Polycystic kidney disease in adults: symptoms

The symptoms of polycystic kidney are divided into renal and extrarenal.

Renal symptoms of polycystic kidney disease in adults

• Acute and permanent pain in the abdominal cavity.
• Hematuria (micro- or macrohematuria).
• Arterial hypertension.
• Infection of the urinary tract (bladder, renal parenchyma, cysts).
• Nephrolithiasis.
• Nephromegalia.
• Renal failure.

The extrarenal symptoms of polycystic kidney disease in adults

• Gastrointestinal:
  • cysts in the liver;
  • cysts in the pancreas;
  • diverticulum of the intestine.
• Cardiovascular:
  • changes in heart valves;
  • intracerebral aneurysms;
  • aneurysm of thoracic and abdominal aorta.

[1], [2], [3], [4], [5]

Renal symptoms of polycystic kidney disease

The first symptoms of polycystic kidneys usually develop at the age of about 40 years, but the onset of the disease can be as much earlier (up to 8 years) and later (after 70 years). The most frequent clinical signs of polycystic kidney disease are pain in the abdominal cavity (or back) and hematuria.

Pain in the abdominal cavity appears in the early stages of the disease, it can be periodic or permanent and varies in intensity. Sharp pains often force patients to take a large number of analgesics, including NSAIDs, which in a similar situation can contribute to the development of hypertension and the reduction of renal function. Often because of the intensity of pain, the introduction of narcotic analgesics is required. The genesis of the pain syndrome is associated with the dilatation of the capsule of the kidneys.

Hematuria, often microhematuria, is the second predominant symptom of polycystic kidney disease in adults. More than one-third of patients periodically experience episodes of macrohematuria. They are provoked by the development of trauma or a massive physical load. The frequency of episodes of macrogemuria increases in patients with sharply increased kidneys and with high arterial hypertension. The presence of these factors should be considered as a risk of developing renal bleeding. Other causes of hematuria include thinning or rupture of blood vessels in the cyst wall, infarcts of the kidney, infection or passage of kidney stones.

Arterial hypertension is detected in 60% of patients with polycystic kidney disease before they develop chronic renal failure. Increased blood pressure may be the first clinical sign of the disease and develop already in adolescents; As the age increases, the frequency of hypertension increases. A characteristic feature of arterial hypertension in polycystic kidney disease is the loss of the circadian rhythm of blood pressure while maintaining high values or even increasing it in the night and early morning hours. Such a pattern of arterial hypertension and its long existence have a damaging effect on the target organs: on the heart, causing the development of left ventricular hypertrophy and insufficiency of its blood supply, which creates a threat of myocardial infarction, as well as kidneys, significantly accelerating the rate of progression of renal failure.

Genesis of arterial hypertension is associated with ischemia leading to activation of RAAS and sodium retention in the body.

Proteinuria, as a rule, is expressed slightly (up to 1 g / day). Moderate and strong proteinuria accelerates the development of renal failure and worsens the long-term prognosis of patients.

Urinary tract infection complicates the course of the disease in about 50% of cases. In women, it develops more often than in men. Infection of the urinary tract can manifest cystitis and pyelonephritis. The development of high fever, increased pain syndrome, the appearance of pyuria without leukocyte cylinders, and insensitivity to the standard for pyelonephritis therapy indicate the spread of inflammation to the contents of the renal cysts. In these situations, the diagnosis is confirmed by ultrasound data, scans with gallium or the results of CT of kidneys.

Early signs of impaired renal function - a decrease in the relative density of urine, the development of polyuria and nocturia.

The extrarenal symptoms of polycystic kidney and the complications of polycystic kidney disease in adults

Along with kidney damage in polycystic disease, anomalies in the structure of other organs are often detected.

Cysts in the liver are the most frequent (38-65%) non-renal symptom of polycystic kidney disease. In most cases, liver cysts do not appear clinically and do not affect the function of the organ.

