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ISSN: 2644-1403

Global Journal of Anesthesia & Pain Medicine

Case Report(ISSN: 2644-1403)

Type I Citrullinemia: Use of the Ani Monitor (Analgesia and Nociception Index) for the Anesthetic Management of a Pediatric Patient with Type I Citrullinemia Volume 3 - Issue 5

Mercedes Benítez Jiménez1*, Ernesto Martinez2, Lola Pato2, María Angeles Rodríguez Navarro1 and Jose Antonio Castillo Bustos1

  • 1Department of Anesthesiology, JM Morales Meseguer University, Spain
  • 2Hospital General universitario Niño Jesús, Madrid, Spain

Received: November 25, 2020;  Published: December 21, 2020

Corresponding author: Mercedes Benítez Jiménez, Department of Anesthesiology, Resuscitation and Pain Therapeutics, General Hospital, JM Morales Meseguer University, Spain

DOI: 10.32474/GJAPM.2020.03.000176

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Abstract

Citrullinemia is an autosomal recessive genetic condition that falls under the spectrum of urea cycle disorders. The defective genes reduce the activity of enzymes needed to process nitrogen effectively. Two types exist: type I (neonatal onset) occurs when the defective arginosuccinate synthase enzyme fails to complete the third step of the urea cycle by converting citrulline and aspartate to L-argininosuccinate. Type II (adult onset) develops with the production of malfunctioning citrine proteins, which serve to transport molecules used in the metabolism of simple sugars, the assembly of protein, and the urea cycle

Introduction| Clinical Case| Discussion| Conclusion| References|

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