Hutchinson Gilford Progeria Syndrome Essay

Hutchinson-Gilford progeria syndrome (HGPS), also referred to as Progeria, was first discovered in 1886 by Jonathan Hutchinson. Later in 1194, it was named by Hastings Gilford (“About Progeria”). The name of the syndrome comes from the word geras, which is a Greek word that translates into “prematurely old” (Gosia, Mehual M, et al. 27). Segmental progeroid syndromes are syndromes in which the individuals who are affected show characteristics of premature, accelerated aging (Ramirez, C L, et al. 155). Other progeroid syndromes like Hutchinson-Gilford progeria syndrome that manifest themselves at different ages include Cockayne syndrome, Emery-Dreifuss syndrome, Werner Syndrome, Seckel Syndrome, Rothmund-Thompson syndrome, and Wiedemann-Rautenstrauch …show more content…
Affected children start to experience extreme problems and delays in growth around nine to 24 months of age. These growth delays include low weight and short stature. They also acquire specific facial features that cause their faces to not be proportioned to their heads. Other characteristic features include faint blueness around the mouth, a small nose, undersized jaw (micrognathia), crowding and deformation of teeth, and bulging eyes. HGPS patients also experience alopecia, which is an autoimmune disorder that results in hair loss. This could cause the eyelashes, eyebrows, and scalp hair to be replaced by small, white hairs. Other typical features include stiffness of the joints, hip dislocations, atherosclerosis, heart disease, loss of subcutaneous adipose layer, strokes, and veins that bulge in the scalp. Patients with HGPS have life threatening complications due to losing the elasticity in their artery walls. They experience symptoms like angina, heart failure, high blood pressure, enlarged heart, and other conditions commonly affiliated with aging and heart disease. Ninety percent of HGPS deaths are directly related to atherosclerosis complications (Hutchinson-Gilford