Dysmetabolic nephropathy - a group of diseases that are characterized by renal impairment due to metabolic disorders.
Depending on the cause of isolated primary and secondary dysmetabolic nephropathy.
Primary are hereditary diseases characterized by progressive course, early development urolithiasis and chronic renal failure.
Secondary dysmetabolic nephropathy may be associated with an increased intake of certain substances in the body, metabolic disorders due to their involvement of other organs and systems (e.g. the gastrointestinal tract), drug therapy and others.
The vast majority of dysmetabolic nephropathies associated with impaired calcium metabolism (from 70 to 90%), about 85-90% of them - with an excess of the oxalate salt acid oxalate (in the form of calcium oxalate), the other with an excess of phosphate (calcium phosphate - 3.10%) are mixed, or - oxalate / phosphate urate.
Primary dysmetabolic nephropathy rare.
Manifestations dismetabolic nephropathy
Calcium oxalate nephropathy
It most often occurs in childhood. Its appearance may be associated with impaired calcium metabolism and the metabolism of oxalate (oxalic acid salts).
Oxalate ingested with the diet or synthesized by the body.
Reasons for the formation of oxalate:
- Increased intake of oxalate from food
- bowel disease - Crohn's disease, ulcerative colitis, Intestinal anastomoses
- Increased production of oxalate the body.
oxalate nephropathy
It is a multifactorial disease. According to various authors, the share of heredity in the development of oxalate nephropathy is up to 70-75%. In addition to genetic important role played by external factors: diet, stress, environmental pressures, and others.
The first manifestations of the disease can develop at any age, even in the neonatal period.
Most often, they are revealed in 5-7 years in the form of detection of oxalate crystals, low content of protein, white blood cells and red blood cells in urinalysis. characteristically increase urine specific gravity.
The overall development of children with oxalate nephropathy, as a rule, does not suffer; but they are characterized by allergies, obesity, Autonomic dystonia with a tendency to a decrease of blood pressure (hypotension), Headaches.
The disease is aggravated during puberty between the ages of 10-14 years, which, apparently, due to the hormonal changes.
Progression of nephropathy oxalate can lead to the formation of kidney stones, kidney inflammation in the development of stratification of the bacterial infection.
phosphate nephropathy
Phosphate nephropathy occurs in diseases associated with impaired calcium and phosphorus metabolism. The main reason phosphaturia - chronic infection of the urinary system.
Often calcium phosphate nephropathy accompanies calcium oxalate, but less pronounced.
Metabolic disorders of uric acid (urate nephropathy)
Per day in the body is formed 570-1000 mg uric acid, one third of the amount which is secreted into the intestine and breaks there bacteria.
The remaining two thirds are filtered in the kidney, the most part is sucked back, and only 6-12% of the filtered amount excreted in the urine.
Primary urate nephropathy caused by hereditary disorders of uric acid metabolism.
Secondary occur as complications of other diseases (eritremii, multiple myeloma, A chronic hemolytic anemia and al.), Are a consequence of the use of certain drugs (thiazide diuretics, cytostatics, salicylates, cyclosporin A et al.) or dysfunction of kidney tubules and physico-chemical properties of the urine (in kidney inflammation, eg).
Urate crystals are deposited in kidney tissue - it leads to the development of inflammation and reduced kidney function.
The first signs of the disease can be detected at an early age, although in most cases there is a long latent throughout the process.
A urinalysis reveal urate, small amounts of protein and erythrocytes. In the presence of large amounts of urine becomes urate brick color.
Violations of cystine metabolism
Cystine, an amino acid is a product of methionine metabolism. There are two main reasons for the increase of cystine concentration in urine:
- excessive accumulation of cystine in the kidney cells
- violation of cystine reabsorption in the renal tubules.
Cystine accumulation in cells is the result of a genetic defect tsistinreduktazy enzyme. It is a sharing violation is systemic and is called cystinosis.
Intracellular and extracellular accumulation of cystine crystals is revealed not only in the kidney tubules and interstitium, but and in the liver, spleen, lymph nodes, bone marrow, peripheral blood cells, nervous and muscular tissue, other bodies.
Violation of cystine reabsorption in kidney tubules observed due genetically defect due to transport through the cell wall of the amino acids - cystine, arginine, lysine, and ornithine.
