Treatment

Unfortunately there is no cure for progeria. All that can be done is monitoring vitals and stages the disease is in. The cardiovascular system is highly monitored as it is one of the systems in the body most effected by this disease. Some children undergo coronary bypass surgery or angioplasty to slow any cardiovascular disease. Other treatments or drugs used is low-dose aspirin to help prevent heart attacks and stroke. Physical therapy to help with joint stiffness and hip problems. High calorie supplements to ensure the child's nutrition is enough and to prevent weight loss. Some children require feeding tubes and the extraction of primary teeth, because adult teeth will come in faster then the baby teeth are lost. There is currently a drug that has been developed for treating cancer and has shown promise in certain studies in correcting the cell defects that cause progeria, however they are still being studied.

Causes

Researchers have found that a single gene, Lamin A (LMNA), mutation is responsible for Hutchinson-Gilford progeria. It makes a protein needed to hold a nucleus together and lacking it can lead to unstable cells, which seems to lead to the rapid aging characteristic. However, progeria isn't passed down in families, it is a occurence that happens to the egg or sperm cell just before conception. So neither parent of a child born with this disorder is the carrier. Some other kinds of progeroid syndromes do run in families though, such as Wiedemann-Rautenstrauch syndrome where the rapid aging begins in the womb, and signs and symptoms are apparent at birth.

Progeria: Introduction

Progeria is a rare genetic disease characterized by the appearance of fast aging in children. The name itself is greek for "prematurely old." The occurence of progeria happens in 1 out of every 4-8 million, effects all races and sexes equally, and occurs all over the world. It is noticed around 18-24 months of age and the signs include growth failure, hair loss, lack of body fat, aged looking skin, stiff joints, hip dislocation, atherosclerosis, and heart disease. Children with progeria have very similar appearance despite different ethnicities and usually die of heart disease around the age of 13, usually having a life-span of between 8-21 years.