renal medullary cystic disease
Introduction
Introduction to renal medullary cystic disease Renal medullary cystic disease refers to the so-called cystic lesions that appear in the renal medulla. The pathological feature is the expansion of the nipple collecting duct, forming numerous cystic cavities of varying sizes. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: kidney stones
Cause
Cause of renal medullary cystic disease
(1) Causes of the disease
It is related to autosomal recessive disease or autosomal dominant inheritance, and there are other unknown factors.
(two) pathogenesis
The pathogenesis of this disease is unclear, and may be a cystic sac cavity formed by the saclike expansion of the collecting duct.
Prevention
Renal medullary cystic disease prevention
Since there is no effective treatment, prevention and treatment of kidney complications and maintenance of renal function are the main preventive purposes.
Patients with this disease are prone to urinary tract infections, especially in women. If the pyelonephritis or cyst infection is induced, the pain in the kidney area is aggravated with obvious fever, hematuria and pyuria. In severe cases, urinary tract sepsis can be caused. Therefore, it is necessary to actively treat the disease and support treatment. Control urinary tract infections and prevent complications such as kidney stones.
Complication
Complications of renal medullary cystic disease Complications kidney stones
1. Medullary Sponge Kidney's clinical manifestations of major complications are kidney stones and kidney infections.
2. Juvenile renal pelvic cystic disease In addition to nephropathy, patients or family members have retinitis pigmentosa, cataract, macular degeneration, myopia or nystagmus.
Symptom
Symptoms of renal medullary cystic disease Common symptoms Drinking hyperparathyroidism secondary infection cysts polyuria renal interstitial fibrosis urinary tract infection chronic renal insufficiency edema pyu
Clinical symptoms
1. Medullary Sponge Kidney: This disease is present at birth, but asymptomatic, urine examination and normal renal function, its clinical manifestations are mainly caused by complications, such as kidney stones and kidney infection, therefore, often Due to acute colic, hematuria, urinary tract irritation, pyuria, etc., the disease was found in further examination. The cystic lesions may have urinary concentrating function and acidification dysfunction, increased urinary calcium excretion, good prognosis, and rare renal failure.
In the past, most patients were diagnosed by IVP. Because half of the patients had kidney stones, a cluster of stones arranged radially in the nipple area was found on the abdominal X-ray film. The contrast agent entered the sac of the nipple, forming a rose pattern, and the renal pelvis often became larger. The width of the kidney is normal or slightly increased.
2. Juvenile Nephronophthisis Medullary Cystic Dis-ease Complex: Most of the disease occurs in children or adolescents. Clinically, the urinary enrichment function appears before renal failure, polydipsia, polydipsia , polyuria and enuresis are characteristic early manifestations, urine can be abnormal except hypotonic, growth retardation, accompanied by anemia, poor renal retention, often hyponatremia and hypovolemia, urinary calcium Increased excretion, resulting in hypocalcemia and hand and foot convulsions, secondary hyperparathyroidism and renal osteodystrophy, gradually developed to renal failure 5 to 10 years after onset.
This type is a common cause of end-stage renal disease in children and young people. It may be a group of joint diseases. In addition to nephropathy, patients or family members have retinitis pigmentosa, cataract, macular degeneration, myopia or nystagmus; The medullary cystic disease is autosomal dominant, and the pathological changes of the four types of kidney are similar, characterized by tubular atrophy, interstitial fibrosis, glomerular sclerosis and small interstitial cyst.
Examine
Examination of renal medullary cystic disease
1. Urine examination: often there are most red blood cells, white blood cells, proteinuria in the late stage, tubular urine, urine specific gravity decreased below 1.010, pus and hematuria can be seen when the infection occurs.
2. Blood biochemical examination: renal dysfunction is often found in the advanced stage, serum urea nitrogen and creatinine are elevated.
3. X-ray inspection:
(1) urinary tract X-ray film: the renal medulla of the renal sputum shows a small number of small stones of varying sizes and sizes, mostly miliary, multiple, clustered or radial, other examinations such as abdomen X-ray plain films showed significant changes in the size of the kidneys on both sides, and there were cystic or calcified shadows.
