Cardiac [^99mTc]Tc-hydroxydiphosphonate uptake on bone scintigraphy in patients with hereditary transthyretin amyloidosis:an early follow-up marker?

Purpose: There is a need for early quantitative markers of potential treatment response in patients with hereditary transthyretin (ATTRv) amyloidosis to guide therapy. This study aims to evaluate changes in cardiac tracer uptake on bone scintigraphy in ATTRv amyloidosis patients on different treatments. Methods: In this retrospective cohort study, outcomes of 20 patients treated with the transthyretin (TTR) gene silencer patisiran were compared to 12 patients treated with a TTR-stabilizer. Changes in NYHA class, cardiac biomarkers in serum, wall thickness, and diastolic parameters on echocardiography and NYHA class during treatment were evaluated. Results: Median heart/whole-body (H/WB) ratio on bone scintigraphy decreased from 4.84 [4.00 to 5.31] to 4.16 [3.66 to 4.81] (p &lt;.001) in patients treated with patisiran for 29 [15–34] months. No changes in the other follow-up parameters were observed. In patients treated with a TTR-stabilizer for 24 [20 to 30] months, H/WB ratio increased from 4.46 [3.24 to 5.13] to 4.96 [3.39 to 5.80] (p =.010), and troponin T increased from 19.5 [9.3 to 34.0] ng/L to 20.0 [11.8 to 47.8] ng/L (p =.025). All other parameters did not change during treatment with a TTR-stabilizer. Conclusion: A change in cardiac tracer uptake on bone scintigraphy may be an early marker of treatment-specific response or disease progression in ATTRv amyloidosis patients.</p

Pharmacotherapy-Based Problems in the Management of Diabetes Mellitus: Needs Much More to be Done!

A total of 856 diabetic patients were evaluated for pharmacotherapy-based problems like for possible drug interactions, adverse drug reactions, and other mismatches, if any. Poor correlation between the advised insulin therapy and patients’ fasting blood glucose levels (12%, n=103) was observed. To most of the patients (41.66%, n= 357), insulin therapy was advised in combination with glucocorticoides, thiazides diuretics, and propranolol. Prescribing beta blocker (propranolol) with insulin is contraindicated. The higher incidence of diabetic foot patients was in the mean age of 57±3.4 years that was controlled with combination therapy of insulin and oral antidiabetics (63.0%, n=516). 11.1% of the treated patients could not take the prescribed therapy due to poor acceptance of insulin therapy due to its syringe needle prick. 41.66% risks of potential drug interactions, 7.93% adverse drug reactions, and 6.6% mismatches were recorded, as per the international approved algorithm, for managing a diabetes mellitus that reflects poor health care system. All these events necessitate for coordinating with other health professionals to make the therapy safer in the better interest of the patients. It is concluded that in practice prescribing pattern carries more risks for patients. It is imperative to improve the practice of pharmacotherapeutics rather than to practice in routine

FIDE report: Dutch Contribution Topic III

Hervorming Sociale Regelgevin

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a delayed, potentially life-threatening, hypersensitivity reaction characterized by a widespread, long-lasting skin eruption, fever, lymphadenopathy, hematological abnormalities, and organ involvement. Time to onset and course are relatively long; relapses may occur. Clinical and biological variability make DRESS a challenging diagnosis. Pathogenesis is not exactly known, but probably reflects a complex interplay of drug and viral-related factors in which genetics and abnormal metabolic pathways of drugs play an important role. Although associated with many drugs, DRESS is mainly observed after a limited number of “high risk” drugs. Early recognition, prompt withdrawal of the culprit, and treatment with corticosteroids are the mainstay of management.</p

Cardiac [99mTc]Tc-hydroxydiphosphonate uptake on bone scintigraphy in patients with hereditary transthyretin amyloidosis: an early follow-up marker?

Purpose: There is a need for early quantitative markers of potential treatment response in patients with hereditary transthyretin (ATTRv) amyloidosis to guide therapy. This study aims to evaluate changes in cardiac tracer uptake on bone scintigraphy in ATTRv amyloidosis patients on different treatments. Methods: In this retrospective cohort study, outcomes of 20 patients treated with the transthyretin (TTR) gene silencer patisiran were compared to 12 patients treated with a TTR-stabilizer. Changes in NYHA class, cardiac biomarkers in serum, wall thickness, and diastolic parameters on echocardiography and NYHA class during treatment were evaluated. Results: Median heart/whole-body (H/WB) ratio on bone scintigraphy decreased from 4.84 [4.00 to 5.31] to 4.16 [3.66 to 4.81] (p <.001) in patients treated with patisiran for 29 [15–34] months. No changes in the other follow-up parameters were observed. In patients treated with a TTR-stabilizer for 24 [20 to 30] months, H/WB ratio increased from 4.46 [3.24 to 5.13] to 4.96 [3.39 to 5.80] (p =.010), and troponin T increased from 19.5 [9.3 to 34.0] ng/L to 20.0 [11.8 to 47.8] ng/L (p =.025). All other parameters did not change during treatment with a TTR-stabilizer. Conclusion: A change in cardiac tracer uptake on bone scintigraphy may be an early marker of treatment-specific response or disease progression in ATTRv amyloidosis patients

Megakaryocytes possess a functional intrinsic apoptosis pathway that must be restrained to survive and produce platelets

Deletion of Bak and Bax, the effectors of mitochondrial apoptosis, does not affect platelet production, however, loss of prosurvival Bcl-xL results in megakaryocyte apoptosis and failure of platelet shedding

Services provision and temporary mobility: freedoms and regulation in the EU

International posting of workers and mobility of self-employed service suppliers lie between outright migration and trade in goods: their regulation, for both distributional and marketcorrecting purposes, is not as difficult to harmonize as that of labour markets, but personal mobility is more visible and socially intrusive than product market interactions. This paper analyzes economic and legal tensions between national regulatory frameworks and international competition in these areas, in both the intra-EU and global contexts, highlighting how interactions between the external and internal roles of the European Commission may foster efficient integration of markets and policies in this and other fields