Biomaterial Database

Dysphoria entero-broncho-pancreatica congenita familiaris (Glanzmann) cystic pancreatic fibrosis; pathological-anatomical part. 1946

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UI	MeSH Term	Description	Entries
D010179	Pancreas	A nodular organ in the ABDOMEN that contains a mixture of ENDOCRINE GLANDS and EXOCRINE GLANDS. The small endocrine portion consists of the ISLETS OF LANGERHANS secreting a number of hormones into the blood stream. The large exocrine portion (EXOCRINE PANCREAS) is a compound acinar gland that secretes several digestive enzymes into the pancreatic ductal system that empties into the DUODENUM.
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013915	Thrombasthenia	A congenital bleeding disorder with prolonged bleeding time, absence of aggregation of platelets in response to most agents, especially ADP, and impaired or absent clot retraction. Platelet membranes are deficient in or have a defect in the glycoprotein IIb-IIIa complex (PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX).	Glanzmann Thrombasthenia,Deficiency of GP 2b 3a Complex,GP IIb-IIIa Complex, Deficiency Of,Glanzmann Thrombasthenia, Type A,Glycoprotein Complex IIb-IIIa, Deficiency Of,Platelet Fibrinogen Receptor, Deficiency of,Platelet Glycoprotein 2b-3a Deficiency,Platelet Glycoprotein IIb-IIIa Deficiency,Thrombasthenia of Glanzmann and Naegeli,GP IIb IIIa Complex, Deficiency Of,Glycoprotein Complex IIb IIIa, Deficiency Of,Platelet Glycoprotein 2b 3a Deficiency,Platelet Glycoprotein IIb IIIa Deficiency,Thrombasthenia, Glanzmann,Thrombasthenias