What Are the Causes of Adult Polycystic Kidney Disease?

Adult polycystic kidney disease is an autosomal dominant hereditary disease that is almost always bilateral. Adult polycystic kidney disease usually does not show symptoms until the age of 40, and 50% of patients over 60 years of age enter end-stage renal failure. In addition to affecting the kidneys, it can also cause cysts in the liver, spleen, pancreas, ovary and other organs, as well as heart valve disease and cerebral aneurysms. Polycystic kidneys are larger than normal kidneys, and the surface is covered with cysts of varying sizes. Because the disease can occur at any age, even the fetus, adult polycystic kidney disease is not accurate.

Basic Information

Visiting department

Nephrology

Multiple groups

Adults

Common locations

kidney

Common causes

Local dilatation between glomerular Bowman's capsule and collecting duct

Common symptoms

Headache, nausea, vomiting, fatigue, weight loss, anemia, hypertension, etc.

Causes of polycystic kidney disease in adults

Renal cyst is a local expansion between the glomerular Bowman's capsule and the collecting duct. When the diameter of the expansion is several millimeters, it will fall off from the original renal tubule and form an independent, fluid-filled cyst. When these cysts enlarge, they will compress the adjacent renal parenchyma, cause ischemia and destroy its function, and block normal renal tubules, eventually leading to progressive damage to renal function.

Clinical manifestations of polycystic kidney disease in adults

Symptoms

The kidneys that have gained weight due to obstruction, infection, or hemorrhage in the cyst cause tension to the renal vascular pedicle, which can cause pain in one or both sides of the lower back and abdomen. Microscopic or gross hematuria is common. When blood clots or stones descend, renal colic can manifest. Patients can find a abdominal mass on their own. Infections (fever, renal pain) are common complications of polycystic kidney disease. Bladder irritation can be the first symptom. With the occurrence of renal insufficiency, headache, nausea, vomiting, fatigue, and weight loss can occur.

2. Signs

Can often touch one or both kidneys, the surface is nodular. With concurrent infections, tenderness may be present. Hypertension may be seen in some patients and the heart may be enlarged. Fever can occur when pyelonephritis or cyst infection occurs. There may be significant anemia and weight loss during the uremia phase. Ophthalmoscope reveals typical changes in hypertension from moderate to severe.

Adult polycystic kidney examination

Laboratory inspection

Urine routinely shows proteinuria and gross or microscopic hematuria. Pyuria and bacteriuria are also common. Progressive decline in urinary concentration. Creatinine clearance tests can show various degrees of impaired renal function. One third of patients with polycystic kidney disease are found with uremia.

2.X-ray inspection

In abdominal plain film, bilateral kidney shadows usually increase, even up to 5 times the normal size, and the disease should be suspected when the kidney length exceeds 16 cm. Tomography during excretory venography is helpful to establish the diagnosis, and tomography can show transparent multiple cysts. In tomography and retrograde urinary tract films, the kidney is often enlarged and the shape of the kidney is very strange (such as spider-like deformities): the kidney is widened, flattened, enlarged and often bends around the surrounding cyst. This change is often mild or even non-existent on one side of the kidney, which can lead to misdiagnosis of a tumor or other kidney disease. When the cyst is complicated by infection, perirenal inflammation can make the kidney shadow and psoas muscle shadow blurred. Angiography can show small curved blood vessels around the cyst and the negative shadow (no vascular distribution) formed by the cyst itself.

3.CT scan

CT has a high accuracy in the diagnosis of polycystic kidney disease, and can detect cysts of 0.3 to 0.5 cm, but it is not the first choice because of the high cost. CT can provide valuable information when cysts become infected and bleed.

4. Renal nuclide test

Gamma-scintillation scans will show many non-vascular "cold spots" in the enlarged renal shadow.

5. Ultrasound

Ultrasound is highly sensitive, non-radioactive, non-invasive, economical, and simple. It is the preferred method of auxiliary examination.

6. Instrument inspection

Cystoscopy can reveal cystitis and sometimes ureteral spurts.

Diagnosis of polycystic kidney disease in adults

Diagnosis can be made based on the cause, clinical manifestations, and related tests.

Adult polycystic kidney disease

Unless there are uncommon complications, conservative and supportive therapies are available.

General measures

Pay attention to rest, avoid smoking, alcohol, strong tea, coffee and chocolate. Low-salt diet is required when you have high blood pressure. Physical work should be done within your ability. When the cyst is large, avoid strenuous exercise and abdominal trauma. When patients are decompensated with renal insufficiency, they need a low-protein diet.

2. Surgery

There is no evidence that removing cysts or reducing cystic compression can improve renal function. If it is found that the cyst is large and oppresses the upper ureter, causing obstruction and further impaired renal function, it can be removed or the internal cystic fluid can be aspirated. When the degree of renal insufficiency is life-threatening for patients, dialysis treatment or kidney transplantation should be considered.

3. Complication treatment

Pyelonephritis should be actively treated to prevent further impairment of renal function. Incision and drainage are needed for cyst infection. When the bleeding on one side of the kidney is severe and very dangerous, nephrectomy or renal artery embolization can be performed, and segmental arterial embolism can also be performed. Concomitant diseases (such as tumors and obstructive stones) require surgical treatment.