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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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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 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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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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Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
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Related Experiment Video

Updated: Apr 20, 2026

Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5

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RAS diseases in children.

Charlotte M Niemeyer1

  • 1Department of Pediatric Hematology and Oncology, Universitätsklinikum Freiburg, Germany charlotte.niemeyer@uniklinik-freiburg.de.

Haematologica
|November 25, 2014
PubMed
Summary

Juvenile myelomonocytic leukemia (JMML) is a childhood cancer driven by mutations in RAS/MAPK pathway genes. Understanding this complex signaling network is crucial for developing new treatments beyond stem cell transplantation.

Area of Science:

  • Genetics
  • Oncology
  • Molecular Biology

Background:

  • RAS genes are critical for cell signaling pathways involved in survival and proliferation.
  • RASopathies are genetic syndromes caused by mutations in the RAS/MAPK pathway, often leading to developmental disorders and increased cancer risk.
  • Juvenile myelomonocytic leukemia (JMML) is a rare childhood leukemia linked to mutations in five key RAS/MAPK pathway genes.

Purpose of the Study:

  • To review the role of RAS/MAPK pathway mutations in JMML.
  • To discuss the clinical characteristics and outcomes of different JMML subtypes.
  • To highlight the need for further research into the RAS/MAPK signaling network for novel therapeutic strategies.

Main Methods:

  • Literature review focusing on genetic mutations in JMML.

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  • Analysis of clinical data and outcomes for JMML patients.
  • Examination of the RAS/MAPK signaling pathway and its involvement in leukemia.

    • Main Results:

    • Germline and/or somatic mutations in PTPN11, CBL, NF-1, KRAS, and NRAS initiate JMML.
    • Different JMML subtypes driven by these mutations exhibit distinct clinical courses.
    • Hematopoietic stem cell transplantation is the primary curative option for most JMML patients.

    Conclusions:

    • Targeting the RAS/MAPK pathway is essential for treating JMML.
    • Further understanding of the pathway's complexities, including crosstalk and feedback loops, is needed.
    • Developing novel pharmacological agents and early clinical trials is crucial for improving JMML outcomes.