• Whatsapp: +8613373397217
  • 24/7 ER : +8613373397217
  • mailbox: bjtsthospital@gmail.com
HOME > Polycystic Kidney Disease > Polycystic Kidney Disease/Trea >

Polycystic kidney disease

  Polycystic kidney disease, also known as Potter (I) syndrome, Perlmann syndrome, congenital renal cystic disease, cystic kidney, bilateral renal hypoplasia syndrome, benign renal polyposis cysts, polycystic disease. Zhu Xianyi first reported in 1941, the disease is not uncommon clinical. There are two types of polycystic kidney disease, autosomal recessive inheritance (infant type) polycystic kidney disease, onset in infancy, the clinical rarer; autosomal dominant type (adult type) polycystic kidney disease, often in the middle-aged Periods are found and can also occur at any age.

  Etiology

  Abnormal genes in 90% of patients with polycystic kidney disease in the short arm of chromosome 16, known as the polycystic kidney 1 gene, the gene product is unclear. Another 10% less than the patient's abnormal gene in the short arm of chromosome 4, known as polycystic kidney 2 gene, the encoded product is unclear. The age at onset, onset of hypertension, and duration of renal failure differed between the two groups.

  The exact cause of this disease is not clear. Although most of the symptoms occur only after the adult, but began to form in the fetal period. Cysts originate in the renal tubules, the fluid properties with the origin of different parts, originated in the proximal tubules, cyst fluid components such as Na +, K +, Cl-, H +, creatinine, urea and plasma similar; originated in the distal The concentrations of Na + and K + in the cyst fluid were lower, and the concentrations of Cl-, H +, creatinine and urea were higher.

  Polycystic kidney disease in polycystic kidney disease in patients with abnormal proliferation of polycystic kidney disease is one of the significant characteristics of a mature or incomplete development of the state, highly suggestive of the regulation of cell development and maturation disorders, the cells in a Immature state, thereby showing strong proliferation. Abnormal transport of epithelial cells is another prominent feature of polycystic kidney, which is the subunit combination, distribution and active expression of Na + -K + -ATPase, which is closely related to cell transport; abnormal cell signal transduction and changes of ion transport channels. Abnormal proliferation of extracellular matrix is ​​the third significant feature of polycystic kidney disease.

  Many studies have shown that these abnormalities are involved in cell growth related to the active factor. But the key to the abnormal links and ways not yet clear. In short, due to genetic defects caused by cell growth and interstitial abnormalities, one of the important pathogenesis of this disease.

  Clinical manifestations

  The size of the kidneys in children with normal or slightly larger form, with age, the number and size of cysts gradually increased and increased, the majority of cases to 40 to 50 years of age when the kidney volume to a considerable extent only symptoms. Mainly for both sides of renal enlargement, kidney pain, hematuria and hypertension.

  Kidney enlargement

  Bilateral renal disease progress asymmetry, the size of the difference, to the late two kidney can fill the entire abdominal cavity, kidney surface cloth has a lot of cysts, kidney-shaped irregular, rugged, hard texture.

  2. Renal area pain

  Often for the back of the oppressive or dull pain, there are severe pain, and sometimes abdominal pain. Pain can be due to physical activity, walking time is too long, sedentary, etc., and increased, after bed can be reduced. Renal hemorrhage, stone movement or infection is the reason for sudden pain.

  3. Hematuria

  About half of the patients were microscopic hematuria, may have onset of gross hematuria, the Department of cyst wall blood vessels rupture caused. Bleeding for a long time Blood clots through the ureter can cause colic. Hematuria often accompanied by white blood cells and proteinuria, urinary protein less, generally not more than 1.0 g / day. Renal infection, pyuria, hematuria increased, low back pain with fever.

  4. Hypertension

  For the common manifestations of polycystic kidney disease, serum creatinine is not increased before about half of the emergence of hypertension, which oppression of the surrounding cysts, activation of renin - angiotensin - aldosterone system. Over the past 10 years, Graham PC, Torre V and Chapman AB have confirmed that the normal renal tissue of the disease, the cyst adjacent to the stroma and cyst epithelial cells increased renin granules, and increased renin secretion. These are closely related to the growth of cysts and the development of hypertension. In other words, the cysts of hypertensive patients grow faster, can directly affect the prognosis.

  5. Renal insufficiency

  Individual cases of renal failure in adolescence that is generally very few before the age of 40 renal dysfunction, 70 years of age still maintain about half of renal function, but hypertensive development of renal failure to significantly shorten the process, there are individual patients 80 years old Can maintain kidney function.

  6. polycystic liver

  Middle age found in patients with polycystic kidney disease, about half of a multi-cystic liver, 60 years of age after about 70%. It is generally believed that its development is slower, and more about 10 years later cystic kidney. The cyst is dilated by the lost bile duct. In addition, the pancreas and ovaries can also occur cysts, colon diverticulum complicated by a higher rate.

