PMID- 16430418
OWN - NLM
STAT- MEDLINE
DCOM- 20060608
LR  - 20181203
IS  - 1155-5645 (Print)
IS  - 1155-5645 (Linking)
VI  - 16
IP  - 2
DP  - 2006 Feb
TI  - Anesthetic management in two siblings with glutaric aciduria type 1.
PG  - 188-91
AB  - Glutaric aciduria type 1 (GA-1) is an inborn error of metabolism that results 
      from a deficiency of glutaryl-CoA dehydrogenase. This disorder mainly manifests 
      in early childhood and most patients with this condition develop a 
      dystonic-dyskinetic syndrome. We report the anesthetic management of two sisters 
      with GA-1, aged 30 and 17 months respectively at the time of surgery, who 
      presented with macrocephaly and psychomotor delay. The children required CSF 
      shunting procedures for hydrocephalus and subdural fluid collections, which were 
      performed under total intravenous anesthesia with propofol and remifentanil.
FAU - Hernandez-Palazon, Joaquin
AU  - Hernandez-Palazon J
AD  - Staff Anaesthesiologist, Department of Anaesthesia, H.U. Virgen de la Arrixaca, 
      Murcia, Spain. joapal@ono.com
FAU - Sanchez-Rodenas, Lorenzo
AU  - Sanchez-Rodenas L
FAU - Martinez-Lage, Juan F
AU  - Martinez-Lage JF
FAU - Collado, Isabel Castano
AU  - Collado IC
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - France
TA  - Paediatr Anaesth
JT  - Paediatric anaesthesia
JID - 9206575
RN  - 0 (Anesthetics, Intravenous)
RN  - 0 (Glutarates)
RN  - 0 (Piperidines)
RN  - EC 1.3.8.6 (Glutaryl-CoA Dehydrogenase)
RN  - H849F7N00B (glutaric acid)
RN  - P10582JYYK (Remifentanil)
RN  - YI7VU623SF (Propofol)
SB  - IM
CIN - Paediatr Anaesth. 2009 Jun;19(6):632-3. PMID: 19645992
MH  - Anesthesia/*methods
MH  - Anesthetics, Intravenous/*administration & dosage/adverse effects
MH  - Cerebrospinal Fluid Shunts/methods
MH  - Child, Preschool
MH  - Dystonic Disorders
MH  - Female
MH  - Glutarates/*metabolism
MH  - Glutaryl-CoA Dehydrogenase/*deficiency
MH  - Head/abnormalities
MH  - Humans
MH  - Hydrocephalus/complications/surgery
MH  - Infant
MH  - Metabolism, Inborn Errors/complications/genetics/*surgery
MH  - Piperidines/administration & dosage/adverse effects
MH  - Propofol/administration & dosage/adverse effects
MH  - Psychomotor Disorders/complications
MH  - Remifentanil
MH  - *Siblings
EDAT- 2006/01/25 09:00
MHDA- 2006/06/09 09:00
CRDT- 2006/01/25 09:00
PHST- 2006/01/25 09:00 [pubmed]
PHST- 2006/06/09 09:00 [medline]
PHST- 2006/01/25 09:00 [entrez]
AID - PAN1629 [pii]
AID - 10.1111/j.1460-9592.2005.01629.x [doi]
PST - ppublish
SO  - Paediatr Anaesth. 2006 Feb;16(2):188-91. doi: 10.1111/j.1460-9592.2005.01629.x.