cystic kidney disease
Introduction
Introduction to cystic nephropathy Cystidney disease (cystickidneydisease) refers to a cystic kidney disease in which single or multiple fluids are present in the kidney tissue. In recent years, there has been a great progress in the diagnosis of cystic nephropathy, which was thought to be rare in the past, and it is now found to be a very common group of diseases. For example, B-mode ultrasound can detect cysts of 0.5 to 1 cm in diameter, while CT can find cysts of 0.3 to 0.5 cm. The so-called cyst of cystic nephropathy is not a closed cyst. It is a segmental expansion of the tubular wall of the renal tubules, and most of them are in communication with small lumens. The classification of cystic kidney disease is characterized by a wide variety of cystic kidney diseases, different causes and different prognosis. basic knowledge The proportion of illness: the incidence rate is about 0.006% - 0.007% Susceptible people: no special people Mode of infection: non-infectious Complications: kidney stones, hypertension, urinary tract infection, renal medullary cystic disease, cataract, macular degeneration, myopia, nystagmus
Cause
Cause of cystic nephropathy
(1) Causes of the disease
1. The cause of polycystic kidney disease is gene deletion. The adult polycystic kidney disease is often the deletion of the chromosome 16 gene. Occasionally, the gene of chromosome 4 is deleted, and the penetrance rate is 100%. Therefore, A single-parent chromosomal deletion will give its children a 50% chance of inheriting the disease. Infant polycystic kidney disease is autosomal recessive, and both parents have genetic changes in the disease to make their children ill, with a 25% probability of onset. .
The evidence related to heredity is that Buck et al. reported that eight brothers and sisters, six of whom were kidney cysts, whose father and uncle died of uremia, and five of them had kidney disease in the next generation; Brasck et al. Polycystic kidney disease was reported in 4 families of 1 family; Reason et al reported that the twins were suffering from this disease; Crawford reported that 17 of 40 families had polycystic kidney disease.
70% to 90% of the disease is bilateral, of the 62 cases reported by Lejars, only 3 cases are unilateral; Oickinson reported a ratio of unilateral to bilateral 1:26; see the polycystic kidney volume in the general specimen examination Compared with normal kidney, it is more than 2 to 3 times larger. It has been reported that 1 case of bilateral polycystic kidney weight is 14436g, Schacht reports that 1 cystic kidney is 7248g, and the appearance of polycystic kidney is irregular cystic nodules. See countless cysts of varying sizes in a honeycomb shape.
Infant type (Potter I type) polycystic kidney cyst is the size of the needle, between the cysts, the kidney tissue is very small, when the cyst is enlarged, the renal parenchyma is atrophied due to compression, so the medulla and cortex are incomplete, the cyst It contains yellow-brown mucus, purulent or bloody liquid, etc. The cyst and the renal pelvis are not connected to each other; the normal kidney tissue is extremely rich in adult cysts, and can be connected with the renal pelvis (adult type is Potter III type), Kasper et al. Potter type II polycystic kidney disease, mostly left kidney, generally no clinical symptoms, but also ureteropelvic obstruction or atresia, while other two types of renal pelvis and ureter are often narrowed due to compression, but not blocked; interlobular Arterial branches also have signs of significant oppression. The cystic wall is composed of cuboidal epithelial cells with a secretory function. There are many small arteries underneath, which can cause hematuria due to increased pressure and rupture.
2. Simple cystic kidney disease is a common clinical disease, and the incidence increases in people over 50 years old. The cause is unknown.
3. Renal medullary cystic disease is associated with autosomal recessive genetic disease or autosomal dominant inheritance, and there are other unknown factors.
