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Nephrotic Syndrome I : Introduction

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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Nephrotic Syndrome II : Assessment and Medical Management01:26

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IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
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Nephrotic Syndrome III : Nursing Management01:24

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Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...
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Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

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Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
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Myocarditis III: Medical Management01:14

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Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
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Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
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The Goeckerman Regimen for the Treatment of Moderate to Severe Psoriasis
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Systemic therapy in juvenile localized scleroderma.

Ivan Foeldvari1, Edoardo Marrani2

  • 1Hamburger Zentrum Für Kinder- Und Jugendrheumatologie, Hamburg, Germany.

Expert Review of Clinical Immunology
|July 18, 2023
PubMed
Summary
This summary is machine-generated.

Early treatment of juvenile localized scleroderma (JLS) is crucial to prevent long-term disability. This review outlines disease assessment and a treat-to-target systemic therapy plan, including methotrexate as a first-line option.

Keywords:
Autologous fat transplantationJAK inhibitorsdamagedisease modifying agentsinactive diseaselocalized sclerodermamorpheaoutcomeremission

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Area of Science:

  • Pediatric rheumatology
  • Dermatology
  • Autoimmune connective tissue diseases

Background:

  • Juvenile localized scleroderma (JLS) is a rare childhood condition causing significant morbidity if untreated.
  • Prompt intervention during the inflammatory phase is essential to prevent permanent damage and functional disability.

Purpose of the Study:

  • To review methods for assessing disease activity and damage in JLS.
  • To propose a systemic treatment escalation strategy based on a treat-to-target approach.
  • To define criteria for inactive disease and medication discontinuation.

Main Methods:

  • Discussion of baseline disease activity assessment for treatment monitoring.
  • Emphasis on evaluating extra-cutaneous involvement.
  • Proposal of a tiered systemic treatment approach, prioritizing methotrexate as first-line therapy.

Main Results:

  • Systemic treatment is recommended for most JLS patients.
  • Methotrexate is the preferred first-line disease-modifying anti-rheumatic drug (DMARD).
  • A treatment escalation plan is necessary for cases of methotrexate intolerance or non-response.

Conclusions:

  • Timely assessment and intervention in JLS are critical.
  • A structured, treat-to-target approach with potential escalation is vital for managing JLS.
  • Further research into prognostic markers is needed to guide treatment decisions.