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Related Concept Videos

Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

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 fluid...
Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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...
Bacterial Gastroenteritis01:18

Bacterial Gastroenteritis

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 receptor...
Atypical Pneumonia01:14

Atypical Pneumonia

Atypical pneumonia, often caused by Mycoplasma pneumoniae, is a form of pulmonary infection that differs from the classical presentation of bacterial pneumonia in both its cause and clinical symptoms. Mycoplasma pneumoniae is a pleomorphic bacterium notable for its lack of a rigid cell wall. This structural characteristic imparts resistance to beta-lactam antibiotics and significantly influences the bacterium’s behavior within the human host.Other pathogens responsible for the disease include...
Hyperosmolar Hyperglycemic State01:21

Hyperosmolar Hyperglycemic State

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...
Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

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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Related Experiment Video

Updated: May 29, 2026

Development of Human Renal Tubular Epithelial Cell Primary Cultures in Monolayers and Three-Dimensional Conditions
06:32

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

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Atypical hemolytic uremic syndrome.

Chantal Loirat1, Véronique Frémeaux-Bacchi

  • 1Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Université Paris VII, Pediatric Nephrology Department, Paris, France. chantal.loirat@rdb.aphp.fr

Orphanet Journal of Rare Diseases
|September 10, 2011
PubMed
Summary

Atypical hemolytic uremic syndrome (aHUS) is a primary complement disorder affecting adults and children. Eculizumab shows promise as a new standard of care, offering improved outcomes for aHUS patients.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Atypical hemolytic uremic syndrome (aHUS) is a rare, severe condition characterized by hemolytic anemia, thrombocytopenia, and renal impairment.
  • Unlike Shiga-toxin-associated HUS, aHUS results from dysregulation of the complement alternative pathway.
  • It affects both children and adults, with a higher prevalence in adults, and can lead to significant morbidity and mortality.

Purpose of the Study:

  • To review the current understanding of aHUS pathophysiology, diagnosis, and treatment.
  • To highlight the role of complement system dysregulation in aHUS.
  • To evaluate the efficacy of emerging therapies, particularly eculizumab.

Main Methods:

  • Review of existing literature on aHUS, including genetic studies and clinical trials.

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  • Analysis of diagnostic criteria for aHUS, differentiating it from other forms of HUS.
  • Assessment of treatment outcomes for plasmatherapy and eculizumab.

    • Main Results:

    • Mutations in complement regulatory genes (e.g., Factor H, MCP, Factor I) and anti-Factor H antibodies are identified in a significant proportion of aHUS patients.
    • Diagnosis requires excluding other HUS causes and investigating complement system abnormalities.
    • Eculizumab, a C5 complement inhibitor, demonstrates impressive efficacy in clinical trials, suggesting a shift in standard care.

    Conclusions:

    • aHUS is primarily a genetic disorder of complement regulation, with varying clinical presentations and genetic underpinnings.
    • Early and accurate diagnosis is crucial for effective management.
    • Eculizumab represents a significant advancement in aHUS treatment, offering improved prognosis compared to historical therapies like plasmapheresis.