title: G6PD Deficiency with Arnold-Chiari Malformation.
abstract: A neonate with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Arnold-Chiari Malformation (ACM) type 2 underwent lumbar meningomyelocele (MMC) repair. Patients with G6PD deficiency are prone to develop haemolysis following any kind of oxidative stress and in ACM, there is a disturbed cranio-spinal pressure relationship. The neonate was managed under general anaesthesia with propofol for induction as well as for maintenance along with fentanyl and oxygen-nitrous mixture.
authors.affiliation: Department of Anaesthesia, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.
authors.forename: Shilpi
authors.initials: S
authors.lastname: Verma
authors.affiliation: Department of Anaesthesia, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.
authors.forename: Pradeep Kumar
authors.initials: PK
authors.lastname: Bhatia
authors.affiliation: Department of Anaesthesia, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.
authors.forename: Vandana
authors.initials: V
authors.lastname: Sharma
authors.affiliation: Department of Anaesthesia, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.
authors.forename: Priyanka
authors.initials: P
authors.lastname: Sethi
authors.affiliation: Department of Physiology, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.
authors.forename: Yogendra Raj
authors.initials: YR
authors.lastname: Singh
mesh_terms: D000758:Anesthesia / Q000379:methods*; D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D001139:Arnold-Chiari Malformation / Q000503:physiopathology / Q000601:surgery*; D058565:Cerebral Ventriculitis; D019299:Decompression, Surgical; D005283:Fentanyl / Q000008:administration & dosage; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood*; D006461:Hemolysis; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D008591:Meningomyelocele / Q000503:physiopathology* / Q000601:surgery*; D009609:Nitrous Oxide; D018384:Oxidative Stress; D010100:Oxygen; D015742:Propofol / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D009609:Nitrous Oxide; D010100:Oxygen; D005283:Fentanyl; D015742:Propofol