Treatment of polycystic kidney

  Although the research on the pathogenesis of autosomal dominant polycystic kidney disease has made great progress in recent years, there is no effective treatment method so far, and the main treatment measures are currently to control complications and delay disease progression. The principles of autosomal dominant polycystic kidney disease treatment are: reduce the birth rate of diseased individuals, early diagnosis, strengthen patient education, regular examination, actively control complications, and take renal treatment in time for patients with end-stage renal disease.  I. General treatment Pay attention to rest, avoid smoking, strong tea, coffee and ethanol-containing drinks, avoid chocolate, low salt diet when there is hypertension, and low protein diet is recommended in the late stage of the disease. Most patients do not need to change their lifestyle or restrict physical activity in the early stage. When the cyst is large, strenuous physical activity and abdominal trauma should be avoided. Patients should be followed up regularly.  II. Control of complications 1. Pain Some patients have transient pain, which can be observed first. If the pain is persistent or heavy, analgesics can be given, but one wave of analgesics is less effective. If the pain is severe and cannot be relieved by analgesics and affects the patient’s life, surgical treatment can be carefully considered.  2. There are 3 types of bleeding: first, intracapsular bleeding, the patient has sudden pain but no hematuria visible to the naked eye; second, the cyst bleeding is connected with the urinary tract, bleeding to a certain extent that breaks the human urinary tract and discharges out of the body, appearing hematuria to the naked eye; third, subcapsular bleeding, large amount, no hematuria, blood pressure may drop. In addition to active treatment for the causes of hematuria such as enlarged cysts, hypertension, urinary tract infections and urinary stones, bed rest is very important. Commonly used hemostatic drugs have little effect and may even form blood clots, leading to urinary tract obstruction or inducing infection. Very few patients with heavy bleeding need blood transfusion therapy. Patients already on hemodialysis who have recurrent episodes of hematuria should be treated with small molecule or heparin-free dialysis. For patients with large bleeding volume and ineffective medical treatment, surgical treatment can be carefully considered.  Hypertension: Hypertension is one of the risk factors for renal impairment and should be effectively monitored, with a therapeutic target of 130/80 mm Hg. The available antihypertensive drugs include angiotensin-converting enzyme inhibitors, angiotensin II receptor antagonists, calcium channel blockers, B-blockers, central antihypertensive agents and diuretics, etc. The combination of antihypertensive drugs is basically the same as that for general hypertension. ARB drugs are preferred because they reduce systemic blood pressure and intracapillary pressure by inhibiting the overactive renin-angiotensin system, which is particularly effective in the early stage of the disease. During application, attention should be paid to monitoring serum creatinine and blood potassium levels to prevent acute renal failure and hyperkalemia. Hypertension in the late stage of autosomal dominant polycystic kidney disease is usually more persistent and requires combination of drugs. For the intractable hypertension that cannot be controlled by the combination of multiple drugs, surgery can be carefully considered.  4.Infection of urinary tract is common, other infections include cholangitis, intestinal diverticulitis and so on. Generally water-soluble antibiotics through glomerular filtration, proximal tubular secretion, fat-soluble antibiotics through the cyst wall diffusion into the cyst, so the treatment. Kidney cyst infection should be combined with water-soluble and fat-soluble antibiotics. Water-soluble antibiotics include ampicillin, aminoglycosides, and second and third generation cephalosporins; fat-soluble antibiotics include compound sulfamethoxazole, ciprofloxacin, chloramphenicol and metronidazole. Early culture of pathogenic bacteria and selection of sensitive antibiotics can be expected to achieve better results. The course of treatment needs more than 2 weeks.  5. Stones encourage patients to drink more water. Depending on the size and location of the stone, endoscopic stone extraction or surgical stone extraction can be performed.  C. Management of extra-renal symptoms 1. polycystic liver to reduce the volume of liver cysts as the principle, can be used ultrasound-guided cyst puncture and fluid aspiration and injection of sclerosing agent, but also surgical treatment, such as laparoscopic cyst decompression and hepatic lobectomy. Cyst infection is treated with cyst fluid puncture and drainage combined with antibiotics for 2-3 weeks.  2. intracranial aneurysm For patients with autosomal dominant polycystic kidney disease with family history of aneurysm at the age of 18-35, magnetic resonance or angiography should be performed. If there is no positive finding, review after 5 years; if there is a positive result, the size of aneurysm should be determined by angiography. 6mm aneurysm should be treated by intervention or surgery. In case of aneurysm rupture and bleeding, the principle of treatment is to prevent rebleeding and cerebral ischemia. Codeine may be used for pain relief and aspirin is contraindicated. 25% of patients experience cerebral ischemia 5-14 d after aneurysm rupture and may be treated with vasoactive drugs or calcium channel blockers as appropriate. Since 35% of patients may rebleed and the morbidity and mortality rate is as high as 50%, the earlier the surgical treatment, the better, so surgery or intervention should be performed within 72h of bleeding.  Gene therapy for polycystic kidney disease is still in the experimental stage. According to its hair conservative treatment ineffective people can use surgical treatment to remove the enlarged cystic disease mechanism, people have been exploring new interventions and studying new a hereditary and congenital kidney disease drugs. Among them, vasopressin V2 receptor antagonists can specifically downregulate cyclic adenosine monophosphate, blocking the effect of cAMP activation of fluid secretion and stimulation of cystic epithelial cell proliferation, and therefore have promising applications for the treatment of polycystic kidney disease, and are currently undergoing clinical trials and becoming the most anticipated class of drugs. Tyrosine kinase inhibitors and PPAR-y agonists are two classes of drugs that are widely used in clinical practice and have also shown good therapeutic effects in several animal models of autosomal dominant polycystic kidney disease, which also have broad application prospects. Sirolimus (rapamycin) is an immunosuppressive agent that has been used clinically. Studies have shown that sirolimus can inhibit the occurrence and enlargement of renal cysts and protect renal function in animal models, and a multicenter clinical trial of sirolimus for autosomal dominant polycystic kidney disease is currently underway abroad. Its references he studied drugs also include statins, metalloproteinase inhibitors, etc.  V. Renal replacement therapy Renal replacement therapy for patients with autosomal dominant polycystic kidney disease after entering end-stage renal disease includes peritoneal dialysis, hemodialysis and renal transplantation. The timing of starting treatment and the choice of treatment modality varies from patient to patient and is not significantly different from those with non-cystic kidney disease.  Chinese medicine treatment Chinese medicine believes that autosomal dominant polycystic kidney disease is caused by stagnation, early stage exists qi stagnation and blood blockage, kidney qi disintegration, lower jiao damp heat; in the middle stage, stasis and blood accumulation, liver yang hyperactivity, essence and blood deficiency, damp heat downward injection; in the end stage, positive deficiency and stasis, liver wind internal movement, dampness and toxicity shut down mainly. In the early stage, we can use leech powder and soapberry to attack the evil, and Liu Wei Di Huang Tang to nourish Yin and tonify the kidney.