Pregnancy with polycystic kidney disease
Introduction
Introduction to pregnancy combined with polycystic kidney Polycysticidosis (polycystickidney) is a hereditary disease. Its pathological features are extensive cyst formation in the renal parenchyma. The connection between the renal tubules and the collecting duct is poor during embryonic development, the secretion of urine is blocked, and the renal tubules form obstructive cysts. . At present, polycystic kidney disease is a hereditary disease associated with abnormal apoptosis. According to genetic characteristics, it is divided into adult polycystic kidney disease (autosomal dominant polycysticidosis, ADPKD) and infantile polycystic kidney disease. (autosomal recessive polycystic kidney disease, autosomalrecessive polycystickidneydisease, ARPKD) two categories. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0001% - 0.0004% Susceptible population: pregnant women Mode of infection: non-infectious Complications: polycystic liver
Cause
Pregnancy with polycystic kidney disease
(1) Causes of the disease
During embryonic development, the renal tubules and the collecting duct or the renal tubules and the renal pelvis are connected to all or part of the kidney before the development of the kidney is terminated, the secretion of secreted urine is blocked, and the glomeruli and renal tubules produce a cystic cyst.
(two) pathogenesis
The disease is an autosomal dominant genetic disease, and its main pathogenesis is still unclear. It may be that the epithelial cells of the mutated epithelial cells secrete extracellular matrix and cause renal cystic lesions. The renal cystic lesions begin with nodular differentiation, and the kidney of 21PKD is confirmed. Excessive hyperplasia, secretion of liquid cysts, ADPKD cystic fluid itself includes active substances that stimulate cystic secretion, the capsule increases with the increase of fluid, oppressing the renal parenchyma, causing clinical symptoms.
Polycystic kidney disease occurs in both kidneys, and unilateral is extremely rare. Even if the renal cyst is unilateral, the pathological examination can also detect early renal cyst changes in the other kidney, and the polycystic renal tubules expand progressively. Lead to cyst formation, obstruction, secondary infection, rupture and chronic renal failure, the affected kidney is often 2 to 3 times larger than the normal side, the liquid in the large cyst can reach several thousand milliliters, and the diameter of the small cyst can be as small as 0.1 cm. When the anatomy, the kidney is honeycomb-shaped, and the capsule and the sac and the sac and the renal pelvis are not connected to each other. The renal parenchyma can be atrophied by the renal cyst compression; the glomerulus is glass-like; most patients have interstitial nephritis.
Prevention
Pregnancy with polycystic kidney disease prevention
1. Prenatal examination of pregnancy with polycystic kidney disease as a high-risk pregnancy, pregnancy complicated with pregnancy-induced hypertension, pyelonephritis, it should be regularly checked and followed up. In addition to routine examinations, attention should be paid to changes in renal function, active prevention and treatment of pregnancy-induced hypertension, and prevention of worsening of polycystic kidney disease. When a urinary tract infection occurs, a broad-spectrum antibiotic should be used, and pyelonephritis can induce renal failure.
2. Terminating the problem of pregnancy Young and uncomplicated pregnant women often have a pregnancy that reaches full term and is delivered vaginally. If the renal function is progressively deteriorated, the termination of pregnancy should be considered. It is estimated that a cesarean section can be performed if the child cannot be delivered vaginally in a short period of time.
Complication
Pregnancy with polycystic kidney disease Complications polycystic liver
Polycystic kidney disease may be associated with polycystic lesions of other organs, such as polycystic liver disease, occasionally cysts in the pancreas and ovaries, often with structural abnormalities of the gastrointestinal tract, blood vessels, and heart valves.
Symptom
Pregnancy with polycystic kidney disease common symptoms pyuria proteinuria cyst hematuria weight loss pregnancy high urinary frequency bleeding dull pain abdominal mass
1. Pain back pain or abdominal pain are the most common symptoms, mostly dull pain, dull pain, fixed on one side or both sides, can be radiated to the lower back or lower abdomen, pain suddenly increased, often intracapsular hemorrhage or secondary infection.
