As much as i love to learn about commonly known dieseases in class one diesease that striked me as i was watching a tv show last year was progeria. Progeria is derived from the Greek work meaning prematurely old. Jonathan Hutchinson and Hastings Gilford discovered the syndrome in 1886. The name Hutchinson Gilford progeria syndrome existed because of this. doctors have discovered a single gene mutation responsible for Hutchinson-Gilford progeria syndrome. The gene is called lamin A this is the protein necessary for holding the center of a cell together. Researchers believe the genetic mutation enables the cell to be unstable, which appears to lead to progeria's characteristic aging process. As newborns with progeria it usually appear normal but as times progess the cchildrens growth ceases and they start developing characteristics of the eldelry.
A study from the Netherlands has shows that 1 in 4 million births (within the Netherlands) are diagnoesed with progeria Currently, there are 80 known cases in the world. Approximately 140 cases have been reported in medical history. Classical Hutchinson-Gilford Progeria Syndrome is usually caused by a odd mutation taking place during the early stages of embryo development. It is almost never passed on from affected parent to child, as affected children rarely live long enough to have children themselves.There's no cure for progeria Regular monitoring for cardiovascular disease may help with managing your child's condition. Some children undergo coronary artery bypass surgery or dilation of cardiac arteries to slow the progression of cardiovascular disease. Drugs known as FTIs, which were developed for treating cancer, have shown hope in laboratory studies in correcting the cell defects that cause progeria. FTIs are currently are being studied in human clinical trials for treatment of progeria.
interstingly Children affected with progeria age rapidly – about 7 to 10 times faster than normal