Related Experiment Video
Updated: Aug 7, 2025
08:44
Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis
Published on: September 7, 2022
2.5K
Hemolytic-Uremic Syndrome in Children.
Olivia Boyer1, Patrick Niaudet2
1Pediatric Nephrology, Necker Enfants Malades Hospital, Université Paris Cité, France; Néphrologie Pédiatrique, Hôpital Necker, 149 Rue de Sèvres, Paris 75015, France.
Pediatric Clinics of North America
|March 7, 2023
Summary
Hemolytic uremic syndrome (HUS) is a severe condition often caused by E. coli. While typical HUS management is supportive, atypical HUS benefits from complement inhibitors like eculizumab.
Area of Science:
- Nephrology
- Hematology
- Infectious Diseases
Background:
- Hemolytic uremic syndrome (HUS) presents with a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure.
- Shiga-toxin-producing bacteria, particularly Escherichia coli (STEC), are the primary cause of typical HUS.
- STEC-HUS is a leading cause of acute renal failure in pediatric populations.
Purpose of the Study:
- To summarize the characteristics and management of typical and atypical HUS.
- To highlight the role of Shiga-toxin-producing bacteria in HUS etiology.
- To discuss the therapeutic advancements in atypical HUS.
Main Methods:
- Literature review of HUS cases and management strategies.
- Analysis of etiological factors, including bacterial toxins and genetic variants.
- Evaluation of treatment outcomes for supportive care and targeted therapies.
Main Results:
- Typical HUS, often linked to STEC from contaminated food, requires supportive care with variable outcomes.
- Atypical HUS (aHUS), accounting for 5% of cases, involves complement pathway dysregulation and has a high risk of end-stage renal disease.
- Genetic variants in complement regulators are implicated in aHUS, leading to a relapsing course.
Conclusions:
- Management of typical HUS remains primarily supportive.
- Atypical HUS necessitates specific treatments targeting the complement system.
- Complement inhibitors, exemplified by eculizumab, have significantly improved the prognosis for atypical HUS patients.
Related Concept Videos
01:24Nephrotic Syndrome I : Introduction
12
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...
12
01:22Lysosomal Hydrolases
3.9K
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
3.9K
01:24Chronic Kidney Disease II: Clinical Manifestations
51
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...
51
01:26Nephrotic Syndrome II : Assessment and Medical Management
13
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...
13
01:29Acute Kidney Injury III: Clinical Manifestations
52
Acute Kidney Injury (AKI) progresses through distinct clinical phases: the oliguric, diuretic, and recovery phases, each marked by unique manifestations and challenges.Oliguric Phase:The oliguric phase is the initial stage of AKI, typically lasting 10 to 14 days. This phase is marked by a significant reduction in urine output, usually less than 400 mL per day, indicating decreased kidney function. Fluid retention is a prominent feature, leading to symptoms such as edema, hypertension, and...
52
01:20Inborn Errors of Metabolism
212
Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
212