Neurological affection and serum neurofilament light chain in wild type transthyretin amyloidosis.

In contrast to inherited transthyretin amyloidosis (A-ATTRv), neuropathy is not a classic leading symptom of wild type transthyretin amyloidosis (A-ATTRwt). However, neurological symptoms are increasingly relevant in A-ATTRwt as well. To better understand the role of neurological symptoms in A-ATTRwt, A-ATTRwt patients were prospectively characterized at Amyloidosis Center Charité Berlin (ACCB) between 2018 and 2023 using detailed neurological examination, quality of life questionnaires, and analysis of age- and BMI-adapted serum neurofilament light chain (NFL) levels. 16 out of 73 (21.9%) patients presented with a severe neuropathy which we defined by a Neuropathy Impairment Score (NIS) of 20 or more. In this group, quality of life was reduced, peripheral neuropathy was more severe, and spinal stenosis and joint replacements were frequent. Age- and BMI matched serum NFL levels were markedly elevated in patients with a NIS ≥ 20. We therefore conclude that highly abnormal values in neuropathy scores such as the NIS occur in A-ATTRwt, and have an important impact on quality of life. Both peripheral neuropathy and spinal canal stenosis are likely contributors. Serum NFL may serve as a biomarker for neurological affection in patients with A-ATTRwt. It will be important to consider neurological aspects of A-ATTRwt for diagnosis, clinical follow-up, and future treatment development.

Interventions Into Reliability-Seeking Health Care Organizations: A Systematic Review of Their Goals and Measuring Methods.

OBJECTIVES: Within the last 2 decades, numerous interventions making use of high-reliability theory have been implemented to increase reliability in healthcare organizations. This systematic literature review first explores the concrete goals on which such interventions focus. Second, the review captures how the achievement of these goals, or alternatively a change, generally an increase in organizational reliability, is measured across different contexts. METHODS: Searches were conducted in PubMed, Academic Search Ultimate, Business SourcePremier, CINAHL, Communication Source, EconLit, ERIC, Medline, Political Science Complete, PsycArticles, APA PsycInfo, PSYNDEX, SocINDEX (via the resource hosterEbscoHost), and Web of Science (through November 22, 2021). Peer-reviewed, English language studies were included, reporting on the implementation of a concrete intervention to increase reliability in a medical context and referring to high-reliability theory. RESULTS: The search first yielded 8896 references, from which 75 studies were included in the final sample. Important healthcare goals stated by the seminal report "Crossing the Quality Chasm" guided the analysis of the included studies. Most of the studies originated from the United States and report on interventions to increase reliability of either organizational units or whole organizations when aiming for safety (n = 65). Other goals reported on include effectiveness, and much less frequently timeliness, patient centeredness, and efficiency. Fifty-eight studies use quantitative measurement exclusively to account for the achievement of these goals; 7 studies use qualitative measurement exclusively, and 10 studies use a mixed-method approach. The operationalization of goals, including the operationalization of organizational reliability, and measurement methods do not follow a unified approach, despite claiming to be informed by a coherent theory. Instead, such operationalizations strongly depend on the overall objective of the study and the respective context. CONCLUSIONS: Measuring the outcomes of high-reliability interventions into healthcare organizations is challenging for different reasons, including the difficult operationalization of partly overlapping goals, the complex, processual nature of achieving reliability, and the limited ability of organizations striving for more reliability if they are already performing on a high level. This review critically assesses the adoption of the goals stated in the seminal report "Crossing the Quality Chasm" and provides insights for organizations and funding providers that strive to evaluate more reliable service provision.