bims-curels Biomed News
on Leigh syndrome
Issue of 2026–07–26
ten papers selected by
Cure Mito Foundation



  1. J Particip Med. 2026 Jul 24. 18 e93720
       Background: Generative artificial intelligence (GenAI) tools are widely accessible to the public, who are engaging with them for a wide range of health care applications. Existing research has focused predominantly on clinician-facing adoption. Far less is known about how patients and family members use GenAI tools, particularly in rare disease contexts, where diagnostic delay, limited specialist access, and unmet informational needs are common.
    Objective: This study aimed to examine the experiences and opinions of adult patients with rare diseases and parents or guardians of children with rare diseases regarding the use of GenAI tools.
    Methods: Between November 2025 and January 2026, we conducted an exploratory mixed methods web-based survey using convenience sampling through rare disease community organizations in the United States. The survey included closed-ended items assessing prior GenAI use, purposes of use, perceived influence on medical decisions and diagnoses, trust, concerns, communication with clinicians, and experiences of harm, alongside open-text questions capturing qualitative reflections. Descriptive statistics were used to summarize quantitative data. Inductive qualitative analysis was applied to the open-text responses.
    Results: A total of 115 respondents completed the survey. A majority of respondents were parents or guardians of a child with a rare disease (n=74, 64.3%), and the remaining respondents were patients with a rare disease (n=41, 35.7%). Slightly more than half of respondents (n=63, 54.8%) reported prior use of GenAI tools in the context of rare disease. Common purposes included exploring new treatments or clinical trials (n=53, 46.1%), interpreting medical tests or clinical notes (n=37, 32.2%), locating specialists or care centers (n=29, 25.2%), and suggesting possible diagnoses (n=28, 24.3%). Nearly one-third of respondents (n=37, 32%) reported some degree of influence of GenAI on their medical decisions. Nearly 10% (n=12) reported contributions of GenAI to a formal diagnosis. Concern about GenAI accuracy was widespread; 71 of 115 (61.8%) respondents reported moderate to extreme concern. Most respondents (n=90, 78.3%) had not discussed AI-generated information with a clinician. Few respondents (n=7, 6.1%) reported experiencing harm. Qualitative analysis identified 3 themes: (1) GenAI as a practical tool for augmenting patient and caregiver expertise and advocacy, (2) conditional trust and bounded use of GenAI with an emphasis on verification and human oversight, and (3) perceived risks, harms, and structural concerns, including inaccuracies, genetic misinterpretation, and privacy and commercialization issues.
    Conclusions: In this exploratory study, patients and families affected by rare diseases were actively experimenting with GenAI tools to support information seeking, preparation, and advocacy while simultaneously expressing substantial caution and concern about the reliability, safety, and appropriate boundaries of use. Our findings contrast sharply with clinician concerns that patients lack the capacity to use GenAI tools judiciously. Notwithstanding, the sample was skewed toward highly educated participants. Future research should prioritize more representative samples to better capture the range of patient and caregiver experiences with GenAI in rare disease care.
    Keywords:  education; general practice; generative AI; large language models; online survey questionnaire; primary care; qualitative research; training
    DOI:  https://doi.org/10.2196/93720
  2. EClinicalMedicine. 2026 Aug;98 104073
    LifeArc Accelerating Rare Disease Trials (ARDT) centreaf
      There are over 10,000 rare diseases collectively affecting an estimated 250-450 million people globally. While these diseases are rare individually, their cumulative impact on patients, families, healthcare systems, and society is substantial. The incorporation of clinical outcome assessments (COAs) in clinical trials can facilitate patient-focused drug development and treatment evaluation by generating meaningful evidence on how patients feel and function. This work was conducted in three phases: a targeted literature review (searched Aug 2025; updated Feb 2026), a multistakeholder workshop (online, Sept 2025) and, finally, an online survey to ratify final recommendations (responses by March 3, 2026). Of 43 individuals invited, 35 (81%) attended the virtual workshop: 11 researchers (including clinical trialists); 12 patients/caregivers; seven industry experts; four individuals from regulatory agencies and one HTA expert. All were based in the UK or USA. Across three sessions, the workshop explored stakeholder perspectives on considerations and appropriate methodological approaches to COA assessment for rare disease drug development to facilitate the generation of recommendations for future use. A threshold of at least 70% votes was chosen, a priori, for inclusion in the final set of recommendations. Here, we describe the potential benefits of COAs, summarise the key challenges, and provide recommendations to facilitate their effective and consistent integration in drug development for rare diseases.
