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Cystic kidney disease



Cystic kidney disease is a heterogeneous group, including congenital and acquired diseases, as well as malformations. We give a classification of cystic kidney disease.

* Cystic renal dysplasia * Polycystic kidney disease

autosomal dominant polycystic disease (adults)

autosomal recessive polycystic disease (children)

• Medullary cystic disease medullary spongy kidney

* nephronophthysis - uremic medullary cystic disease

* Acquired (due to dialysis) cystic disease

* Localized (simple) kidney cysts

* Renal cysts in syndromes of congenital abnormalities (for example, tuberous sclerosis)

* Glomerulocosis disease

* Extraparenchymal renal cysts (lobe-cup cyst, lymphatic cyst of the kidney gate)

Cysts develop as a result of a genetic or acquired abnormality, leading to prolonged cell proliferation, transepithelial fluid secretion, rearrangement of the extracellular matrix and the formation of cysts.

Cystic-renal dysplasia (the term "dysplasia", which in other chapters means the stage of development of tumors, here means a violation of the development of an organ during embryogenesis and the postpartum period). The disease is caused by anomalous differentiation of metanephros, characterized by persistence in the kidney of abnormal structures (cartilage, undifferentiated mesenchyme, immature collecting tubes) and anomalous organization of the lobules. In most cases, it is accompanied by ureteral pelvis obstruction, agenesis and urethral atresia, and other urinary tract abnormalities. This kidney dysplasia occurs as a sporadic disease, not associated with familial predisposition. It can be single or double sided. With this anomaly, the kidneys are enlarged, have an irregular shape and a polycystic appearance due to cysts of different sizes - from microscopic to large (several centimeters in diameter). Cysts are lined with flattened epithelium. Along with normal nephrons, many nephrons with immature tubules are found. A characteristic feature of the histological structure of such a kidney is the presence of islands of undifferentiated mesenchyme, often with cartilage and immature collecting ducts.

Autosomal dominant polycystic kidney disease in adults. This is a relatively common disease, affecting approximately 1 person in 1000. They suffer from 10% of those in need of kidney transplantation and chronic hemodialysis. Such a disease is always bilateral. Initially, only separate portions of nephrons are involved in the formation of cysts. Therefore, the kidney function remains intact until the age of 40-50. In most patients, hypertension develops or chronic renal failure progresses. In an autosomal dominant polycystic kidney disease, bilateral renal enlargement is observed. Kidneys can reach very large sizes and have a mass of up to 4 kg each. Their outer surface is deformed by a set of cysts 3-4 cm in diameter, between which there are areas of the intact parenchyma. Under the microscope, you can detect functioning nephrons. Cysts are filled with clear serous or turbid red-brown fluid (the result of hemorrhages). They are formed from tubules and are usually lined by transitional epithelium. In this disease, azotemia is observed for a long time, slowly progressing to uremia.
About 30% of patients die from renal failure, 30% from complications of hypertension (heart disease, hemorrhages in the brain, ruptures of aneurysms) and 30% from causes not related to the underlying disease.

Autosomal recessive polycystic kidney disease in children. It is not genetically related to polycystic adult kidney disease and is inherited in an autosomal recessive manner. Perinatal, neonatal, infantile and juvenile subtypes of the disease are known, which are allocated depending on the time of manifestation and the presence of associated liver changes.

The buds are enlarged, have a smooth surface. The incision in the cortex and medulla shows numerous small cysts, which creates a spongy appearance of the tissue of the organ. Enlarged elongated tubules are located at right angles to the surface of the kidney and replace the cortex and medulla. Histologically detect saccular or cylindrical expansion of the collecting tubes. Cysts are lined with cubic cells, indicating their origin from the collecting tubes. The disease is always bilateral.

In almost all cases, there are numerous cysts in the liver, lined by epithelium. In addition, the proliferation of portal bile ducts is noted.

Cystic diseases of the brain substance of the kidneys. The two main types of cystic disease of the medulla of the kidneys are spongy medulla of the kidney and nephronophthysis. The term “spongy substance of the kidney” reflects the multiple cystic expansion of the collecting ducts in the medulla. Nephronophthysis - the uremic complex in cystic medulla - is a group of progressive kidney diseases that usually begin in childhood. Their general characteristic: a different number of cysts of the medulla, accompanied by significant atrophy of the tubules of the cortex and interstitial fibrosis. Macroscopically, the kidneys are small with a granular surface. In the medulla visible cysts, especially numerous in the cortico-medullary region. Small cysts are also found in the cortex. They are lined with flat or cubic epithelium and surrounded by inflammatory cells or connective tissue.

Acquired cystic disease (associated with dialysis). In the kidneys of patients with end-stage kidney disease, requiring prolonged dialysis (blood purification from metabolic slags using the “Artificial Kidney” apparatus), numerous cysts in the cortex and medulla are sometimes detected. Cysts with a diameter of 0.5-2 cm contain a clear liquid and often calcium oxalate crystals, lined with hyperplastic or flattened tubular epithelium. They are probably formed as a result of obstruction of the tubules due to interstitial fibrosis or blockage of the tubules with oxalate crystals.

Simple cysts. Both single and multiple cysts are usually found in the cortex. Most often they have a diameter of 1-5 cm, but can reach a size of 10 cm or more. They are translucent, surrounded by a gray, shiny, smooth membrane and filled with a clear liquid. Microscopically, these membranes consist of a single layer of cubic or flattened epithelium, which in most cases is completely atrophied. Simple cysts are most often found in the post-mortem examination.

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Cystic kidney disease

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