With high frequency (up to 80% or more), especially in the stage of chronic renal failure, the patients develop gastrointestinal lesions. In comparison with the general population, at a polycystosis in 5 times more often diverticulums of an intestine and a hernia are found out.

In one third of patients with polycystic kidney disease, aortic and mitral valvular changes are diagnosed , while the lesion of the tricuspid valve is rare.

In some cases, cysts of the ovaries, uterus, esophagus and brain are detected.

Attention is drawn to the high (8-10%) frequency of cerebral vascular lesions with the development of aneurysms. This indicator doubles, if the patients are diagnosed with a hereditary heredity for the damage to the vessels of the brain.

The rupture of aneurysms with the development of subarachnoid bleeding is a common cause of death of these patients under the age of 50 years. The risk of aneurysm rupture increases with increasing size and is considered high for an aneurysm of more than 10 mm. The presence of such education is considered an indication for surgical treatment.

Currently, for the diagnosis of cerebral vascular lesions in polycystic kidneys successfully apply MRI of the brain. Using this method, it is possible to diagnose aneurysms of cerebral vessels less than 5 mm in size. The method is recommended as a screening for examination of persons with a hereditary burden due to cerebrovascular complications.

The most common complications of polycystic kidney disease:

• bleeding into cysts or retroperitoneal space;
• infection of cysts;
• formation of kidney stones;
• development of polycythemia.

Bleeding to cysts or retroperitoneal space clinically manifested by macrogemuria and pain syndrome. The causes of their development can be high arterial hypertension, physical stress or abdominal injury. Episodes of bleeding into the cysts, while observing the protective regime, often pass by themselves. If there is a suspicion of bleeding to the retroperitoneal space, ultrasound diagnosis, computed tomography or angiography is performed, and when the complications are confirmed, the issue is resolved surgically.

The main risk factor for infection with renal cysts is urinary tract infection; less often the source of infection becomes hematogenous infection. In the overwhelming majority of cysts are identified Gram-negative flora. The necessity of penetration of an antibacterial substance into the cyst creates difficulties in the treatment of infected cysts. These properties are only lipophilic antibacterial drugs with a dissociation constant that allows the substance to penetrate into the acidic environment of the cyst for 1-2 weeks. These include fluoroquinolones (ciprofloxacin, levofloxacin, norfloxacin, ofloxacin) and chloramphenicol, as well as combined sulfonamide with trimethoprim - co-trimoxazole (trimethoprim-sulfamethoxazole). Aminoglycosides and penicillins hardly penetrate into the cysts, do not accumulate in them, and therefore these drugs are ineffective.

Nephrolithiasis complicates the course of polycystic kidneys in more than 20% of patients. Most often in polycystic disease, urate, oxalate or calcium stones are found. The reasons for their formation are violations in the metabolism and passage of urine.

The frequent complication of polycystic kidney disease is polycythemia. Genesis it is associated with excessive production of the brain substance of the kidney of erythropoietin.

Progression of renal failure

In the vast majority of patients with polycystic kidneys before age 30, the functional state of the kidneys remains normal. In subsequent years, in almost 90% of cases, varying degrees of renal insufficiency develops. It is now shown that the rate of progression of chronic renal failure is largely determined by genetic factors: the genotype of polycystic kidney disease, sex and race. Experimental and clinical studies show that with the 1 st type of polycystic kidney, terminal renal failure develops 10-12 years earlier than with polycystosis of the second type. In males, terminal renal failure develops 5-7 years faster than in women. A higher rate of progression of chronic renal failure was noted in individuals of African-American race.

In addition to genetic features, hypertension plays an important role in the progression of renal failure. The mechanism of the effect of high blood pressure on the kidney function in polycystic kidney does not differ from that in other kidney pathologies.

It is very important for a doctor to be able to recognize the symptoms of polycystic kidney disease, since an incorrect diagnosis can cost a patient a life.