    Keywords:  Clinical outcome assessment; Clinical trials; PRO; Patient-focused drug development; Patient-reported outcome; Rare diseases
    DOI:  https://doi.org/10.1016/j.eclinm.2026.104073
  3. S D Med. 2025 Nov;78(11): 495-498
      Medical literature suggests that a shift towards shared decision-making has been ongoing for more than 40 years and has coincided with a transition in medicine from paternalism toward greater patient autonomy in decision making. While many definitions of shared decision-making have been developed, most describe a collaborative process where the physician's medical expertise and the patient's values and goals are considered when evaluating and deciding on treatment options. Patient decision aids and other tools, including ethics consultations, may be used to promote the shared decision-making process. Ongoing challenges for physicians include variation among patients concerning the extent to which the patient wants to participate in making decisions, and shifting patient perspectives on their treatment decisions. The use of the internet, social media and the growth of direct to consumer advertising, as well as the rapidly expanding use of artificial intelligence, may create additional challenges to shared decision-making. Finally, challenges may develop if perspectives are misaligned and the patient requests drugs or treatments that conflict with a physician's personal ethics or deeply held beliefs. Shared decision-making is a fluid process requiring an ongoing commitment and engagement with patients.
  4. Ther Adv Rare Dis. 2026 Jan-Dec;7:7 26330040261469197
       Background: Thymidine kinase 2 deficiency (TK2d) is an ultra-rare autosomal recessive mitochondrial disease characterized by progressive myopathy.
    Objectives: To understand patient experiences and the impact of TK2d on patient quality of life (QoL), and to explore support needs.
    Design: A cross-sectional international online survey.
    Methods: The survey for the Assessment of TK2d Patient Perspectives (ATP) study, co-created with patient advocates, included multiple-choice questions, verbal rating scales, and open free-text questions. Patients of all ages with a self-reported genetic diagnosis of TK2d were eligible to participate, either directly or through a caregiver proxy. The patient, caregiver proxy, or bereaved caregiver proxy answered questions on demographics, signs/symptoms, impacts on health-related QoL (HRQoL), support needs, healthcare resource use, and overall experience of living with TK2d. Quantitative data were summarized with descriptive statistics. Qualitative data were analyzed using inductive thematic analysis.
    Results: Responses for 32 patients (24 patient and 8 caregiver proxy responses) were collected between September 2023 and February 2024. All patients experienced myopathic symptoms. The most frequently reported impact of TK2d was on patients' ability to perform basic activities of daily living (26/32), including difficulties in walking (22/32), eating/swallowing (19/32), and breathing (25/32). The proportions of patients reporting a moderate or severe impact of breathing and walking difficulties on HRQoL, and the proportions requiring medical devices, were higher for those with an earlier age of TK2d symptom onset (⩽2, vs >2 to ⩽12, or >12 years). For most patients, TK2d also had a negative impact on mood, social and leisure activities, and employment/education. Progressive loss of abilities, increasing dependency on others, and medical equipment use contributed to mental and emotional burdens.
    Conclusion: These quantitative and qualitative analyses of patients' lived experiences highlight the substantial, progressive, and wide-ranging burden of TK2d.
    Keywords:  burden; patient experience; quality of life; thymidine kinase 2 deficiency
    DOI:  https://doi.org/10.1177/26330040261469197
  5. Hastings Cent Rep. 2026 Jul-Aug;56(4):56(4): 19-24
      For patients who are suffering, the bedside presence of a family member can provide comfort, and many people hold that there is moral value in being present with a conscious, suffering patient. Yet what is the moral significance of the absence of family members when a patient is minimally conscious or unconscious and not aware of their absence? Clinicians are often troubled when family members and surrogate decision-makers who are able to spend a significant amount of time at an unconscious, seriously ill patient's bedside do not do so. Clinicians feel frustrated that they must bear the burden of witnessing the patient's actual or perceived suffering while the family escapes this burden and therefore appears to fail to uphold a duty to the patient. What are we to make of this point of view? What is the source of this frustration? What is the nature of the perceived duty? Is it morally defensible to request or require family members to be present with an unresponsive patient to bear witness to their actual or perceived suffering? If so, on what grounds?
    Keywords:  clinical ethics; doctor‐family relationship; end‐of‐life decision‐making; family duty; suffering; surrogate decision‐making
    DOI:  https://doi.org/10.1002/hast.70037
  6. Arch Physiol Biochem. 2026 Jul 19. 1-29
      Context: Diabetic kidney disease (DKD) presents substantial challenges in early detection and clinical monitoring. Mitochondrial dysfunction plays a pivotal role in DKD pathogenesis, and mitochondrial DNA copy number (mtDNA-CN) reflects this dysfunction. Objective: To evaluate the translational potential of mtDNA-CN as a biomarker for DKD, focusing on its diagnostic, prognostic, and monitoring utility across biological specimens, and examine the critical challenges of its clinical translation. Materials and Methods: We systematically synthesize evidence from cellular experiments, animal models, and clinical studies that assess mtDNA-CN alterations in DKD, with a comparative analysis of specimen types and methodologies. Results: Studies demonstrated that mtDNA-CN in blood and urine are correlated with the onset and progression of DKD. Rigorous standardization must be implemented across specimen collection, pre-analytical processing, analytical testing, detection and data reporting. Conclusion: MtDNA-CN is a promising and measurable biomarker for DKD. Combined with conventional markers, it could enhance clinical utility.
