Wolf-Hirschhorn (4p) Syndrome

Genetic Disorder Assignment - SeeJay Padilla

What is Wolf-Hirschhorn Syndrome?

Wolf-Hirschhorn Syndrome, also known as 4p deletion syndrome, is a condition that affects many parts of the body. Affected children with this disorder experience delayed growth and development and intellectual disability. Slow growth begins before birth. Infants usually have problems with feeding and gaining weight, and they have weak and underdeveloped muscles.

What causes this disorder, and where does it occur?

Wolf-Hirschhorn Syndrome is inherited chromosomal deletion that happens randomly during the event of forming reproductive cells or in early embryonic development. There are also prenatal tests to determine if the infant will have Wolf-Hirschhorn Syndrome.

The chromosome that this disorder occurs on chromosome 4. Wolf-Hirschhorn syndrome is caused by a deletion of genetic material near the end of the short (p) arm of chromosome 4.

What are the symptoms?

Affected children experienced delayed growth and underdevelopment. The children are delayed with motor abilities, like sitting, standing and walking. They also have a rather short stature. The infants tend to have problems with feeding and gaining weight. They experience intellectual disabilities, and usually will need help with daily actions. Patients also tend to have seizures.

The population affected by this disorder?

About 1 in every 50,000 infants are diagnosed with WHS. This might be an understatement as it's likely that some individuals are never diagnosed.

Wolf-Hirschhorn syndrome affects both genders, however, it happens almost twice as much in females, than males.

What will the patients need, and life expectancy?

Children with this disorder will probably have delayed development in motor skills such as sitting, standing and walking. Group physical therapy, social therapy, and genetic counseling. Some children can walk and talk, but others need help with all that.

21% of these children die within the first two years after birth. Though most can live out their lives. Some recorded ages of the children were in their 30's and 40's though life expectancies have been getting longer than recorded in the 60's and 70's.

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Prevention and cures?

There isn't much prevention for this disorder, but genetic counseling can determine whether or not your family has any genetic disorders, which leads to family planning.

The disorder has no cure, but there are treatments for the seizures, like valproic acid. Standard care should be provided for skeletal anomalies, heart defects, and hearing loss.