Pediatric giant bladder-giant ureter syndrome

Introduction

Introduction of a huge bladder in children - huge ureteral syndrome The huge bladder-large ureteral syndrome, also known as the huge bladder, has a huge ureteral sign, which is characterized by a huge thickening of the bladder-ureter, but no urinary tract obstruction and vesicoureteral reflux, and no urinary tract obstruction. Some people think that the huge bladder Or a huge ureter should be diagnosed as a separate disease. With the advancement of inspection techniques, it has been found that the expanded bladder is also reversible, and the ureteral dilatation is also reversible. Regarding the view of no urinary tract obstruction, obstruction is also found. At the same time, the extent to which the ureter and bladder are enlarged can be diagnosed, and there is no standard. Neurogenic bladder is sometimes difficult to distinguish from this condition. basic knowledge Sickness ratio: 0.0001% Susceptible people: children Mode of infection: non-infectious Complications: urinary tract infection, urinary incontinence, anemia

Cause

Huge bladder in children - the cause of huge ureteral syndrome

(1) Causes of the disease

There have been many explanations:

1. Parasympathetic ganglion cells in the distal ureter wall reduce the reduction of parasympathetic ganglion cells in the distal ureteral wall, dysplasia or absence, leading to loss of ureteral motility and functional obstruction, but not confirmed by histology.

2. The end longitudinal muscle of the ureteral wall is lacking (normal ring muscle), thus causing functional obstruction, and it is believed that the longitudinal muscle deficiency is caused by the compression of the ureter in the middle kidney tube 12 weeks after the embryo.

3. In the distal ureter muscle layer, there are abnormal collagen fibers in the distal ureter muscle layer and nerves are normal, but abnormal collagen fibers in the muscle layer interfere with the stratified cell layer arrangement, hindering the transmission of peristaltic waves and producing functions. Sexual obstruction, these have to be further research in histology.

4. Genetic factors There are reports of mother and daughter suffering from this disease, so whether there is a family genetic predisposition remains to be studied.

(two) pathogenesis

The significant expansion of the ureter is characterized by more common unilateral, bilateral patients account for about 20%, but the weight can be inconsistent, its characteristics are:

1. The lower or the ureter has different degrees of expansion, the wall is thin, and there is no obvious distortion.

2. No organic ureteral obstruction.

3. No lower urinary tract obstruction.

4. No vesicoureteral reflux.

5. No neurogenic bladder.

6. There is no anatomical stenosis in the ureteral bladder junction.

7. After the urine is drained, the difference in ureteral tension cannot be retracted to normal (the diameter of the ureter in children is less than 0.7 cm).

Prevention

Huge bladder in children - prevention of huge ureteral syndrome

This disease is a congenital anomaly. There is no definite preventive measure at present. People with family history should do genetic counseling work. With reference to preventive measures for birth defects, prevention should be carried out from pre-pregnancy to prenatal, and pre-marital medical check is to prevent birth defects. Play a positive role, the size of the role depends on the inspection items and content, mainly including serological tests (such as hepatitis B virus, Treponema pallidum, HIV), reproductive system tests (such as screening for cervical inflammation), general physical examination (such as blood pressure, ECG) ) and ask about the family history of the disease, personal medical history, etc., do a good job in genetic disease counseling, pregnant women to avoid harmful factors, including away from smoke, alcohol, drugs, radiation, pesticides, noise, volatile harmful gases, toxic and harmful heavy metals, etc. In the process of antenatal care during pregnancy, systematic screening of birth defects is required, including regular ultrasound examination, serological screening, etc., and if necessary, chromosome examination. Once abnormal results occur, it is necessary to determine whether to terminate the pregnancy; The safety of the fetus in the uterus; whether there is sequelae after birth, whether it can be treated, Etc. After how to take practical measures for diagnosis and treatment.

Complication

Huge bladder in children - complications of huge ureteral syndrome Complications, urinary tract infection, urinary incontinence, anemia

Recurrent urinary tract infections, urinary incontinence, anemia, nutritional disorders, etc.

Symptom

Children's huge bladder - huge ureteral syndrome symptoms Common symptoms urinary frequency hematuria huge bladder abdominal pain urgency urinary incontinence urinary pain pyuria high fever nausea

Symptoms of urinary tract infection, hematuria and abdominal pain are common, often lead to pyuria, turbidity, frequent urination, urgency, dysuria, with varying degrees of waist, abdominal pain, and even gross hematuria, recurrent urinary tract infections and antibiotic treatment It is not easy to heal, and it is an important manifestation of this symptom. There are pathogenic bacteria in urine culture, urinary incontinence can occur in children, severe symptoms such as systemic poisoning, high fever, etc., often accompanied by nausea and vomiting, poor appetite, growth retardation, anemia, occasionally Can be abdomen and abdomen soft mass, bladder angiography can be seen bladder expansion and ureteral reflux, venous secretory angiography can be seen in the bladder and ureter giant expansion, intravesical pressure determination is higher than normal.

