Polycystic kidney disease (PKD) is the most common monogenic inherited kidney disease in humans. According to European and American countries, polycystic kidney disease is the 4th cause of end-stage renal failure. According to the statistics in Shanghai, China, dialysis patients due to polycystic kidney disease accounted for 5.6% of the whole dialysis population, which also ranked the 4th after chronic glomerulonephritis, diabetic nephropathy and benign renal small arteriosclerosis. According to the genetic pattern, polycystic kidney disease can be divided into autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD is characterized by numerous fluid cysts of varying sizes in both kidneys, which grow progressively, destroying the normal structure and function of the kidneys and eventually leading to end-stage renal failure. The pathology of ARPKD is characterized by markedly enlarged kidneys on both sides, containing many radially arranged prismatic cysts with biliary proliferation, enlarged portal areas, and fibrosis. Death often occurs in infancy and early childhood, with a few surviving into adulthood. In clinical practice, the most common polycystic kidney disease is autosomal dominant polycystic kidney disease, which is also the current research hotspot, and the polycystic kidney disease mentioned below refers to autosomal dominant polycystic kidney disease. The genetic background of polycystic kidney disease There are two main causative genes of autosomal dominant polycystic kidney disease, called PKD1 and PKD2. PKD1 gene is located in the short arm of chromosome 16 (16p13.3), and its encoded protein is called polycystin-1, and PKD1 gene mutation causes 85%-90% of ADPKD. PKD2 is located on the long arm of chromosome 4 (4q22), and its encoded protein is called polycystin-2 (polycystin-2), PKD2 mutation accounts for 5%-15% of ADPKD caused by PKD1 gene mutation. 2. Clinical manifestations of polycystic kidney disease About 85% of ADPKD patients have family history. Symptoms are most common with discomfort or pain in the abdomen, flank and back, mostly vague or dull pain. Sudden increase in pain may be due to rupture and bleeding of the cyst or secondary infection. About 60% of ADPKD patients have visual or microscopic hematuria, which is often induced or aggravated after trauma, strenuous labor or infection. Hypertension is one of the common clinical manifestations of ADPKD, accounting for about 60% to 90%. The abdominal mass is an important sign of ADPKD, which can be felt bilaterally in 50%~80% of cases and unilaterally in 15%~30% of cases, and has a tight texture on palpation and moves with breathing. Clinical symptoms of renal impairment usually appear between the ages of 20 and 60, and about 50% of patients develop end-stage renal disease by the age of 60. ADPKD affects multiple organs and tissues in addition to the kidneys. About 1/3 of patients may have liver cysts, and the number of cysts increases with age, with clinical manifestations such as pain, discomfort and early fullness in the liver area. Cysts can also occur in the pancreas, spleen, esophagus, thyroid, endometrium, seminal vesicles, and epididymis, etc. These isolated cysts may not cause clinical symptoms. Heart valve abnormalities, cardiac hypertrophy and congenital heart disease are another common extra-renal manifestation of ADPKD. 83% of patients with end-stage ADPKD have barium enema confirmed colonic diverticula. Aneurysms can occur in the cerebral arteries, abdominal aorta, thoracic aorta, atrial septum, and coronary arteries, and are saccular or spindle-shaped. Intracranial aneurysms are the most common, with an incidence of about 12% in patients with ADPKD. Diagnosis of polycystic kidney disease The diagnosis of ADPKD is mainly based on family genetic history, imaging examination and genetic diagnosis. (1) Family genetic history: 95% of ADPKD patients have autosomal dominant genetic characteristics, i.e., the disease develops from generation to generation with equal incidence in men and women, and patients are heterozygous with almost 100% epizootic rate, but 5% to 10% of ADPKD patients have no family genetic history. (2) Imaging examination: B-type ultrasonography is the most commonly used method to diagnose ADPKD, which is convenient, inexpensive and non-invasive. CT and magnetic resonance imaging (MRI) are valuable in the diagnosis of ADPKD and can provide useful information when bleeding or infection occurs in cysts. (3) Genetic diagnosis: there are mainly methods such as gene chain analysis, direct detection of gene mutation and single nucleotide polymorphism detection. 4.Blood pressure control of polycystic kidney disease Hypertension is one of the common clinical manifestations of polycystic kidney disease and also the earliest clinical manifestation of polycystic kidney disease. It is reported abroad that the increase of blood pressure in patients with polycystic kidney disease is more than ten years earlier than that in general population, and 60% of patients have increased blood pressure before the abnormal kidney function. If they enter the stage of end-stage renal disease, almost all patients have hypertension. Polycystic kidney disease is similar to other types of kidney disease in that hypertension is an important factor in the progression of renal function and a major risk factor for cardiovascular complications. In recent years, a series of clinical studies have been conducted in the treatment of polycystic kidney disease, but currently the most feasible clinical intervention is blood pressure control in patients with polycystic kidney disease. Although early and effective antihypertensive therapy is considered to be an important measure to delay disease progression and reduce cardiovascular complications, there is no complete uniformity in which antihypertensive drugs should be applied in the treatment of hypertension in patients with polycystic kidney disease. It has been confirmed that the RAAS system is continuously activated in patients with polycystic kidney disease, and glomerular hyperfiltration is observed in the early stage of the disease, indicating that the activation of the RAAS system plays an important role in the development of hypertension in patients with polycystic kidney disease. A clinical study of HALT-PKD has also been initiated in the United States, focusing on the effects of antihypertensive drugs ACE/ARBs on disease progression in patients with polycystic kidney disease. Meanwhile, some scholars have found that the sympathetic nervous system is also activated in patients with polycystic kidney disease. in a randomized, double-blind, prospective study of 46 hypertensive patients with polycystic kidney disease, Zeltner et al. found no significant difference in the effects of applying the ACEI drug ramipril and the beta-blocker metoprolol group on renal function, urinary albumin excretion rate, and LVMI after 3 years of follow-up. However, strict blood pressure control, such as mean arterial pressure (MAP) control below 97 mmHg, inhibited the increase in LVMI and reduced urinary albumin excretion, suggesting that strict blood pressure control may protect the cardiac and renal organs from further damage. In conclusion, strict blood pressure control is essential to protect the cardiac and renal functions of patients with polycystic kidney disease. ACEI and ARB are the ideal drugs of choice, and other commonly used drugs include calcium channel antagonists, β-blockers and central antihypertensive drugs.