Kaltsifilaksiya is rare, potentially life-endangering syndrome of which increase in sensitivity of fabrics to calcium is characteristic. Development of kaltsifilaksichesky reactions leads to bystry progressing of a kaltsinoz, thrombosis of small and average arteries, ischemia of skin and hypodermic cellulose, internals. The sites of fabrics captured by pathological process are sharply painful, nekrotizirutsya gradually and izjyazvlyatsya. Diagnostics includes performance of x-ray pictures of the struck areas, a histologic research. Conservative treatment assumes correction of mineral exchange, surgical – excision of affected areas within healthy fabrics.
The term "kaltsifilaksiya" was introduced into circulation by the Canadian researcher Hans Selye who created experimental model of kaltsifilaksichesky reactions to animals. In English-speaking literature for definition of a disease the term "kaltsifitsiruyushchy uraemic arteriopatiya" is used. The syndrome mainly is diagnosed for patients of the senior age group with a terminal stage of a renal failure. Frequency of occurrence of pathology among patients on dialysis makes, according to different data, from 1 to 4%. 64% from them perish in the first year after emergence of clinical symptoms of pathology from sepsis or quickly progressing polyorgan insufficiency.
The role of kaltsifilaksichesky reactions in dermatology is studied insufficiently. In the works G. Selye considered a sensitization to calcium as protective reaction during which adjournment of lime can increase resistance of fabrics to influence of the damaging factors. Leads a combination of several reasons to development of pathological process:
- Severe damage of kidneys. The disease develops at dialysis patients and at the patients who underwent an operation on transplantation of a kidney. In its basis - violation of exchange of mineral salts in an organism, first of all calcium and phosphorus which is aggravated with application of dialysis environments with calcium salts in structure.
- Secondary . Malfunction of okoloshchitovidny glands against the background of a renal failure is observed rather often. Change of concentration of a paratgormon breaks balance of bone and extra bone exchange of calcium and phosphates, accelerates a bone resorption, promotes an exit of minerals in blood, stimulates adjournment of salts in walls of vessels.
Diabetes, obesity, application of glucocorticosteroids, immunodepressants, anticoagulants like varfarin, polyneuropathy, endocrine diseases, a monoclonal gammapatiya increases probability of formation of kaltsifilaksichesky reactions. The more risk factors is available for the patient, the disease progresses quicker, the treatment is more difficult and the forecast is worse.
Development of pathological process is the cornerstone two phenomena: a sensitization to calcium and influence of the so-called "allowing" factors provoking sedimentation of calcic salt in a vascular wall. Local increase in concentration of minerals in intim of vessels leads to change of a phenotype of cages of a vascular wall. Gladkomyshechny cages gradually regenerate in cells of a bone tissue. Arteries lose elasticity, their gleam is narrowed. The stenosis of arteries progresses rather slowly. At this stage of external manifestations there is no disease or they are insignificant.
Development sharp painful ischemic purples is connected with thrombosis. Emergence of blood clots is promoted by delay of a blood-groove in stenozirovanny arteriola, damage of an internal vystilka of vessels, hyper coagulation. The progressing violation of blood circulation causes ischemia of fabrics and their subsequent necrosis.
Pathological process takes mainly back and side surfaces of shins, buttocks, a stomach, forearms and hands, men have a penis. Skin in the field of defeat turns pale, becomes considerable more dense to the touch, sometimes gets a marble shade. The centers are sharply painful. Intensive painful feelings can complicate movement of the patient. At damage of calves patients cannot go, are forced to move on a wheelchair. Opioid analgetics can be necessary for knocking over of pains. At diabetic polyneuropathy pain can be absent.
Gradually having deficiency of oxygen and nutrients of fabric is died off with formation of ulcers. The intermediate stage of formation of an ulcer is the bubble with a sluggish tire filled with serous contents with blood impurity. When the bubble is broken off, the erozirovanny surface is bared. Ulcers are deep, get into hypodermic cellulose. At the edges of a skin wound there is a formation of a scab. Skin around an ulcer gradually changes color with marble-white on dark red, cyanotic or violet. The necrosis is characterized by black coloring of integuments. The defeat zone steadily extends and can occupy the whole anatomic areas. Process progresses within several weeks or months.
Rather rare option of course of a kaltsifilaksiya is involvement in pathological process of internals, in particular, of terminal departments of a large intestine. At such form of a disease the centers of ischemia are formed in an abdominal cavity that is followed by emergence of intensive belly-aches, bleeding from intestines.
The main complications of a kaltsifitsiruyushchy arteriopatiya are sepsis, the progressing polyorgan insufficiency, the necrosis of fabrics demanding carrying out a nekrektomiya. In the absence of medical care gangrene of fingers, a penis can end with their spontaneous amputation. In 1-2% of cases owing to damage of coronary arteries there comes cardiac arrest. At an intestinal form of pathology of bleeding from digestive tract force doctors to perform one for another band operations, to delete nekrotizirovanny departments of a gut, to restore intestinal passability. Taking into account the terminal stage of a renal failure and other heavy accompanying pathology which is available for the patient the result of surgical interventions, big on volume, not always favorable.
