Progeria: A Bizarre Children’s Disorder
It’s always disheartening to learn about diseases and disorders that affect children. Progeria is one such genetic disorder that can cause little ones to age rapidly. The word “rapid” doesn’t seem adequate in describing how quickly the aging process occurs. These children age at a pretty unbelievable speed which seems supersonic when compared to the rest of humankind. If a child possesses the genetic code, symptoms become apparent before he or she turns two years of age. It’s rare to find children with progeria who live longer than 13 years.
Progeria defined
Progeria is also known as Hutchinson-Gilford progeria syndrome, or (HGPS). At birth, children appear normal, but around 12 months of age hair loss and slow growth begin to appear. During their short life span, these children experience arthritis, heart disease, and other conditions commonly found in the elderly. Eventually, they succumb to their ailments and they die from heart problems or strokes. Regrettably, there is not a cure, although research continues and there is promise in the treatment of this condition.
Common symptoms of Progeria
At birth a child may seem normal, but as he approaches his first birthday symptoms may begin to appear. If your child experiences hair loss and skin changes along with slow growth and delayed fine or gross motor skills, you may want to call a doctor. Other symptoms include the following:
- Slow growth (height and weight below average)
- Hair loss, including eyebrows and eyelashes
- Protruding eyes
- Thin lips
- Scleroderma, or the hardening of skin, especially on extremities and on the trunk of the body
- Micrognathia, or a lower jaw that is notably small
- A voice that is high-pitched
- A narrow face
- Vein visibility
- Stiffness in joints
- Hip dislocation
- Resistance to insulin
- Irregular heartbeat
- Delayed tooth growth
- Decreased muscle mass and body fat
The cause of Progeria
The mutation of a single gene known as lamin A, or LMNA, is responsible for Hutchinson-Gilford progeria syndrome. The mutation is thought by researchers to make cells unstable. LMNA produces a protein that binds the nucleus of a cell together. Because the cells are unstable, it is believed that the protein isn’t effective and cannot bind the nucleus together. It is the cause of the characteristic aging appearance. Many genetic mutations are passed on from one family member to another, but this is not the case when it comes to progeria. Instead, it is believed to be something that happens by chance, and impacts a single egg or sperm just before they are joined. No parents are carriers of this disorder.
Complications that may arise with Progeria
Atherosclerosis, or severe hardening of the arteries, occurs in children with progeria. It is largely detrimental because it inhibits the body’s ability to carry oxygen and nutrient rich blood to tissues and organs. It can also make it difficult for blood to flow freely throughout the body. The most common cause of death among children with progeria is related to complications from atherosclerosis. Cardiovascular problems can occur, causing heart attack and congestive heart failure. Cerebrovascular problems can also occur, which happens when arteries and veins supplying blood to the brain are ineffective, resulting in stroke. Other health concerns such as Alzheimer’s disease and far-sightedness do not develop because of progeria.
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