glycogenosis


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glycogenosis

 [gli″ko-jĕ-no´sis]
generalized glycogenosis Pompe's disease.
hepatorenal glycogenosis Gierke's disease.
myophosphorylase deficiency glycogenosis McArdle disease.

gly·co·ge·no·sis

(glī'kō-jĕ-nō'sis),
Any of the glycogen deposition diseases characterized by accumulation of glycogen of normal or abnormal chemical structure in tissue; there may be enlargement of the liver, heart, or striated muscle, including the tongue, with progressive muscular weakness. Seven types (Cori classification) are recognized, depending on the enzyme deficiency involved, all of autosomal recessive inheritance, but with a different gene for each enzyme deficiency. [MIM designations: I, *232200, *232220, *232240; II, *232300; III, *232400; IV, *232500; V, *232600; VI, *232700; VII, *232800].

glycogenosis

(glī′kə-jĕ-nō′sĭs)
n. pl. glycogeno·ses (-sēz′)

glycogenosis

Glycogen storage disease, see there.

gly·co·ge·no·sis

(glī'kō-jĕ-nō'sis)
Any glycogen deposition disease characterized by accumulation of glycogen of normal or abnormal chemical structure in tissue; there may be enlargement of the liver, heart, or striated muscle, including the tongue, with progressive muscular weakness.
Synonym(s): dextrinosis.

Glycogenosis

An alternate term for glycogen storage disease. The plural form is glycogenoses.

gly·co·ge·no·sis

(glī'kō-jĕ-nō'sis)
A glycogen deposition disease characterized by accumulation of glycogen of normal or abnormal chemical structure in tissue; may be enlargement of the liver, heart, or striated muscle, including the tongue.
References in periodicals archive ?
Batch, "Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes," Journal of Paediatrics and Child Health, vol.
Manfredi, "Elevation of transaminases as an early sign of late-onset glycogenosis type II," European Journal of Pediatrics, vol.
Glycogen storage disease (GSD) or glycogenosis include hereditary diseases caused by abnormalities of the enzymes that regulate the synthesis and degradation of glycogen.
Included in this group are disorders presenting as a predominant hypoglycaemia like glycogenosis, gluconeogenesis defects and hyperinsulinism, the fatty acid oxidation disorders, and the congenital lactic acidaemias (deficiencies of pyruvate carboxylase, pyruvate dehydrogenase, Krebs cycle and mitochondrial respiratory chain disorders).
Individuals with glycogen storage disease or with poorly controlled diabetes have excessive storage of glycogen in their livers (glycogenosis) and increased risk of liver cancer (Adami et al.
These disorders affect the breakdown of glycogen or glucose (complex and simple carbohydrates) and are also called glycogenosis disorders.
We diagnosed the patient as Mauriac syndrome with the findings of growth retardation, poorly controlled diabetes, hepatomegaly, and hepatic glycogenosis. Genetic analysis was performed to exclude congenital glycogen storage disease type-1 (GSD-1).
Note that some nuclei have cleared chromatin (nuclear glycogenosis), which can be seen in various metabolic conditions, such as diabetes mellitus (Hematoxylin and Eosin staining at 400x magnification).
Long-term course of hepatic glycogenosis. A retrospective study of 76 cases.
Glycogenosis Type II: Protein and DNA analysis in five South African families from various ethnic origins.