FAMILIAL PERIODIC DISEASE WITH RENAL DAMAGE

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Abstract
A 16-year-old boy was hospitalized with symptoms and signs of acute glomerulonephritis. He had suffered since childhood from recurring attacks of fever with abdominal pain and rigidity. Treatment with various antibiotics was unavailing. Anemia increased and renal failure progressed until death, and autopsy disclosed an appendectomy scar, general amyloidosis of the secondary type, terminal pericarditis, and bronchopneumonia. The father and one brother of the patient had each undergone appendectomy for similar recurrent episodes of abdominal pain, and another brother had a similar history but without appendectomy. A fourth brother was healthy, as was the mother. It is believed that the four cases described were instances of familial periodic disease or recurrent polyserositis and that this possibility should be kept in mind in patients with symptoms of either nephritis or appendicitis.

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