Hemorrhagic diathesis in pediatrics is called the pathology of the circulatory system, accompanied by the same manifestation - spontaneous bleeding. In children, such a disease is quite common. The disease is suffering both adults and children.

Hemorrhagic diathesis (vasculitis) is divided into 2 groups: congenital and acquired.

Genetic retreat, having heredity, leads to the occurrence of hemorrhagic diathesis of congenital nature. Blood disease or vascular pathology may cause acquired disease.

The classification allocates the following groups of this pathology:

1. Diathesis provoked by the wrong thrombocyte hemostasis. Thrombocytopathy I. are composite of this group. Violated immunity, renal diseases, viral infection and liver pathology provoke their occurrence.
2. The classification of the second group includes diathesis, provoked by blood clotting disorders: hemophilia, fibrinolytic purpura. Diseases appear due to prolonged therapy with anticoagulants and fibrinolithics.
3. The classification of the third group covers diathesis in which the walls of the vessels are destroyed: vasculitis, Teleangectasy.
4. The classification of the fourth group has various forms, the factors of the appearance of which may be irregular blood clotting or T rhombotary hemostasis.

Symptoms

Hemorrhagic vasculitis have different symptoms. In disruption of the insightful function of the walls of the vessels, the symptoms can be manifested by small racks throughout the body and on the mucous membranes, abdominal pain, blood in the urine. The appearance of pain and swelling in the joints may also indicate this disease.

Second group of disease accompanied by sudden bleeding and hemorrhage (subcutaneous, extensive or small). Hemorrhages can be different coloring. Blood coagulation disorders may be caused by the development of anemia, which also happens a sign of diathesis.

Hemophilia - are the third type of this pathology. Symptoms: from the first year after the appearance of the baby, subcutaneous hemorrhage at easy injury as well hemorrhage in the articular cavity (Hemartrosis).

Symptoms of children of this disease can be observed as follows:

• rash all over the body;
• tendency to form a hematoma with minor bruises;
• girls in a half-grade age are observed menstrual blood loss that exceed the norm (symptoms of menorrhagia).

Repeatedly in children have such symptoms as blood outpouring in internal organs (kidney, etc.) who accompanied by pain in the abdomen and vomit with blood impurities.

Diagnostics

The diagnosis is carried out to identify the reasons that cause bleeding. For this, doctors guide a sick child on laboratory examination of urine, blood, coagulogram and biochemistry.

When this pathology is detected:

• general blood and urine tests;
• biochemical analysis of blood and urine;
• the study of blood to the trace elements that are contained in it;
• establishing a period for general blood height;
• promcroth and thrombin tests;
• tombooplastin generation test;
• correctional samples in the autoagulogram on the determination of the degree of shortage in the plasma of the desired pathogen;
• immunological tests.

A comprehensive examination will provide the highest quality results based on which, you can establish an accurate diagnosis.

Laboratory

Lab diagnostics is crucial. If the laboratory indicators were normal in patients who have increased bleeding, does not mean that hemorrhagic disease is absent. Changes in laboratory research can be seen during the period of a hemorrhagic fragment. Based on the re-study.

One of the laboratory tests, which is carried out for the study of hemostasis, is not sufficiently sensitive (for example, the detection of the healsability period). The results of the test that determines the activated partial thromboplastin time (AFTT) is modified by hemophilia only with a decrease in the missing factor to the indicator at least 10% of the standard.

Bloodfaction is manifested when the capacity of some factor is lower than this critical indicator. With autoeritrocyte sensitization, even resorting to modern methods, the violation of the hemostasis system is not possible.

Differential

Differential diagnosis is the easiest way to classify the manifestations of the disease, since many forms of hemorrhagic vasculitis are accompanied by similar signs, but require different treatment. Differential diagnostics uses the following data:

• analysis of the presence of links between the manifestation of symptoms of syndrome and the use of medicines, vaccinations, various pathogenic effects;
• evaluation of the presence of bleeding after surgery and injury;

Differential diagnosis helps the doctor to determine on what time the disease appeared, as well as its duration and characteristic features.

Instrumental

Instrumental diagnostics is carried out using a general blood test, which it will help to identify postgemorrhagic normochromic or hypochromic anemia. In a consequence of data study, specialists are prescribed such diagnostic methods: the radiography of the chest organs, an ultrasound study (ultrasound) of the abdominal cavity, as well as the ECG.

Uzi are prescribed with the manifestations of kidney lesions. Sick children are often found, an anomaly of the development of the urinary system organs, the incorrect excretory and accumulative functioning of the kidney and others. The results obtained will help to draw up forecasts regarding the disease, as well as monitor the projection of treatment.

A patient's survey is also conducted, which contributes to the determination of the nature of pathology: hereditary or acquired, acute or chronic form, causes of an allergic reaction, etc.

• Be sure to read:

Physical examination reveals the type of bleedingindicating the damage to the specific hemostasal link (vascular with a vasculitically purple type, plateletinic-patech-spot, coagulation during hematomas).

Treatment

Treatment is assigned exclusively by the doctor after determining the type of diathesis. Also, treatment depends on the bleeding mechanism. Proponial assistance to parents to a child with hemorrhagic vasculite should include such measures and actions:

• provide the baby peace and bed mode;
• establish the correctness of the power, which should be enriched with a large number of calories;
• let the child drink more fluid;
• when bleeding, use a tampon.

Preparations

In case of vasculitis or thrombocytopenic, purple in treatment corticosteroid drugs are useds. It is recommended to use such drugs as: antispasmodics, disagrements, neutrometabolic medicines. The treatment complex contributes to the improvement of the condition of the child's body, depending on the degree of development of the disease.