With disease progression, symptoms defined urolithiasis and accession infection - inflammation of the kidneys.
Diagnostics
Laboratory and instrumental diagnostics dismetabolic nephropathy is based on
- identifying salt crystals urinalysis,
- increasing the concentration of certain salts in the biochemical study of urine
- study antikristalloobrazuyuschey ability urine (AMCEN)
- conducting tests calciphylaxis and peroxide in the urine,
- renal ultrasound.
Identification salt crystals only in general urine analysis is not a basis for diagnosis dysmetabolic nephropathy. It should be borne in mind that the selection of crystals in the urine of children is often transient and is not associated with metabolic disorders.
To confirm the diagnosis dysmetabolic nephropathy in detecting crystals of salts in urinalysis performed urine biochemical research.
Test calciphylaxis reveals violations of cellular calcium metabolism. Test for peroxide in the urine reflects the active process of cell membrane peroxidation.
The changes revealed by ultrasound of the kidneys, as a rule, there is little specific. Ability to detect kidney mikrokameshkov or inclusions.
Treatment dismetabolic nephropathy
Treatment of any dismetabolic nephropathy can be reduced to four basic principles:
- normalization of lifestyle;
- proper drinking regime;
- diet;
- specific therapies.
Receiving a large amount of liquid is a universal method of treating any dysmetabolic nephropathy, since it facilitates the reduction of solubles concentration in urine.
One of the goals of treatment is to increase night urination volume, which is achieved by taking the fluid before bedtime. Preference should be given to simple or mineral water.
Diet can greatly reduce the salt load on the kidneys.
Specific therapy should be aimed at the prevention of crystal formation, salt excretion, normalize metabolic processes.
Treatment of the oxalate nephropathy
Advice on nutrition
- When treating patients with oxalate nephropathy assigned potato cabbage diet, at which reduced food intake of oxalate and the load on the kidneys.
- It is necessary to eliminate jelly, strong meat broth, spinach, cranberries, beets, carrots, cocoa and chocolate.
- It is recommended to introduce apricots diet, prunes, pears.
- Mineral waters used such as Slavyanovskaya and Smirnovskaya at 3-5 ml / kg / day. 3 admission rate 1 month 2-3 times a year.
drug therapy
Drug therapy includes membrane-drugs and anti-oxidants. Treatment should be long-lasting.
- Pyridoxine (vitamin B6) is administered in a dose of 1-3 mg / kg / day. for 1 month on a quarterly basis.
- Vitamin B6 has a membrane stabilizing effect due to participation in the metabolism of fats as an antioxidant and metabolism of amino acids. It is also expedient purpose magne B6 preparation the rate of 5-10 mg / kg / day. course for 2 months, 3 times a year.
- Membrane stabilizing action renders vitamin A, which normalizes the interaction of proteins and lipids of the cell membrane. The daily dose of vitamin A 1,000 IU in the year a child's life course - 1 month on a quarterly basis.
- Tocopherol acetate (vitamin E) is a powerful antioxidant that enters into the body from outside and generated by the body itself. It must be remembered that the excessive intake of vitamin E may inhibit food products at its inner negative feedback mechanism. Vitamin E strengthens the protein-lipid communication cell membranes. Is assigned with vitamin A at a dose of 1-1.5 mg / kg of body weight per day.
As used Membrane dimephosphone and ksidifon.
Dimefosfon applied in a dose of 1 ml 15% solution per 5 kg body weight, 3 doses per day. Course - 1 month, 3 times a year.
Ksidifon prevents the deposition of insoluble calcium salts. Administered in a dose of 10 mg / kg / day. 2% solution in 3 doses. Course - 1 month, 2 times a year.
Tsiston has high efficiency, especially when crystalluria. Tsiston administered in a dose of 1-2 tablets 2-3 times a day, a course of 3 to 6 months.
In addition, magnesia is assigned, especially at elevated oxalate content in a dose 0.15-0.2 g / day.
Treatment of urate nephropathy
Diet
- In the treatment of urate nephropathy rich diet provides for an exception purine bases products (liver, kidney, meat broths, peas, beans, nuts, cocoa, etc.).
