Document Detail

Preemptive living donor liver transplantation in glycogen storage disease Ia: case report.
MedLine Citation:
PMID:  18929868     Owner:  NLM     Status:  MEDLINE    
PATIENT: The patient was a 5-year-old boy in whom GSD-Ia was diagnosed at age 10 months. Clinical symptoms included frequent hypoglycemic episodes, hyperlipidemia, hyperuricemia, and growth retardation, which were poorly controlled using conventional treatments. At age 5 years, frequent massive nasal bleeds developed, which led to severe anemia. The patient was brought to our institute for living donor liver transplantation (LDLT). Because GSD-Ia usually responds to dietary and medical treatments, we had a long discussion to determine whether preemptive LDLT was indicated. Transplantation was performed using the left lateral liver segment from the patients mother. The weight of his native liver was almost 2 kg. After reperfusion of the graft, the blood glucose concentration rapidly increased, and regular glucose was administered throughout the operation. The posttransplantation course was uneventful. The patient had no episodes of hypoglycemia with a regular diet. Total cholesterol, triglyceride, and uric acid concentrations also reverted to normal without medication. The patient had a few episodes of nasal bleeding after transplantation, which stopped spontaneously. He was discharged from our hospital with normal liver function.
CONCLUSION: Patients with GSD-Ia should be considered for preemptive LDLT to improve their quality of life when clinical symptoms do not respond to appropriate treatment.
S Kaihara; H Ushigome; K Sakai; A Yoshizawa; S Nobori; T Suzuki; M Okamoto; T Ochiai; N Yoshimura
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Transplantation proceedings     Volume:  40     ISSN:  0041-1345     ISO Abbreviation:  Transplant. Proc.     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-10-20     Completed Date:  2008-12-01     Revised Date:  2013-05-20    
Medline Journal Info:
Nlm Unique ID:  0243532     Medline TA:  Transplant Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2815-7     Citation Subset:  IM    
Department of Surgery, Kobe City General Hospital, Kobe, Japan.
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MeSH Terms
Blood Glucose / metabolism
Child, Preschool
Enteral Nutrition
Glycogen Storage Disease Type I / surgery*
Hepatectomy / methods
Liver Transplantation* / physiology
Living Donors*
Tissue and Organ Harvesting / methods
Treatment Outcome
Reg. No./Substance:
0/Blood Glucose

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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