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Batten disease

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Search Period All yearsLast 12 monthsLast 5 years Topic All topicsGeneralNutrition and dietAlternative treatment methods Type of research Any type of researchStrongest evidence only (29)Meta-analysis (6)Treatment guideline (3)Systematic review (10)Randomized trial (9)Phase 3 study (1)Clinical trial (58)Review article (128)Early study (phase 1 or 2) (21)Case report (105)Laboratory or animal research (71)Commentary or editorial (35) Studies currently recruiting participants only (24)

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Publications and studies (974)

• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. CLN8 Mutations Presenting with a Phenotypic Continuum of Neuronal Ceroid Lipofuscinosis-Literature Review and Case Report. (2021/06/23) ♡
• Corrigendum: Cathepsin D Variants Associated With Neurodegenerative Diseases Show Dysregulated Functionality and Modified α-Synuclein Degradation Properties. (2021/06/23) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. A Typology of Existing Machine Learning-Based Predictive Analytic Tools Focused on Reducing Costs and Improving Quality in Health Care: Systematic Search and Content Analysis. (2021/06/22) ♡
• Recognizing differentiating clinical signs of CLN3 disease (Batten disease) at presentation. (2021/06/01) ♡
• An Exploratory Study of Goals of Care Conversations Initiated with Seriously Ill Veterans in the Emergency Room. (2021/06/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Evaluating and modulating TFEB in the control of autophagy: toward new treatments in CNS disorders. (2021/06/01) ♡
• A novel deletion variant in CLN3 with highly variable expressivity is responsible for juvenile neuronal ceroid lipofuscinoses. (2021/06/01) ♡
• Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease. (2021/06/01) ♡
• Juvenile Batten disease: A diagnostic conundrum. (2021/06/01) ♡
• Electroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses. (2021/05/30) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Inborn Errors of Metabolism Associated With Autism Spectrum Disorders: Approaches to Intervention. (2021/05/28) ♡
• Pearls & Oy-sters: Levodopa-Responsive Adult NCL (Type B Kufs Disease) Due to CLN6 Mutation. (2021/05/25) ♡
• Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Knockout of the CMP-Sialic Acid Transporter SLC35A1 in Human Cell Lines Increases Transduction Efficiency of Adeno-Associated Virus 9: Implications for Gene Therapy Potency Assays. (2021/05/19) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa. (2021/05/14) ♡
• Investigating health-related quality of life in rare diseases: a case study in utility value determination for patients with CLN2 disease (neuronal ceroid lipofuscinosis type 2). (2021/05/12) ♡
• Cerliponase Alfa for the Treatment of Atypical Phenotypes of CLN2 Disease: A Retrospective Case Series. (2021/05/01) ♡
• Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome. (2021/05/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. A lysosomal enigma CLN5 and its significance in understanding neuronal ceroid lipofuscinosis. (2021/05/01) ♡
• Generation of pathogenic TPP1 mutations in human stem cells as a model for neuronal ceroid lipofuscinosis type 2 disease. (2021/05/01) ♡
• Low Serum Albumin Predicts Severe Outcomes in COVID-19 Infection: A Single-Center Retrospective Case-Control Study. (2021/05/01) ♡
• Systematic reviewiAll research on one question is searched according to fixed rules and compared side by side, so that no studies with poor outcomes are missed. Guidelines on the diagnosis, clinical assessments, treatment and management for CLN2 disease patients. (2021/04/21) ♡
• Investigation of type I interferon responses in ANCA-associated vasculitis. (2021/04/15) ♡
• Neurofilament light chain levels correlate with clinical measures in CLN3 disease. (2021/04/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Macula as a Window to Diagnosis. (2021/04/01) ♡
• Revealing the clinical phenotype of atypical neuronal ceroid lipofuscinosis type 2 disease: Insights from the largest cohort in the world. (2021/04/01) ♡
