Adult polycystic kidney disease is an autosomal dominant disease, almost all bilateral (accounting for 95% of the total number of cases). Although babies have been reported to have adult polycystic kidney disease in the literature, but the disease occurs in infants unlike adults. Infantile polycystic kidney disease is an autosomal recessive hereditary disease. It prevents the survival of the kidney in a short period, while the adult polycystic kidney usually does not show symptoms before the age of 40. The same form of multiple cysts can be seen in the liver, spleen and pancreas. The size of the polycystic kidney is larger than that of the normal kidney, and its surface is covered with cysts of different sizes.
It has been suggested that the cyst is due to the structure of the collecting duct and the renal tubule and the junction between them and has defects in the development process. A blind excretory duct is associated with a functioning glomerulus and becomes a cyst. When these cysts are enlarged, the adjacent renal parenchyma is compressed, causing ischemia to destroy its function and block normal tubules, resulting in progressive impairment of renal function.
(1) symptoms: because of obstruction, infection or bleeding of cyst, the kidney is pulled by the heavy weight of the blood vessel, which can cause the pain of one side or both sides of the kidney. Microscopic or macroscopic hematuria is common and can be very serious, and the cause is unknown. When the clot or stone goes down, it can manifest renal colic. The patient can find a lump of abdominal mass on his own. Infection (plug, fever, renal pain) is a common complication of polycystic kidney disease. Symptoms of bladder irritation can be the first symptom. With the occurrence of renal insufficiency, headache, nausea, vomiting, fatigue and weight loss can occur.
(2) signs: often touch one or both kidneys, the surface is nodular. Tenderness may be present when complicating infection. Hypertension can be found in 60% to 70% of patients, and evidence of cardiac enlargement can be found immediately. Fever occurs when pyelonephritis or cyst is present. Uremia can have obvious anemia and weight loss. Fundus examination revealed typical moderate to severe changes in hypertension.
(1) laboratory examination: anemia can be caused not only by chronic blood loss, but also due to the inhibition of hematopoiesis. Routine urine visible proteinuria and hematuria, pyuria and bacteriuria are common. Progressive loss of urine concentrating function. Renal clearance tests can show various levels of impaired renal function. About 1/3 of patients with polycystic kidney disease were found in uremia.
(2) x ray examination: in the abdominal radiograph, bilateral renal shadow usually increases, even up to 5 times the normal size. When the length of the kidney exceeds 16cm, it should be suspected. In the discharge of tomography venography was helpful in establishing the diagnosis, tomography can display transparent cysts. In tomography and retrograde radiography, the kidney is enlarged, while the shape of the renal calyx is very bizarre (such as spider like deformity): the calyx is widened, flattened, enlarged, and often bent around the cyst around it. This change usually occurs on one side of the kidney, even less, and can easily lead to misdiagnosis as a tumor or other kidney disease. Cyst with infection, renal inflammation can make kidney and psoas muscle was blurred as. Angiography shows small negative vessels around the cyst and the negative image of the cyst itself (without vascular distribution).
(3) CT scan: CT is ideal for non-invasive diagnostic techniques for the diagnosis of polycystic kidney disease in the past. Multiple cysts, cysts in the thin wall, and enlarged kidneys make this imaging modality very accurate at diagnosis (95%).
(4) renal scintigraphy: gamma scintigraphy will show an enlarged renal image with many avascular "cold spots"".
(5) ultrasonography: ultrasonography is superior to excretory urography and renal radionuclide examination in the diagnosis of polycystic kidney disease.
(6) instrument examination: cystoscopy can detect cystitis, which contains abnormal components in urine. Sometimes you can see ureterostoma spurting. Ureteral catheterization and retrograde urography are rarely used.
Conservative and supportive therapies may be used unless there are minor complications.
(1) general measures: patients should have low protein diet (protein intake of 0.5 ~ 0.75/kg/d), and forced to drink at least 3000ml liquid every day. Physical labor should act, prohibit strenuous exercise. When the patient is in the decompensation stage of renal insufficiency, the patient should be treated with uremia. At this point, hypertension should be controlled and can be marked with dialysis.
(2) surgery: there is no evidence that the removal of cysts or the reduction of cyst pressure can improve renal function. If a large cyst is found to compress the upper ureter, causing obstruction and further damage to the renal function, it may be removed or pumped out of its internal capsule. Dialysis treatment or renal transplantation should be considered when the extent of renal dysfunction is threatening the patient's life.
(3) the treatment of complications: pyelonephritis should be treated closely to prevent further impairment of renal function. Incision and drainage should be performed when the cyst is infected. When the bleeding of one side of the kidney is serious and very dangerous, we can do renal resection or renal artery embolization, or do segmental arterial embolization. Concomitant diseases, such as tumors and obstructive stones, require surgical treatment.
This disease occurs in children with a poor prognosis, and the prognosis is much better in most patients who show signs of clinical symptoms after 35~40 years of age. Although there is a large difference between these patients, but these patients usually die within 5~10 years after the diagnosis, unless effective dialysis or renal transplantation is performed.
(1) hydronephrosis (caused by congenital or acquired ureteral obstruction): bilateral abdominal masses and impaired renal function. But renal pelvis venography and ultrasonography will show that these findings are markedly different from those of polycystic kidney disease.
(2) bilateral renal tumors: this disease is rare, but it is very common in the urine film with polycystic kidney disease. When one side of the kidney in the polycystic kidney is smaller or the urography does not show distortion, it is difficult to differentiate from the renal tumor on one side. But the tumor is usually confined to only one part of the kidney, whereas the cyst in the polycystic kidney is covered with the entire kidney. Unilateral renal tumors may be normal in their general renal function, whereas the renal function of the polycystic kidney is often impaired. CT or renal angiography can be used to identify the two diseases. Scintigraphy or ultrasonography is also helpful in differential diagnosis.
(3) Von Hippel-Lindau disease (cerebellar angioma cyst, retinal angioma and pancreatic tumor or cyst): Double cyst or adenocarcinoma of the kidney can develop. Urography or renal tomography with X may show polycystic kidney disease, which can be diagnosed according to other characteristics. CT, angiography, ultrasonography, or scintigraphy can be used to confirm the diagnosis.
(4) tuberous sclerosis (paroxysmal convulsions, mental retardation, and sebaceous adenoma): sebaceous adenoma often affects the skin, brain, retina, skeleton, liver, heart and kidneys. The lesion of the kidney is usually multiple and bilateral, and a lipoma can be seen microscopically. In uremic stage, urography is easy to suggest cystic kidney, other features and CT and ultrasound can be identified.
(5) simple renal cyst: usually unilateral, with normal renal function. Urography can be seen as a single lesion, whereas polycystic kidneys are bilateral and multiple lesions.
(1) pyelonephritis: it is the common complication of polycystic kidney, the reason is unknown. No symptoms can occur. There are few or no purulent cells in the urine. Smear staining or quantitative culture can be used for diagnosis. Gallium citrate -67 scan can determine the location of the infection, including the location of the abscess.
(2) cyst infection: it can cause pain in the renal region, tenderness, and fever. It is not easy to identify whether it is a polycystic kidney infection or pyelonephritis, and it would be helpful to do gallium scanning at this time.
(3) hematuria: very few cases of persistent hematuria, and even life-threatening.
Adult polycystic kidney, eat what food is good to the body:
1, vitamin high fruits and vegetables.
2, fruits and vegetables rich iron , such as carrots.
3, can hold stone fossils of vegetables and food, such as black fungus.
4, a diuretic effect of food.
5, alkaline food is also more use, can play a supporting role in the treatment.