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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

  1. Diseases of the kidneys and urinary system. Prostate diseases
    1. Microscopic characteristics of acute pyelonephritis 1. interstitial edema 2. leukocyte infiltration 3. lymphocytic infiltration 4. tubule atrophy 5. necrosis of the epithelium of the pelvis and tubules 2. Acute post-streptococcal glomerulonephritis is accompanied by 1. doubling of GBM 2. formation of half moon 3. development of hypercellularity. Chronic glomerulonephritis in post-streptococcal
  2. Kidney disease
    Guided by the structural and functional principle, two main groups of kidney diseases, or nephropathies, can be distinguished: glomerulo- and tubulopathies, which can be acquired and hereditary. Glomerulopathies are diseases with primary and primary damage to the renal glomeruli, characterized by impaired filtration. Acquired glomerulopathies include glomerulonephritis,
    Kidney diseases are numerous and varied. The modern classification of kidney diseases, based on the structural-functional principle, provides for the allocation of 5 groups of diseases: glomerulopathy, tubulopathy, stromal disease, neoplasms and congenital anomalies. The basis of glomerulopathy is the primary and primary lesion of the glomeruli, leading to a violation of the filtration of urine.
    • Guided by the structural and functional principle, glomerulopathies and tubulopathies, which can be acquired and hereditary, as well as diseases with primary involvement of interstitium, pyelonephritis and kidney stone disease, are distinguished. • Nephrosclerosis completes the course of many kidney diseases and underlies chronic kidney disease.
  5. Tubulo-interstitial kidney disease
    Tubulo-interstitial kidney disease is a group of diseases of various etiologies in which tubules and interstitial tissue are affected mainly and are caused by toxic damage to the kidneys, malignant neoplasms, pathology of the immune system, vascular disorders, hereditary diseases and infections. The last group of causes includes acute and chronic pyelonephritis.
  6. Abstract. Laboratory diagnosis of kidney disease, 2010
    Purpose of work: to find out which laboratory methods are most significant in the diagnosis of kidney disease. Tasks: - Identify which of the indicators, most clearly indicates pathological
  7. Kidney disease
    Normal kidneys are a paired, continuously functioning organ. Constantly forming urine, they remove metabolic waste products, regulate the water-salt metabolism between blood and other tissues, participate in the regulation of blood pressure and acid-base balance of the blood, and perform endocrine functions. On a section of a kidney it is clearly visible how immediately under a thin connective tissue capsule
  10. Cystic dysplasia
    The absolute morphological criterion for renal dysplasia is the presence of hyaline cartilage and primitive ducts. Cystic dysplasia is characterized by the location in the parenchyma of cysts, separated by the renal tissue and connective tissue layers. Most often, newborns develop polycystic kidney disease and the “Finnish” type of congenital nephrotic syndrome (microcystic kidney disease). DEFINITION Congenital
  11. Early active detection of kidney and urinary tract diseases
    The primary health care team provides level 1 screening, including complaints analysis (rapid questionnaires), results of a clinical analysis of freshly released urine. Risk groups are young children, pregnant women, men over 50 years old, women over 60 years old. If pathology is detected (proteinuria, leukocyturia, hematuria) after a doctor's examination
  12. LESSON 7 TOPIC. Diseases of the kidneys, urinary tract, male reproductive system
    Motivational characteristic of the topic. Knowledge of the morphological manifestations of diseases and syndromes of the kidneys, urinary organs and organs of the male reproductive system is necessary for the successful assimilation of the pathology of these organs in clinical departments. In the practical work of the doctor, this knowledge is necessary for the clinical and anatomical analysis of sectional cases. The general purpose of the lesson. Learn by morphological characteristics
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