- Preference should be given to dairy products and vegetable origin.
- An important prerequisite for successful treatment is sufficient fluid intake - from 1 to 2 liters per day. Preference should be given a weakly alkaline and poorly mineralized waters Decoction (horsetail, dill, birch leaf, lingonberry leaf clover, knotweed et al.), Oats broth.
For optimal acidity of urine can use citrate mixture (Ural Y blemaren, magurlit, solimok et al.).
When urate nephropathy is important to reduce the concentration of uric acid. For this purpose, means reducing the synthesis of uric acid - allopurinol, nicotinamide.
Pediatric use of allopurinol is limited because of the possible complications of the skin, liver, blood.
Under the strict supervision of allopurinol administered at a dose of 0.2-0.3 g / day. 2-3 hours for 2-3 weeks then the dose is reduced. The duration of the general course - up to 6 months.
Nicotinamide is a weaker drug than allopurinol, but better tolerated; appointed at a dose of 0,005-0,025 g 2-3 times a day for 1-2 months repeat courses.
Uric acid is output as orotic acid, tsiston, etamid, Cystenalum, Phytolysinum et al.
Treatment of phosphate nephropathy
Treatment of phosphate nephropathy should be directed to the urine acidification (mineral water - seltzer, ARZNI, suar-Dzau et al.; preparations - Cystenalum, ascorbic acid, methionine).
Assigned restricted diet foods rich in phosphorus (cheese, liver, eggs, chicken, beans, chocolate, etc.).
cystinosis treatment
Treatment of cystinosis and cystinuria includes diet, vysokozhidkostny treatment and drug therapy to urine alkalinization and increase the solubility of cystine.
The purpose of diet therapy - to prevent excessive intake of cystine child predecessor - methionine and other sulfur-containing acids.
It will also exclude (or severely limit) of baby food diet rich in methionine and sulfur-containing amino acids products - cheese, fish, eggs, meat and others.
Since methionine is necessary for the growth of the child's body, prolonged use of a strict diet is not possible, so in 4 weeks on start diet therapy diet of the child expands and is close to normal, but a rigorous exception of fish, cheese and eggs.
The amount of liquid should be at least 2 liters / day., Especially important to take fluid before bedtime.
For urine alkalizing used citrate mixture, sodium bicarbonate solution, blemaren, alkaline mineral water.
To improve the solubility and prevent crystallization cystine assigned penicillamine. It has some toxicity, so at the beginning of therapy are assigned low dose - 10 mg / kg / day. 4-5 techniques further dose increases for a week to 30 mg / kg / day, and when cystinosis -. 50 mg / kg / day.
penicillamine treatment should be under the control of cystine content in leukocytes and / or tsianidnitroprussidnogo test (test for cystine in the urine where cystine concentration should be up to 150-200 mg / l). Upon reaching these indicators penicillamine dose is reduced to 10-12 mg / kg / day.
penicillamine treatment is carried out for a long time, for years. As penicillamine inactivates pyridoxine concurrently assigned Vitamin B6 (pyridoxine) at a dose of 1-3 mg / kg / day. for 2-3 months with repeated courses.
To stabilize the membranes of renal tubules appointed vitamin A (6,600 IU / day) and vitamin E (tocopherol, 1 drop of 1 year life of a 5% solution per day) for 4-5 weeks with repeated courses.
There is a positive effect of the use of penicillamine instead of less toxic analog thereof - kuprenil in reduced dose in combination with ksidifonom and other Membrane.
Antibiotic therapy is indicated for accession infection.
If cystinosis successfully used a kidney transplant, which takes place prior to the development of end-stage chronic renal failure. Kidney transplantation can significantly increase the lifespan of patients - up to 15-19 years, but the deposition cystine crystals observed in the graft, which ultimately leads to the defeat and transplanted kidneys.
Forecast
Forecast at dismetabolic nephropathy generally favorable.
In most cases, the corresponding mode, diet and drug therapy can achieve stable normalization of indicators in urine.
In the absence of treatment or when the ineffectiveness of the most natural outcome dismetabolic nephropathy is kidney stones and inflammation of the kidneys.
The most common complication is the development of nephropathy dismetabolic urinary tract infection in the first place pyelonephritis.
