bims-tyki2d Biomed News
on Thymidine kinase 2 deficiency
Issue of 2026–09–13
five papers selected by
Zoya Panahloo, UCB



  1. Ther Adv Rare Dis. 2026 Jan-Dec;7:7 26330040261478640
      The inaugural United Mitochondrial Disease Foundation (UMDF) Mitochondrial Medicine 2025 Masterclass focused on primary mitochondrial diseases (PMDs) especially primary mitochondrial myopathies (PMMs) and thymidine kinase 2 deficiency (TK2d). The Masterclass featured leading US experts in the field, providing the latest scientific and clinical knowledge, as well as patients and caregivers sharing their lived experience of mitochondrial diseases. In this report, we summarize the key highlights of each presentation. An overview of PMM featuring the etiologies of different PMMs and key features of notable PMMs, was followed by a presentation discussing the practical clinical processes of diagnosing PMM, how the roles of clinicians have evolved as diagnostic technology has improved, and actions clinicians can take to maximize the chances of an early, accurate diagnosis. Real-world case studies highlighted variations in disease presentation among the wide range of PMMs, which was followed by an in-depth review of clinical assessments and symptom management for PMM across organ systems. Multisystemic disorders like PMM require multidisciplinary management, in both a chronic and acute setting, and two experts discussed the practical workflow for clinicians to build a multidisciplinary model of care at their hospitals, the roles and responsibilities of the lead coordinator and each subspecialist, and practical steps that clinicians can take to manage acute care coordination and decrease acute care utilization. A hypothetical case study of TK2d (based on real patients) brought together the different aspects of PMM discussed during the Masterclass, while the patient perspective presentations allowed patients and caregivers to discuss the real-world impact that the diagnostic and care journey had on them and their families. This Educational Masterclass provided detailed knowledge of PMM designed to provide the next generation of clinicians and investigators with practical, actionable guidance and resources they could utilize to make a difference in the lives of patients with PMM.
    Keywords:  TK2d; mitochondrial disease; mitochondrial myopathy
    DOI:  https://doi.org/10.1177/26330040261478640
  2. Drug Discov Today. 2026 Sep 08. pii: S1359-6446(26)00204-7. [Epub ahead of print] 104799
      
    DOI:  https://doi.org/10.1016/j.drudis.2026.104799
  3. Neurotherapeutics. 2026 Sep 10. pii: S1878-7479(26)00242-4. [Epub ahead of print] e01072
      
    Keywords:  Deoxycytidine; Deoxynucleoside; Deoxythymidine; POLG; Pyrimidine; TK2
    DOI:  https://doi.org/10.1016/j.neurot.2026.e01072
  4. Ther Adv Rare Dis. 2026 Jan-Dec;7:7 26330040261486869
      Rare diseases collectively affect approximately 6% of the global population, yet 95% of affected individuals lack access to effective treatments. Beyond this persistent therapeutic gap, patients and caregivers face a broad spectrum of unmet needs, spanning psychosocial challenges, access to reliable diagnostic information, care coordination, and long-term follow-up, which remain poorly characterised in both clinical and policy contexts. Three interrelated challenges underpin this problem. First, validated instruments capable of systematically capturing unmet needs across diverse patient groups, including adults, minors, and caregivers, are largely absent. Second, even when patient data are collected, they are rarely integrated into clinical trial design, regulatory evaluation, or reimbursement deliberations in a structured and reproducible manner. Third, the responsible governance of rare disease data, particularly across institutional and national boundaries, faces unresolved ethical, legal, and organisational barriers. This perspective paper outlines the conceptual foundations of the INFORM-RD research project (a patient-informed clinical platform to inform patient-centred decision-making for rare diseases), embedded at KU Leuven, Leuven Institute for Rare Diseases and University Hospitals Leuven, Belgium. Drawing on the KCE NEED framework and leveraging a cohort of over 30,000 people with rare diseases, INFORM-RD aims to: 1) develop and validate a scalable methodology for unmet need data collection, 2) translate these data into actionable decision-support tools for clinicians, regulators, and payers, and 3) establish an ethical and legal governance architecture enabling responsible and scalable data sharing at national and international levels. By positioning patients, including underrepresented groups such as children and persons with cognitive limitations, as co-creators throughout the research process, INFORM-RD offers a transferable blueprint for need-driven rare disease care across Europe.
    Keywords:  clinical and regulatory decision-making; healthcare policy and regulation; patient-centred care; rare disease data governance; rare diseases; unmet needs
    DOI:  https://doi.org/10.1177/26330040261486869
  5. BMJ Digit Health Ai. 2026 ;2(1): e000104
      Although artificial intelligence (AI) shows considerable promise in healthcare, its introduction and safe and effective exploitation involve several challenges. We argue that to maximise the benefits of AI and understand, track and mitigate potential harms there must be a shift in how AI is evaluated. Evaluation should be more closely linked to the local contexts in which AI is deployed and used and better integrated with clinical practice. To support this shift, we propose three interrelated levels of evaluation: evaluation of implementation, which examines transformations in contextually situated practices; evaluation of optimisation, which explores changes to clinical pathways over extended periods; and evaluation of scaling, which focuses on coordination through regional and national structures. This approach offers significant opportunities to advance both healthcare practice and the epistemology of medical knowledge.
    Keywords:  Artificial intelligence; Evidence-Based Medicine; Health Information Systems
    DOI:  https://doi.org/10.1136/bmjdh-2026-000104