What is Polycystic Kidney Disease?
Polycystic kidney disease is a congenital disease characterized by the replacement of renal tissue with many different sized cysts. The disease, as a rule, is bilateral, often is hereditary.
Pathogenesis during Polycystic Kidney Disease
The etiopathogenesis of the Polycystic kidney is based on abnormal ontophilogenesis with incomplete and incorrect fusion of straight and convoluted tubules, difficulty in the outflow of urine and transformation into cystic formations. Along with abnormal nephrons, there are also properly formed nephrons.
Polycystic kidney disease is combined with other abnormalities of the kidneys and other organs.
Pathological Anatomy
The appearance of polycystic kidneys resembles a bunch of grapes. Morphologically, there are cysts of various sizes (sometimes up to 6-8 cm in diameter) that fill almost the entire organ. The pelvis and calyx are deformed, elongated. The kidneys are significantly enlarged, their mass can reach 1-2 kg. The occurrence of large cysts in the area of the gates of the kidneys causes hemodynamic disorders.
Symptoms of Polycystic Kidney Disease
In most cases, the disease is asymptomatic for a long time and is often detected by accident at autopsy. The first symptoms of the disease can be general and local. Common fatigue, thirst. increased blood pressure, sometimes slight fever. Blood pressure initially increased slightly and inconsistently. Local symptoms include lower back pain, and sometimes urination disorders. On palpation of the abdominal cavity, enlarged tuberous kidneys are found. In the urine, small proteinuria is detected, micro-, less often, macrohematuria. The functional ability of the kidneys is slightly reduced.
The clinical picture of chronic renal failure with polyuria and thirst, azotemia, anemia further develops. Arterial hypertension becomes high and stable, which is associated with increasing renal ischemia. The functional ability of the kidneys is a lower-1 wife, isohypostenuria is noted. Massive hematuria may occur. Joins chronic pyelonephritis, often exacerbating. Cysts can suppurate; calculi form in the kidneys.
Forecast
Polycystic kidney disease progresses slowly. Even in cases of chronic renal failure, patients live for several years. Joining chronic pyelonephritis, very high hypertension significantly worsen the prognosis. In approximately 20% of cases, polycystic disease does not manifest itself and can be detected by accident.
Diagnosis of Polycystic Kidney Disease
The presence of tuberous tumor formations in both renal regions is a pathognomonic sign of polycystic kidney disease and confirms the diagnosis. However, palpation of enlarged kidneys is not always possible, especially in the early stages of the disease. A decisive role in the diagnosis of polycystic is played by x-ray examination, primarily excretory urography, preferably in the infusorian version, in which the elongation and extension of the pelvis and calyx are determined. In unclear cases, retrograde pyelography is performed. Kidney scans and isotopic renography also help clarify the diagnosis.
Differential diagnosis is carried out with chronic nephritis and chronic pyelonephritis, especially in the presence of renal failure. If only one kidney is palpable, it is necessary to differentiate polycystic kidney disease with tumors. Crucial in all cases is an X-ray examination.
Polycystic Kidney Treatment
Conservative treatment comes down to fighting infection and chronic renal failure. The treatment of hypertension is essential.
Surgical treatment is effective only in the compensated stage of the disease and comes down to opening and emptying large cysts. The kidneys are reduced in size, their blood circulation and function are improved. The positive results of kidney transplantation are described.