(two) pathogenesis
Polycystic kidney
The pathogenesis of this disease is unclear. Hilde-brand et al believe that polycystic kidney disease may be caused by the expansion of Bowman's capsule, or it may be caused by the expansion of the renal tubule, which is a glomerulus developed from the posterior kidney germ. The communication with the collection tube developed by the Wolffian tube is obstructed; Bialestack suggests that some cysts are abnormally enlarged nephrons, called giant kidney units, and some cysts are considered to have excretory function; Bricker et al. The chemical analysis of the liquid in the cyst proved that the components contained in it were close to the urine; Norris et al. believed that many temporary nephrons could not be atrophied normally, and some nephrons were locally narrowed and segmented, thus forming different sizes. Cyst; Hepler et al. believe that the blood circulation of the kidney is not normal, resulting in renal parenchymal degeneration; Hidd-brant believes that the secretion (derived from renal tissue and some glomeruli) and excretory (from the kidney) The collecting tube of ureteral buds, renal pelvis, etc.) lose contact with each other during development, the secretory part becomes a blind end, and its secretions are not discharged, so most cysts are formed; others think it is a machine Factors such as local inflammation caused by fetal period fiber excretory duct obstruction, or because of tube obstruction and insoluble calcium salts, so that poor results of urine caused by renal tubular expansion, there are the following two statements could explain some clinical phenomena:
(1) Lambert through the continuous section of the kidney, detailed study of infants and adults with cystic kidneys, pointed out that polycystic kidney cysts have three forms and sources: 1 glomerular cyst; 2 tubular cyst; 3 drain cyst, cyst The wall is lined with cuboidal or flat cells. The interstitial fibers are rich in connective tissue, the medulla and cortex are incomplete, and most of the normal nephron disappears. He found all cysts in the cystic kidney of the baby that are connected to the renal tubules. The function of the small tubules are not connected, the cystic nephron has no function; adult kidney, in addition to the above types of cysts, while some cysts have their own function of the renal tubules and renal pelvis, normal renal tissue between the cysts Extremely rich, and functional activities, so early without clinical symptoms, even when the adjacent kidney tissue is compressed and atrophy can maintain function until the cyst continues to increase, when the renal atrophy occurs, the kidney function declines and dies However, there is still no proper explanation for the cause.
(2) Dammin believes that the glandular system has excessive formation of ductal epithelial cells during normal embryonic development. In the period of development, glandular epithelial cells generally degenerate, digest and disappear, and the initial stage of degeneration is to complete the epithelial tube. Segmentation, if the segment is isolated without retreating, the cyst is formed, that is, the segmental expansion of the proximal end of the renal tubule in the renal cyst, which is still connected with the glomerular filtrate, which is considered to be the posterior kidney ovary and ureter The theory that fetal buds cannot be connected is the opposite, and it can explain that polycystic kidney disease can have multiple cystic diseases of the liver, spleen, pancreas and even ovary, uterus and bladder.
2. The pathogenesis of simple renal cyst may be the tubular diverticulum formed by the day after tomorrow.
3. Renal medullary cystic disease The pathogenesis of this disease is unclear, and may be a cystic sac cavity formed by the saclike expansion of the collecting duct.
Prevention
Cystic nephropathy prevention
Because there is no effective treatment for patients with cystic nephropathy, prevention and treatment of renal complications and maintenance of renal function are the main preventive purposes. Patients with this disease should avoid close contact activities, especially collision and compression, to prevent cyst rupture.
Patients with this disease are prone to high blood pressure, urinary tract infections, urinary calculi, especially women, such as induced pyelonephritis or cyst infection, kidney pain increased with obvious fever, hematuria and pyuria, severe cases can lead to urinary tract sepsis, therefore, It is necessary to actively symptomatic and supportive treatment, control high blood pressure, prevent urinary tract infection, prevent and treat complications such as kidney stones, and prolong the normal survival of patients.
Complication
Cystic nephropathy Complications Kidney stones Hypertension Urinary tract infection Renal medullary cystic disease Cataract macular degeneration Myopia nystagmus
1. Major complications of polycystic kidney disease:
(1) Kidney stones: The low back pain of this disease is usually dull pain, colic with hematuria accompanied by gross hematuria.
(2) Multiple cysts in other organs: About half of the ADPKD patients found in middle age have polycystic liver disease, about 70% after 60 years old. It is generally considered to be slower to develop, and more cystic kidneys are about 10 years later. The cyst is It is formed by the expansion of the labyrinthine bile duct. In addition, the pancreas and ovary can also be complicated by cysts, and the colonic diverticulum rate is also high.
(3) cerebral artery aneurysm: 10% to 40% of patients with this hemangioma, often due to rupture of hemangioma, further examination of cerebral hemorrhage, in addition, thoracic aortic aneurysm and heart valve disease (such as valvular insufficiency) And prolapse) is also more common, in infantile polycystic kidney disease, may be associated with portal hypertension and alveolar dysplasia.