Preparations such as: ditinon, heparin, use of glorying, intravenous gluconate and calcium chloride, patchicardine, belt, etc. are prescribed during hemorrhagic syndrome on the background of diseases of the gastrointestinal tract.

To get rid of bleeding, doctors recommend the reception of drugs that contribute to improving the blewerage. To stop bleeding will be involved by medicines with antiseptic effects: Thrombin, Pakhicpine, the use of a special film with and hemostatic sponge.

The use of drugs that contribute to the increase in blood clotting, and the antiseptics will help not only stop the bleeding, but also leads to instantaneous tightening of the Russian Academy of Sciences.

Vitamins

Treatment with vitamins, such as , Vitamin RR, Rutin is used in the event of avitaminosis.Vitamins of group K - anti-hymorrhagic drugs that contribute to the normalization of blood-insulability are involved in the biosynthesis process, therefore they are prescribed in the presence of all types of GD.

Blood transfusion

Transfusion of blood or plasma is carried out in hemophilia. If the patient is disturbed by constant bleeding, there may be a surgical intervention to remove the spleen. In the event of anemia, the diet and reception of iron drugs are recommended.

Folk remedies

People's methods of hemorrhagic diatic therapy set: baths, various ointments, herbs etc. But do not forget that first of all you should consult a doctor. Self-treatment is harmful to the child's health.

One of the funds of traditional medicine is the treatment of decoction, where the main component will be a bay leaf. Ten large laurel leaves pour one liter of water and bring to a boiling on a slow fire for 3 minutes. If you use the water bath method, then 10 minutes. Grind rosehip and add a teaspoon to the composition. Leave the mixture for the night, and in the morning you can give the baby, 3 times a day on 1 tablespoon.

The bay decoction can be diluted with tea, juice, water or with any other drink that uses your child in the amount of one tablespoon. The course of therapy by the people's method is 6 months.

The decoction can be used to prevent. For the prevention of hemorrhagic diathesis also apply: therapeutic physical culture, phytotherapy and physiotherapy procedures.

I. Hemorrhagic diathesis caused by a violation of thrombocytopower or thrombocyte hemostasis (thrombocytopathy).

• Thrombocytopenic purpura (idiopathic and acquired).
• Symptomatic thrombocytopenia (leukemia, hemorrhagic aleicia, radiation disease, etc.).
• Thrombocytopathy (violation of aggregation and adhesive and other platelet functions).
• Hemorrhagic thrombocytemia.

II. Hemorrhagic diathesis caused by a violation of blood coagulation and fibrinolysis or coagulation hemostasis (coagulopathy).

1. Violation of thromboplastin formation, or the 1st phase of blood coagulation.

• Hemophilia A, B and C.

2. Violation of thrombinization, or the 2nd phase of blood coagulation (dispatch).

• Hypoproactivity (Paragemophilia).
• Hypoproconvertinemia.
• Factor deficiency (Stuart Puera).

Hypoprothrombinemia (hemorrhagic diathesis of newborns; endogenous K-avitaminosis under mechanical jaundice; liver damage; medication or dicumorium hemorrhagic diathesis after an overdose of indirect anticoagulants). Violation of thrombin formation (drug hemorrhagic diathesis after an overdose of direct anticoagulants of the type of heparin).

3. Violation of fibrinetsis, or the 3rd phase of blood coagulation.

Afibrinogenemic purple (congenital). Fibrinogenopathy (acquired hypofibrinogenemia). Fibrin stabilizing (XIII) factor.

4. Violation of fibrinolysis.

Fibrinolytic bleeding and hemorrhages caused by acute fibrinolysis due to thrombohemorgic syndrome (disseminated intravascular coagulation syndrome, consumption coagulopathy) and overdose of thrombolytic drugs.

5. Violation of blood coagulation in different phases caused by circulating anticoagulants (antithromboplastins, inhibitors of factors VIII and IX, antithrombins).

III. Hemorrhagic diathesis caused by the damage to the vascular wall (vasopathy).

Hemorrhagic vasculitis (Shenlein-Genoch's disease). Hemorrhagic purple associated with infectious-toxic, infectious-allergic, dystrophic and neuroendocrine influences.

Hemorrhagic angiomatosis (Randu-Osler-Weber's disease), C-Avitaminosis (grief).

By 3.S. Barkagan, with hemorrhagic diatelas, such basic types of bleeding should be distinguished.

1. Hematomic. It is characteristic of violations of the internal blood coagulation mechanism - hereditary (hemophilia) and acquired (the appearance of circulating anticoagulants in the blood). Sometimes it is observed in the overdose of anticoagulants (retroperitone hematomas).
2. Capillary, or microcirculatory. Characterized for thrombocytopenia and thrombocytopathy, as well as the deficiency of plasma factors of the prothrombin complex (V, VII, X, II), hypo- and disfibrinegenemia; It is manifested by petechial-spotted hemorrhages in the skin, mucous membranes, bleeding of gums, uterine, nose.
3. Mixed capillary-hematomic. It is characteristic of disseminated intravascular blood coagulation (thrombohemorrhagic syndrome), Willebrand disease (deficiency of the VIII, vascular factor and a violation of the adhesive-aggregation function of platelets), an overdose of anticoagulants. It is manifested in main hematomas and patechial-spotted hemorrhages.
4. Purple. Observed with hemorrhagic vasculitis and other limitels. It is manifested mainly by symmetrically located small point and erythemical hemorrhages.
5. Micrangiomatous. Due to hereditary and acquired vessel dysplasia (Randy-Osler's disease, symptomatic capillary). It is characterized by persistent repetitive bleeding of the same localization.

Not all of the hemorrhagic diathesis listed above can be attributed to urgent states, however, with many of them, at certain periods, hemorrhagic syndrome is so pronounced that urgent therapy is necessary.

