Background: We aimed to describe the electrophysiological progression rate of chronic idiopathic axonal polyneuropathy (CIAP) and look into the potential role of human leukocyte antigen (HLA) genetic susceptibility in its development.
Methods: We recruited 57 patients with CIAP (mean age at diagnosis 67, mean follow-up 7 years). The assessments included clinical and electrophysiological data and HLA-DQ genotyping.
Results: The DQA1*05 allele was found more frequently in patients than in healthy controls (odds ratio, 1.96, P = .011). In patients with length-dependent CIAP, a linear effect of time on the electrophysiological findings was found in the superficial radial (3.2% mean annual decrement, P < .001), sural (4.7% mean annual decrement, P = .002) and tibial nerve (6.1% mean annual decrement, P = .007) amplitudes, independently from age or gender.
Conclusions: Patients with length-dependent CIAP, show a linear progression over time. Interesting associations of HLA-DQA1*05 allele with length-dependent CIAP and non-DQ2/DQ8 with idiopathic sensory ganglionopathy were found. These merit further investigation in larger cohorts and may suggest a role of the immune system in the pathogenesis of CIAP.
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http://dx.doi.org/10.1002/mus.27164 | DOI Listing |
J Family Med Prim Care
November 2024
Pediatric Department, Jeddah, Saudi Arabia.
Celiac disease (CD) is a chronic illness. Blood testing for tissue transglutaminase antibodies is the initial screening test for the diagnosis of CD, and upper gastrointestinal endoscopy and duodenal/jejunal biopsy are used to confirm CD. Intussusception (IS) is the process in which a proximal segment of the bowel invaginates through the lumen of a distal segment.
View Article and Find Full Text PDFJ Family Med Prim Care
November 2024
Flinders University College of Medicine and Public Health, Adelaide, Australia.
Up to 10% of uveitis cases occur in children, with notable implications due to the risk of chronicity and vision loss. It can result from infections, autoimmune and autoinflammatory diseases, trauma, or masquerade syndromes. Primary care providers are vital in early detection, symptom management, and timely specialist referral.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Nanjing Hospital of Chinese Medicine Affiliated to Nanjing University of Chinese Medicine, Nanjing University of Chinese Medicine, Nanjing, Jiangsu, China.
Background: Meniere's disease (MD) is an idiopathic chronic inner ear disease that seriously impacts patients' physical and mental health. Medications may be effective for a proportion of patients, and additional effective treatments are still needed. This review aimed to evaluate the efficacy of acupuncture treatment for MD.
View Article and Find Full Text PDFCase Rep Genet
December 2024
Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Aichi, Japan.
Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare disorder caused by heterozygous pathogenic variants and is characterized by both progressive heterotopic ossification of the soft tissues and congenital malformations of the great toe. In addition to pathological skeletal metamorphosis, patients with FOP experience diverse neurological symptoms such as chronic pain and involuntary movements; however, little is known about the association between FOP and epileptic seizures. We report the case of a young boy with FOP who sustained multiple major fractures due to epileptic loss of consciousness.
View Article and Find Full Text PDFAdv Ther
December 2024
Professor Emeritus, Tohoku University, Sendai, Japan.
Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial pneumonia, which is characterised by progressive worsening of dyspnoea and lung function. Nintedanib treatment is recommended to slow IPF disease progression. The aim of this post-marketing surveillance (PMS) study was to evaluate the safety and effectiveness of nintedanib over 24 months in patients with IPF in a real-world setting in Japan.
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