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How to understand Russell Silver Syndrome


April 3, 2009

Russell Silver Syndrome is a condition that is present at birth in which the infant has difficulty with growing and feeding. The facial features of children with Russell Silver Syndrome are distinctive and some have asymmetric arms and legs. Russell Silver Syndrome does not affect a person life expectancy but many patients as teenagers and adults are shorter than average.

Cause of Russell Silver Syndrome

The exact cause of Russell Silver Syndrome is not actually known, however, it is present more often in families that have a medical history of the syndrome. In around 10% of those with Russell Silver Syndrome have a defect in the chromosome 7. The syndrome can affect all races along with males and females alike.

Symptoms of Russell Silver Syndrome

The main symptoms is the failure to grow normally however, other symptoms are also present which include:

•  Delayed bone age

•  Low birth weight

•  Poor growth

•  Short height

•  Different length arms

•  Different length legs

•  Pinky finger is curved or fused

•  Ring finger is curved or fused

•  Short, stubby fingers

•  Short, stubby toes

•  Cafe-au-lait

•  Short arms

Facial Features

•  Small triangular face

•  Wide forehead

•  Small, narrow chin

•  Prominent nasal bridge

•  Down-turning corners of the mouth

•  Normal sized head

Kidney problems and other health problems

•  Failure to thrive

•  Gastroesophageal reflux disease

•  Horseshoe kidney

•  Hydronephrosis

•  Posterior urethral valves

•  Renal tubular acidosis

•  Swelling of the esophagus

Treatment of Russell Silver Syndrome

Generally, the best treatment while in infancy is to ensure the infant is consuming enough calories in order to grow. In some cases, a feeding tube may be necessary.

In many cases, human growth hormone therapy will also be given to help the body produce enough of this vital hormone in which to grow properly.

Some children may have other problems due to Russell Silver Syndrome and may need therapy in other areas to help with language problems or other educational problems.

Complications that can arise with Russell Silver Syndrome

Patients with Russell Silver Syndrome may develop other complications such as learning disabilities, if the jaw is small speaking and chewing can be difficult, and emotional problems due to their appearance.

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