Hemorrhagic diathesis. Classification, etiology, pathogenesis, clinic, diagnosis, treatment. Volodicheva Elena Mikhailovna The main hematologist of the Tula region candidate honey. Sciences 1.

The hemorrhagic diatic syndrome N hemorrhagic diathesis is the syndrome, the main clinical signs of which are increased bleeding, a tendency to repeated bleeding and hemorrhage, spontaneous or after insignificant injuries. 2.

N Hemorrhage occurs in violation of the hemostasis system N hemostasis is a biological system that provides, on the one hand, the preservation of the liquid state of the blood, and on the other to the warning and stop of the bleeding by maintaining the structural integrity of the walls of blood vessels and a fairly rapid thrombing of the latter. four

The components of the hemostasis system: plasma factors of coating in the blood plasma. There are 13 non-fermentation factors N cellular factors. Platelets are involved in all phases of the hemostatic process. Also identified erythrocyte, leukocyte factors of completion N vascular component. Damage to the vascular wall leads to the release of the active thromboplastic substance. . N 6.

The first way is a violation of the thrombocyte hemostasal link - a decrease in the number of platelets (thrombocytopenia) or a violation of their functional state (thrombocytopathy). Most often in clinical practice, a thrombocytopenic purple (Verlgood disease) is found in the second way - a violation of the plasma pattern of pathogenesis due to the shortage of coagulation of coagulation or an antoslerting system - coagulopathy. A typical representative of this group is hemophilia. Third way - violations of the vascular wall of vasopathy; Hemorrhagic vasculitis. 7.

Classification. 1. Coagulopathy: n hemophilia A, B, with n hypoconvertermia, disadvantage of factors V, III, X, XIII 2. Violation of the megacariocyte-thrombocyte system N thrombocytopenia (ITP, symptomatic) N thrombocytopathy 3. Violation of the vascular system: n hemorrhagic vasculitis N disease Randy Osler. eight

Clinical types of bleeding N At the heart of clinical manifestations of hemorrhagic diathesis lies hemorrhagic syndrome. Carefully assembled history and objective research makes it possible to identify the type of bleeding, which is of great importance for the differential diagnosis of hemorrhagic diathesis. Severe five clinical types of bleeding. nine

N n n n For hematomic type, the following features are characterized: - massive, deep, intense and painful hemorrhages in large joints, muscles, under the aponeurosis and fascia, into subcutaneous and retroperitoneal fiber; - Profuses spontaneous post-traumatic or postoperative bleeding, including from internal organs (gastrointestinal, renal), which often occur immediately after surgery or injury, and after a few hours. Patechial-spotted (bruise) type is characterized by: - \u200b\u200bsurface hemorrhages in the skin, they are not tense, painless, do not squeeze and do not destroy the surrounding tissues; - Blood grooves (bruises) on the skin, which are more petechs in size, but also not tense and painless; Petechia and bruises arise spontaneously or with the slightest traumatization. - Dexted, nasal and uterine bleeding. 10

N n n n n for mixed, bruise-hematomic type, characteristic: - Patechial rashes and bruises that occur earlier by hematoma; - The hematomas in the retroperitoneal and subcutaneous tissue, as a rule, are few, but large sizes, there are practically no hemorrhage in the joints and their deformation. Vasculitically purple type is characterized by: - \u200b\u200bhemorrhagic skin rashes, most often symmetrical; The elements of rashes are limited, slightly raised over the skin, their appearance is often preceded by blisters or bubbles, which are then soaked with blood; Hemorrhagic elements can merge, epidermis over them is necrotic with the formation of a crust; After the disappearance of the rash remains the foci of skin pigmentation; - bleeding from internal organs - gastrointestinal, renal. An angiomatous type is characterized by: - \u200b\u200bpersistent and repeating bleeding of one or two, less often more localizations (for example, nasal, pulmonary); - the lack of spontaneous and post-traumatic hemorrhages in the skin, subcutaneous tissue. eleven

Inner Path Contact Surface External Fabric Factor XII VMK VII XI XII IX Insurance Fibrin XA VA Phospholipid CA² Protrombinase XIII Soluble Fibrin Protrombin Thrombin Fibrinogen Fibrin Monomer 13

Simulation of hemostasis paths Inner path: n Activated partial thromboplastinet (AFTT). External Way: N Prothrombin Time (1935, Quick). FV-MW 330 000, FVIIMW 50 000, FX-MW 58 800 and FII Approximate Tests: Promotable Time (PT); ABTV; Thrombin time; Quantitative determination of fibrinogen 14

Protrombin index - is determined in the presence of calcium ions and excess tissue thromboplasty The formation of a bunch depends only on the activity of the prothrombin complex (factors: II, V, VII and X) prothrombin activity in Kvika 0, 1 ml of plasma + 60 with prothrombin index: donor / patient ratio · 100% 0, 2 ml TKS * * - Thrombinkal mixture 15

Interpretation of the results The reason for the elongation of PV: n administration of oral anticoagulants; n liver disease; n vitamin K; N DVS; N Healthy deficiency Promcrin, f. VII, X or V. Shortion of PV is marked with thrombosis 16

ACTV - characterizes the inner path of hemostasis (1953): Factors VIII, IX, XII; N Preasallikryin (Fitcher Factor) N High Molecular Kininogen (F. Fitzgerald) Test is sensitive to the deficiency of all coagulation factors, except for VII, to heparin to specific and nonspecific inhibitors. N 0, 1 plasma + 0, 1 ERILID / KAOLIN 3 min + 0, 1 Calcium chloride 17

