I. What is polycystic kidney?
The autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are divided into two types of polycystic kidney disease: ARPKD, which often develops in infants and children and rarely lives into adulthood. ADPKD is one of the most common monogenic genetic diseases in human beings and is autosomal dominant, with an incidence of about 1/400 to 1/1000, accounting for the third most common monogenic genetic disease in human beings, with the following genetic characteristics: it is transmitted from generation to generation, with equal probability of disease in men and women, and a 50% chance of disease in children if one parent has the disease. Polycystic kidney is characterized by the presence of numerous and growing fluid-filled cysts in both kidneys, which progressively increase in size and eventually destroy the structure and function of the kidneys, leading to end-stage renal failure. 50% of patients develop end-stage renal failure (uremia) at the age of 60, accounting for about 8% to 10% of all patients with end-stage renal failure, and is the fourth leading cause of end-stage renal failure worldwide. It is the fourth major cause of end-stage renal failure in the world.
Second, the clinical manifestations of polycystic kidney clinically most patients can have a family history, the performance varies and can be accompanied by multiple cysts in other organs, the most common being liver cysts. The common manifestations are.
(1) Pain in the lower back and abdomen: it is the most common early symptom, and the pain is aggravated if there is bleeding or infection in the cyst.
(2) Hypertension: Hypertension is one of the most common early manifestations of ADPKD, and the level of blood pressure is proportional to the size of the kidney and the number of cysts, and it keeps rising with age.
(3) Abdominal masses: enlarged kidneys can be palpated on abdominal palpation with uneven surface, and in some patients, the kidneys may even reach the pelvis.
(4) Renal function impairment: In the early stage of polycystic kidney patients, renal function is mostly in the normal range, but as the disease progresses, renal insufficiency, failure and even uremia begin to appear. about 45% of patients over 60 years old progress to uremia. About 60% to 80% of patients eventually have renal failure and enter the uremic phase, and must rely on long-term hemodialysis or kidney transplantation.
(5) Stones: About 1/5 of patients have stones.
(6) Infection: Infection can occur in the renal parenchyma or cyst, mostly unilateral, and can cause symptoms such as high fever, Korean war, and back pain.
(7) Hematuria: It occurs in about half of the people with different degrees, and stones and infection are the main causes of hematuria.
The above symptoms are common symptoms of polycystic kidney patients, and patients can show one or several of them, but some of them can have no clinical manifestations.
Third, the diagnosis method of polycystic kidney and the indication of surgery for family members with family history of polycystic kidney, ultrasound or CT examination is feasible to confirm the presence of polycystic kidney; patients who are diagnosed with polycystic kidney need to regularly review renal function, imaging, renal function and frequently measure blood pressure, if patients have no conscious symptoms, normal renal function, small cysts and no other complications, they can temporarily observe the diet and avoid the application of nephrotoxic drugs in life; and Regular re-examination of ultrasound, renal function, etc. in order to find abnormalities and deal with them in time; if the patient has symptoms (such as back pain, hematuria, fever, or hypertension, etc.), obvious damage of renal function (elevated blood creatinine or serious reduction of GFR in both kidneys) or combined with other organ damage, then timely surgery is required. The purpose of surgery for polycystic kidney is mainly twofold.
(1) to reduce the symptoms.
(2) to slow down the progress of the disease and improve renal function.
The treatment of polycystic kidney can be temporarily observed for early polycystic kidney patients. Pay attention to diet and avoid the application of nephrotoxic drugs in life. However, ultrasound and kidney function should be reviewed regularly.
Drug treatment: mostly symptomatic treatment. Such as antihypertensive drugs to treat hypertension, antibiotics to treat infection, erythropoietin to treat anemia, kidney-protective drugs to treat renal insufficiency, sodium bicarbonate to correct acidosis, etc. At present, there is no specific drug for the treatment of polycystic kidney.
Surgery: Surgery can be used to remove enlarged cysts, but surgery cannot cure polycystic kidney.
There are two main purposes of surgery.
1, to reduce the internal pressure of the kidney, to slow down the rate of decline of kidney function and to help control blood pressure, etc.
2, to reduce the patient’s back pain and other uncomfortable symptoms.
The surgical methods are: ultrasound-guided cyst puncture and aspiration, open cyst decompression, laparoscopic cyst decompression, etc. Depanalization and decompression not only can reduce patients’ symptoms, but also can improve kidney function and slow down the rate of kidney function decline. Laparoscopic surgery, as a minimally invasive procedure, has gradually become the standard procedure for the treatment of polycystic kidney. It has.
① less trauma and less pain.
② fast recovery and short hospitalization period.
③It is possible to perform laparoscopic treatment for patients with open surgery for a second time. The surgery is mainly for patients with early and middle stage polycystic kidney. For those with obvious abdominal symptoms in the late stage, surgery can also be chosen as long as there are no other contraindications to surgery and they can tolerate anesthesia. Professor Jin Xunbo firstly proposed the new concept of combined renal peritoneal debridement on the basis of laparoscopic polycystic kidney decompression, in which renal peritoneal debridement is performed on the basis of cyst decompression to further release the kidney compression, which helps to improve renal blood supply and enhance the treatment effect. The age of surgery is preferably below 60 years old. If the patient is older and cannot tolerate anesthesia, etc., surgical treatment should be chosen with extreme caution, and ultrasound-guided cyst puncture and aspiration can be considered, which is relatively less invasive.
Surgery only serves to delay the deterioration of renal function and does not fundamentally stop the production of cysts, nor is it possible to treat all cysts. The so-called “recurrent” cysts are only small cysts that existed at the time of the first surgery but could not be distinguished by the naked eye and could not be treated.