What Are The Symptoms Of Hutchinson-Gilford Syndrome?

Hutchinson-Gilford syndrome (HGPS) is a rare and progressive disorder that causes children to age prematurely, often with an onset within the first few years of their life. This disorder is passed through parental genes to a child. The mutation of the LMNA gene is responsible for causing Hutchinson-Gilford syndrome. This particular mutation causes the body to incorrectly produce a protein needed for the nucleus of a cell to stay together. As a result, the cells in the body of individuals with this condition are unstable, and this abnormality leads to rapid aging. The life expectancy of a Hutchinson-Gilford syndrome patient is thirteen years on average, but some may live up to twenty years. Strokes or cardiovascular problems are commonly the cause of death in affected individuals. Children with this syndrome often appear normal when they are born, but symptoms begin to appear between ten and twenty-four months old in most cases.

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Slowed Growth

A child affected by Hutchinson-Gilford syndrome is usually born with an average weight, height, and body proportion. Within the first year of the child's life, however, they will experience stagnation in growth. They will gain less weight and will be much shorter for their age then they should be. Children affected by this condition will have no body fat and very little muscle. Between the ages of eighteen months and two years, the child will become very thin and have a larger head than normal. The child's primary teeth will come in several years later than usual. The growth of the child will slow down as their cells fail to duplicate at a regular rate.

The LNMA gene provides the code on how to produce parts of the filamentous lamina that are called laminins A and C. Functional laminins A and C are required for the cell's filamentous lamina to replicate DNA accurately, structurally support the nucleolus, and correctly transcribe RNA. A mutation in the LNMA gene results in a loss of the ability to produce normally functioning laminins A and C. When the cells inside of the tissues cannot replicate, the tissues of the body will fail to grow.

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