Interpretation of the results The reason for the elongation of the AFTV: deficiency of the factors of the internal coagulation path; Presence of coagulation inhibitors; Liver diseases; Vitamin K; DVS; Introduction heparin. Shortening is observed during thrombosis and internal combustion 18

Hemophilia A, B, S. Hemophilia - congenital coagulopathy, characterized by deficiency of factors: N VIII (hemophilia A) N ix (hemophilia in, crystram disease) N xi (hemophilia C). nineteen

Hemophilia - a disease characterized by a quantitative or qualitative deficit of coagulation factors VIII (hemophilia A), IX (hemophilia c) N n n Antihemophilic factor VIII (Factor VIII) MV 270, 000 - 340, 000 Is synthesized by endothelial liver cells? VIII: With an associated plasma with a Willebrand factor FVIII - inner path Normal concentration of about 0. 5 μg / ml NNN Factor IX (Crystram Factor) MV 72, 000 It is synthesized in the liver thermally labilene and stable when stored vitamin K-dependent normal concentration 3 μg / ml 20.

History of hemophilia is rooted in the distant past The first mention of hemophilia is contained in the Babylonian Talmud (1500 years ago) the term - hemophilia appeared in 1823 21

Etiopathogenesis. N Hemophilia A is 80% N Hemophilia at 19% N Hemophilia C is 1% hemophilia A and B is transmitted recessively combined with X-chromosome. Mostly men are sick, women are carriers of a gene and half of their sons may suffer from hemophilia. Hemophilia with is transmitted to autosomal-recycling, so the faces of both sexes are sick with the same frequency. 22.

wives. N P 1 H x H H x X, H H xx, healthy. wears. husband. --H x y, healthy. H x H Hu sick 23

25

Population of Russia and the calculated number of patients with hemophilia 6 764 67 645 000 77 654,000 According to the State Statistics Committee, the results of the All-Russian Census of 2002 in the country lives 145 million 290 thousand people 26

The severity of bleeding depends on the degree of deficiency of factors. Normally, it is 50 -100% latent form N with a decrease of up to 20 -50% there is a tendency to increased bleeding at large injuries, a pronounced form N at a level of 5 -20% arise heavy bleeding during injuries. Heavy form n if it ranges within the limits From 1 -5%, spontaneous bleeding occurs very severe form n. The complete lack of factor. N 27.

Clinic. Increased bleeding appears from the first months of the child's life n. Bleeding appears on the background of injuries: cuts, bruises, various accommodations n deep hemorrhage, abundant bleeding at the injury place, hemorrhage into large joints (hemarthrosis), leading to the development of contractures and ankylose joints (often kneecon and ankle), dangerous massive intertensive, subfascial, retroperitoneal hematomas, hymaturia. N 28.

30

31

32

33

34

Diagnostics. 1. N N 2. 3. Coagulogram: Reducing blood coagulation time Increase the active partial thromboplastin time decrease in auto-adaging, coagulation activity Prombrombin, thrombin time is normal. Reducing the level and activity of factors VIII, IX, XI Immunological test for determining the antigen of factors with homologous antibodies. 35.

What is the activity of factor VIII? It is measured in international units in the bottle (250 IU, 500 IU, 1000 IU); n in 1 ml. Normal plasma is 1 meter of VIII; N 1 meter VIII introduced by 1 kg. Body weight increases the level of factor VIII by ~ 2%. N 38.

Therapeutic dose: Hemophilia A - 25 -50 IU / kg every 12 hours; n hemophilia in - 25 -50 IU / kg every 24 hours; N 39.

The replacement therapy of blood coagulation factors VIII or IX is carried out by the patient itself or on the fact of hemorrhage or with a prophylactic goal. 40.

Treatment. SPP containing Factors VIII, IX, XI 2. Cryprecipitate (about 100 units in 1 portion) 3. Anti-emophilic plasma (concentrate of factors VIII, IX), Agemphyl A, in local therapy: 1. Minimalization of pain, seams, ice 2. When hemarity-immobilization, ice, elevated position General therapy: 1. Epsylon-aminocapronic acid, synthetic progestins Treatment of anemia: Eritrit transfusion. masses. 1. 41.

Etiopathogenesis of ITP. The main cause of bleeding is thrombocytopenia. Causes of thrombocytopenia: 1. The depressing effect of the spleen on the formation of platelets - submerged megacariocytes are found in the bone marrow point. N 43.

2. The accelerated destruction of platelets in the spleen is the lifespan of platelets a few hours instead of 7-10 days 3. With hereditary thrombocytopenia, the defects of the membrane structure, the violation of energy processes in them leads to rapid destruction 4. At the acquired ITP, antitrombocitary antibodies are produced in the spleen. Ig. G. 44.

The course of the disease: N chronic, recurrent N acute (hapton) viral infection or individual drugs (sulfonamides, butadion, chinin) play the role of hapten associated with platelet. The resulting antibodies determine the destruction of platelets and the occurrence of increased bleeding. 45.

Clinic ITP. N The clinical picture is manifested at platelet levels below 100 x10 * 9 / l. At platelet levels below 50 x10 * 9 / l, vital bleeding may occur. . 46.

Small-point subcutaneous hemorrhages - petechia, ekkimosis, are located asymmetrically n bleeding from mucous membranes: gums, nasal bleeding n bleeding from gematurium ns hematuria N blood hemorrhage in injection places n long bleeding after extraction of teeth N postgimorgic anemia. N 47.

48

49

Diagnosis of ITP. N n N General blood test: Reducing the number of erythrocytes and hemoglobin Reducing the number of platelets (below 100 x10 * 9 / l) with an autoimmune process. ENLITH MORPHOLOGICAL CHANGES IN TROOMETS: Increased dimensions. fifty

N Coagulogram: an increase in bleeding in Duke and Ivi, reducing the reproduction, blood coagulation time and the kaolintefalin test in the normal N Miorogram: the number of megacariocytes is increased by N sample of the Dixon-detection of antithrombocyte antibodies. 51.

