ArticleThe Journal of pediatrics2015
Responses to Fasting and Glucose Loading in a Cohort of Well Children with Spinal Muscular Atrophy Type II.
Article in The Journal of pediatrics, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
What it found
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed.
- Survival Motor Neuron Protein Requirement-Supply Mismatch in Spinal Muscular Atrophy: A Conceptual Framework.Advances in therapy · 2026Review
- Spinal muscular atrophy: Biology, pathogenesis, and therapeutic advances.Therapeutic advances in neurological disorders · 2026Review
- Comorbidities in spinal muscular atrophy and their impact on the course of the underlying disease: a real-life observational study.BMC musculoskeletal disorders · 2025Observational
- Adiponectin and Leptin-Considerations in Adult Patients with Spinal Muscular Atrophy Type 3.Diagnostics (Basel, Switzerland) · 2025Article
- Peripheral defects precede neuromuscular pathology in theJournal of neuromuscular diseases · 2024Article
- Glucose and Lipid Metabolism Disorders in Adults with Spinal Muscular Atrophy Type 3.Diagnostics (Basel, Switzerland) · 2024Article
- Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy.Human molecular genetics · 2024Article
- Mitochondrial Dysfunction in Spinal Muscular Atrophy.International journal of molecular sciences · 2022Review
- Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy.Gene therapy · 2022Article
- The Role of Sphingomyelin and Ceramide in Motor Neuron Diseases.Journal of personalized medicine · 2022Review
- Metabolic Dysfunction in Spinal Muscular Atrophy.International journal of molecular sciences · 2021Review
- Revisiting the role of mitochondria in spinal muscular atrophy.Cellular and molecular life sciences : CMLS · 2021Review
- Spinal muscular atrophy: Broad disease spectrum and sex-specific phenotypes.Biochimica et biophysica acta. Molecular basis of disease · 2021Review
- The Relationship between Body Composition, Fatty Acid Metabolism and Diet in Spinal Muscular Atrophy.Brain sciences · 2021Review
- Review
- Discovery of a CNS penetrant small molecule SMN2 splicing modulator with improved tolerability for spinal muscular atrophy.Scientific reports · 2020Article
- Muscle regulates mTOR dependent axonal local translation in motor neurons via CTRP3 secretion: implications for a neuromuscular disorder, spinal muscular atrophy.Acta neuropathologica communications · 2019Article
- Abnormal fatty acid metabolism is a core component of spinal muscular atrophy.Annals of clinical and translational neurology · 2019Article
- Low-Intensity Running and High-Intensity Swimming Exercises Differentially Improve Energy Metabolism in Mice With Mild Spinal Muscular Atrophy.Frontiers in physiology · 2019Article
- Light modulation ameliorates expression of circadian genes and disease progression in spinal muscular atrophy mice.Human molecular genetics · 2018Article
Corrections and comments
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Authors and funding
4 authors.
Funding
Abstract
objectiveTo examine the impact of fasting and glucose tolerance on selected metabolic variables in children with spinal muscular atrophy (SMA) type II in a well state, secondary to reports of glucose regulation abnormalities in SMA. STUDY
designIn this prospective pilot study, 6 children aged 7-11 years with SMA type II participated in an oral glucose tolerance test and a supervised medical fast during 2 overnight visits at the University of Utah. At baseline, a dual-energy x-ray absorptiometry scan was performed to determine body composition. Laboratory test results were obtained at baseline and in response to the respective interventions. Data analysis was descriptive. Prefasting and postfasting data were evaluated using the Wilcoxon signed-rank test.
resultsBased on the dual-energy x-ray absorptiometry scan, all 6 children were variably obese at baseline. All 6 exhibited hyperinsulinemia, and 3 of 6 met formal American Diabetes Association criteria for impaired glucose tolerance. According to homeostatic insulin resistance calculations, 5 of the 6 participants were insulin-resistant. All 6 participants tolerated a monitored fast for 20 hours without hypoglycemia (blood glucose <54 mg/dL). Free fatty acid levels increased significantly from prefasting to postfasting, whereas levels of several plasma amino acids decreased significantly during fasting.
conclusionChildren with SMA type II defined as obese using objective variables are at increased risk for impaired glucose tolerance regardless of whether or not they visually appear obese. Further studies are needed to determine the prevalence of impaired glucose tolerance and tolerance for fasting within the broader heterogeneous SMA population and to develop appropriate guidelines for intervention.
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