Segmental hypoplasia

August 12, 2017 17:51 | Genetic Diseases

next a rare variant of renal diz-embryogenesis, characterized by the proliferation of segmental areas hypoplastic kidney tissue, a syndrome, which in the literature is called «Ask-Upmark kidney."The main manifestation of the disease is severe hypertension, which is celebrated usually in children under the age of 10 years.

There are 5 groups of disease, depending on the severity of the process on the part of one or both kidneys.This division is based primarily on the results of X-ray contrast examination.

The first group of patients combined with unilateral segmental hypoplasia when compensatory hypertrophy is detected on the opposite side.It was noted in these patients increased renin release diseased kidney, kidney removal at the same time gives a fairly good clinical effect.The second group includes patients with bilateral segmental hypoplasia, who noted a distinct asymmetry of the lesion.Against the most severe prognosis patients considered to belong to the third group.With this defeat there is a bi

lateral symmetrical kidney disease.Typically, these patients there is a constant proteinuria, no compensatory hypertrophy, observed early decline in kidney function.The structure of the fourth group includes patients with relatively advanced disease hidden.Segmental hypoplasia combined with obstructive uropathy of the fifth group.

It is believed that the basis of pathology is a violation of embryogenesis renal vessels.The latter is characterized by impaired blood supply to certain segments of the renal tissue, resulting in a change of differentiation revealed segmental renal structures.

Nefritopodobnaya "unidentified" dysplasia (morphologically hypoplastic dysplasia).In connection with the widespread introduction in nephrology practice method morfobiopticheskogo research it became apparent that the signs dizembriogeneza renal parenchyma are uncommon discovery, and you need targeted attention to the subtle signs of differentiation of renal structures.Otherwise, these manifestations may dizembriogeneza properly considered.Along with the well-rounded forms of renal dysplasia, there are options to indicate that the term "unidentified dysplasia" was proposed.The very introduction of the term involves the need for research aimed at clinical and morphological identification of these forms of renal dizembriogeneza.

kidney disease often detected by chance during clinical examination, registration in an institution or in connection with the development of a concomitant disease.The most common and the most for the first manifestation of disease emergence is a urinary syndrome.Often detected proteinuric (protein in the urine release) version of the disease.Loss of protein per day in some children reaches 10 In some cases, there is a constant in combination with hematuria excretion protein to 1 g per day.This option bladder syndrome is most typical for children with nefritopodobnoy dysplasia.Some patients in the early stages of the disease prevails isolated proteinuria and haematuria (appearance of protein in the urine and blood, respectively), and subsequently awarded an original staging of the disease.Prevailing in such cases the diagnosis is glomerulonephritis.In the treatment of such children are antibacterial and hormonal preparations, and the visible effect of this is not observed.

Almost all children with this pathology can be seen small anomalies of development, similar to that observed in hereditary nephritis.Quite often, the number of such anomalies greater than 7. In half of the children observed anatomical abnormalities of the structure of the urinary system in the form of pathological intrarenal vessels, kidneys increased mobility, doubling kidney ureter contraction, signs of underdevelopment pyelocaliceal systems.

most persistent radiographic signs of pathology - a distinct unevenness kidney circuits (lobulation).In 2/3 of children are slowing excretion of contrast agent during excretory urography, and they are not associated with an abnormality of the upper urinary tract.The dimensions of the kidneys often in the normal range, in some children, they slightly increased or reduced.Renograficheskogo These studies confirm the reduction of the secretory and excretory functions of the kidneys.

Histological examination of kidney tissue taken during biopsy reveals acinar arrangement of glomeruli and reducing their size compared with age-related regulations.In some children, there are signs of nephrotic syndrome, often incomplete, and hypertension (high blood pressure).Determine the single reason that could have a teratogenic effect during embryo development is not possible.