Examine

Pediatric huge bladder - examination of huge ureteral syndrome

1. Blood tests for urinary tract infections often have a significant increase in the total number of white blood cells and neutrophils, with a response protein >20 mg/L.

2. Urine routine examination clean middle-stage urinary centrifuge microscopic examination of white blood cells 5 / Hp prompt urinary tract infection, if you see white blood cell cast, suggesting pyelonephritis, severe urinary tract inflammation, may have transient proteinuria, some sick children can Have hematuria or terminal hematuria.

3. Bacterological examination Urinary culture requirements should be done before the application of antibiotics. Do not drink more water before urinating. In the process of urine retention, it should be strictly operated according to normal operation to avoid urine pollution. Urine culture can be contaminated by bacteria in the anterior urethra and urethra. It is necessary to clean the middle urine and colony count before treatment. If the colony count 105/ml has the significance of diagnosing urinary tract infection, 104105/ml is suspicious, but patients with urinary tract irritation have urinary white blood cells. Increased, urine culture colony count of 103 ~ 104 / ml should also consider the diagnosis of urinary sensation, in addition, some Gram-positive cocci, such as Streptococcus mutans, slow division, such as 103 / ml can also diagnose urinary sensation, for infants and Newborns and children who are suspected of having urinary dysfunction and have difficulty in urinary bladder can perform pubic bladder puncture culture. Positive culture has diagnostic significance. If the urine for bacteria culture cannot be sent in time, it should be temporarily placed in the refrigerator at 4 °C. Otherwise, Will affect the results, a large number of diuretic or have applied antibacterial treatment, it will affect the results of urine culture, urine culture, such as culture positive should be done drug sensitivity test, guide treatment.

4. Direct smear of urine to find bacteria. Use a drop of evenly fresh urine to dry on a glass slide, stain with methylene blue or gram. Under the high magnification or oil mirror, if there is 1 bacteria per field, it means the count of urine colonies. >105/ml, according to the urinary sediment smear Gram stain and bacterial morphology can be used as a reference for the choice of drug treatment.

5. bactericidal urine test urine nitrite reduction test, can be used as screening test, the positive rate can reach 80%.

6. Blood culture infection symptoms should be accompanied by blood culture at the same time, according to symptoms, signs and imaging examination of the urinary tract, can make a correct diagnosis.

7. Intravenous urography can show a large thickening of the dilated ureter, renal pelvis and renal pelvis may be normal, but also can be expanded to varying degrees, but the general development is lighter than the normal side, delayed ureteral drainage can be delayed, there may be renal scar.

8. Urinary bladder urethra angiography The bladder shape is normal, no reflux, no obstruction of the urethra, but severe infection, may have vesicoureteral reflux, inflammatory changes of the bladder.

9. B-ultrasound can be found in the dilated ureter, and can understand both kidneys and bladder.

10. Percutaneous nephrolithotomy B ultrasound shows renal ureteral hydrops, intravenous urography is not clear can be used for percutaneous nephrolithotomy, repeated observation under the screen is very helpful for diagnosis, can fully understand the degree of expansion and its presence or absence Obstruction and obstruction.

11. Radionuclide examination Kidney scan is also helpful for understanding renal function, ureteral excretion and obstruction. Currently, diuretic renal scanning has been widely used in the examination of urinary tract diseases.

12. There is no abnormality in cystoscopy, and there is no obstruction in the ureteral cannula.

Diagnosis

Diagnosis and differential diagnosis of huge bladder-large ureteral syndrome in children

diagnosis

According to the clinical occurrence of recurrent urinary tract infection, combined with venous secretory angiography and cystography, a preliminary diagnosis can be made. The diagnosis conditions proposed by Tongji Xinfu are:

1 congenital;

2 bilateral ureteral dilatation;

3 bladder expansion;

4 irreversible;

5 no urinary tract obstruction;

6 no neurogenic bladder;

7 no dysuria.

Differential diagnosis

1. Obstructive giant ureter congenital ureteral stricture, valve, atresia, ectopic opening, etc., acquired ureteral polyps, stones, infection, trauma, etc., can cause obstructive giant ureter.

2. Reflux giant ureter primary or secondary vesicoureteral reflux, such as secondary congenital posterior urethral valve, urethral stricture, urethral diverticulum, neurogenic bladder, lower urinary tract obstructive disease, etc., neurogenic It is difficult to distinguish the bladder from the disease.

3. Secondary non-obstructive giant ureter such as diabetes, diabetes insipidus, etc., long-term polyuria leads to ureteral dilatation, in order to identify the neurogenic bladder, ureteral electromyography and X-ray TV can be used to continuously demonstrate the motility of the ureter, In addition, cystoscopy and cystography can be performed to confirm the presence or absence of lower urinary tract obstruction.

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