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Liver Transplant may be the Answer for Lathosterolosis

by Dr. Simi Paknikar on  March 15, 2014 at 12:16 PM Health In Focus   - G J E 4
Liver transplant may be the answer for a rare disorder called lathosterolosis, statesa case report published online by the American Journal of Transplantation.
Liver Transplant may be the Answer for Lathosterolosis
Liver Transplant may be the Answer for Lathosterolosis
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Lathosterolosis is a rare disorder with currently only one living patient diagnosed with the condition. It occurs due to a defect in cholesterol synthesis and leaves the patient with multiple congenital anomalies, mental retardation and liver failure.

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Dr Pier Luigi Calvo of the Cittá della Salute e della Scienza in Italy and his colleagues did a liver transplant in the only living girl with the condition when she progressed to liver failure by the ages of 7 years. Prior to this, the girl already suffered from autistic behavior, difficulty with walking and cataract in the eyes.

Following the transplantation, cholesterol synthesis in the girl returned to normal within a year and her walking improved. Further deterioration in her mental state stopped around five years after the transplant.

The physicians thus suggest that there is a link between the normal functioning of the liver and the brain. Diseases related to abnormal cholesterol metabolism which are associated with abnormal brain functioning can probably be managed by timely intervention in the form of liver transplantation. However, the transplantation may not be able to reverse the mental changes that have already occurred.

Liver transplant is commonly done for conditions that cause extensive liver damage. These include hepatitis B and C infections, alcoholic liver disease and liver cancers. Drugs which suppress immunity are given following the transplant to prevent rejection.

Source: Medindia
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