| [ Summary ] |
The main features of autosomal dominant polycystic kidney disease (ADPKD) are its phenotypic variability and its proliferative neoplasm-like character. The basic pathology can be divided into disorders, characterized by weak walls and by cysts. The former, includes vascular diseases, such as intracranial aneurysms, diverticula of the colon and dilatation of the common bile duct. The latter, include cysts in the kidneys, liver, pancreas, seminal vesicles, and the arachnoid membrane. The complications with these disorders include rupture of intracranial aneurysms, renal failure, hyper tension, gross hematuria, cystic bleeding, cystic infection and urinary calculi. The management of high blood pressure and renal function is considered to be the most important factor in control of this condition. The management of both renal and extra-renal manifestations requires careful consideration of both phenotypic variability and familial clustering, for example, MRA screening for intra cranial aneurysms in patients with a family history of intracranial bleeding. Another example, is that in the selection of treatment for symptomatic abdominal distention, consideration should be given to whether the mass effect is caused by one or few huge cysts or by numerous small cysts. We also need to know whether the patient has good renal functions. Prolonged administration of antibiotics, such as cyprofloxacin, may be necessary to effectively treat cystic infections in patients with ADPKD. |