Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Scintigraphic abnormalities in glycogen storage disease.

J H Miller, G F Gates, B H Landing

    Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine
    |April 1, 1978
    PubMed
    Summary

    Serial scintigraphy in glycogen-storage disease type 1 (GSD-1) reveals liver enlargement and focal defects. Careful monitoring is crucial for detecting potential malignant changes like hepatic-cell carcinoma.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    Glycogen storage disease. I. Familial cardiac glycogen storage disease: report of two cases and discussion of relation to other forms of abnormal glycogen deposition.

    Bulletin. International Association of Medical Museums·2014
    Same author

    Plastic injection techniques in pediatric pathology, with particular reference to roentgenographic study of injected specimens.

    Bulletin. International Association of Medical Museums·2014
    Same author

    The intraperitoneal toxicity of some glycols, glycol ethers, glycol esters, and phthalates in mice.

    Federation proceedings·2010
    Same author

    The intraperitoneal toxicity of some glycols, glycol ethers, glycol esters, and phthalates in mice.

    The Journal of pharmacology and experimental therapeutics·2010
    Same author

    The development of structure and function in the postnatal human cerebral cortex from birth to 72 months: changes in thickness of layers II and III co-relate to the onset of new age-specific behaviors.

    Pediatric pathology & molecular medicine·2002
    Same author

    Bone SPECT of the back after lumbar surgery.

    Clinical nuclear medicine·1999

    Area of Science:

    • Hepatology
    • Pediatric Endocrinology
    • Diagnostic Imaging

    Background:

    • Glycogen-storage disease type 1 (GSD-1), or von Gierke's disease, is a metabolic disorder affecting glucose metabolism.
    • Patients with GSD-1 often exhibit hepatomegaly, splenomegaly, and renomegaly.
    • There is an increased risk of developing hepatic complications, including tumors.

    Purpose of the Study:

    • To evaluate the utility of serial scintigraphy in characterizing liver, spleen, and kidney abnormalities in GSD-1 patients.
    • To assess the role of scintigraphy in identifying and differentiating benign from potentially malignant liver lesions in GSD-1.

    Main Methods:

    • Serial liver, spleen, and kidney scintigraphy was performed on fifteen patients diagnosed with GSD-1.
    • Scintigraphic findings were correlated with the presence and nature of hepatic lesions.

    Related Experiment Videos

    Main Results:

    • A consistent pattern of enlarged liver with diminished radionuclide uptake, splenomegaly with increased uptake, and renomegaly was observed.
    • Seven patients showed focal liver defects of varying sizes on scintigraphy.
    • Small or stable defects were associated with benign hepatic adenomas, while growing, large lesions indicated malignant transformation.

    Conclusions:

    • Serial scintigraphy is a valuable tool for assessing organ involvement in GSD-1.
    • Routine liver scintigraphy, including scintiangiography, is recommended for early detection of hepatic-cell carcinoma in GSD-1 patients.
    • Distinguishing benign from malignant hepatic lesions is critical for appropriate patient management.