic type NF-1. However, no studies have compared the surgical outcomes between EF and GR procedure for EOS with NF-1. The purpose of this study was to compare the surgical outcomes of EF versus GR for early onset and dystrophic scoliosis with NF-1. Methods: This was a retrospective multicenter study; 26 EOS patients with dystrophic ty...
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Scioliosis/Kyphosis Source Type: research
ConclusionsTrametinib was an active and feasible treatment for progressive pLGG leading to disease control in all patients. However, treatment related toxicity interfered with treatment in individual patients, and disease control after MEKi withdrawal was not sustained in a fraction of patients. Our data support in-class efficacy of MEKi in pLGGs and necessity for upfront randomized testing of trametinib against current standard chemotherapy regimens.
Source: Journal of Neuro-Oncology - Category: Cancer & Oncology Source Type: research
ConclusionBezold –Jarisch Reflex as a result of narrow thoracic inlet caused by cervical kyphosis and compensatory hyperlordosis of the upper thoracic spine has never been reported. This case highlights the need to introspect into thoracic inlet morphology in severe cervicothoracic deformities. Thoracic inlet deco mpression is an efficient way of addressing this unique complication.
Source: European Spine Journal - Category: Orthopaedics Source Type: research
Publication date: Available online 28 September 2020Source: NeuroImage: ClinicalAuthor(s): Natalie R Boonzaier, Patrick W Hales, Felice D'Arco, Bronwen C Walters, Ramneek Kaur, Kshitij Mankad, Jessica Cooper, Alki Liasis, Victoria Smith, Patricia O'Hare, Darren Hargrave, Christopher A Clark
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
Publication date: Available online 1 October 2020Source: Stem Cell ResearchAuthor(s): Kiymet Bozaoglu, Wei Shern Lee, Kristina M Haebich, Kathryn N North, Jonathan M Payne, Paul J Lockhart
Source: Stem Cell Research - Category: Stem Cells Source Type: research
mel In this Special Issue of Cancer, a series of 10 papers (seven papers, three reviews) on Neurofibromatosis is presented by international leaders in this field of research [...]
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Editorial Source Type: research
Authors: Rago A, Tordi A, Siniscalchi A, Andrizzi C, Campagna S, Toritto TCD PMID: 32989179 [PubMed]
Source: Blood Research - Category: Hematology Tags: Blood Res Source Type: research
Head and neck manifestations of neurofibromatosis type 1 (NF1) often result in significant facial deformities, nerve compression, and airway obstruction. The treatment of these facial deformities with orthognathic combined with TMJ surgery is not well-reported in the literature.
Source: Journal of Oral and Maxillofacial Surgery - Category: ENT & OMF Authors: Source Type: research
Neurofibromatosis type 1 (NF-1; von Recklinghausen disease) is an autosomal dominant genetic disorder characterized by multiple caf é-au-lait macules and neurofibromas [1]. Malignant neoplastic complications are common causes of death in NF-1 patients. Vascular complications are very rare but can lead to life-threatening hemorrhage if they occur [2,3]. Among the various vascular complications, there have been only a few case re ports of NF-1 patients with ruptures of artery aneurysms in the neck region that compromised upper airway [4,5].
Source: The American Journal of Emergency Medicine - Category: Emergency Medicine Authors: Source Type: research
PMID: 33002413 [PubMed - as supplied by publisher]
Source: Canadian Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Can J Ophthalmol Source Type: research
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