52

Treatment of ITP. Glucocorticosteroids: Prednisolone 40 -60 mg / day according to scheme n In Effectiveness of GCS, immunosuppressant-methipred, vincristine, azathioprine, cyclophosphane N splectomy of readings are used: increasing anemia, the ineffectiveness of medicines. . N 53.

N thrombocytopathy is a disease in which qualitative and functional properties are violated - adhesion and platelet aggregation, resulting in a violation of hemostasis. During thrombocytopathy, the number of platelets within the normal range. N among hemorrhagic diathesis is found in 36% of cases. 54.

Clinic thrombocytopathy. Subcutaneous petechial-spotted hemorrhages, hematomas located asymmetrically n bleeding from mucous membranes: gums, nasal bleeding n bleeding from gematuria NE hematuria N blood crying of blood hemorrhage in injection places n long bleeding after extraction of teeth N postgimorgic anemia. N 55.

Diagnostics. n General blood test: 1. Reducing the number of erythrocytes and hemoglobin 2. The number of platelets is normal 3. In the morphology of platelets, granules and processes are not determined. 56.

Treatment. Diet with WIT C, P, A, ASHIP NO AMINOCAPRONIC Acid 6 -12 g / day n ditinon, vikasol N Pamba, Pambecam N ATF acid, magnesium sulfate N riboxin, Inosine-F n Androxon, adrenoxyl N Thrombocytic mass n Local stop blood. N 61.

Pathogenesis. Hemorrhagic vasculitis refers to immunocomplex diseases, as it is due to the damaging effect of low molecular weight immune complexes (IR). With their formation, there is a predominance of antigen. IC and the complement activated by them cause microtrombovasculites with fibrinoid necrosis, perivascular swelling, microcirculation blockade. 64.

Clinical current. Clinical flow is distinguished: n skinny or simple form N articular shape N Abdominal form N renal form N Lightning form for the flow of the disease distinguish: 1. Ostly 2. Chronic, recurring. 65.

Clinic. N Skin shape Defeat skin fine-point, symmetric petechia, mainly on the lower limbs and buttocks. Rash monomorphic, with a distinct inflammatory basis, keeps 4 -5 days, after leaving pigmentation. 66.

N The articular shape N the place of lesion of the joints is a synovial shell. n There is a sharp pain, swelling, violation of the function of the joint N Abdominal shape occurs in the hemorrhage into the mucous membrane of the stomach, intestine, mesentery. There are severe abdominal pain, simulating the picture of an acute abdomen, may increase body temperature, sometimes vomiting appears. Blood is determined in feces. 67.

n The renal form N flows along the type of acute or chronic jade, sometimes takes a protracted shape and moves to CPN. It is possible to develop arterial hypertension, nephlastic syndrome N lightning (cerebral) form develops with hemorrhage into a cerebral shell or vital areas. 68.

Diagnostics. n Clinical manifestations: monomorphic finely purified, symmetric rashes that are not disappearing with hyperfibrinogenemia, an increase in the content of IR, cryoglobulins, gammaglobulins N positive test of auto-adaging N on a coagulogram shortening the total coagulation of blood, prothrombin and thrombin time. 69.

Treatment: HEPARIN 7500 -15000 UR / SUT V / B or P / C under blood clotting N heparinoids: Sulodekside, mortgagen N Power plasmapheresis N steroid hormones: prednisolone 20 -60 mg / day according to scheme n Microcirculation improvement: Trental, pentoxifyllin N Strengthening the vascular wall: ascorbic acid, rutin. N 70.

23. Conditional and unconditional reflexes in a child of 1 year of life.

24. The laws of the increase in mass, body length, head of the head, chest.

25. Assessment of the physical development of children. The concept of acceleration.

26. Anatomy-physiological features of the skin, subcutaneous fiber, lymph nodes. Methods of examination. Semiotics.

27. Anatomy-physiological features of the musculoskeletal system. Examination methods. Semiotics.

28. Anatomy-physiological features of blood circulation bodies. Methods of examination. Semiotics.

29. Anatomy-physiological features of respiratory organs in children. Methods of examination. Semiotics.

30. Features of peripheral blood in children at various periods of childhood. Semiotics.

31. Anatomy-physiological features of the liver, gallbladder and spleen in children. Methods of examination. Semiotics.

32. Anatomy-physiological features of digestive organs in children. Methods of examination. Semiotics.

33. Anatomy-physiological features of urica organs and urinary in children. Examination methods. Semiotics.

34. Natural feeding and its advantages for the normal development of the breast child.

35. Mode and diet of a nursing mother.

36. Lactation. Composition and calorie content of colostrum and mature female milk.

37. Difficulties, absolute and relative contraindications with natural feeding from mother and child.

38. Pring. Dates of administration. Character. Correction of vitamins and mineral salts.

40. Mixed feeding, its characteristic. Doctors.

41. Artificial feeding, its characteristic. The deadlines for the introduction of valbami.

42. Composition and calorieness of breast milk, high-quality differences from cow's milk.

43. Characteristics of the main nutritional mixtures for feeding children 1 year of life.

44. Features of feeding children 1 year of life with Rickets

45. Features of feeding children 1 year of life with hypotrophy.

46. \u200b\u200bFeatures of feeding children 1 year of life with exudative diathesis

