Liver transplantation for type Ib glycogenosis with reversal of cyclic neutropenia

We describe a case of glycogen storage disease type Ib in 32-year old male patient with poor metabolic control in spite of medical and nutritional management and the use of recombinant granulocyte stimulating factor. Because of this, liver transplantation was considered as a definitive treatment. We...

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Bibliographic Details
Published in:Clinical nutrition (Edinburgh, Scotland) Scotland), 2001-08, Vol.20 (4), p.375-377
Main Authors: MARTINEZ-OLMOS, M.Á., LÓPEZ-SANROMÁN, A., MARTÍN-VAQUERO, P., MOLINA-PÉREZ, E., BÁRCENA, RAFAEL, VICENTE, E., CANDELA, Á., PALLARDO-SÁNCHEZ, L.F.
Format: Article
Language:English
Subjects:
Adult
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Emergency and intensive care: metabolism and nutrition disorders. Enteral and parenteral nutrition
enteral nutrition, liver transplantation
Glycogen Storage Disease Type II - complications
Glycogen Storage Disease Type II - surgery
Glycogen Storage Disease Type II - therapy
glycogen storage diseases-type I
Granulocyte Colony-Stimulating Factor - therapeutic use
Humans
Intensive care medicine
Liver Transplantation
Male
Medical sciences
Neutropenia
nutritional management
Treatment Outcome
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Summary:We describe a case of glycogen storage disease type Ib in 32-year old male patient with poor metabolic control in spite of medical and nutritional management and the use of recombinant granulocyte stimulating factor. Because of this, liver transplantation was considered as a definitive treatment. We comment on the metabolic results of liver transplantation performed, with reversal of hypoglycemia, hyperuricemia, hypertrigliceridemia and cyclic neutropenia, all of which persist 4 years post-transplant. In view of this case, we believe that liver transplantation is a feasible option to consider in patients with type Ib glycogenosis as a definitive therapeutic procedure.
ISSN:0261-5614
1532-1983
DOI:10.1054/clnu.2001.0432