(4) complications such as hypertension or repeated urinary tract infections.
2. The main complications of single cystic kidney are mainly due to cysts located in the renal artery at the renal gate, causing an increase in plasma renin, which can lead to hypertension; or secondary infection of renal pelvis obstruction.
3. Complications of renal medullary cystic disease:
(1) Medullary Sponge Kidney The main complications are kidney stones and kidney infections.
(2) In addition to renal lesions in juvenile renal pelvic medullary cystic disease, patients or family members have common complications such as retinitis pigmentosa, cataract, macular degeneration, myopia or nystagmus.
Symptom
Symptoms of cystic nephropathy Common symptoms Corticosteroids increased polyuria hyponatremia convulsions drinking white blood cells urinary proteinuria dull pain
1. Clinical manifestations of polycystic kidney disease
The kidney size and shape are normal or slightly larger when the disease is young. The number and size of cysts gradually increase and increase with age. In most cases, the kidney volume increases to a considerable extent until 40 to 50 years old, and the symptoms are mainly Lateral kidney enlargement, pain in the kidney area, hematuria and hypertension.
(1) Kidney enlargement: The renal lesions on both sides are asymmetrical, and the size is different. In the late stage, the two kidneys can occupy the entire abdomen, and there are many cysts on the surface of the kidney, which makes the kidney shape irregular, uneven, and hard.
(2) Pain in the kidney area: it is an important symptom, often it is pressure or dull pain in the lower back, and there is also severe pain, sometimes abdominal pain. The pain can be aggravated by physical activity, walking time is too long, sedentary, etc., and can be relieved after bed rest. Intrarenal hemorrhage, stone movement or infection is also the cause of sudden severe pain.
(3) Hematuria: About half of the patients have microscopic hematuria, which may have paroxysmal gross hematuria. This is caused by rupture of the cyst wall. When hemorrhage is frequent, blood clots can cause colic through the ureter. Hematuria is often accompanied by leukocyteuria and proteinuria. The amount of urine protein is small, generally not more than 1.0g / d, pyuria in the kidney infection, hematuria increased, low back pain with fever.
(4) Hypertension: a common manifestation of ADPKD, about half of the hypertension occurs before serum creatinine is elevated, which is related to the cystic compression of surrounding tissues and activation of the renin-angiotensin-aldosterone system. For nearly 10 years, Graham PC Torre V and Chapman AB have confirmed the normal tissues in the kidney of the disease. The cysts are adjacent to the interstitial and cystic epithelial cells, and the renin pigment is increased, and the renin secretion is increased. These are closely related to the growth of cysts and the occurrence of hypertension, in other words, In patients with hypertension, cysts grow faster and can directly affect the prognosis.
(5) Renal insufficiency: Kidney dysfunction occurs sooner or later in this disease. In some cases, renal failure occurs in adolescence. Generally, there is little renal dysfunction before the age of 40. About 70% still maintain renal function at the age of 70, but hypertension The process of developing renal failure is greatly shortened, and some patients can still maintain kidney function at the age of 80.
(6) Polycystic liver disease: About half of ADPKD patients found in middle age have polycystic liver disease, about 70% after 60 years old, it is generally considered that its development is slow, and more cystic kidney is about 10 years later, its cyst is lost by The bile duct is expanded. In addition, cysts can occur in the pancreas and ovaries, and the colonic diverticulum has a higher rate of concomitant.
(7) cerebral artery aneurysm: 10% to 40% of patients with this hemangioma, often due to rupture of hemangioma, further examination of cerebral hemorrhage, in addition, thoracic aortic aneurysm and valvular heart disease (such as valvular insufficiency) And prolapse) is also more common.
Adult polycystic kidney disease usually develops in early adulthood, often with hematuria, hypertension or renal insufficiency. Abdominal palpation can reveal large polycystic kidney disease, renal function is mostly progressive, hypertension, obstruction or Pyelonephritis is an important cause of accelerated renal dysfunction. Autosomal recessive (infant) polycystic kidney disease occurs in infancy, clinically rare, mostly in infancy, and very few people with mild disease can live to adulthood.
The degree of anemia in this disease is often lighter than that of uremia caused by other causes. The reason is that the cyst of the kidney can generally produce erythropoietin.
