Anesthetic Experience of Percutaneous Nephrolithotomy for Renal Calculi in a Patient with Lesch-Nyhan Syndrome: A case report / 대한마취과학회지
Korean Journal of Anesthesiology
;
: 520-523, 2007.
Article
in Korean
| WPRIM
| ID: wpr-193257
ABSTRACT
Lesch-Nyhan syndrome (LNS) is a rare, X-linked recessive inherited disorder caused by a deficiency of the enzyme hypoxanthine-guanine-phophoribosyltransferase, leading to excessive purine production and elevation of uric acid. Clinical manifestations include mental retardation, spasticity, choreathetosis, compulsive self-mutilation, renal calculi followed by obstructive nephropathy, and arthritis. Patient with LNS may have increased risk of aspiration pneumonia, acute renal failure and unexpected sudden death. We accomplished successful general anesthesia in a case of LNS requiring percutaneous nephrolithotomy due to renal calculi.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pneumonia, Aspiration
/
Arthritis
/
Uric Acid
/
Nephrostomy, Percutaneous
/
Kidney Calculi
/
Death, Sudden
/
Acute Kidney Injury
/
Anesthesia, General
/
Lesch-Nyhan Syndrome
/
Intellectual Disability
Limits:
Humans
Language:
Korean
Journal:
Korean Journal of Anesthesiology
Year:
2007
Type:
Article
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