Kidney Dysplasia in Children - Diagnosis and Treatment of Disease
Kidney dysplasia in children is a process of abnormal development of the genitourinary system, which is accompanied by a decrease in the volume of the kidney, abnormal tissue development and impaired urinary function.
Etiology
Basically, the cause of kidney dysplasia in children is, genetic disorders or various adverse effects on the fetus in the womb. Most often, it is not possible to establish the initial cause of the development of pathology.
To date, renal dysplasia is usually divided into simple and cystic.
Localization is simple:
- focal;
- total;
- are segmented.
Simple focal dysplasia
Simple focal dysplasia does not give almost no symptoms, so it can be detected only with biopsy.
Externally the kidney does not change, its dimensions remain the same as well as the surface. The centers of renal dysplasia can be detected only microscopically in the cortex and juxtamendullar zone, very rarely they can be found in the cerebral layer.
Total dysplasia
It is almost 3% of all registered congenital diseases of the genitourinary system. Macroscopically, this pathology is almost not manifested: the shape of the kidney does not change, the surface is smooth, sometimes it is possible to detect cystic foci with catarrhal exudate.
This type of pathology manifests itself as a urinary syndrome and is characterized by the development of renal failure. A child with such a diagnosis has a large number of small anomalies of development, slightly behind in development, mostly physical, from his peers, diagnosed immunodeficiency states and lowering blood pressure.
Depending on the mass, total dysplasia is divided into aplastic and hypoplastic.
This is important! Aplastic dysplasia in children refers to cystic dysplasia. This disease is characterized by a critical reduction in size almost and disabling its functions. Two-sided aplastic dysplasia, in most cases, leads to a lethal outcome. Developed renal dysplasia in the fetus is accompanied by the defeat of other organs and systems. The most common lesion of the reproductive system and digestive system.
Hypoplastic dysplasia is characterized by the appearance of a urinary syndrome and early development of CRF.
Morphologically observed: a decrease in the total mass of the kidney, lobular surface and not always clearly visible boundaries between the layers. During the investigation of the material of the affected kidney, primitive changes in the structures are detected microscopically: a decrease in the size of the glomeruli, atrophy of the vascular loops and a thickening of the capsule.
Clinically, hypoplastic dysplasia occurs early enough - at 4-7 months. In newborn children, this pathology manifests itself only as signs of dysplasia, more at an older age they are joined by sclerotic lesions of the glomeruli, hyalinosis, hyperplasia of the arteries and thyroid-like changes in the tubules.
Simple segmental dysplasia
This type of renal dysplasia is extremely rare, up to 0.03% of all diseases affecting the kidneys. This pathology is characterized by a decrease in the size of the organ and the number of pyramids, the appearance of a transverse groove on the outer surface of the hypoplastic segment. Segmental dysplasia is accompanied by the development of sclerosis, fibrosis, hyalinosis glomerulus, cellular infiltration and atrophy of the epithelium.
The leading sign of the development of pathology is the manifestation of hypertension at an early age, more common in girls. Children mostly complain of frequent headaches, convulsions, nausea and occasional dizziness.
From the clinical symptoms, the first to show pain syndrome, characterized by aching pain in the lower abdomen. Relatively early there is polyuria and polydipsia, in some cases there is a sharp decrease in the body weight of the child and a lag in growth. The urinary syndrome manifests itself mainly in proteinuria.
Cystic renal dysplasia
Multi-cystic is characterized by a sharp increase in the size of the kidney due to the large number of cysts, between which there is practically no parenchyma. This kind of pathology is manifested by dull pain in the abdomen and the development of hypertension. Bilateral renal damage leads to death, one-sided - to the defeat of other organs and systems of the body.
Medullary renal dysplasia is characterized by a decrease in the organ and preservation of embryonic lobulation. Microscopically detected a large number of glomeruli, proliferation of connective tissue and infiltration. Quite often during the diagnosis there is thinning of the cortex, enlargement of the cerebral cyst due to the presence of cysts with a diameter of more than 1-1.5 cm,
Clinically, the disease manifests itself at the age of 3 to 6 years, most often appears "symptom complex Fanconi" - polyuria, subfebrilebody temperature, developmental delay, frequent vomiting, acidosis, anemia, uremia and polydipsia.
Multilocular dysplasia is focal cystic dysplasia, which is most often localized in one of the poles of the kidney and has the appearance of a multi-compartment cyst, divided inside by septa and separated from the healthy tissue by a capsule.
Clinically, there is a pain syndrome in the abdomen and lower back, as well as syndromes associated with the digestive organs. This is due to the fact that the enlarged kidney can squeeze the surrounding organs.
Cortical dysplasia is the so-called "microcystosis of the kidney" which is characterized by the presence of small glomerular and tubular cysts with a diameter of up to 3 mm. The organ with such a disease will not change its original shape and structure. Cortical dysplasia is accompanied by early development of renal failure.
Treatment of kidney dysplasia in children is aimed primarily at eliminating the symptoms of chronic insufficiency and general malaise. In rare cases, transplant organ.
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