دورية أكاديمية
Cutaneous amyloid is a biomarker in early ATTRv neuropathy and progresses across disease stages
العنوان: | Cutaneous amyloid is a biomarker in early ATTRv neuropathy and progresses across disease stages |
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المؤلفون: | Roy Freeman, Alejandra Gonzalez‐Duarte, Fabio Barroso, Marta Campagnolo, Sharika Rajan, Jennifer Garcia, Jee Young Kim, Ningshan Wang, Lucas Orellana, Christopher Gibbons |
المصدر: | Annals of Clinical and Translational Neurology, Vol 9, Iss 9, Pp 1370-1383 (2022) |
بيانات النشر: | Wiley, 2022. |
سنة النشر: | 2022 |
المجموعة: | LCC:Neurosciences. Biological psychiatry. Neuropsychiatry LCC:Neurology. Diseases of the nervous system |
مصطلحات موضوعية: | Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571, Neurology. Diseases of the nervous system, RC346-429 |
الوصف: | Abstract Objective To determine the sensitivity and specificity of cutaneous amyloid deposition in relation to patient‐reported measures in the earliest disease stage of hereditary ATTR amyloidosis (ATTRv). Methods In a cross‐sectional study, we analyzed 88 individuals with TTR mutations, 47 of whom were in the earliest disease stage and without clinically evident neuropathy, 12 healthy controls, and 13 disease controls with diabetes. All participants' neuropathy symptoms and signs were assessed using validated patient and clinician‐reported measures and 3‐mm skin punch biopsies were immunostained using protein gene product 9.5 and Congo Red. Results Amyloid can be detected in the earliest disease stages in up to 86% of patients with ATTRv amyloidosis. Amyloid was not detected in healthy individuals or individuals with diabetic peripheral neuropathy supporting a sensitivity of 86% and a specificity of 100%. The cutaneous deposition of amyloid correlates with neuropathy sensory symptoms, measured with the Neuropathy Total Symptom Score‐6 (R = 0.46, p |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2328-9503 |
Relation: | https://doaj.org/toc/2328-9503 |
DOI: | 10.1002/acn3.51636 |
URL الوصول: | https://doaj.org/article/8b09026792cb4814b07a1bf400e47db4 |
رقم الأكسشن: | edsdoj.8b09026792cb4814b07a1bf400e47db4 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 23289503 |
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DOI: | 10.1002/acn3.51636 |