Segmental hypoplasia

The next rather rare variant of renal dis-embryogenesis, characterized by segmental spread of hypoplastic kidney tissue, is syndrome, which in the literature is called the "Ask-Upmark kidney".The main manifestation of the disease is severe hypertension, which is noted in children usually under the age of 10 years.

There are 5 groups of the disease, depending on the severity of the process from one or both kidneys. This division is based mainly on the results of radiopaque studies.

The first group includes patients with unilateral segmental hypoplasia, when compensatory hypertrophy is detected on the opposite side. It was in these patients noted increased renin release of the affected kidney, the removal of the kidney while giving a fairly good clinical effect. The second group includes patients with bilateral segmental hypoplasia, who have a distinct asymmetry in the lesion. The most severe in relation to the prognosis are the patients who are included in the third group. With this lesion, bilateral symmetrical renal damage is observed. Usually, such patients have permanent proteinuria, there is no compensatory hypertrophy, an early decrease in renal function is observed. The fourth group includes patients with a relatively hidden development of the disease. Segmental hypoplasia in combination with obstructive uropathy is the fifth group.

There is an opinion that at the heart of the pathology is the violation of renal vascular embryogenesis. The latter characterizes the violation of the blood supply of certain segments of the renal tissue, as a result of which a segmental change in the differentiation of renal structures is revealed.

Jade-like "unidentified" dysplasia( morphological and hypoplastic dysplasia).In connection with the widespread introduction of the method of morphobiotic investigation into nephrological practice, it became evident that the signs of dysembryogenesis of the renal parenchyma are often a find, and careful attention should be paid to the subtle signs of a disorder in the differentiation of renal structures. Otherwise, these manifestations of disembryogenesis may be incorrectly evaluated. Along with clearly delineated forms of renal dysplasia, there are variants for designating which the term "unidentified dysplasia" was proposed. Already the very introduction of this term provides for the need for research aimed at the clinical and morphological identification of these forms of renal dysembryogenesis.

The pathology of the kidneys is more often detected accidentally during clinical examination, registration in a children's institution or in connection with the development of some concomitant disease. The most common and the very first manifestation of pathology is the urinary syndrome. Often find a proteinuric( with the release of protein in the urine) variant pathology. The loss of protein per day in some children reaches 10 g. In some cases there is a constant hematuria in combination with the excretion of protein to 1 g per day. This variant of the urinary syndrome is most typical for children with jade-like dysplasia. At the first stages of the disease, isolated proteinuria or hematuria predominate in some patients( the appearance of protein and blood in the urine, respectively), and subsequently a specific stage of the disease is noted. The predominant diagnosis in such cases is glomerulonephritis. In the process of treatment, such children receive antibacterial and hormonal drugs, and there is no visible effect from this.

Almost all children with this pathology can see small anomalies of development, close to those that are noted in hereditary nephritis. Quite often, the number of such anomalies is more than 7. Half of the children have anatomical abnormalities in the structure of the urinary system in the form of pathologies of the kidney vessels, increased mobility of the kidneys, doubling of the kidneys with narrowing of the ureter, signs of hypothyroidism.

The most consistent X-ray signs of pathology are the distinct irregularity of the kidney contours( lobulation).In 2/3 of the children, a decrease in the excretion of contrast medium during excretory urography is noted, and they are not associated with an abnormality of the development of the upper urinary tract. The size of the kidneys is often within normal limits, in some children they are slightly enlarged or reduced. The data of the renography study confirm the decrease in the secretory and excretory functions of the kidneys.

Histological examination of the kidney tissue taken during biopsy reveals a clustered arrangement of glomeruli and a decrease in their size in comparison with age standards. Some children have signs of nephrotic syndrome, more often incomplete, and hypertension( increased blood pressure).Identify a single cause that could have a teratogenic effect during embryo development, it is not possible.