Mallory bodies in a patient with type Ia glycogen storage disease. 1987

S Itoh, and Y Ishida, and S Matsuo

Mallory bodies were determined by light microscopy and confirmed by electron microscopy in the liver of a 37-yr-old woman with type Ia glycogen storage disease. They were found mainly in the swollen hepatocytes of the centrilobular region in the liver associated with periportal and centrilobular pericellular fibrosis. Ultrastructurally, the shape of small circular structures found in the Mallory body was similar to that of the circularly disorganized rough endoplasmic reticulum seen around the Mallory body. Although Mallory bodies are seen in several liver diseases other than alcoholic liver injury, there has been only one report of finding them in the liver in type I glycogen storage disease.

UI MeSH Term Description Entries
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D004721 Endoplasmic Reticulum A system of cisternae in the CYTOPLASM of many cells. In places the endoplasmic reticulum is continuous with the plasma membrane (CELL MEMBRANE) or outer membrane of the nuclear envelope. If the outer surfaces of the endoplasmic reticulum membranes are coated with ribosomes, the endoplasmic reticulum is said to be rough-surfaced (ENDOPLASMIC RETICULUM, ROUGH); otherwise it is said to be smooth-surfaced (ENDOPLASMIC RETICULUM, SMOOTH). (King & Stansfield, A Dictionary of Genetics, 4th ed) Ergastoplasm,Reticulum, Endoplasmic
D005260 Female Females
D005953 Glycogen Storage Disease Type I An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood. Glucose-6-Phosphatase Deficiency,Glucosephosphatase Deficiency,Glycogenosis 1,Hepatorenal Glycogen Storage Disease,von Gierke Disease,Deficiency, Glucosephosphatase,Gierke Disease,Gierke's Disease,Glycogen Storage Disease 1 (GSD I),von Gierke's Disease,Deficiencies, Glucose-6-Phosphatase,Deficiencies, Glucosephosphatase,Deficiency, Glucose-6-Phosphatase,Disease, Gierke,Disease, Gierke's,Disease, von Gierke,Disease, von Gierke's,Gierkes Disease,Glucose 6 Phosphatase Deficiency,Glucose-6-Phosphatase Deficiencies,Glucosephosphatase Deficiencies,von Gierkes Disease
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006818 Hyalin A clear, homogenous, structureless, eosinophilic substance occurring in pathological degeneration of tissues. Hyalin Substance,Hyaline,Hyaline Substance,Hyalins,Substance, Hyalin,Substance, Hyaline
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001706 Biopsy Removal and pathologic examination of specimens from the living body. Biopsies

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