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
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