Biomaterial Database

[Clinically undiagnosed glycogen storage disease type I as cause of postoperative death (author's transl)]. 1977

H Nier, and R Sailer, and H Müntefering

A 30-year-old patient was admitted to hospital for cholecystectomy with a diagnosis of complete obstruction of the cystic duct. The preoperative clinical and chemical findings were normal. Following cholecystectomy the patient recovered normally from the anaesthetic. Three hours later sudden cardiac arrest occurred. Necropsy revealed glycogen storage disease type I. Hypoglycaemia and metabolic acidosis had probably led to hypokalaemia which is considered as the cause of cardiac failure.

UI	MeSH Term	Description	Entries
D007003	Hypoglycemia	A syndrome of abnormally low BLOOD GLUCOSE level. Clinical hypoglycemia has diverse etiologies. Severe hypoglycemia eventually lead to glucose deprivation of the CENTRAL NERVOUS SYSTEM resulting in HUNGER; SWEATING; PARESTHESIA; impaired mental function; SEIZURES; COMA; and even DEATH.	Fasting Hypoglycemia,Postabsorptive Hypoglycemia,Postprandial Hypoglycemia,Reactive Hypoglycemia,Hypoglycemia, Fasting,Hypoglycemia, Postabsorptive,Hypoglycemia, Postprandial,Hypoglycemia, Reactive
D007008	Hypokalemia	Abnormally low potassium concentration in the blood. It may result from potassium loss by renal secretion or by the gastrointestinal route, as by vomiting or diarrhea. It may be manifested clinically by neuromuscular disorders ranging from weakness to paralysis, by electrocardiographic abnormalities (depression of the T wave and elevation of the U wave), by renal disease, and by gastrointestinal disorders. (Dorland, 27th ed)	Hypopotassemia,Hypokalemias,Hypopotassemias
D011183	Postoperative Complications	Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery.	Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D002763	Cholecystectomy	Surgical removal of the GALLBLADDER.	Cholecystectomies
D003645	Death, Sudden	The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions.	Sudden Death
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
D006323	Heart Arrest	Cessation of heart beat or MYOCARDIAL CONTRACTION. If it is treated within a few minutes, heart arrest can be reversed in most cases to normal cardiac rhythm and effective circulation.	Asystole,Cardiac Arrest,Cardiopulmonary Arrest,Arrest, Cardiac,Arrest, Cardiopulmonary,Arrest, Heart,Asystoles
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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