Related Experiment Videos

Pediatric antiphospholipid antibodies and antiphospholipid syndrome

Beverley J Hunt1

  • 1Department of Haematology & Louise Coote Lupus Unit, Guys' & St Thomas' Foundation Trust, London, UK. beverley.hunt@gstt.nhs.uk

Seminars in Thrombosis and Hemostasis
|August 23, 2008
PubMed

Insights

Pediatric Antiphospholipid Syndrome (APS) shares similarities with adult APS but has unique antibody patterns. Recurrent thrombotic events are less frequent in children, though management remains challenging due to limited evidence.

Area of Science:

  • Pediatric Rheumatology
  • Hematology
  • Immunology

Background:

  • Antiphospholipid syndrome (APS) affects children and adults, presenting with diverse thrombotic events, primarily deep vein thrombosis and stroke.
  • Transient antiphospholipid (aPL) antibodies are more common in children post-infection compared to adults.
  • Children with "true" aPL antibodies experience fewer recurrent thrombotic events than adults, possibly due to a less prothrombotic childhood hemostatic state.

Purpose of the Study:

  • To highlight the unique aspects of pediatric Antiphospholipid Syndrome (APS).
  • To discuss the challenges in managing thrombotic events in children with APS.
  • To explore the potential of a pediatric APS registry for data collection.

Main Methods:

  • Review of existing literature on pediatric Antiphospholipid Syndrome.
  • Comparison of clinical presentation and outcomes between pediatric and adult APS.
  • Discussion of current management strategies and their limitations.

Main Results:

  • Pediatric APS presents with similar thrombotic sites as adult APS, predominantly deep vein thrombosis and stroke.
  • Children more frequently exhibit transient, non-thrombogenic aPL antibodies, often following infections.
  • Recurrent thrombotic events appear less frequent in children with APS compared to adults.

Conclusions:

  • Management of pediatric APS is complex due to a lack of evidence-based medicine.
  • Optimal anticoagulation duration and intensity remain unresolved issues, with a target INR of 2-3 commonly used.
  • Multicenter randomized controlled trials are difficult to conduct; a pediatric APS registry (e.g., Ped-APS Register) is a feasible alternative for data generation.

Related Concept Videos

Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
Pharmacokinetics in Pediatric Patients: Drug Distribution01:17

Pharmacokinetics in Pediatric Patients: Drug Distribution

Drug distribution in the pediatric population exhibits unique challenges and considerations due to the physiological differences between children, particularly neonates and infants, and adults. A crucial aspect of pediatric pharmacology is understanding how these differences impact the pharmacokinetics of various drugs, necessitating age-specific dosing strategies to ensure efficacy and safety.Neonates and infants have a higher total body water content, ~75%–90% of their body weight, compared...
Phosphoinositides and PIPs01:42

Phosphoinositides and PIPs

Phosphoinositides are a group of phospholipids containing a glycerol backbone with two fatty acid chains and a phosphate attached to a myoinositol sugar ring. The inositol head group extends into the cytoplasm, where it is modified by adding phosphate groups to form phosphatidylinositol phosphates or PIPs.
Different phosphoinositides are synthesized and recruited on the cytosolic face of the plasma membrane. The localization of specific phosphoinositides concentrated in separate membrane...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
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...