2. Simple renal cyst
Often in adulthood, usually without clinical symptoms, does not affect kidney function, and occasionally due to hematuria, the vast majority of patients are accidentally found in B-mode ultrasound or CT examination, urine examination is normal, hematuria is rare, such as Hematuria has three possibilities: 1 coexistence of malignant tumors; 2 caused by urinary tract infection; 3 cysts rupture, and often accompanied by pain in the kidney area.
When the cyst is large, it can also cause pain or discomfort in the kidney. Large cysts (>5cm in diameter) can compress the surrounding normal kidney tissue, reduce local blood flow, increase renin secretion, and develop hypertension, but renal function tests are generally performed. In the normal range.
For this disease, the main task of clinicians is to differentiate from other types of renal cystic diseases, especially to exclude cystic tumors. Whether simple renal cysts can cause hematuria is still controversial. Some scholars believe that the hematuria of patients with this disease may be caused by Caused by other concomitant diseases, in a few patients with simple renal cysts, high renin hypertension is caused by cysts located at the renal hilum.
3. The clinical manifestations of renal medullary cystic disease are as follows
(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., further examination revealed this disease, cystic lesions can occur in a wide range of urine concentration and acidification, increased urinary calcium excretion, good prognosis, 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, and more. Drinking, 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, urine Increased calcium excretion, leading to hypocalcemia and hand and foot convulsions, secondary hyperparathyroidism and renal osteodystrophy, gradually develop 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 cystic nephropathy
1. Polycystic kidney: urine test often has pyuria (94%), hematuria (43%), proteinuria (93%), tubular urine (11%), urine specific gravity below 1.010 accounted for 40%, with pyuria Urine culture can be found in Escherichia coli, etc., and kidney dysfunction often occurs in the late stage.
2. Single cystic kidney: urine test may have pyuria and hematuria, but generally no renal insufficiency.
3. Renal medullary cystic disease: urine examination often has a decrease in urine specific gravity, below 1.010, pus, hematuria, proteinuria, tubular urine, late renal dysfunction, blood urea nitrogen, elevated creatinine .
4. Polycystic kidney
(1) Pathophysiological changes of polycystic kidney disease: The disease is generally affected by both kidneys and is rarely unilaterally affected. The weight of a single kidney in a normal adult is about 150g. In the asymptomatic adult polycystic kidney disease, the weight of a single polycystic kidney The average is 256g; in patients with symptomatic adult polycystic kidney disease, the average weight of a single polycystic kidney is 465g. In adult polycystic kidney disease, diffuse cysts are common in the kidney, regardless of the size of the renal cortex and medulla. Cysts, shaped like a bunch of grapes, cystic epithelial cells have localized hyperplasia, polypoid formation, abnormal proliferation of extracellular matrix, cysts formed by proximal tubule bulging, cystic fluid components like plasma; cysts formed by distal tubules, cyst fluid Medium sodium, low chlorine content, and high concentration of urea and creatinine. In patients with symptomatic polycystic kidney disease, with the increase of age, the number of cysts increases, the cystic cavity increases, and the diameter is 2~3cm. Up to 20 ~ 30cm, the whole kidney is almost occupied by cysts, and the cysts with a diameter of >3cm often contain bloody fluid or blood clots.
The polycystic kidney section showed that the cysts were consistently distributed in the cortex and medulla, and the renal pelvis and renal pelvis were often significantly deformed. In severe cases, almost no obvious residual renal tissue was observed, but in patients with mild symptoms, the disease often associated with multiple simple cysts. (such as simple renal cysts, isolated multi-sector cysts) confused.
Microscopic observation showed that the normal kidney tissue was compressed by the adjacent cyst. On the basis of vascular sclerosis or pyelonephritis, glomerular sclerosis, tubule atrophy and interstitial fibrosis were performed. It is difficult to identify the tissue source of the cyst unless the cyst remains. The normal position of the original tissue and the morphological features of the epithelium, cysts from the renal sac sometimes contain deformed small squamous glomerular vascular plexus; cysts derived from the deep concentrating system are often thin-walled; occur in the subcapsular collecting system The cyst wall is thicker and often wraps around dense fibrous connective tissue. The specific plant lectin binding assay can be used to identify the tissue source of the cyst as a proximal tubule, a collecting duct or the like.