• Clinical trialiResearch in patients, without randomization between groups. Useful, but less certain than a randomized trial. Physical activity, mental health and well-being of adults during initial COVID-19 containment strategies: A multi-country cross-sectional analysis. (2021/04/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Status dystonicus associated with CLN8 disease. (2021/04/01) ♡
• GFAP hyperpalmitoylation exacerbates astrogliosis and neurodegenerative pathology in PPT1-deficient mice. (2021/03/30) ♡
• Rapid and Progressive Loss of Multiple Retinal Cell Types in Cathepsin D-Deficient Mice-An Animal Model of CLN10 Disease. (2021/03/21) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. The converging roles of Batten disease proteins in neurodegeneration and cancer. (2021/03/19) ♡
• Evaluation of genetic diversity and management of disease in Border Collie dogs. (2021/03/18) ♡
• Global network analysis in Schizosaccharomyces pombe reveals three distinct consequences of the common 1-kb deletion causing juvenile CLN3 disease. (2021/03/18) ♡
• Exploring dementia and neuronal ceroid lipofuscinosis genes in 100 FTD-like patients from 6 towns and rural villages on the Adriatic Sea cost of Apulia. (2021/03/18) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Successful treatment of cannabinoid administration against refractory epilepsy in Batten disease: a case report. (2021/03/01) ♡
• Re-emergence of BTV serotype 4 in North Macedonia, July 2020. (2021/03/01) ♡
• Early-stage study (phase 1 or 2)iEarly research in a small group, primarily aimed at safety and dosage. Whether it actually works still needs to be determined afterward. Clinical Pharmacokinetics and Pharmacodynamics of Cerliponase Alfa, Enzyme Replacement Therapy for CLN2 Disease by Intracerebroventricular Administration. (2021/03/01) ♡
• Neuronal ceroid lipofuscinosis: genetic and phenotypic spectrum of 14 patients from Turkey. (2021/03/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. A case of neuronal ceroid lipofuscinosis type 8 associated with central precocious puberty. (2021/03/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Hyperlipofuscinosis With Subretinal Fibrosis and Choroidal Vascular Remodeling in Stargardt Disease. (2021/03/01) ♡
• Neuronal Ceroid Lipofuscinoses in Children. (2021/03/01) ♡
• Spinal manifestations of CLN1 disease start during the early postnatal period. (2021/02/01) ♡
• Family Caregiver Support of Patient Self-Management During Chronic, Life-Limiting Illness: A Qualitative Metasynthesis. (2021/02/01) ♡
• Randomized researchiParticipants were divided into groups by lottery and compared with each other. This reduces the chance that a difference is due to something other than the treatment. T cell and antibody responses induced by a single dose of ChAdOx1 nCoV-19 (AZD1222) vaccine in a phase 1/2 clinical trial. (2021/02/01) ♡
• Automatic quantification of lymphocyte vacuolization in peripheral blood smears of patients with Batten's disease (CLN3 disease). (2021/01/25) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Paroxysmal sympathetic hyperactivity following status epilepticus in a 22-year-old with Juvenile Neuronal Ceroid Lipofuscinosis: A case report. (2021/01/19) ♡
• Randomized researchiParticipants were divided into groups by lottery and compared with each other. This reduces the chance that a difference is due to something other than the treatment. Safety and efficacy of the ChAdOx1 nCoV-19 vaccine (AZD1222) against SARS-CoV-2: an interim analysis of four randomised controlled trials in Brazil, South Africa, and the UK. (2021/01/09) ♡
• Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. AAV9 Gene Therapy Increases Lifespan and Treats Pathological and Behavioral Abnormalities in a Mouse Model of CLN8-Batten Disease. (2021/01/06) ♡
• Comparative analysis of the gut microbiota composition in the Cln1(R151X) and Cln2(R207X) mouse models of Batten disease and in three wild-type mouse strains. (2021/01/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Autosomal dominant neuronal ceroid lipofuscinosis: Clinical features and molecular basis. (2021/01/01) ♡
• Clinical trialiResearch in patients, without randomization between groups. Useful, but less certain than a randomized trial. Better characterization of operation for ulcerative colitis through the National surgical quality improvement program: A 2-year audit of NSQIP-IBD. (2021/01/01) ♡

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