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Transthyretin amyloidosis (ATTR) is a severe and rare disease characterized by the progressive deposition of misfolded transthyretin proteins, causing irreversible organ damage. Transthyretin amyloidosis can present as a hereditary ATTR or acquired wild-type ATTR form. Genetic testing is critical for determining a hereditary predisposition and subsequently initiating appropriate family screening. In France, strict regulations govern genetic testing that aim to protect patients and their families affected by hereditary diseases such as ATTR. However, challenges persist in establishing an effective genetic testing pathway. A multidisciplinary group of French experts convened to discuss the challenges associated with an ATTR genetic diagnosis and to propose improvement strategies. Key challenges include the lack of pathway standardization, communication gaps between healthcare professionals (HCPs) and patients, and difficulties in complying with regulatory requirements. Concerns about patient data safety and outsourced testing quality further complicate matters. Proposed strategies included the development of stakeholder mapping tools for HCPs and patients, educational programs to improve literacy on genetic testing regulations, increase disease awareness among medical geneticists and genetic counselors, and strengthening HCP-patient communication through educational materials. These initiatives aim to streamline the genetic testing pathway, enhance compliance with regulations, and ultimately provide optimal support for patients and families with ATTR.
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http://dx.doi.org/10.1186/s13023-024-03370-z | DOI Listing |
Clin Infect Dis
September 2025
Section of Epidemiology, Alaska Division of Public Health, Anchorage, Alaska, USA.
Background: Borealpox virus (previously known as Alaskapox virus) is an Orthopoxvirus species first identified in a patient living near Fairbanks, Alaska, in 2015; the source of the patient's infection was unknown. Six additional borealpox cases have been identified through 2023.
Methods: We conducted interviews to ascertain travel history and potential exposures for the six patients, trapped small mammals for orthopoxvirus testing, and performed a phylogenetic analysis of viral DNA sequences.
Microbiol Spectr
September 2025
Department of Clinical Microbiology, Hospital Clínic of Barcelona-ISGlobal, University of Barcelona, Barcelona, Spain.
Unlabelled: Accurate methods to assess viral viability are crucial for determining isolation duration and antiviral therapy in immunocompromised patients. Although cell culture (CC) is the gold standard, it has limitations. Cycle threshold (Ct) values from genomic RNA (gRNA) RT-PCR and subgenomic RNA (sgRNA) RT-PCR have been proposed as markers of active viral replication.
View Article and Find Full Text PDFBackground: Thiopurine S-methyltransferase (TPMT) is crucial for metabolising thiopurine drugs. This study aimed to establish the cutoff values for TPMT activity in a cohort of healthy individuals. We defined normal TPMT activity ranges and identified clinically applicable thresholds to distinguish individuals with normal TPMT function from those with reduced or deficient activity.
View Article and Find Full Text PDFGenet Med Open
July 2025
Faculty of Biology Medicine and Health, University of Manchester, United Kingdom.
Purpose: Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disorder. This study aimed to analyze the genotype distribution of FCS-causing genes in the United Kingdom.
Methods: Data were anonymously collated from 2 genetic testing laboratories providing national genetic diagnosis services for severe hypertriglyceridemia in the United Kingdom.
Transl Neurosci
January 2025
The Affiliated Brain Hospital, Guangzhou Medical University, No. 36, Ming Xin Road, Guangzhou, Guangdong, P. R. China.
Background: Multiple sclerosis (MS) is a major demyelinating disorder that affects the central nervous system. A growing body of evidence has revealed the involvement of coagulation pathway in the pathogenesis of MS. However, the causal association between coagulation factors and MS is still unclear.
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