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Related Concept Videos

Pharmacokinetics in Pediatric Patients: Drug Distribution01:17

Pharmacokinetics in Pediatric Patients: Drug Distribution

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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,...
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Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

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Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
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Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

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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...
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Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

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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...
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Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

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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...
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Pediatric severe factor XI deficiency: A multicenter study.

Assaf Arie Barg1,2, Sarina Levy-Mendelovich1,2, Ivan Budnik3

  • 1Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Pediatric Blood & Cancer
|December 27, 2021
PubMed
Summary
This summary is machine-generated.

Factor XI (FXI) deficiency in children generally has a favorable outcome. However, severe FXI deficiency may be linked to bleeding risks, emphasizing the need for careful surgical hemostasis.

Keywords:
bleedingdiagnosisfactor XI deficiencypediatricthrombosistreatment

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Area of Science:

  • Hematology
  • Pediatric Medicine
  • Rare Diseases

Background:

  • Factor XI (FXI) deficiency is a rare autosomal recessive bleeding disorder.
  • Limited data exists on pediatric clinical features of FXI deficiency.
  • High prevalence in Israel facilitated a large multicenter cohort study.

Purpose of the Study:

  • Investigate hemostatic challenges unique to children with severe FXI deficiency.
  • Explore the clinical impact of FXI deficiency in pediatric patients.
  • Assess bleeding risks and management strategies in children.

Main Methods:

  • Retrospective evaluation of medical files from children with FXI level <15% across five tertiary centers.
  • Data collection included demographics, bleeding episodes, surgeries, treatments, and lab features.
  • Analysis focused on clinical characteristics and outcomes in severe FXI deficiency.

Main Results:

  • Sixty children with a median FXI level of 4% were included.
  • Three children experienced intracranial hemorrhage; two had major bleeds.
  • No surgical bleeding complications occurred with hemostatic treatment (tranexamic acid/FFP); 25% excessive bleeding without it (p=0.002).

Conclusions:

  • FXI deficiency in children typically has a favorable prognosis with no spontaneous bleeds or perinatal ICH.
  • Adequate hemostasis is crucial for surgical procedures in children with FXI deficiency.
  • Pediatric FXI deficiency severity correlates with bleeding tendency, unlike adult studies.