Impact of Non-Cardiac Clinicopathologic Characteristics on Survival in Transthyretin Amyloid Polyneuropathy

ConclusionSurvival in patients with ATTR-PN is highly variable and affected by non-cardiac baseline characteristics, such as autonomic dysfunction, large fiber involvement, late-onset disease, and non-Val30Met mutation. Careful interpretation of these findings is warranted given that this synthesis did not control for differences between studies. Survival in patients with ATTR-PN remains poor among those who are untreated or with delayed diagnosis.
Source: Neurology and Therapy - Category: Neurology Source Type: research

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Publication date: June 2020Source: Clinical Lymphoma Myeloma and Leukemia, Volume 20, Issue 6Author(s): Carolina Marini, Eolia Brissot, Abdulhamid Bazarbachi, Rémy Duléry, Simona Sestili, Giorgia Battipaglia, Clémence Médiavilla, Annalisa Paviglianiti, Ramdane Belhocine, Francoise Isnard, Simona Lapusan, Rosa Adaeva, Anne Bannet, Zoé van de Wiegert, Anne Vekhoff, Tounes Ledraa, Ollivier Legrand, Myriam Labopin, Agnes Bonnin, Annalisa Ruggeri
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Conclusions. Most change occurred for middle and older age groups, complex manual wheelchairs, and standard power mobility devices. This study supports the need for a separate Medicare category of Complex Rehabilitation Technology for those with severe disabilities. PMID: 32442049 [PubMed - as supplied by publisher]
Source: Assistive Technology - Category: Rehabilitation Tags: Assist Technol Source Type: research
Publication date: August 2020Source: Research in Developmental Disabilities, Volume 103Author(s): Lars Orbach, Moritz Herzog, Annemarie Fritz
Source: Research in Developmental Disabilities - Category: Disability Source Type: research
Publication date: August 2020Source: Research in Developmental Disabilities, Volume 103Author(s): Anna Wallisch, Lauren Little, Evan Dean, Winnie Dunn
Source: Research in Developmental Disabilities - Category: Disability Source Type: research
Authors: Merideth MA, Introne WJ, Wang JA, O'Brien KJ, Huizing M, Gochuico BR Abstract Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by defective biogenesis of lysosome-related organelles. Clinical manifestations include a bleeding diathesis due to a platelet delta storage pool deficiency, oculocutaneous albinism, inflammatory bowel disease, neutropenia, and pulmonary fibrosis. Ten genes associated with HPS are identified to date, and each gene encodes a protein subunit of either Biogenesis of Lysosome-related Organelles Complex (BLOC)-1, BLOC-2, BLOC-3, or the Adaptor Protein...
Source: Platelets - Category: Hematology Tags: Platelets Source Type: research
This article reviews recently published literature focusing on the pattern of malignancy in children with PID, incidence, and risk factors for developing malignancy post-HCT for PID and possible strategies to reduce the risks. EXPERT OPINION: Survival post HCT for PID has improved dramatically in the last 20 years and the genomic revolution has led to an expanding number of indications. To improve long-term quality of life attention needs to focus on late effects, including the possibility of malignancy occurring more frequently than expected in the general population, understand the risks and improve the process ...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
Authors: Dickson A, Manning J Abstract The role of the external clinical advisor is critical to the adjudication of complex claims in the processes of the Accident Compensation Corporation (ACC). This is particularly true of claims for treatment injury that occur during birth, which are often very complicated. In most cases external clinical advisors are non-treating doctors, whose opinion strongly guides the hand of ACC. This viewpoint considers the impact of the role of the external clinical advisor by using extracts from an external clinical advisor's report to show how a power imbalance can be enacted in ACC de...
Source: New Zealand Medical Journal - Category: General Medicine Tags: N Z Med J Source Type: research
In this study, we focused on dorsal mesoderm elongation, one of the critical early development events for trunk formation, and we established in vitro autonomous elongating tissues from human induced pluripotent stem cells (hiPSCs). This artificial tissue elongation is regulated by MYOSIN II and FGF signaling, and is diminished by methylmercury or retinoic acid (RA), similar to in vivo human developmental disabilities. Moreover, our method for differentiation of hiPSCs requires only a short culture period, and the elongation is cell number-independent. Therefore, our in vitro human tissue elongation system is a p...
Source: Chemosphere - Category: Chemistry Authors: Tags: Chemosphere Source Type: research
During idle times your computer can run COVID-19 experiments to help predict effectiveness of chemical compounds in inhibiting functions of viral proteins as a possible treatment for Coronavirus
Source: Disabled World - Category: Disability Tags: Medical Calculators Source Type: news
Authors: Choi JH, Paik WH Abstract Living donor liver transplantation is the most common type of liver transplantation in Asia. Post-transplant biliary stricture is frequent in living donor liver transplantation, and endoscopic management is considered to be the treatment of choice. However, endoscopic management is still challenging in patients who undergo right lobe living donor liver transplantation because of the anatomical alteration. In this article, we reviewed the recently updated results for proper endoscopic biliary drainage in post-living donor liver transplantation anatomical biliary stricture and compa...
Source: Clinical Endoscopy - Category: Gastroenterology Tags: Clin Endosc Source Type: research
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