(2) intravenous urography: a small fusiform cyst filled with contrast agent can be seen around the renal cone and small sputum. Most of the cysts are arranged in a fan-like form of grape cluster or bouquet; the renal sputum is widened and the cup is enlarged.
(3) retrograde pyelography: showing enlargement of the renal pelvis cup.
4. CT and MRI examination: CT and MRI are generally not used as the initial examination method. Because of the large amount of radiation received, the price is more expensive, but the advantage is that it can display different densities of tissues and provide detailed anatomical structure. CT can better Defining the location and nature of the lesion is a helpful test. It is replacing traumatic retrograde and anterior pyelography. MRI is also like CT, and it can clearly show the density of each tissue, clear the cause of obstruction and the nature of the lesion. .
5. Ultrasound examination: Because this is a non-invasive examination, it is highly sensitive to the diagnosis of renal cystic changes. It is also suitable for the examination of polycystic kidney disease. Ultrasound examination can reveal changes in renal volume, deformation of renal pelvis and renal pelvis. The cyst wall is thicker or has varying degrees of calcification and obstruction.
Diagnosis
Diagnosis and diagnosis of renal medullary cystic disease
diagnosis
The diagnosis of this disease is more difficult. The following diagnostic points are: the lesions are mostly bilateral, the age of onset is 40 to 60 years old, and can also be seen in children, more common in men.
1. May have a positive family history.
2. There is often a history of discharge of tiny stones.
3. Clinical manifestations, often asymptomatic, sometimes have the following performance:
(1) hematuria: mostly painless microscopic hematuria, when the stone is discharged, it can be gross hematuria, which can appear repeatedly.
(2) persistent lumbar pain and discomfort: the retention of urine in the renal tubules of the lesion, caused by increased internal pressure, stimulate the renal collecting system when the stone is discharged, causing paroxysmal renal colic.
(3) pyuria: secondary infection may have microscopic or gross pyuria.
(4) Chronic renal insufficiency: anemia, edema and other manifestations often occur in the late stage of the development of the lesion.
Especially children and adolescents with unexplained renal insufficiency, thirst, polydipsia, polyuria and increased sodium and kidney stones and urinary tract infections, individual pigmented retinitis, cataract, macular degeneration, myopia or nystagmus.
Differential diagnosis
The disease must be identified with the following diseases.
1. Renal sputum cyst: There may be lumbar pain and microscopic hematuria, pyuria, but urography shows that the cyst is connected with the renal pelvis or renal pelvis, and the contrast agent is empty; the cyst is mostly unilateral.
2. Renal calcium salt deposition: urinary X-ray film can be seen in the diffuse calcium deposits in the renal cone, but for the performance of a variety of diseases in the kidney, there are clinical features of the primary disease; often accompanied by renal damage; There is no fan-shaped cyst in the venography of the urinary tract.
3. Kidney tuberculosis: urinary tract angiography can also occur in the renal parenchyma, but the cystic cavity occurs mostly on one side, large volume, with the renal pelvis, renal pelvis and renal pelvis destruction, funnel stenosis or atresia; bladder irritation is obvious Acid-resistant bacilli can be found in the urine sediment smear.
4. Necrotic renal papillitis: venous urography can also be found in the small sac of the nipple, but the cystic cavity and the renal pelvis can be connected, which can be multiple, the kidney shadow increases; there are diabetes, urinary tract obstruction, infection, allergies, Oral phenacetin and other medical history; rapid onset, severe systemic symptoms; necrotic nipple tissue can be found in the urine.
5. Pyelonephritis purulent cyst: there is low back pain and hematuria, pyuria, but urography narrows the kidney, irregular expansion of the renal pelvis and renal pelvis, stenosis and distortion, progressive renal dysfunction and hypertension; combined with long-term urinary tract infection The medical history can be identified.
6. Retrograde renal pelvic tract angiography caused by renal tubular reflux: contrast agent is fan-shaped in the renal pyramidal area, but repeated X-rays disappeared after a few days, the disease must also pay attention to polycystic kidney disease, simple renal cyst, isolated Sexual multi-room cysts, liver or kidney hydatidosis, kidney tumors, hydronephrosis and other identification.
