What is polycystic kidney disease?

With the continuous advancement of modern science and technology, the level of medicine is getting higher and higher. Diseases that could not be discovered and treated before can be easily discovered and treated with modern advanced medical equipment. Polycystic kidney disease is a disease that is difficult to detect. It is possible to have congenital polycystic kidney disease, or it may be inherited. It is a disease that is difficult to detect. So what exactly is polycystic kidney disease? Let me introduce it to you below.

Polycystic kidney disease is also known as Potter (Ⅰ) syndrome, Perlmann syndrome, congenital renal cystic tumor disease, cystic kidney, bilateral renal dysgenesis syndrome, benign multilocular cyst of the kidney, and polycystic disease. The disease was first reported by Zhu Xianyi in my country in 1941 and is not uncommon in clinical practice. There are two types of polycystic kidney disease: autosomal recessive (infantile) polycystic kidney disease, which occurs in infancy and is clinically rare; and autosomal dominant (adult) polycystic kidney disease, which is often discovered in young and middle-aged people but can also occur at any age.

Clinical manifestations

The size and shape of the kidneys of patients with this disease are normal or slightly larger when they are young. The number and size of cysts gradually increase and increase with age. In most cases, symptoms do not appear until the kidney volume has grown to a considerable extent when the patient is 40 to 50 years old. The main symptoms are bilateral kidney enlargement, renal pain, hematuria and hypertension.

1. Kidney enlargement

The renal lesions on both sides progress asymmetrically and differ in size. In the late stage, the two kidneys may fill the entire abdominal cavity. There are many cysts on the surface of the kidneys, making the kidneys irregular in shape, uneven, and hard in texture.

2. Pain in the kidney area

It is often a feeling of pressure or dull pain in the lower back or back, but there may also be severe pain, and sometimes abdominal pain. The pain may be aggravated by physical activity, walking for too long, sitting for a long time, etc., and may be relieved by lying in bed. Bleeding in the kidney, movement of stones, or infection can also cause sudden, severe pain.

3. Hematuria

About half of the patients present with microscopic hematuria, and may have paroxysmal gross hematuria, which is caused by rupture of blood vessels in the cyst wall. When bleeding is heavy, blood clots may pass through the ureter and cause colic. Hematuria is often accompanied by leukocytosis and proteinuria. The amount of urine protein is small, generally not exceeding 1.0 g/day. In case of intrarenal infection, pyuria is obvious, hematuria worsens, and low back pain is accompanied by fever.

4. Hypertension

It is a common manifestation of polycystic kidney disease. Before the serum creatinine increases, about half of the patients develop hypertension, which is related to the cyst compressing the surrounding tissues and activating the renin-angiotensin-aldosterone system. In the past 10 years, Graham PC, Torre V and Chapman AB have confirmed that the number of renin granules in normal kidney tissue, cyst-adjacent stroma and cyst epithelial cells in this disease is increased, and renin secretion is increased. These are closely related to the development of cyst growth and hypertension. In other words, cysts grow faster in people with hypertension, which can directly affect the prognosis.

5. Renal insufficiency

In some cases, renal failure occurs in adolescence. Generally, renal function declines rarely occurs before the age of 40. About half of the people still maintain renal function at the age of 70. However, the process of progression to renal failure is greatly shortened in those with hypertension. Some patients can still maintain kidney function at the age of 80.

6. Polycystic liver disease

About half of the polycystic kidney patients diagnosed in middle age have polycystic liver disease, and about 70% after the age of 60. It is generally believed that it develops slowly and is about 10 years later than polycystic kidney. The cyst is formed by dilation of the labyrinthine bile duct. In addition, cysts may also occur in the pancreas and ovaries, and the incidence of colon diverticula is relatively high.

7. Physical examination

During physical examination, one or both kidneys may be palpable and appear nodular. There is tenderness when there is infection. 50% of patients had increased waist circumference.