(2) imaging examination: including X-ray abdominal plain film examination, CT and MRI examination, angiography [intravenous pyelography (IVP), ureteral pyelography, radionuclide angiography], etc. for routine examination, the disease Renal pyelography (or retrograde angiography if the kidney lacks excretory function), it can be found that the early renal pelvis is caused by cystic compression of the renal pelvis, and the renal pelvis is extended, and the renal pelvis is widened. The border is obvious. Other examinations, such as the plain X-ray film, show that the size of the kidneys on both sides changes significantly, and there are cyst-like or calcified shadows.
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, provide detailed anatomical structure, and CT can better define the location and nature of lesions. It is a helpful method of examination. It is replacing traumatic retrograde and anterior pyelography. MRI is also like CT, and it can clearly show the density of each tissue and determine the cause of obstruction and the nature of the lesion.
(3) Ultrasound examination: Ultrasound examination is very effective in diagnosing renal pelvis and renal pelvis enlargement. It is a priority method for diagnosing renal cystic changes and hydronephrosis. Because it is a non-invasive examination, it is highly sensitive to the diagnosis of renal cystic changes. Sexuality is also applicable to the examination of polycystic kidney disease. Ultrasound examination can reveal an increase in renal volume, cysts, renal pelvis and renal pelvis often deformed significantly, and thicker or different degrees of calcification and obstruction of the cyst wall. Ultrasound can also be used for the disease. Screening.
5. Simple renal cyst
(1) pathological examination: can be seen in unilateral or bilateral kidneys, cysts can be single or multiple, but in each patient generally no more than 5, cysts can be located in the renal cortex or medulla, not connected with the renal pelvis, the wall Thin, clear border, grassy yellow transparent liquid, no red blood cells, white blood cells, calcification can appear on the wall of the capsule.
(2) B-ultrasound examination: the simple renal cyst is a liquid dark area, and the cyst can be single or multiple, which can be unilateral or bilateral.
(3) CT, MRI and fine needle aspiration: CT and MRI findings of tumor-induced renal cysts are different from simple renal cysts. Tumor-like renal cysts often have thick walls, irregular cyst walls, and echoes in the cystic tumor area. There is no echo enhancement on the posterior wall, the interface between the cyst and the renal parenchyma is not smooth, the boundary is not clear enough, and the contrast can be enhanced after intravenous injection of the contrast agent. The cystic fluid of the tumorous renal cyst is bloody or black, and the tumor cells, lactate dehydrogenase and cholesterol are visible. The content is increased.
6. Renal medullary cystic disease
(1) imaging examination: including X-ray abdominal plain film examination, CT and MRI examination, angiography (intravenous pyelography, ureteral pyelography, radionuclide angiography), etc. are routine examination methods, the disease is treated by pyelography ( If the kidney lacks excretory function, it must be retrospectively examined. It can be found that the renal pelvis is compressed by the cyst in the early stage, and the semilunar deformity of the renal pelvis is seen. The late renal pelvis and renal pelvis are extended, the renal pelvis is widened, the boundary is obvious, and other examinations are performed. For example, the abdominal X-ray film shows that the size of the kidneys on both sides changes significantly, and there are cyst-like or calcified shadows.
CT and MRI are generally not used as the initial examination method. The advantage is that the location and nature of the lesion can be better defined, and the density of each tissue can be clearly displayed, and the etiology and pathological properties of the obstruction can be clearly defined, which is beneficial for the exclusion of lesions such as urinary tumors.
(2) Ultrasound examination: renal volume changes, renal pelvis and renal pelvis deformation, and thicker or different degrees of calcification and obstruction of the cyst wall.
Diagnosis
Diagnosis and diagnosis of cystic nephropathy
Diagnostic criteria
1. Diagnosis of polycystic kidney disease
Should be based on its clinical manifestations such as bilateral kidney enlargement, urinary abnormalities, high blood pressure, etc. should be suspected of the disease, if there is a family history, this disease can be more suggestive, B-mode ultrasound, CT and magnetic resonance imaging can be found A characteristic double renal cyst can be established by diagnosis.
The number of early renal cysts in this disease is not much, it can be unilateral. If the number of renal cysts increases or extrarenal cysts appear within a few years, the diagnosis of ADPKD is also certain. In recent years, DNA such as 3'HVR, PGP and 24-1 was applied. The probe, the gene linkage analysis method for the diagnosis of cyst genes is extremely reliable, and can detect heterozygous family members and asymptomatic patients.
