Biomaterial Database

The Hutchinson-Gilford Progeria Syndrome: a case report. 2010

I Russo-Menna, and C Arancibias

Department of Anesthesiology, Division of Pediatric Anesthesiology, Virginia Commonwealth University, Richmond, VA 23298, USA. irussomenna@vcu.edu

The HGPS (Hutchinson Gilford Progeria Syndrome) is a rare genetic disorder with an incidence of 1 per 8 million live births. Originally described in 1886, less than 100 cases have been reported. The syndrome is characterized by premature aging with changes in many organs. The diagnosis is usually made by age 2, the mean survival age is 13.4 years and the most common cause of death is myocardial infarction. Recent genetic advances have identified the cause as a defect in the LMNA gene of chromosome 1.

UI	MeSH Term	Description	Entries
D007442	Intubation, Intratracheal	A procedure involving placement of a tube into the trachea through the mouth or nose in order to provide a patient with oxygen and anesthesia.	Intubation, Endotracheal,Endotracheal Intubation,Endotracheal Intubations,Intratracheal Intubation,Intratracheal Intubations,Intubations, Endotracheal,Intubations, Intratracheal
D011371	Progeria	An abnormal congenital condition, associated with defects in the LAMIN TYPE A gene, which is characterized by premature aging in children, where all the changes of cell senescence occur. It is manifested by premature graying; hair loss; hearing loss (DEAFNESS); cataracts (CATARACT); ARTHRITIS; OSTEOPOROSIS; DIABETES MELLITUS; atrophy of subcutaneous fat; skeletal hypoplasia; elevated urinary HYALURONIC ACID; and accelerated ATHEROSCLEROSIS. Many affected individuals develop malignant tumors, especially SARCOMA.	Hutchinson-Gilford Syndrome,Hutchinson Gilford Progeria Syndrome,Hutchinson-Gilford Progeria Syndrome,Hutchinson Gilford Syndrome,Hutchinson-Gilford Progeria Syndromes,Progeria Syndrome, Hutchinson-Gilford,Progeria Syndromes, Hutchinson-Gilford
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003793	Dental Restoration, Permanent	A restoration designed to remain in service for not less than 20 to 30 years, usually made of gold casting, cohesive gold, or amalgam. (Jablonski, Dictionary of Dentistry, 1992)	Dental Fillings, Permanent,Dental Filling, Permanent,Dental Permanent Filling,Dental Permanent Fillings,Dental Restorations, Permanent,Filling, Dental Permanent,Filling, Permanent Dental,Fillings, Dental Permanent,Fillings, Permanent Dental,Permanent Dental Filling,Permanent Dental Fillings,Permanent Dental Restoration,Permanent Dental Restorations,Permanent Filling, Dental,Permanent Fillings, Dental,Restoration, Permanent Dental,Restorations, Permanent Dental
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000768	Anesthesia, General	Procedure in which patients are induced into an unconscious state through use of various medications so that they do not feel pain during surgery.	Anesthesias, General,General Anesthesia,General Anesthesias
D014081	Tooth Extraction	The surgical removal of a tooth. (Dorland, 28th ed)	Extraction, Tooth,Extractions, Tooth,Tooth Extractions