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Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
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The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
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Juvenile Scleroderma.

Aruna Bhat1

  • 1Department of Pediatric Rheumatology, Narayana Health City, Bangalore, Karnataka, India. hegdearuna@hotmail.com.

Indian Journal of Pediatrics
|November 15, 2023
PubMed
Summary

Juvenile Scleroderma, encompassing localized scleroderma and systemic sclerosis in children, requires early diagnosis and effective management. Prompt identification and treatment are crucial for improving quality of life and survival rates in affected children.

Keywords:
Digital ulcersJuvenile sclerodermaNail-fold capillaroscopyRaynaud’s phenomenonSystemic sclerosis

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Area of Science:

  • Pediatric rheumatology
  • Dermatology
  • Fibrosing disorders

Background:

  • Juvenile Scleroderma includes localized scleroderma and systemic sclerosis, rare chronic fibrosing conditions in children.
  • While histopathology is similar, systemic sclerosis presents with distinct vasculopathy, organ involvement, and higher morbidity/mortality.
  • Raynaud's phenomenon and digital ulcers are key indicators of systemic sclerosis, significantly impacting patients' quality of life.

Purpose of the Study:

  • To highlight the importance of early identification and management of Juvenile Scleroderma.
  • To discuss the distinct clinical features and outcomes of localized scleroderma versus systemic sclerosis in children.
  • To emphasize the role of emerging treatments and research in improving patient survival and well-being.

Main Methods:

  • Review of clinical characteristics and histopathological findings.
  • Analysis of disease progression, morbidity, and mortality factors.
  • Evaluation of current and emerging therapeutic strategies.

Main Results:

  • Juvenile Scleroderma encompasses distinct forms with varying internal organ involvement and severity.
  • Early inflammatory phase detection is critical for effective intervention.
  • Raynaud's phenomenon with digital ulcers is a significant marker for systemic sclerosis.

Conclusions:

  • Early identification, effective treatment, and vigilant surveillance are paramount for better outcomes in Juvenile Scleroderma.
  • Advancements in vascular and immunosuppressive therapies, alongside biomarker development, are improving survival rates.
  • Multidisciplinary approaches are essential for managing the complex needs of children with Juvenile Scleroderma.