Glycogen storage diseases in Thai patients: Phramongkutklao Hospital experience.

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dc.contributor.authorKamolsilp, Mahattanaen_US
dc.date.accessioned2009-05-27T20:48:01Z
dc.date.available2009-05-27T20:48:01Z
dc.date.issued2005-11-25en_US
dc.descriptionChotmaihet Thangphaet.en_US
dc.description.abstractThere are 3 cases of liver type glycogen storage diseases. All of them presented with protruding abdomen, failure to thrive, doll face and mark hepatomegaly. Laboratory findings were hypoglycemia, metabolic acidosis, abnormal liver function test, hyperlipidemia and prolonged bleeding time in GSD Ia. GSD III has no hypoglycemia and borderline hyperuricemia. Glucagon stimulation test helps to differentiate typing. The aim of treatment is to prevent hypoglycemia, suppress lactic acid production, decrease blood lipid and uric acid levels and enhances statural growth by uncooked cornstarch. Complications such as epistaxis and suspected liver adenoma have to be closely followed up. Genetic counseling for both types GSD are autosomal recessive with recurrence risk of 25%. Prenatal diagnosis by enzymes assay or molecular diagnosi are not available in this hospital.en_US
dc.description.affiliationDepartment of Pediatrics, Phramongkutklao Hospital, Bangkok, Thailand.en_US
dc.identifier.citationKamolsilp M. Glycogen storage diseases in Thai patients: Phramongkutklao Hospital experience. Journal of the Medical Association of Thailand. 2005 Nov; 88 Suppl 3(): S295-301en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/43657
dc.language.isoengen_US
dc.source.urihttps://www.mat.or.th/journal/all.phpen_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshGlycogen Storage Disease Type I --complicationsen_US
dc.subject.meshGlycogen Storage Disease Type III --complicationsen_US
dc.subject.meshHumansen_US
dc.subject.meshThailanden_US
dc.titleGlycogen storage diseases in Thai patients: Phramongkutklao Hospital experience.en_US
dc.typeCase Reportsen_US
dc.typeJournal Articleen_US
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