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Establishment of normal myofiber size distribution in children and young adults
Michael W Lawlor1,2, Marta Margeta3, Karra A Jones4
1Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, United States.
Insights
Establishing normal myofiber size ranges in children is crucial for diagnosing muscle disorders. This study provides essential pediatric skeletal muscle fiber size standards for accurate clinical diagnosis.
Area of Science:
- Pediatric Pathology
- Skeletal Muscle Histology
- Diagnostic Standards
Background:
- Myofiber size abnormalities are key indicators in skeletal muscle biopsies, particularly when fibers are small.
- Assessing pediatric myofiber size has been challenging due to limitations in tools for analyzing numerous samples.
- Accurate reference ranges are needed for diagnosing pediatric neuromuscular conditions.
Purpose of the Study:
- To establish normative ranges for myofiber size in children and young adults.
- To provide a standardized dataset for diagnostic and research purposes in pediatric muscle pathology.
- To facilitate the identification of abnormal myofiber sizes in clinical practice.
Main Methods:
- Analysis of 349 normal pediatric skeletal muscle biopsy specimens (4 weeks to 25 years).
- Histological assessment using H&E staining and minFeret diameter measurement via Visiopharm software.
- Grouping data into 18 age cohorts to define sex-specific normal ranges.
Main Results:
- Established normative ranges for myofiber size (minFeret diameter) across 18 pediatric age cohorts.
- Demonstrated the utility of these ranges through a pilot study comparing normal and abnormal cases.
- Provided a user-friendly dataset for diagnostic application.
Conclusions:
- The study successfully established reliable, age- and sex-specific reference ranges for pediatric myofiber size.
- These standards aid in the accurate diagnosis of conditions involving abnormal myofiber dimensions.
- This dataset serves as a valuable tool for pediatric muscle pathology research and clinical evaluation.
Abstract:
Abnormalities of myofiber size are often of diagnostic significance on skeletal muscle biopsies, mainly when myofibers are excessively small. The establishment of standards for myofiber size in children has been hampered until recently by the lack of tools to assess large numbers of fibers across a sizeable number of samples. This study describes the assessment of myofiber size in 349 histologically normal patient biopsy specimens obtained between 4 weeks and 25 years of life and corresponding primarily to locations in the thigh/quadriceps/vastus lateralis region. Biopsy specimens were selected for inclusion based on histologically normal light microscopic findings and minimal technical artifacts. H&E-stained slides were scanned and evaluated for minFeret diameter using a Visiopharm software app (APP #10164). MinFeret diameter fiber size data were then grouped into 18 age cohorts to establish normal ranges for males and females within each age cohort. A pilot study to compare known abnormal cases to these normal ranges was then performed to demonstrate how cases with abnormal fiber size might compare to these standards. This dataset provides a user-friendly and applicable set of standard fiber size ranges to assist in diagnostic and scientific work in children and young adults.
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