General Anesthesia for Extracorporeal Shockwave Lithotripsyin Child with Lesch-Nyhan Syndrome / 영남의대학술지
Yeungnam University Journal of Medicine
;
: 78-83, 2008.
Article
in Korean
| WPRIM
| ID: wpr-201628
ABSTRACT
Lesch-Nyhan syndrome is an inborn error of purine metabolism resulting from hypoxanthine-guanine-phosphoribosyltransferase (HGPRT) deficiency and leading to excess purine production and uric acid over-production. It is a very rare X-linked recessive disorder, characterized by movement disorder, cognitive deficits, and self-injurious behavior. However, because of the high incidence of calculi, patients may present for surgery of urinary tract, and have increased risk of difficult intubation, aspiration pneumonia, renal insufficiency or sudden death. We report the case of a 5-year-old boy with Lesch-Nyhan syndrome who underwent successive extracorporeal shockwave lithotripsy under general anesthesia.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pneumonia, Aspiration
/
Purines
/
Uric Acid
/
Urinary Tract
/
Lithotripsy
/
Calculi
/
Child, Preschool
/
Incidence
/
Self-Injurious Behavior
/
Death, Sudden
Type of study:
Incidence study
/
Prognostic study
Limits:
Child
/
Humans
Language:
Korean
Journal:
Yeungnam University Journal of Medicine
Year:
2008
Type:
Article
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