Biomaterial Database

Glycogen storage disease (type-IV) with cirrhosis and metastatic intrathoracic neuroblastoma. 1994

A K Sarkar, and S Sarkar, and V Asawa, and A K Munshi

Department of Pediatrics, Institute of Post Graduate Medical Education and Research and S.S.K.M. Hospital, Calcutta.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008103	Liver Cirrhosis	Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.	Cirrhosis, Liver,Fibrosis, Liver,Hepatic Cirrhosis,Liver Fibrosis,Cirrhosis, Hepatic
D008297	Male		Males
D009362	Neoplasm Metastasis	The transfer of a neoplasm from one organ or part of the body to another remote from the primary site.	Metastase,Metastasis,Metastases, Neoplasm,Metastasis, Neoplasm,Neoplasm Metastases,Metastases
D009447	Neuroblastoma	A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)	Neuroblastomas
D006011	Glycogen Storage Disease Type IV	An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.	Amylopectinosis,Andersen Disease,Brancher Deficiency,Glycogenosis 4,Andersen's Disease,Deficiency, Brancher,Gbe1 Deficiency,Glycogen Branching Enzyme Deficiency,Glycogen Storage Disease Type 4,Glycogenosis IV,Type IV Glycogenosis,Amylopectinoses,Andersens Disease,Brancher Deficiencies,Deficiencies, Brancher,Deficiencies, Gbe1,Deficiency, Gbe1,Disease, Andersen,Disease, Andersen's,Gbe1 Deficiencies,Glycogenoses, Type IV,Glycogenosis 4s,Glycogenosis IVs,Glycogenosis, Type IV,Type IV Glycogenoses
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013899	Thoracic Neoplasms	New abnormal growth of tissue in the THORAX.	Neoplasm, Thoracic,Neoplasms, Thoracic,Thoracic Neoplasm