IPLab:Lab 5:Neurofibromatosis

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Contents

Clinical Summary

This 45-year-old divorced white male came to the emergency room with severe hepatic cirrhosisCirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules. and aspiration pneumoniaIn alcoholics, aspiration pneumonia is common--bacteria enter the lung via aspiration of gastric contents.. Shortly after admission he developed cardiac arrhythmiasArrhythmias are abnormal heart rhythms. and died. Significant past history included alcohol abuse, cirrhosisCirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules., and neurofibromatosis. He had no family history of neurofibromatosis, but his condition was diagnosed at age 17 when he developed neurofibromas along the lateral chest wall. There was no history of continued follow-up after this initial diagnosis.

Autopsy Findings

The patient was covered with variably sized subcutaneous nodules ranging from 0.5 to 2.5 cm in diameter. Other significant findings included micronodular hepatic cirrhosisCirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules. (liver weight: 1760 gramsA normal liver weighs 1650 grams (range: 1500 to 1800 grams).), ascites (500 ml)Ascities is the accumulation of fluid with in the abdominal cavity. There should be less than 30 ml of fluid in the peritoneal cavity., and splenomegaly (weight260 grams)Splenomegaly means enlargement of the spleen. The normal spleen weighs 155 grams.: 260 grams).

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Virtual Microscopy

Study Questions


Additional Resources

Reference

Journal Articles

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