Résumé
Hepatic cysts are the most common extrarenal manifestations of adult polycystic kidney disease, but the hepatic cyst infection in a patient with polycystic kidney disease is rare. The infection may remain localized or may be followed by acute cholangitis or septicemia. The best management for infected hepatic cyst is drainage in combination with antibiotic therapy. We experienced a case of infected hepatic cyst in a patient with polycystic kidney disease in a 46-year-old man receiving hemodialysis. He presented with fever and abdominal pain and was found to have an infected liver cyst. Treatment with antibiotics and percutaneous drainage of the cyst resulted in clinical improvement. Thus, we report a case of infected hepatic cyst in a patient with polycystic kidney disease with review of the literature.
Sujets)
Adulte , Humains , Adulte d'âge moyen , Douleur abdominale , Antibactériens , Angiocholite , Drainage , Fièvre , Foie , Polykystoses rénales , Polykystose rénale autosomique dominante , Dialyse rénale , SepsieRésumé
Adult polycystic kidney disease is a genetic disease characterized by bilateral multiple renal cysts and is transmitted as an autosomal dominant traits. The disease usually manifests in the fourth decade of life. The affected patients usually die with end stage renal failure, cardiovascular assaults and infection etc. Cardiovascular assaults include ruptured berry aneurysm, spontaneous intracranial hemorrhage, acute myocardial infarction, dissecting aortic aneurysm and hypertensive heart failure. Fatal intracranial hemorrhage and hypertensive heart failure occur in 15% and 2% of patients, respectively. Genetically-determined structural weakness in the arterial wall have been suggested as a possible factor in the genesis of berry aneurysm. We have experienced three autopsy cases with cardiovascular causes of sudden death. Two cases have intracranial hemorrhage (1 berry aneurysm and 1 intracerebral hemorrhage) and the remaining one is dead with hypertensive cardiomyopathy. All of them are aged over 40 years and associated with polycystic liver disease. Histologically, myriad cysts of enlarged bilateral kidney are lined by single layer of cuboidal cells with focal hyperplastic epithelial configuration. Diffusely scattered liver cysts are also lined by flat to cuboidal epithelium and often associated with portal fibrosis.
Sujets)
Adulte , Humains , Anévrysme de l'aorte , Autopsie , Cardiomyopathies , Hémorragie cérébrale , Mort subite , Épithélium , Fibrose , Défaillance cardiaque , Anévrysme intracrânien , Hémorragies intracrâniennes , Rein , Foie , Maladies du foie , Infarctus du myocarde , Polykystose rénale autosomique dominante , Insuffisance rénaleRésumé
Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.