The importance of hereditary and familial incidence, justify the classification of congenital kidney disease proposed by Perkoffa (see the following images). Although relatively rare in the general population, congenital kidney disease must be known, because it allows early diagnosis and treatment of other family members and provides genetic counseling.
Many kidney disease, which can occur as a hereditary listed in 30tom chapter. (Some illnesses with a known mode of inheritance.) Selected diseases will be briefly presented.
First Congenital chronic nephritis
Symptoms of the disease usually appear in childhood, with episodes of hematuria, usually after an upper respiratory tract. Renal failure often develops in men, but only rarely in women. Survival for more than 40 years is rare.
In many families, together with kidney disease occur deafness and abnormalities of the eyes, frequent complications were urinary tract infection.
Histology reminiscent of glomerulonephritis. Often there are fat-filled cells (foam cells) or macrophages, which are derived from or tubular cells. This characteristic appears to kortikomeđularnom date.
Laboratory findings are commensurate with existing renal insufficiency whose.
Treatment is symptomatic.
Second Cystic kidney disease
Congenital abnormalities of renal structure, must always be considered in any patient with hypertension, pyelo, nephritis or renal insufficiency. Events abnormalities of renal structure in connection with priđodadm disease whose course and prognosis of renal disorders altered structure.
Polycystic kidney disease
Polycystic renal disease is often familial and procedures in addition to the kidney and liver, and pancreas.
It is thought that the formation of cysts in the kidney cortex due to a disturbance in the binding collection and konvolutnih individual tubules of the nephron. New cysts are not created, but those that exist beyond the pressure and made the destruction of adjacent tissues. Cysts can be found in the liver and pancreas. The incidence of aneurysms of cerebral blood vessels is higher than normal.
Cases of polycystic disease are discovered during investigation for hypertension, using diagnostic tests in patients with pyelonephritis or hematuria, or researching the family of patients with polycystic disease. Sometimes, lumbar pain, caused by bleeding into the cyst, may draw attention to kidney disorders. Other symptoms and signs are those that are often seen in hypertension or renal insufficiency. At physical examination, it is easy to grope extended, irregular kidneys.
The urine can be found leukocytes and erythrocytes. With bleeding into the cyst, bleeding may occur in the urinary tract. Biochemical blood tests reflect the degree of renal insufficiency. X-ray examination showed enlarged kidneys urography demonstrates a classic elongated calix, renal pelvis, stretched over the surface clean.
There is no specific treatment and surgery is contraindicated if there is no obstruction of the ureter with adjacent cyst. Hypertension, infection and uremia are treated in the usual way.
Although the disease may become symptomatic during childhood or early adolescence, often revealed in the fourth or fifth decade. If there are no fatal complications in the form of nfekcije hypertension and urinary tract infection, uremia develops very slowly and live longer than patients with renal failure due to other causes.
Renal cystic disease of the heart
With increasing frequency, the two syndromes are discovered, because their diagnostic features became better known.
Medullary cystic disease
Familial disease that can cause symptoms during adolescence. Anemia is usually the initial manifestation, and azotemia, acidosis, and hiperfosfatcmija fast becoming manifest. Hypertension can also occur. Finding urine is no abnormality, though there is often a lack of opportunities urinary concentrating. Many small cysts are scattered through the medulla of the kidney.
Sponge kidneys are not producing symptoms and are detected by the characteristic appearance of the urogramu. At the intravenous pyelogram see the expansion cup and papilla and small cavities inside the pyramid. Many small stones often meet cysts and infections can be persistent. Quo ad vitam prognosis is good.
Many kidney disease, which can occur as a hereditary listed in 30tom chapter. (Some illnesses with a known mode of inheritance.) Selected diseases will be briefly presented.
First Congenital chronic nephritis
Symptoms of the disease usually appear in childhood, with episodes of hematuria, usually after an upper respiratory tract. Renal failure often develops in men, but only rarely in women. Survival for more than 40 years is rare.
In many families, together with kidney disease occur deafness and abnormalities of the eyes, frequent complications were urinary tract infection.
Histology reminiscent of glomerulonephritis. Often there are fat-filled cells (foam cells) or macrophages, which are derived from or tubular cells. This characteristic appears to kortikomeđularnom date.
Laboratory findings are commensurate with existing renal insufficiency whose.
Treatment is symptomatic.
Second Cystic kidney disease
Congenital abnormalities of renal structure, must always be considered in any patient with hypertension, pyelo, nephritis or renal insufficiency. Events abnormalities of renal structure in connection with priđodadm disease whose course and prognosis of renal disorders altered structure.
Polycystic kidney disease
Polycystic renal disease is often familial and procedures in addition to the kidney and liver, and pancreas.
It is thought that the formation of cysts in the kidney cortex due to a disturbance in the binding collection and konvolutnih individual tubules of the nephron. New cysts are not created, but those that exist beyond the pressure and made the destruction of adjacent tissues. Cysts can be found in the liver and pancreas. The incidence of aneurysms of cerebral blood vessels is higher than normal.
Cases of polycystic disease are discovered during investigation for hypertension, using diagnostic tests in patients with pyelonephritis or hematuria, or researching the family of patients with polycystic disease. Sometimes, lumbar pain, caused by bleeding into the cyst, may draw attention to kidney disorders. Other symptoms and signs are those that are often seen in hypertension or renal insufficiency. At physical examination, it is easy to grope extended, irregular kidneys.
The urine can be found leukocytes and erythrocytes. With bleeding into the cyst, bleeding may occur in the urinary tract. Biochemical blood tests reflect the degree of renal insufficiency. X-ray examination showed enlarged kidneys urography demonstrates a classic elongated calix, renal pelvis, stretched over the surface clean.
There is no specific treatment and surgery is contraindicated if there is no obstruction of the ureter with adjacent cyst. Hypertension, infection and uremia are treated in the usual way.
Although the disease may become symptomatic during childhood or early adolescence, often revealed in the fourth or fifth decade. If there are no fatal complications in the form of nfekcije hypertension and urinary tract infection, uremia develops very slowly and live longer than patients with renal failure due to other causes.
Renal cystic disease of the heart
With increasing frequency, the two syndromes are discovered, because their diagnostic features became better known.
Medullary cystic disease
Familial disease that can cause symptoms during adolescence. Anemia is usually the initial manifestation, and azotemia, acidosis, and hiperfosfatcmija fast becoming manifest. Hypertension can also occur. Finding urine is no abnormality, though there is often a lack of opportunities urinary concentrating. Many small cysts are scattered through the medulla of the kidney.
Sponge kidneys are not producing symptoms and are detected by the characteristic appearance of the urogramu. At the intravenous pyelogram see the expansion cup and papilla and small cavities inside the pyramid. Many small stones often meet cysts and infections can be persistent. Quo ad vitam prognosis is good.
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