47. Features of feeding children 1 year of life with anemia.

48. Congenital heart defects, etiology, classification

49. UPU: Open arterial duct

50. UPU: DMPP

51. UPU: Davyd

52. UPU: Tetrad Fallo

53. UPU: Coarse Aorts

54. UPU: Stenosis of the pulmonary artery

55. Distrophy, definition, classification

56. Hypotrophy. Definition, etiopathogenesis, classification.

57. Hypotrophy, clinic, treatment.

58. Parabitrophy, definition, etiopathogenesis, clinic and treatment

59. Rahit in children. Etiology, pathogenesis, clinic.

60. Rahit in children. Treatment and prevention

61. Spasofilia. Etiology, pathogenesis, clinical options, treatment and prevention

62. Exudative catarrhal diathesis, clinical manifestations. Treatment and prevention.

63. Allergic diathesis, clinical manifestations. Treatment and prevention.

64. Lymphatic and hypoplastic diathesis, clinical manifestations. Treatment and prevention

65. Neuro-arthritic diathesis, clinical manifestations. Treatment and prevention.

66. Waiting. Ethiopathogenesis, classification, diagnostics.

67. Waited. Clinic, treatment, prevention

68. Differential diagnosis of waiting and normal anemia.

69. Acute pneumonia. Etiopathogenesis, classification, clinic

70. Acute pneumonia. Diagnostics, principles of antibacterial therapy

71. Diagnostic criteria for acute pneumonia in children.

72. Differential diagnosis of acute pneumonia and bronchitis

73. Acute bronchitis in children. Classification. Etiopathogenesis. Clinic. Treatment.

74. Acute simple bronchitis. Features of the clinic, diagnostic criteria. Principles of treatment.

75. Acute obstructive bronchitis. Features of the clinic, diagnostic criteria. Principles of treatment.

76. Bronchiolitis. Features of the clinic, diagnostic criteria. Principles of treatment.

77. Recurrent bronchitis. Diagnostic criteria. Tactics of treatment.

78. Chronic bronchitis in children. Definition, etiology, pathogenesis, clinic, treatment.

79. Respiratory failure in children. Causes, clinic, severity. Urgent Care

80. Bronchial asthma. Ethiopathogenesis, classification.

81. Bronchial asthma, clinic, criteria of gravity and assessment of gravity

82. Bronchial asthma, concept of complete and incomplete control of asthma, evaluation of the function of external respiratory

83. Bronchial asthma. Pinzies of basic therapy.

84. Bronchial asthma. Principles of symptomatic therapy.

85. Bronchial asthma. Asthmatic status. Urgent Care

86. Acute rheumatic fever in children. Etiology, pathogenesis, classification.

87. Acute rheumatic fever in children. Diagnostic criteria, syndromes in the clinic of Orl

88. Chronic rheumatic heart disease in children. Definition. Classification. Clinic.

89. Acute rheumatic fever. Power treatment

90. Acute rheumatic fever. Primary and secondary prevention.

91. Acute heart failure in children. Classification, clinic, emergency care.

92. System red lupus. Criteria for diagnosis, classification, treatment

93. Dermatomiosit. Diagnostic criteria. Classification. Treatment.

94. Sclerodermia. Criteria for diagnosis, classification, treatment

95. Juvenile rheumatoid arthritis in children. Ethiopathogenesis, classification, clinic.

96. Yura. Power treatment. Prevention.

97. Acute glomerulonephritis in children. Etiology, pathogenesis, classification, clinical forms, stage treatment.

98. Chronic glomerulonephritis in children. Etiology, pathogenesis, classification, clinical forms, treatment.

99. Acute pyelonephritis in children. Etiology, pathogenesis, classification, features of the clinic in children of early and older. Treatment and prevention.

100. Chronic pyelonephritis in children. Etiology, pathogenesis, classification, clinic. Treatment and prevention.

101. Urinary tract infections. Diagnostic criteria.

102. Differential diagnosis of pyelonephorite and cystitis

103. Differential diagnosis of pyelonephritis and glomerulonephritis

104. OPN in children. The reasons. Classification. Clinic. Urgent Care. Indications for hemodialysis.

105. CPN, classification, clinic.

106. Hemorrhagic vasculitis in children. Etiology, pathogenesis, classification, clinic, treatment and prevention.

107. Thrombocytopenic purpura in children. Etiology, pathogenesis, classification, clinic, treatment.

108. Hemophilia in children. Etiology, pathogenesis, classification, clinic, treatment

109. Differential diagnosis of hemorrhagic diathesis

110. Chronic gastroduodenites in children. Ethiopathogenesis, classification

111. Chronic gastroduodenitis, clinic, modern diagnostic methods

112. Chronic gastroduodenitis. Page treatment and prevention. Eradication schemes h. Pylori.

113. Georny disease in children. Ethiopathogenesis, classification.

114. Georny disease in children. Clinic, features of the course in children at the present stage.

115. Panish disease. Complications. Diagnostics. Power treatment. Emergency care for gastric bleeding.

116. Chronic cholecystitis in children. Etiology, pathogenesis, classification, clinic, diagnosis. Page treatment and prevention

117. GCB in children. Etiopathogenesis, features of the clinic.

118. GCB in children. Diagnostic criteria. Principles of treatment

119. High-voltage dysfunction of the gallbladder in children. Etiopathogenesis, clinic, stage treatment and prevention

120. Hypersmotor dysfunction of the gallbladder. Etiopathogenesis, clinic, treatment.

121.Ascaridosis

122. Trichocephalosis

123. Enterobiosis.

124. Sugar diabetes in children. Etiology, pathogenesis.

125. SD in children. Diagnostic criteria. Clinic

126. SD in children. Compensation criteria. Complications

127. SD in children. Principles of treatment

128. Hyperglycemic Coma. Causes, clinic, emergency therapy.

129. Hypoglycemic Coma. Causes, clinic, emergency therapy.

130. Differential diagnosis of ketoacidochie and hypoglycemic coma.

131. Difteria in children. Forms of rare localization. Clinic, diagnosis, bacteridation, epidemiological significance. Treatment and prevention.