It is difficult to make the diagnosis of a kaltsifilaksiya to the dermatologist. Often involve doctors of other specialties in diagnostics: surgeon, nephrologist, oncologist, endocrinologist. At the initial stages the disease on the manifestations reminds a number of other pathological processes therefore it is necessary to carry out several various diagnostic tests and to estimate their results in total:
- Radiological research. X-ray pictures of shins, a chest and abdominal cavity are carried out. On roentgenograms vessels with lime deposits are well visible. In a chest cavity comes to light aortas, in an abdominal cavity - mezenterialny vessels, the arteries feeding with blood a liver and other bodies. In disputable or not clear cases resort to performance of a computer tomography.
- Biopsy of kidneys. In samples of tissue of the kidney taken on a histologic research it is found and . Deposits of lime settle down mainly on average a layer of a vascular wall. The research is applied to early diagnosis of pathology, specification a condition of kidneys. A histologic research of skin not informatively as changes in it are not specific.
- Determination of level of calcium (Sa) and phosphorus (P). Serumal concentration of Ca and phosphates are used for assessment of risk of development of a kaltsifikation of soft fabrics, vessels and kidneys at already established diagnosis. Kaltsifilaksichesky crisis can develop at the low work of concentration Ca and P. High rates are not always a kaltsifilaksiya sign.
- Research of level of a paratgormon. Exchange of calcium in an organism is regulated by hormones of parathyroid glands. Development of pathology usually is associated with a secondary giperparatireoz which develops at patients with a renal failure. But also decrease in function of parathyroid glands can negatively affect mineral exchange.
In dermatology local manifestations of a kaltsifilaksiya need to be differentiated with a dystrophic and metastatic kaltsinoz of skin, a system sklerodermiya, development lightning purples at the DVS-syndrome, a varfarinovy (geparinovy) necrosis, some types of vaskulit. To surgeries of a difdiagnostik it is carried out with putrefactive phlegmon, a thrombembolia of large arteries.
Treatment of a kaltsifilaksiya
The effect of the carried-out therapy can be gained only at an integrated approach. Medical tactics can change depending on a condition of the patient, the accompanying pathologies, the speed of progressing of ischemia. As a rule, the doctor should consider several serious diseases which are available for the patient at once, each of which is capable to lead to a bystry decompensation of a state and death. Are among the main methods of treatment:
- Correction of therapy of the main disease. Any medicines which provoke lime adjournment are excluded: iron medicines, vitamin D, anticoagulants, corticosteroids and so forth. If drugs cannot be cancelled completely, their dose is corrected. The number of procedures of a hemodialysis increases with 3 to 5 in a week. The ionic players of dialysis solutions are changed to reduce concentration of minerals in blood.
- Treatment of wounds. For prevention of spread of a gnoyerodny infection in fabrics of area of an ulceration process solutions of anti-septic tanks, impose ointments with antibiotics. The died-off fragments delete surgically. Due to the high probability of penetration of bacteria into blood with development of sepsis preventively appoint antibiotics of a broad spectrum of activity.
- Subtotal resection of parathyroid glands. The method is applied when adjournment of lime in vessels is revealed at early stages of formation of pathological process, and according to biochemical blood test sharp increase in a paratgormon in blood is noted. At late stages of a disease advantage of operation, as a rule, minimum.
- Physical therapy. Procedures of hyperbaric oxygenation which are appointed the repeating courses allow to accelerate healing of ulcers. The good effect can be gained from oxygen cocktails. Increase in concentration of oxygen in fabrics maintains viability of cages, stimulates healing process that increases effectiveness of conservative and surgical treatment.
- Purpose of bisfosfonat. Is an experimental method. The main property of bisfosfonat – prevention of loss of minerals a bone tissue. The less calcium is washed away from a bone, the lower it concentration in blood serum. There are messages on effective application of one of medicines of group of pamidronovy acid for treatment of a kaltsifilaksiya at the patients having a renal failure of heavy degree.
- Symptomatic treatment. It is appointed individually in each case. Can include decrease in body weight at obesity, control and correction of level of sugar of blood at diabetes, purpose of the fabric activator of a plazminogen at patients with vein thromboses of the lower extremities, sodium thiosulphate for decrease in content of calcium in blood, infusional therapy for elimination of intoxication which arose owing to disintegration of fabrics.
Forecast and prevention
It is impossible to remove the cause of development of a kaltsifilaksiya, but there is an opportunity to balance mineral exchange so that to prevent loss of crystals of salts from blood and at the same time not to provoke osteoporosis at the patients suffering from dysfunction of kidneys. The forecast and survival of patients are higher in cases of early identification of pathological changes in vessels before development of clinical symptoms (ischemia and an ulceration), the compensated condition of patients on other diseases which are available for them (to diabetes, arterial hypertension, etc.). Ways of prevention of a kaltsifilaksiya are not developed. It is recommended to ask in due time for medical care, to take the medicine appointed by the doctor, to undergo necessary inspections and procedures.