Primary hyperoxaluria( oxalose)
Primary hyperoxaluria is a hereditary metabolic pathology characterized by periodic exacerbations, oxalate-calcium nephrolithiasis( kidney stones), which gradually leads to chronic renal failure.
Symptoms of the disease are first detected in early childhood - hematuria( the appearance of blood in the urine), leukocyturia( the appearance of leukocytes in the urine), renal colic. On the roentgenogram of the abdominal cavity, dense multiple concretions( stones) in the kidneys are found. In some cases, oxalosis is first manifested by a delay in the growth of the child and signs of uremia( this is the last stage of kidney failure), renal tubular acidosis( a violation of the acid-base balance in the body as a result of impaired renal function).In another part of the people suffering from this disease, the primary hyperoxaluria( the appearance in the urine of a large number of oxalates - oxalic acid salts) was manifested only in adulthood in the form of kidney failure. In oxaluria, the kidneys are the main target organs, i.e., organs susceptible to pathological changes.
The defeat of the kidneys with oxalose is manifested mainly by changes in the urine( protein, the appearance of a small number of red blood cells, leukocytes).The general condition of the child remains satisfactory. Sometimes pain syndrome, enuresis( bedwetting) is recorded. The emergence of these symptoms is due to the fact that crystals of oxalates have an irritant effect on the urinary tract. Bacteriuria( appearance of bacteria in the urine) is usually absent, functional tests of the kidneys do not detect abnormalities. Load tests( water, saline, ammonium chloride loading) can reveal the failure of kidney function. Prolonged damaging effects of crystals can lead to the development of inflammatory changes in both the urinary tract and in the kidney( interstitial nephritis).Thanks to this, conditions are created for triggering the inflammatory reaction, first an abacterial, and in the subsequent - with a layering of microbial infection. The general condition of the child worsens, an "inexplicable" increase in body temperature( no higher than 38 ° C), pain syndrome, dysuric disorders( changes in frequency of urination, volume and quality of urine), a decrease in the concentration ability of the kidneys. With the progression of the disease, the development of pyelonephritis and renal-stone disease is likely.
The incidence of renal pathology among relatives of the first degree of kinship in families with hyperoxaluria is 34.2%.
Calcium oxalate is the main component of kidney stones in almost 75% of cases of kidney stone disease. However, in most people with a recurrent type of disease, hyperoxaluria is not detected. They have significant fluctuations in the content of oxalic acid in the urine during the day. At the same time, the urine of healthy people, as a rule, is oversaturated with oxalate and calcium ions, which explains the significant frequency of formation of kidney stones in them.
The basis of treatment of oxalate diathesis is a rational diet that reduces the functional burden on the kidneys. A potato-cabbage diet is proposed that provides for the exclusion of saturated extractive meat dishes, oxalic products such as deciduous vegetables( sorrel, lettuce, spinach) from the patient's diet, tea, cocoa, chocolate, cranberries, carrots, beets. Recommended "fresh" fruit - pear, prunes, dried apricots.
The use of ipraside causes a decrease in the excretion of oxalate in the urine. Justified the appointment of allopurinol, the purpose of which is to reduce the intensity of excretion through the kidneys of uric acid, which can promote the crystallization of calcium oxalate and the formation of kidney stones. Combined use of pyridoxine and magnesium oxide is widely used. The effectiveness of pyridoxine can be traced only in some people with this disease. A positive result is observed when xyliphon is used orally, which increases the solubility of calcium salts, inhibits the growth of sodium citrate crystals.