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Pompe disease <disease> Severe glycogen storage disease, also called glycogenosis II, is caused by deficiency of alpha-1,4-glucosidase, the lysosomal enzyme responsible for glycogen hydrolysis.
Even though the nonlysosomal glycogenolytic system is normal, glycogen still accumulates in the lysosomes in almost all tissues, but produces especially severe effects in skeletal and cardiac muscle leading to early mortality.
Specific enzyme assay in muscle cells, leukocytes or amniocytes confirms the diagnosis.
(29 Dec 1997)
Pompe's disease Glycogenosis due to lysosomal alpha-1,4-glucosidase deficiency, resulting in accumulation of excessive amounts of glycogen of normal chemical structure in heart, muscle, liver, and nervous system.
Synonym: generalised glycogenosis, Pompe's disease.
(05 Mar 2000)
Pompe, J <person> 20th century Dutch physician.
See: Pompe's disease.
(05 Mar 2000)
pompelmous Origin: D. Pompelmoes; cf. G. Pompelmuse, F. Pamplemousse, and F. Pompoleon.
<botany> A shaddock, especially. One of large size.
Source: Websters Dictionary
(01 Mar 1998)
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Pompe's d. glycogen storage d., type II.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Pompe's disease glycogen storage disease (type II).
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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Pompe ancient city southeast of Naples that was buried by a volcanic eruption from Vesuvius
Pompe a port city in southern England on the English Channel
Pompe Roman general and statesman who quarrelled with Caesar and fled to Egypt where he was murdered (106-48 BC)
Pompe Roman general and statesman who quarrelled with Caesar and fled to Egypt where he was murdered (106-48 BC)
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