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Related Concept Videos

Nephrotic Syndrome I : Introduction01:24

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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Hyperosmolar Hyperglycemic State01:21

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Hyperosmolar Hyperglycemic State, or HHS, is a serious and life-threatening complication of type 2 diabetes mellitus. It is characterized by three main features: severe hyperglycemia, profound dehydration, and elevated serum osmolality, all occurring without significant ketoacidosis.HHS typically develops in older adults or individuals with limited access to fluids. This may result from illness, cognitive impairment, or medications such as diuretics or corticosteroids. These factors reduce...
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Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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Bacterial Gastroenteritis01:18

Bacterial Gastroenteritis

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Bacterial gastroenteritis, characterized by diarrhea, abdominal cramps, and vomiting, is often caused by ingestion of contaminated food or water and is frequently associated with pathogenic Escherichia coli strains. These microbes exploit two principal mechanisms to inflict disease.Shiga toxin–producing E. coli, also referred to as STEC—notably O157:H7—release Shiga toxins that target ribosomes, blocking protein synthesis. The B subunit of the toxin binds the host glycolipid...
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Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

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Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
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Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

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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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Related Experiment Video

Updated: Apr 20, 2026

Development of Human Renal Tubular Epithelial Cell Primary Cultures in Monolayers and Three-Dimensional Conditions
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Development of Human Renal Tubular Epithelial Cell Primary Cultures in Monolayers and Three-Dimensional Conditions

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[Atypical hemolytic uremic syndrome].

Miquel Blasco Pelicano1, Santiago Rodríguez de Córdoba2, Josep M Campistol Plana1

  • 1Servicio de Nefrología y Trasplante Renal, Hospital Clínic, Barcelona, España.

Medicina Clinica
|December 1, 2014
PubMed
Summary

Atypical hemolytic uremic syndrome (aHUS) involves complement dysregulation, leading to thrombotic microangiopathy. Understanding these mechanisms aids in early diagnosis and targeted therapies, improving patient outcomes.

Keywords:
Alternative pathway of complement systemAtypical hemolytic uremic syndromeEculizumabPlasma therapySíndrome hemolítico urémico atípicoTratamiento plasmáticoVía alternativa del complemento

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Area of Science:

  • Nephrology
  • Immunology
  • Hematology

Context:

  • Hemolytic uremic syndrome (HUS) is a condition marked by low platelets, hemolytic anemia, and kidney dysfunction.
  • Kidney pathology reveals thrombotic microangiopathy (TMA) due to endothelial cell injury.
  • HUS is classified into typical (bacterial-induced) and atypical (aHUS) forms.

Purpose:

  • To review the clinical entity of atypical hemolytic uremic syndrome (aHUS).
  • To elucidate the role of complement dysregulation in aHUS pathogenesis.
  • To discuss differential diagnosis and therapeutic strategies for aHUS.

Summary:

  • Atypical hemolytic uremic syndrome (aHUS) is often linked to complement gene mutations, leading to a worse prognosis and end-stage renal disease.
  • Recurrence or de novo aHUS can occur post-kidney transplantation.
  • Complement dysregulation is a key factor in endothelial damage and TMA development in most aHUS cases.

Impact:

  • Advances in understanding aHUS pathogenesis and novel therapies are transforming its natural history.
  • Improved diagnostic and etiological treatment strategies are becoming available.
  • This review provides insights into aHUS diagnosis, pathogenesis, and treatment options.