2. Hematuria and proteinuria 25% to 50% of patients have hematuria, which can be manifested as microscopic hematuria or gross hematuria. Hematuria is mainly caused by rupture of the vessel wall by excessive traction. 70% to 90% of patients have protein. Urine, generally not much, 24h quantitative often below 2g.
3. High blood pressure occurs in 70% to 75% of patients with hypertension, so pregnant women with pregnancy often have pregnancy-induced hypertension, which can cause polycystic kidney disease to worsen.
4. Abdominal mass During pregnancy, with the uterus growing up, the abdomen is not easy to lick and swollen polycystic kidney disease, but in non-pregnant body weight loss patients, 50% to 80% can be paralyzed and abdominal mass.
5. Infected polycystic kidney disease often combined with urinary tract infection, acute infection or suppuration manifested as chills, high fever, frequent urination, pyuria and so on.
6. In advanced cases of renal failure, renal failure due to cystic compression, pyelonephritis and other causes of renal parenchyma.
Examine
Pregnancy with polycystic kidney disease
1. There is no abnormality in the urine routine in the early stage of urine. There may be microscopic hematuria in the middle and late stage. Some patients have proteinuria, and there are white blood cells and pus cells in the stones and infection.
2. Determination of impaired renal concentrating function in the early stage of urine osmotic pressure measurement.
3. Determination of renal function Serum creatinine and urea nitrogen were progressively elevated with the loss of renal compensatory function, and creatinine clearance was also a sensitive indicator.
4. Abdominal plain film has enlarged renal shadow and irregular appearance. X-ray during pregnancy has certain influence on the fetus, so it is not used as an auxiliary diagnosis method for polycystic kidney disease during pregnancy.
5. B-ultrasound shows a large number of liquid dark areas in the kidney area.
6. IVU bilateral renal pelvis and renal pelvis are deformed by a spider, and the renal pelvis is flat and wide. The neck is elongated and thin, often curved. When the kidney is insufficiency, the development time of the kidney is delayed or even not developed.
7. CT shows a double kidney enlargement, the shape is lobulated, by most liquid-filled parenchyma cysts, liver, spleen, pancreatic cysts can also be found at the same time.
Diagnosis
Diagnosis and differentiation of pregnancy complicated with polycystic kidney
diagnosis
According to the family history and clinical manifestations of polycystic kidney disease, the possibility of polycystic kidney disease can be made. Combined with urine and ultrasonography, the diagnosis of polycystic kidney disease can be established. After diagnosis of polycystic kidney disease, attention should be paid to other parts of the lesion, such as Capsule liver.
Differential diagnosis
1. Hydronephrosis can also be manifested as lumbar pain and cystic mass in the waist and abdomen, but urography shows that the renal pelvis and renal pelvis are dilated, there is no separation, no compression, elongation changes, the renal cortex is thin, and can be clear The cause of obstruction, B-ultrasound showed that the kidney volume increased, the renal parenchyma became thinner, and the middle was a liquid dark area.
2. Simple renal cysts are mostly unilateral. The IVU examination shows a local enlargement of the renal shadow, the hemisphere is prominent at the edge, and the renal pelvis or renal pelvis has a curved impression. The B-ultrasound shows the clear circular liquidity of the inner edge of the renal parenchyma. In the dark area, CT examination showed that the kidney was partially round, the wall was thin and smooth, and the cystic space was not strengthened.
3. Multi-atrial renal cysts may have hematuria, low back pain, high blood pressure and lumbar and abdominal masses, but no renal dysfunction. B-ultrasound and CT examination are limited to a single cystic mass in the unilateral kidney, with many intervals inside. The cyst is divided into multiple small rooms that are not connected to each other.
4. Renal tumors have hematuria, low back pain and kidney lumps, but no chronic renal dysfunction. The masses often occur in the unilateral kidney, and B-ultrasound and CT examination show a substantial margin of unclear margin. Renal angiography can detect the edge of the mass. The blood vessels are enlarged, and the plaque-like contrast agent is scattered in the mass.