  7. Physical examination

  Physical examination can touch the side or bilateral kidneys, nodular. With tenderness when infected. 50% of patients with increased waist circumference.

  Staging

  Polycystic kidney disease is a kind of hereditary nephropathy, its incidence and development also has certain rules, polycystic kidney disease has the following rules.

  Period of occurrence

  The disease is a genetic disease, usually born with cysts, but smaller, difficult to detect, 20 years old before the general difficult to find, but if the family of polycystic kidney disease cases, should be early examination, and early observation of the cyst growth status . Attention to maintenance.

  2. Growth period

  Patients in the 30 to 40 years of age, cysts will have a faster growth, the medical period known as the growth period. Growth should strengthen the observation, Western medicine treatment of this period there is no way, but symptomatic treatment, such as high blood pressure, which appears to be very passive. In this period should still be active treatment, treatment is aimed at the use of strong blood circulation by the role of traditional Chinese medicine, the cyst no longer grow or delay the growth rate of cysts, to extend the life of the role of patients, it can be said that this is Chinese medicine promoting blood circulation to delay the critical period of cyst growth.

  3. Swelling period

  Patients after the age of 40, the cyst will be further growth and swelling, when the cyst more than 4cm later, to the cyst ulceration, known as the enlargement period. With the expansion of the cyst will appear more clinical symptoms, such as low back pain, proteinuria, hematuria, elevated blood pressure, then should be closely observed in the treatment of this period is a critical period of Chinese and Western treatment. Can be used to promote blood circulation detoxification Xiezhuo, by removing the harm to renal function of the cyst fluid to protect the purpose of renal function, so polycystic kidney disease is the combination of Chinese and Western medicine treatment of renal protection critical period.

  Rupture period

  Such as cysts continue to grow, in some external factors, there will be ulceration, ulceration should be immediately after hospitalization for treatment, and actively control the infection, to prevent sepsis and acute deterioration of renal function, in order to facilitate other symptomatic treatment.

  5. Uremia period

  For uremia treatment, protection of renal function, late peritoneal dialysis or hemodialysis.

  Classification and characteristics

  1. Perinatal type

  Perinatal renal cystic lesions have been serious, involving 90% of the collection tube, while a small amount of portal vein around the fiber proliferation, perinatal death.

  2. Neonatal type

  Involving 60% of the collecting duct, with mild portal vein fibrous proliferation. 1 month after birth symptoms, died of renal failure in a few months.

  3. Baby type

  Infantile manifestations of renal enlargement, 25% of tubular involvement, liver, splenomegaly with moderate portal vein around the fiber proliferation. 3 to 6 months after birth symptoms, died in childhood due to renal failure.

  4. Junior type

  Juvenile type in the 13 to 19-year-old symptoms. Kidney damage is relatively minor, only 10% of the renal tubular cystic degeneration, occasionally developed into renal failure. Severe fibrosis in the portal vein. Generally around the age of 20 due to liver complications, portal hypertension death.

  an examination

  1. Urine routine

  No abnormal early, late stage microscopic hematuria, some patients with proteinuria. With stones and infection when there are white blood cells and pus cells.

  2. Determination of urine osmolality

  Early lesions only a few cysts, there may be impaired renal function, suggesting that the changes are not entirely related to renal structural damage may be related to the adverse reaction of the kidney against antidiuretic hormone. Decreased renal function before the glomerular filtration rate decreased.

  3. Serum creatinine

  With the loss of renal compensatory capacity was progressively increased. Creatinine clearance rate for the more sensitive indicators.

  4. KUB film

  Show kidney enlargement, irregular shape.

  5.IVP

  Showing signs of renal pelvic compression deformation, pyramidal calyceal shape was strange spider-like, calyceal flat and wide, neck elongation thinner, often curved.

  6.B super

  Showing a large number of kidneys have a dark area.

  7. CT

  Showing increased kidneys, the shape was lobulated, the majority of liquid-filled thin-walled cyst.

  diagnosis

  Adult polycystic kidney disease (polycystic kidney disease) diagnostic criteria can be divided into the main diagnostic basis and auxiliary diagnosis.

  1. The main diagnostic basis

  ① renal cortex, medulla covered with numerous sizes of liquid cysts; ② autosomal dominant autosomal dominant polycystic kidney disease (ADPDK) family history; ③ gene linkage analysis was positive results.

  2. Auxiliary diagnostic basis

  ① polycystic liver; ② renal insufficiency; ③ pancreas or spleen cyst; ④ heart valve abnormalities; ⑤ intracranial aneurysm; ⑥ abdominal hernia.

  treatment

  At present, there is no way to stop the development of the disease. Early detection, to prevent the occurrence and development of complications, timely and correct treatment of complications has emerged is essential.

