Adult polycystic kidney disease

Introduction

Introduction to adult polycystic kidney Adult polycystic kidney disease is an autosomal dominant hereditary disease that is almost bilateral (95% of the total number of cases). Although it has been reported in the literature that infants have adult polycystic kidney disease, the disease occurs in infants different from adults. Infant polycystic kidney disease is an autosomal recessive hereditary disease, which prevents short survival. Adult polycystic kidney disease is usually 40 years old. No symptoms appear before, and multiple cysts of the same shape can be seen in the liver, spleen and pancreas. The volume of polycystic kidney is larger than that of normal kidney, and the surface is covered with cysts of different sizes. basic knowledge The proportion of illness: 0.001% Susceptible people: good for adults Mode of infection: non-infectious Complications: pyelonephritis hematuria

Cause

Adult polycystic kidney disease

Cause:

The cyst is caused by the structure of the collection tube and the tubule and the junction between them, and there is a defect in the development process. A blind end of the drainage tube is connected with the functional glomerulus to become a cyst. When these cysts are enlarged, the cysts are enlarged. It oppresses the adjacent renal parenchyma, causing ischemia and destroying its function and occluding normal renal tubules, eventually leading to progressive damage to renal function.

Prevention

Adult polycystic kidney disease prevention

The disease occurs in children, the prognosis is very poor, most patients with clinical signs and symptoms after 35 to 40 years old, the prognosis is much better, although there is a big difference between them, but these patients are usually 5 ~ after diagnosis Death within 10 years, unless effective dialysis treatment or kidney transplantation is performed. A reasonable treatment diet for patients with polycystic kidney disease is important for controlling the progression of renal function deterioration. Use a low-salt diet 2 to 3 grams of salt per day is appropriate, eat less potassium, phosphorus, diet, low protein, low-fat diet, eat more vitamin-rich and plant crude fiber diet, keep the stool smooth. Hope to adjust your lifestyle and diet under the guidance of your doctor, and do more with less to restore your kidney function.

Complication

Adult polycystic kidney disease Complications, pyelonephritis, hematuria

(1) pyelonephritis: a common complication of polycystic kidney disease, the cause is still unclear, there may be no symptoms, there are few or no pus cells in the urine, smear staining or quantitative culture can make a diagnosis, gallium citrate- 67 scanning photography can determine the location of the infection including the location of the abscess.

(2) Cyst infection: can cause pain in the kidney area and tenderness, and fever, it is not easy to identify polycystic kidney infection or pyelonephritis. At this time, gallium scanning photography will be very helpful.

(3) hematuria: In a very small number of cases, active persistent gross hematuria may occur, and may even be life-threatening.

Symptom

Adult polycystic kidney symptoms Common symptoms Nodular waist soreness and inability to hematuria Hemorrhage cyst Weight loss Low back pain with kidney area sputum pain Heart enlargement bilateral intra-renal pain nausea

A kidney that gains weight due to obstruction, infection, or cystic hemorrhage can cause pain in one or both sides of the kidney. Hematuria is common and can be quite serious under the microscope or the naked eye. The cause is still unclear. Renal colic can be expressed when a blood clot or stone descends. The patient can find a belly mass on his own.

Examine

Adult polycystic kidney disease examination

(1) Laboratory examination: Anemia can not only be caused by chronic blood loss, but also caused by inhibition of hematopoietic function caused by uremia. Urine routinely shows proteinuria and macroscopic or microscopic hematuria, pyuria and bacteriuria. It is common, the urinary enrichment function is progressively reduced, and the renal clearance test can show various degrees of impaired renal function. About one-third of patients with polycystic kidney disease are found to be uremia.

(2) X-ray examination: in the abdominal plain film, bilateral renal shadows usually increase, even up to 5 times the normal size, the kidney should be suspected of having a length of more than 16 cm, and performing tomography during excretory venography Helps to establish a diagnosis, tomography can show transparent multiple cysts, in tomography and retrograde urography, the kidneys often increase and the shape of the renal pelvis is very strange (such as spider-like deformity): renal pelvis widening , flattened, enlarged and often curved around the surrounding cysts, this change is often lighter or even does not occur in one side of the kidney, which may lead to misdiagnosis as a tumor or other kidney disease, when the cyst is complicated by infection, peri-renal inflammation can Renal shadows and the shadow of the psoas muscle are blurred. Angiography can show the "negative" shadow (no blood vessel distribution) formed by the small blood vessels that bend around the cyst and the cyst itself.

(3) CT scan: In the past, non-invasive diagnostic techniques for the diagnosis of polycystic kidney disease, CT is the most ideal, multiple cystic fluid in the thin cyst wall and more normal kidneys, making this An imaging method is extremely accurate (95%) at the time of diagnosis.

(4) Renal Nuclide Examination: A gamma-scintillation scan will show that there are many "cold spots" in the enlarged kidney shadow that are free of blood vessels.

(5) Ultrasound examination: Ultrasound imaging is superior to excretory urography and renal nucleus examination in the diagnosis of polycystic kidney disease.

(6) Device examination: Cystitis can be found in cystoscopy. At this time, urine contains abnormal components, and sometimes ureteral spurting is seen. Ureteral intubation and retrograde urography are rarely used.

Diagnosis

Diagnostic identification of adult polycystic kidney

diagnosis

According to the medical history, clinical manifestations and laboratory data is not difficult to make a diagnosis.

Differential diagnosis

(1) hydronephrosis (caused by congenital disease or acquired ureteral obstruction): bilateral flank abdominal mass and renal dysfunction may occur, but pyelography and ultrasonography will show these manifestations Polycystic kidney disease is very different.

(2) bilateral renal tumors: this disease is rare, but in the urinary tract angiography and polycystic kidney can be very imagined, when the polycystic kidney in one side of the kidney is small or urography is not showing distortion, with a The identification of lateral renal tumors is difficult, but the tumor is often confined to a certain part of the kidney, while the cyst of the polycystic kidney is full of the whole kidney. The total renal function of the unilateral renal tumor can be normal, while the total polycystic kidney is normal. Renal function is often impaired, CT or renal angiography can be used to identify the two diseases, scintigraphy or ultrasound can also help to differentiate the diagnosis.

(3) Von Hippel-Lindau disease (cerebellar hemangioma cyst, retinal angiomatosis and pancreatic tumor or cyst): multiple cysts or adenocarcinoma of the kidney can progress progressively, urography or renal tomography The technique can show polycystic kidney disease. According to other characteristic manifestations, the diagnosis can be made. CT, angiography, ultrasound imaging or scintigraphy can confirm the diagnosis.

(4) Nodular sclerosis (paroxysmal convulsions, mental retardation and sebaceous adenoma): sebaceous adenomas often involve the skin, brain, retina, bones, liver, heart and kidneys. Kidney lesions are usually multiple bilateral Angiolipoma can be seen under the microscope. Urinary tract urography during uremia is easy to suggest cystic kidney. Other characteristic manifestations and CT and ultrasound images can be identified.

(5) simple renal cyst: usually single-sided single, normal renal function, urography can be shown as a single lesion, while polycystic kidney disease is bilateral and multiple lesions.

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