Progeria

Progeria, the name of a daunting rare and fatal disease can also be referred to more specifically as Hutchinson-Gilford Progeria Syndrome, or the acronym of this term, HGPS (Chara, 2002; Guang-Hui et al., 2011; Gruenbaum, 2009). Progeria originates from the Greek word “geras” which means “old age” (Gulli & Mallory, 2002). The prefix “pro-” means to advance forward so the combination of these two terms create the meaning “prematurely old” (Wynbrandt & Ludman, 2000). Dr. Hastings Gilford is credited with coining the name in 1904, combining his and Dr. Jonathan Hutchinson’s names. (Gulli & Mallory, 2002; Wynbrandt & Ludman, 2000). Dr. Hutchinson, in 1886, and Dr. Gilford, in 1897, are the first scientists known to study Progeria. Both the men…

Introduction Hutchinson Gilford Progeria Syndrome (HGPS) is a fatal disease that is characterized by the appearance of rapid aging in children. HGPS is rare, affecting roughly 1 in 8 million live births. HGPS can be identified by early symptoms that include slow development, limited growth, alopecia, localized scleroderma, and a distinct facial structure which includes a small face, shallow jaw, and pinched nose. As the disease progresses, new symptoms arise, such as hearing and vision…

Progeria In Greek, progeria, means prematurely aged. There are two types of progeria. The most common, is Hutchinson-Guilford. Wiedemann-Rautenstrauch syndrome is the second type of progeria, which appears in children while they’re still in utero (Erickson Gabbey, 2016). For years, scientists had researched in an attempt to learn the cause of this disease that affects children. After decades of scientific research, the gene for Progeria Syndrome was found in April 2003 by a group of researchers…

Pathophysiology of Hutchinson-Gilford Progeria Syndrome In order to conduct genetic research studies in hopes of finding a cure for the fatal disease progeria, one must understand how the disease affects the human body. Patients diagnosed with progeria demonstrate clinical signs of accelerated aging, including skin atrophy, osteoporosis, loss of subcutaneous fat and muscle, arthritis, poor growth, and alopecia. These manifestations are presented as a result of defects in the body’s processing…

change their lives forever. Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), was initially discovered in England in 1886 and is an extremely rare and fatal childhood disease, affecting only 1 in 20 million individuals[1]. There are anywhere from 200-400 children worldwide at any given time living with the condition[1,2], and it seems to affect both genders equally[1]. Another…

Background Progeria is an extremely rare form of dwarfism, affecting a mere 200-250 children worldwide at a time (Gordon, 2013).Classically known as Hutchinson-Gilford progeria syndrome, HGPS is a disease that causes the body to "prematurely age". Manifestations of this disease affects the skin, musculoskeletal system, and vasculature. The term progeria is derived from the Greek word geras, meaning "old age" (Shah, 2015). Dr. Jonathan Hutchinson first described this syndrome in 1886, and…

Progeria By:America Ortiz Hutchinson-Gilford progeria, also known as progeria is a disease that causes children to age rapidly. Progeria is very rare. There are less than 1,000 cases a year in the U.S. According to Mayo Clinic a child with progeria has a lifespan of thirteen years. Children with progeria often have fragile bones, stiff joints, and hip dislocations. Plus, children with progeria have may have delayed and abnormal tooth formation, which causes them to have different looking…

Progeria also known as Hutchinson-Gilford Progeria syndrome is an extremely rare genetic disease where a person is born with the characteristics of an aged body. The word Progeria comes from the greek words “pro” meaning premature and “geras” meaning old age. This genetic disease only occurs in an estimated 1 in 8 million individuals, and there are no hereditary factors or environmental factors which affect the cause of progeria. The average life of an individual with Progeria is usually between…

Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic mutation that causes children to age rapidly and die at a young age. “Progeria” is derived from Greek and translates to prematurely old. It is a mutation in the LMNA gene, which produces the Lamin A protein. The Lamin A protein is responsible for the support of the nucleus, In a HGPS free person, the nucleus produces Lamin A properly and is strong and stable, but in someone who has Progeria the cell produces progerin which makes the…

Genetic testing is a common medical practice used by doctors and researches that identifies irregular mutations and/or defective genes that are susceptible to causing harmful genetic disorders. “One of a Kind” and “Advances in Kids’ Early Aging Disease” are two well-written articles that examine different genetic disorders and the threats they pose on children. Seth Mnookin, author of “One of a Kind,” tells the story of Bertrand Might, who at the time was the only known patient suffering from a…