Biomaterial Database

Report on a case of Hutchinson-Gilford progeria, with special reference to orthopedic problems. 1992

F Fernandez-Palazzi, and A T McLaren, and D F Slowie

Head Service C of Orthopedics, Hospital San Juan de Dios, Caracas, Venezuela.

Hutchinson-Gilford progeria is a very rare syndrome of premature aging and often features many orthopedic abnormalities. This is a case report on a young boy suffering from progeria. His orthopedic history included bilateral talus deformities of the feet, bilateral dislocated hips, pes planus, a fractured femur (which healed without complications), aseptic necrosis in the left nuclear head of the femur, bilateral fixed hip flexion deformities, bone dysplasia, osteoporosis and osteolysis.

UI	MeSH Term	Description	Entries
D008297	Male		Males
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
D001847	Bone Diseases	Diseases of BONES.	Bone Disease,Disease, Bone,Diseases, Bone
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D005264	Femoral Fractures	Fractures of the femur.	Femoral Fracture,Fracture, Femoral,Fractures, Femoral
D005530	Foot Deformities	Alterations or deviations from normal shape or size which result in a disfigurement of the foot.	Metatarsal Deformity,Deformities, Foot,Deformities, Metatarsal,Deformity, Foot,Deformity, Metatarsal,Foot Deformity,Metatarsal Deformities
D006617	Hip Dislocation	Displacement of the femur bone from its normal position at the HIP JOINT.	Hip Displacement,Hip Dysplasia,Dislocation, Hip,Dislocations, Hip,Displacement, Hip,Displacements, Hip,Dysplasia, Hip,Dysplasias, Hip,Hip Dislocations,Hip Displacements,Hip Dysplasias
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man