  1. General treatment

  Under normal circumstances, the patient check out polycystic kidney disease, the first to maintain an optimistic attitude, if not yet affect the normal life of patients, and usually need to pay attention not to eat or salty, spicy and other stimulating food, rest time to law , The mood should be smooth and optimistic; if the impact on the patient's normal life, usually pay attention to the above a few, but also for treatment, and the sooner the better, or any of its development to renal failure uremia, too late.

  2. cyst decompression to the top

  This operation relieves cyst compression of the renal parenchyma, protects most of the remaining nephrons from extrusion and further damage, so that renal ischemic conditions have improved, some of the renal function units have been restored, delaying the development of the disease. Surgery is the key to success as soon as possible surgery, cyst decompression must be thorough, do not give up the small cysts and deep cyst decompression. Bilateral should be surgery, general bilateral surgery interval of more than six months. Advanced disease, such as renal impairment has been in azotemia, uremia, regardless of whether there is a merger of hypertension, decompression treatment is meaningless, anti-surgery can increase the severity of the disease.

  3. Chinese medicine treatment

  Chinese medicine in the treatment of polycystic kidney disease in conservative treatment (taking traditional Chinese medicine), the effect is very good. Chinese medicine using the overall concept and syndrome differentiation, that polycystic kidney disease is the result of external and internal factors, through the cascade diversion, and gradually let the cyst fluid discharge, to gradually narrow the purpose of the cyst. Although the traditional Chinese medicine can not overcome the genetic problem, but the effect of conservative treatment is unmatched by Western medicine, and basic non-toxic side effects, not easy to relapse.

  4. Dialysis and transplantation

  Into end-stage renal failure, dialysis should be immediately treated, the preferred hemodialysis. The survival rate of kidney transplantation in polycystic kidney disease is similar to that in other patients. However, the disease associated with polycystic kidney disease increases the difficulty of postoperative treatment and affects the transplanting effect.

  5. Hematuria treatment

  Hematuria occurs, in addition to a clear reason as soon as possible to give treatment, should reduce the activity or bed rest. Dialysis or dialysis patients, such as recurrent severe and uncontrollable hematuria, may consider the use of transcatheter renal artery embolization.

  6. The treatment of infection

  Renal parenchymal infection and cyst infection is the main complication of the disease, the general principle of the joint application of antibiotics.

  7. combined upper urinary tract stones treatment

  According to the location and size of stones according to the principle of treatment of urinary tract stones.

  8. Hypertension treatment

  Renal ischemia and renin-angiotensin-aldosterone system activation, is the main cause of hypertension should be based on the choice of antihypertensive drugs.

  9. Micro-Chinese medicine treatment

  Treatment of congenital polycystic kidney disease with micro-osmotic herbal ointment rather than surgical treatment is the reason why even large cysts oppress the renal parenchyma by surgery (decompression or decompression surgery) Temporary solution, but can not solve the big cyst removed after the small cysts due to pressure reduction but will rapidly increase the problem. Surgical therapy is only an expedient, with limitations.

  prevention

  1. Prevention of colds

  Kidney disease patients with polycystic kidney disease is very painful, because with other kidney disease is not the same, polycystic kidney disease is a lifelong disease, to be accompanied by a lifetime, even if the extra attention, the family care more care , Still can not stop the cysts continue to enlarge the objective reality. At this point, such as colds, especially repeated colds will make kidney damage in patients with polycystic kidney disease increased one point, played a worse role in worsening, but also accelerate the progress of renal injury.

  2. Control diet

  Reasonable diet in patients with polycystic kidney disease is very important to control the deterioration of renal function. Use of low-salt diet 2 to 3 grams a day is appropriate to eat salt, eat less potassium, phosphorus diet, to low-protein, low-fat diet, eat rich in vitamins and crude fiber diet to maintain smooth stool.

  3. Prevention of trauma

  Polycystic kidney cysts will continue to enlarge, will lead to increased intracystic cyst pressure, forcing the patient's kidneys are also increasing, intra-abdominal pressure increases. At this point any minor trauma, such as sprains, bruises, falls, etc. will increase intra-abdominal pressure or trauma directly on the impact of external cysts to promote cysts with high internal pressure rupture, bleeding, it is easy to induce infection .

  4. Control of blood pressure

  Most polycystic kidney disease in patients with impaired renal function before there will be high blood pressure, we call it polycystic kidney disease: the emergence of hypertension will accelerate the damage of renal function, while high blood pressure will also heart, brain Vascular injury, there will be multiple cystic kidney associated with cerebral hemorrhage caused by cerebral hemorrhage and other serious complications of stroke, so control of blood pressure to slow the deterioration of renal function, prevention of complications is essential.

Tags:

Articles
Kidney DiseaseMore >>