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Continuous Renal Replacement Therapy for Treatment of Severe Attacks of Inborn Errors of Metabolism

Ayse Filiz Yetimakman1, Selman Kesici2, Murat Tanyildiz1

  • 1Division of Pediatric Intensive Care, Department of Pediatrics, Hacettepe University, Ankara, Turkey.

Journal of Pediatric Intensive Care
|August 13, 2019
PubMed

Insights

Continuous renal replacement therapy (CRRT) can be lifesaving for children experiencing severe metabolic crises from inborn errors of metabolism. Early CRRT intervention improves outcomes and restores metabolic balance in most pediatric patients.

Area of Science:

  • Pediatric Critical Care Medicine
  • Metabolic Disorders
  • Nephrology

Background:

  • Inborn errors of metabolism can precipitate life-threatening metabolic crises in children.
  • Continuous renal replacement therapy (CRRT) is a potential life-saving intervention in these critical situations.
  • Assessing patient risk and response to CRRT is crucial for optimizing management.

Purpose of the Study:

  • To evaluate the effectiveness of CRRT in managing severe metabolic crises in pediatric patients.
  • To analyze the correlation between pre-CRRT risk scores and patient survival.
  • To determine the success rate of CRRT in restoring metabolic balance.

Main Methods:

  • Retrospective analysis of clinical data from pediatric patients undergoing CRRT for metabolic crises.
  • Calculation of Pediatric Risk of Mortality (PRISM) scores within the first 24 hours.
  • Calculation of Pediatric Logistic Organ Dysfunction (PELOD) scores within 24 hours prior to CRRT initiation.

Main Results:

  • CRRT successfully restored metabolic balance in 72% of the studied pediatric patients.
  • Lower pre-CRRT PELOD scores were significantly associated with improved survival (p=0.02).
  • CRRT demonstrated effectiveness in managing metabolic crises despite various comorbid factors.

Conclusions:

  • CRRT is an effective therapeutic option for managing severe metabolic crises in children with inborn errors of metabolism.
  • Early initiation of CRRT before the development of severe complications is recommended.
  • Risk stratification using scores like PELOD may aid in identifying patients who benefit most from CRRT.

Keywords:
acute metabolic crisescontinuous renal replacement therapyinborn errors of metabolism

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