132. Difteria. Etiology, pathogenesis, pathological anatomy. Classification of clinical forms.

133. Diftera of the rotogling: catarrhal, localized, common, features of their flow. Differential diagnosis. Polyneuropathy during diphtheria

134. DIYPTECHIY DIFTHETTY POWERSICY, TOXIC 1-3 degrees. Serotherapy, therapy of complications.

135. Large Diftere. Clinic, Stage, Differential Diagnosis. Treatment, indications for surgical intervention.

136. Differential diagnosis of meningococcal meningitis with purulent bacterial meningitis of other etiology

137. Differential diagnosis of purulent and serous meningitis in children.

138. Scarlatina.

139. Cort. Etiology, epidemiology, pathogenesis, classification. Typical Corey Clinic.

140. Cort. Etiology, pathogenesis, clinic of mitigated, light, abortive measles. Diagnosis, role in the epidemic process.

141. Cort. Clinical picture, diagnosis, complications, treatment. Prevention.

142. Cort. Secondary and primary pneumonia for measles. Diagnosis and treatment.

143. Specific prevention of measles on the national vaccination calendar. Indications and contraindications.

144. Streptococcal infection. Scarlatina in children. Treatment of scarletins and its complications. Prevention.

145. Poklush. Etiology, epidemiology, pathogenesis, classification

146. Poklush. Classification, clinic, treatment, prevention. Vaccines ADH and AAKDS. Contraindications.

147. Exicosis in children with acute intestinal infections. Clinic. Treatment. Reghydration principles.

148. National calendar of the preventive vaccinations of Russia

149. Epidemic parotitis. Epidemiology, pathogenesis, etiology, classification, clinic, treatment.

150. Epidemic parotitis. Complications, treatment, prevention

151. Submaxillit, sublingvit, pancreatitis with epidemic parotitis. Clinic, treatment, prevention.

152. Wind gas. Etiology, epidemiology, pathogenesis, clinic, treatment and prevention.

153. The chickenpox is severe. Windmill encephalitis. Clinic, treatment.

154. Respiratory syntial infection in children.

155. Influenza. Etiology, pathogenesis, classification, clinic in young children. Treatment.

156. Neurotoxicosis with influenza. Clinic, treatment

157. Influenza: complications in children, clinic, diagnosis, treatment. Specific prophylaxis. Types of vaccines. Contraindications.

158. Adenovirus infection. Etiology, pathogenesis, classification, chapel of the pharyingsonunctival fever. Diagnosis, treatment.

159. Supporting clinical symptoms of tonsilotlopharygitis with adenovirus infection

160. Paragripp in children. Etiology, epidemiology, pathogenesis, classification. The clinic of the concomitant laryngotracheobronchite I and II degree.

161. Paragripp in children. Decompensated stenosing laryngotracheobronchitis. Treatment

162. Enterovirus infections in children. Etiology, leading syndromes. Treatment and diagnostics.

164. Acute sluggish paralysis. Differential diagnosis with polio

165. Herpes ishing in children. Etiology and pathogenesis. Clinic. Vaccines Obvak and Variorix. Indications.

166. Viral hepatitis A. Etiology, epidemiology, clinic, treatment. Prevention

167. Hepatitis A basic therapy in children. Specific prophylaxis.

168. Viral hepatitis B. Etiology, epidemiology, clinic, treatment. Non-specific prevention. Vaccination against viral hepatitis B. Indications and contraindications. List of vaccines.

169. Complications of viral hepatitis B. Clinic, treatment

170. Poliomyelitis. Etiology, classification, clinical picture. Treatment and prevention.

171. Poliomyelitis. Epidemiology. Paralytic clinic. Differential diagnosis with sluggish paralysis in enterovirus infection and diphtheria. Specific prophylaxis

172. Viral hepatitis A. Angry forms. Clinical and laboratory diagnostics. The role in the spread of infection.

173. Delta infection in children. Epidemiology, clinic, complications. Treatment and prevention.

174. Vaccision Associated Poliomyelitis. Clinic. Diagnostics. Prevention.

175. Acute Shigellies in children. Etiology, pathogenesis, epidemiology, classification. Features of the clinic in children 1 year of life. Treatment and prevention.

176. Atypical shapes of chiegellas in children. Clinic. The role in the spread of infection in children's teams. Prevention.

177. Salmonellosis is in-hospital in children. Clinic, diagnosis, treatment and prevention

178. Salmonellosis in children. Etiology, epidemiology, classification. Treatment and prevention.

179. Salmonellosis in children. Lightweight and moderate forms. Clinic, treatment, prevention.

180. Salmonellosis in children. Rare forms. Clinic, diagnosis, treatment.

181. Eschhericia in children. Etiology, Epidemiology, Pathogenesis, Clinic, Classification, Treatment, Prevention.

182. Complications for acute intestinal infections in young children. Treatment.

183. Rotavirus infection in children. Etiology. Epidemiology. Clinic, diagnosis, treatment and prevention

184. Oral rehydration at the Oka. Indications for conducting. Complications

185. Meningococcal infection. Etiology, epidemiology, pathogenesis, classification, clinic, diagnosis, treatment.

186. Meningococcal infection. Etiology, epidemic. Localized forms. Clinic. Treatment

187. Meningococcal infection. Meningitis. Clinic, diagnostics. Treatment at the chipboard stage and in the hospital.

188. Meningococcal infection. Meningochacemia. Infectious toxic shock. Clinic. Treatment.

189. Krasnuha in children. Ethiopathogenesis, Epidemiology, Clinic, Differential Diagnosis, Treatment and Prevention. The role in the development of embrypathia.

