Exploring the Gut Microbiota-Muscle Axis in Duchenne Muscular Dystrophy.

The gut microbiota plays a pivotal role in maintaining the dynamic balance of intestinal epithelial and immune cells, crucial for overall organ homeostasis. Dysfunctions in these intricate relationships can lead to inflammation and contribute to the pathogenesis of various diseases. Recent findings uncovered the existence of a gut-muscle axis, revealing how alterations in the gut microbiota can disrupt regulatory mechanisms in muscular and adipose tissues, triggering immune-mediated inflammation. In the context of Duchenne muscular dystrophy (DMD), alterations in intestinal permeability stand as a potential origin of molecules that could trigger muscle degeneration via various pathways. Metabolites produced by gut bacteria, or fragments of bacteria themselves, may have the ability to migrate from the gut into the bloodstream and ultimately infiltrate distant muscle tissues, exacerbating localized pathologies. These insights highlight alternative pathological pathways in DMD beyond the musculoskeletal system, paving the way for nutraceutical supplementation as a potential adjuvant therapy. Understanding the complex interplay between the gut microbiota, immune system, and muscular health offers new perspectives for therapeutic interventions beyond conventional approaches to efficiently counteract the multifaceted nature of DMD.

[Pathogenetic role of intestinal microbiocenosis in the pathogenesis of hereditary myodystrophy]. / Patogeneticheskaia rol' kishechnogo mikrobiotsenoza v klinike nasledstvennykh miodistrofii.

The effect of the inclusion of probiotic preparations for the correction of disturbances in normal intestinal microflora into the complex therapy of patients wish Duchenne's childhood muscular dystrophy and Becker's myopathy was analyzed. Probiotic therapy made it possible to improve the clinical state of patients, manifested by an increase in muscular strength and accompanied by positive shifts in electromyographic, immunological, biochemical, hormonal characteristics. Intestinal microbiocenosis plays seemingly a certain role in the formation of hereditary pathology.

Histochemistry and morphometric analysis of muscle fibers from patients with duchenne muscular dystrophy (DMD) / Histoquímica y análisis morfométrico de las fibras musculares de p0acientes con distrofia muscular de duchenne (DMD)

The aim of the study was to analyze the muscle fibers by histochemistry and morphometric methods from patients with Duchenne muscular dystrophy (DMD). Muscle biopsies were taken from the vastus lateralis muscle of five boys between 13 and 15-years of age, with clinical diagnosis of DMD. The histochemistry was performed using myofibrillar ATPases (9.6, 4.6 and 4.3). To morphometrical analysis a computerized semiautomatic system and software Image-Lab was used. ATPase staining showed atrophy of muscle fibers. Fibrosis and adipose deposition occurred in variable degree depending of muscular involvement. The morphometrical analysis showed an increase of size (percentage) to type I fiber than other types in all patients. Furthermore, the type I fiber had a larger cross-sectional area and mean diameter than type IIa and IIb fibers. Both histochemistry and morphometric analysis could be important tools for qualitative and quantitative diagnostics of muscle fibers attacked in this type of disease.

El objetivo del estudio fue analizar las fibras musculares mediante histoquímica y métodos morfométricos en pacientes con distrofia muscular de Duchenne (DMD). Se tomaron biopsias musculares del músculo vasto lateral de cinco niños entre 13 y 15 años de edad, con diagnóstico clínico de DMD. La histoquímica se realizó mediante ATPasa miofibrilar (9.6, 4.6 y 4.3). Para el análisis morfométrico se utilizó un sistema semiautomático computarizado y software de imagen de laboratorio. La tinción de ATPasa mostró una atrofia de las fibras musculares. La fibrosis y depósito adiposo se observó en grado variable dependiendo del compromiso muscular. El análisis morfométrico mostró un aumento de tamaño (porcentaje) de fibras tipo I en todos los pacientes. Además, la fibra tipo I tuvo un área de sección transversal y diámetro medio mayor que las fibras tipos IIa y IIb. Tanto la histoquímica y el análisis morfométrico pueden ser herramientas importantes para el diagnóstico cualitativo y cuantitativo de las fibras musculares comprometidas en este tipo de enfermedad.