Polycystic kidney disease with extrarenal manifestations (clinical case)
A.R. BOGDANOVA
Kazan (Volga region) Federal University, Kazan
Contact details:
Bogdanova A.R. — PhD (medicine), Associate Professor of the Department of Internal Diseases
Address: 76 Karl Marks St., Kazan, Russian Federation, 420012, tel.:+7-927-246-63-94, e-mail:_alinochka@mail.ru
Polycystic kidney disease is a genetically determined pathological process associated with the formation and progression of cysts in kidneys. The disease has two types — autosomal dominant and autosomal recessive. Autosomal dominant polycystic kidney disease, dominating among the causes of irreversible deterioration of the kidneys filtration function, can remain asymptomatic for a long time, manifesting itself mainly as arterial hypertension and minimal changes in urine (microhematuria). The growth of renal cysts leads to the appearance of hypercreatininemia, usually in the fifth decade of patients’ life. Of particular importance in the timely diagnosis of autosomal dominant polycystic kidney disease are its extrarenal manifestations — cysts of the liver and spleen, aneurysms of intracranial vessels, often complicated by rupture with fatal hemorrhage, hernias of the linea alba, anomalies of the heart valves. The presented clinical case clearly demonstrates the development of polycystic kidney disease with multiple extrarenal manifestations.
Key words: polycystic kidney disease, kidney cysts, gross hematuria, extrarenal manifestations.
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