    Keywords:  Diabetic kidney disease; biomarker; clinical translation; mitochondrial DNA copy number
    DOI:  https://doi.org/10.1080/13813455.2026.2702001
  7. Front Digit Health. 2026 ;8 1807607
      Accurate medical coding is essential for disease registries, particularly in the context of rare conditions. Manually transforming electronic health records data into standardized codes is time-consuming and resource intensive. This study evaluates an automated coding system using synthetic health records data and explores the potential benefits and challenges of introducing this tool into rare diseases registries activities. We developed a hybrid architecture combining a symbolic component with medical knowledge graphs and an ensemble of three widely used Large Language Models with a critical review mechanism. Ninety-nine synthetic Italian-language clinical reports were coded by the system. Subsequently, a multidisciplinary expert panel performed a double-coding validation of extracted terms, categorizing automated results into four groups: correct, incorrect, inaccurate, or missing codes. The system extracted a total of 479 terms (264 diagnosis codes and 215 procedure codes) mapped to ICD-9-CM classification. The expert panel, considered as the gold standard, identified 500 terms (302 diagnosis codes and 198 procedure codes). Chi-square analysis highlighted statistically significant differences between diagnosis and procedure coding in at least one of the four groups of results (p=0.001). The system achieved an accuracy of 70.53% for diagnoses, compared to 78.28% for procedures. Additionally, the relative frequency of the various incorrect codes is generally consistent and uniform, except for two incorrect procedure codes that are particularly prevalent. Considering all the findings, we critically point out the potential contribution and impact of an automated coding system into rare diseases registries process, examining the benefits and barriers that could facilitate or hamper progress in this specialized field. The automated coding system demonstrated reasonable accuracy with health records synthetic data. Key challenges include limited ICD-9-CM codes, particularly for rare diseases, overreliance on nonspecific residual codes, and tendency to generate details not present in reports. Opportunities for future improvement may include adopting the ICD-10/ICD-11 classification, implementing reliability metrics and a multi-ontology approach, thus promoting data interoperability according to FAIR principles. An automated coding system, properly improved, may have an essential impact for rare disease registries and are welcomed by several initiatives such as the EHDS.
    Keywords:  ICD; artificial intelligence; automated coding system; disease registry; precision medicine; rare diseases
    DOI:  https://doi.org/10.3389/fdgth.2026.1807607
  8. Soc Sci Med. 2026 Jul 16. pii: S0277-9536(26)00664-7. [Epub ahead of print]405 119588
      Randomised Controlled Trials (RCTs) are considered the "gold standard" for producing medical evidence due to their emphasis on standardisation, control, and scientific rigour. However, staff often face challenges in adhering to protocols, as running trials can generate tensions with caring for participants as individuals. In this article, drawing on Annemarie Mol's concept of the 'logic of care', we explore how care and research are continually negotiated within clinical trials. We conducted a 17-month ethnography at a UK clinical trial centre. The study draws on 330 h of observation of 170 staff and participants to examine how healthcare professionals navigate competing demands while managing hypertension. Our findings show that strict adherence to research protocols was maintained when deemed essential for running the trial, even when doing so generated discomfort or potential harm for participants. However, there were also moments in which staff prioritised participant wellbeing, for example by offering dietary advice, even when such actions risked influencing trial outcomes. Our analysis offers four contributions to this literature. First, it illustrates how the logic of care and the logic of research co-exist within the everyday conduct of trials. Second, our study highlights how the relationship between care and research is not resolved through the prioritisation of one logic over the other, but through ongoing negotiation. Third, our findings show how these tensions are embodied in the role of the clinician-researcher. Fourth, our analysis contributes to broader debates on the ethics of clinical research.
    Keywords:  Clinical trials; Ethnography; Logic of care; Logic of research; Patient care; Randomised controlled trials; Research ethics
    DOI:  https://doi.org/10.1016/j.socscimed.2026.119588
  9. Sleep Adv. 2026 Jul;7(Suppl 1): A19-A24
      The treatment landscape for narcolepsy is rapidly evolving with the introduction of new therapies featuring unique mechanisms of action and innovative formulations of established treatments. The recent approval of a once-nightly sodium oxybate formulation (SO-ER) has expanded the spectrum of oxybate therapies to 3, including the traditional immediate-release formulation (OXB) and a low-sodium version containing calcium, magnesium, potassium, and sodium oxybates (LXB). While the availability of diverse options allows clinicians to tailor oxybate-based treatments, it also raises challenges in selecting and fine-tuning these therapies. Involving patients in the decision-making process through shared decision-making (SDM) platforms can be an effective approach to optimizing oxybate therapy. This review article aims to provide clinicians with a comprehensive overview of the unique characteristics of available oxybate formulations and potential strategies for delivering empathetic care that incorporates SDM into the selection and optimization of oxybate-based treatment regimens.
    Keywords:  Oxybate; cataplexy; narcolepsy; sleepiness
    DOI:  https://doi.org/10.1093/sleepadvances/zpag034