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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 spongiform kidney

* nephronophthisis - uremic medullary cystic disease

* Acquired (due to dialysis) cystic disease

* Localized (simple) kidney cysts

* Kidney cysts in congenital malformations (e.g. tuberous sclerosis)

* Glomerulocystic disease

* Extraparenchymal renal cysts (pelvic and pelvic cysts, lymphangitic cysts of the renal collar)

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

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 the organ during embryogenesis and the postpartum period). The disease is caused by an anomaly in the differentiation of metanephros, characterized by persistence in the kidney of abnormal structures (cartilage, undifferentiated mesenchyme, immature collecting tubes) and anomalous organization of lobules. In most cases, it is accompanied by ureteropelvic obstruction, urethral agenesis and atresia, and other urinary tract abnormalities. The indicated renal dysplasia is a sporadic disease not associated with a family 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 (a few centimeters in diameter). Cysts are lined with flattened epithelium. Along with normal nephrons, there are many nephrons with immature tubules. 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 that affects approximately 1 person per 1000. It affects 10% of people who need a kidney transplant and chronic hemodialysis. Such a disease is always bilateral. Initially, only individual portions of nephrons are involved in the formation of cysts. Therefore, kidney function remains intact until the age of 40-50. Most patients develop hypertension or chronic renal failure progresses. In autosomal dominant polycystic kidney disease, bilateral renal enlargement is observed. The kidneys can reach very large sizes and have a mass of up to 4 kg each. Their outer surface is deformed by many cysts with a diameter of 3-4 cm, between which there are areas of preserved parenchyma. Functioning nephrons can be detected under a microscope. The cysts are filled with a clear serous or cloudy reddish-brown fluid (the result of hemorrhages). They are formed from tubules and are usually lined with transitional epithelium. With 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, cerebral hemorrhage, rupture of aneurysms) and 30% from causes that are not related to the underlying disease.

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

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

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

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

Acquired cystic disease (associated with dialysis). In the kidneys of patients with end-stage kidney disease, which requires prolonged dialysis (purification of blood from metabolic toxins using the "Artificial Kidney" apparatus), numerous cysts in the cortical and brain substance are sometimes detected. Cysts with a diameter of 0.5-2 cm contain a clear liquid and often crystals of calcium oxalate, lined with hyperplastic or flattened tubular epithelium. Perhaps they are formed as a result of tubular obstruction due to interstitial fibrosis or blockage of the tubules by oxalate crystals.

Simple cysts. Both single and multiple cysts are usually found in the cortical substance. More 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 detected during pathological examination.

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Cystic kidney disease

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