Patients with mild symptoms are often misdiagnosed as simple renal cysts, solitary multiple cysts and other simple cysts. Family history and concurrent hepatic cysts can help differential diagnosis.
Hematuria should be identified with new diseases such as neoplasms, kidney stones and other hematuria. Pay attention to polycystic kidney stones or cysts, etc. Coagulation screening (PT, APTT and platelets) should be performed to rule out bleeding disorders. Patients with a family history of subarachnoid hemorrhage have a cerebrovascular MRI.
The diagnosis of this disease mainly relies on CT scan to exclude kidney tumors. MRI examination can help identify other cystic diseases and congenital hydronephrosis. In addition, MRI can determine the location, distribution, number, size, and size of cysts. The kidney, kidney and kidney are connected.
2. Simple renal cyst
According to clinical manifestations, it is mainly secondary urinary tract infection caused by hypertension and renal pelvic obstruction.
The diagnosis of this disease mainly depends on B-mode ultrasound or CT examination, especially in the elderly, a single cyst should be differentiated from the tumor on the basis of the cyst or cyst formed by malignant tumor necrosis. Therefore, the suspected type should be carefully observed for type B. Ultrasound or CT imaging, if necessary, cyst puncture, cystic fluid is blood or brown, lactate dehydrogenase increased, suggesting malignant tumors, such as finding tumor cells, the diagnosis is more reliable, after the cystic fluid is drawn, the same amount of contrast agent is injected. It is also helpful to identify benign or malignant cysts. The simple lining of the cyst is smooth, occasionally incomplete paralysis, malignant tumor wall thickness, irregular lining and unevenness.
It is not uncommon for the elderly to have several cysts on one or both sides of the kidney. Because of the large number of cysts, it is called multiple simple renal cysts. Many clinicians misdiagnosed as polycystic kidney disease (ADPKD). Ziegler et al. It is not easy to count the number of cysts in the cystic kidney. The following points will help identify:
(1) Simple renal cysts The size and shape of the kidneys are basically normal unless there is a large cyst.
(2) simple renal cysts are mostly unilateral, such as bilateral, the number of cysts is not much, easy to count the number.
(3) There are no ADPKD patients in immediate family members.
(4) There is no kidney stone, and there is very little high blood pressure.
(5) no liver, pancreatic cyst or intracranial aneurysm coexist.
(6) There are more normal kidney tissues between the cysts and the cysts. In addition, children in the ADPKD family have 1 or 2 cysts in the kidney during examination, which strongly suggests ADPKD, because simple renal cysts are rare in children. After 2 to 3 years, the number of cysts is increased and the diagnosis of ADPKD is confirmed.
3. Renal medullary cystic disease
Diagnosis is difficult, the following points can be used as diagnostic clues and basis:
(1) Positive family history.
(2) Kidney dysfunction in children and adolescents with unknown causes.
(3) thirst, polydipsia, polyuria and increased sodium and kidney stones and urinary tract infections.
(4) The size of the kidney is small. Because the cyst is small and small, it is easily overlooked in the imaging examination and should be taken alert.
(5) retinitis pigmentosa, cataract, macular degeneration, myopia or nystagmus.
Differential diagnosis
1. Differential diagnosis of polycystic kidney disease: this disease must be associated with simple renal cyst, solitary multiple cyst, multiple simple cysts, kidney tuberculosis, liver or kidney hydatidosis, kidney tumor, hydronephrosis, chronic nephritis, Renal pyelonephritis and other organ cysts in the abdominal cavity are identified, and those who have hematuria must be identified with other diseases such as neoplasms and kidney stones that cause hematuria.
2. Differential diagnosis of simple renal cyst: the disease needs to be differentiated from polycystic kidney disease, neoplastic renal cyst, cystic adenoma or cystic tumor.
3. Differential diagnosis of renal medullary cystic disease: this disease must be associated with polycystic kidney disease, simple renal cyst, isolated multi-sector cyst and other multiple simple cysts, renal tuberculosis, liver or kidney hydatidosis, kidney tumor, renal pelvis Identification of stagnant water, chronic nephritis, pyelonephritis and other organ cysts in the abdominal cavity.