190. Congenital rubella syndrome in children.

191. Hemophilic infection in children. Etiology, epidemiology, classification. Clinic, diagnosis, treatment. Prevention

192. Pneumococcal infection. Etiology, epidemiology, classification. Clinic meningitis, diagnosis, treatment. Specific prophylaxis.

193. Epstein-Barr disease. Infectious mononucleosis in children. Etiology, epidemiology, pathogenesis, clinic, course, treatment

194. Difteria: early and late complications. Clinic. Differential diagnosis. Treatment.

195. Rules for the storage and introduction of vaccines and sera

109. Differential diagnosis of hemorrhagic diathesis

Hemorrhagic diathesis (GD) are a group of hereditary or acquired diseases, for whom the tendency to recurrent bleeding and hemorrhages of various durability and intensity are characteristic.

To the development of hemorrhagic syndrome, with DG, there are violations of various links of a complex hemostasis cascade, most often - the absence or lack of individual factors of blood coagulation (proseagulants), an excess of physiological anticoagulants and fibrinolytic agents.

The hemostasis system ensures the warning and stopping of bleeding by maintaining the structural target of the wall of the blood vessels and sufficiently rapid thrombing during damage. These functions provide 3 functional structural components of the hemostasis system: the walls of blood vessels, uniform elements of blood, primarily platelets, and plasma enzyme systems (coaginating, fibrinolytic, kallicrein-kininic, etc.).

2 hemostasis mechanisms are isolated:

1. Primary (microcitar, vascular-thrombocystary) hemostasis, which provides a stopping of bleeding from proximal and terminal arteriols, preacapillars, true capillaries and veins by means of temporary vascular spasm, adhesion and viscous platelet metamorphosis with the formation of platelet cork (white platelet blood clomba), its subsequent Seals and cuts. The resulting white thrombocitory thrombus is tightened by damaged edges of small vessels, holds them from dilatation and does not let the liquid part of the blood.

2. Secondary (macrocyal, final) hemostasis, which is ensured by the blood coagulation system and completes full hemostasis in macro-sides, started on the vascular-thrombocytarium.

Conducting a differential diagnosis of DG, it should take into account the data of the anamnesis, physical examination of the patient and rely on the laboratory detection of hemostasis disorders, which allows to verify the diagnosis.

When polling the patient with hemorrhagic syndrome, it is necessary:

1) establish the acquired or hereditary nature of the disease;

2) to clarify the timing, the abandonment, duration and features of the course of the disease (the appearance in early children's, youthful age or in adults, acute or gradual development of hemorrhagic syndrome, chronic or recurrent);

3) find out the causes of the occurrence or enhancement of bleeding, localization, the order of the appearance of the elements of rash and change their coloring, the effectiveness of the treatment;

4) assess the presence of bleeding after operations and injuries, menorgia, gastrointestinal and other bleeding;

5) to analyze the presence of communication between the appearance of symptoms of hemorrhagic syndrome and drug intake, vaccinations, various pathogenic effects, accompanying diseases (liver diseases, infectious septic process, leukemia, injury, shock, etc.);

6) Find out the preferential localization, severity and type of bleeding.

If there is information about the hereditary nature of the disease, the severity of symptoms among family members (penetrantiness) should be assessed, the presence of other genetic defects. This is due to the frequent combination of hereditary DG with other anomalies: telegangectasis - with hyperelistosis of the skin, weakness of the ligament apparatus, the prolapse of the mitral valve; hereditary thrombocytopenia - with skeleton anomalies, impaired immune system and pigment exchange; Hemophilia - with color violations.

An objective examination of the patient should be directed to the diagnosis of diseases that could lead to GD, as well as an assessment of the severity of hemorrhagic syndrome. It should be borne in mind that the clinical manifestations of the DGD depend on which hemostasis link is affected, and the correct assessment of the type of bleeding will greatly facilitate the differential diagnosis of DG, since it will purposely apply tests verifying the diagnosis.

With genetically determined hereditary teleangectation (Randy-Osler's disease) due to thinning of the basal membrane of small blood vessels on mucous membranes, lips and skin are formed small noded vascular formations that are easily bleeding and are a source of abundant and difficult to bind bleeding. Sometimes teleangectations are combined with cerebellar disorders and immune failure (Louis Bar syndrome).

If there are visible teleangectasius, the diagnosis is not difficult. Endoscopic examination is carried out to detect teleangectasia on the mucous membrane of the digestive tract. Hemostasis indicators usually do not differ from the norm.

For acquired GD of vascular genesis (Purple Shenlein-Genoch, hypersensive vasculitis, hemorrhagic vasculitis of infectious-toxic, infectious-inflammatory genesis, etc.) is characterized by a vasculitically purple type of bleeding, more often with a symmetric arrangement of rashes. Often, other types of rash (blister, papules) are identified. Arthralgia, hematuria, abdominal disorders (pain syndrome, intestinal bleeding) are also characterized by fever. With a slight course of hemostasis disorders may be absent. In severe flow, as a rule, signs of chronic disnsimated insvisitive coagulation syndrome (DVC syndrome) are found - the presence of fibrin-monomeric complexes, positive protamine sulphate and ethanol tests. In fulminant form, hypoofibrinogenemia, thrombocytopenia and coagulopathy of consumption, detects, which indicates the presence of deployed DVS syndrome.

For DG due to the lack of blood platelets or their high-quality inferiority, the petechial-spotted type of bleeding with the rapid appearance of hemorrhages when pressing on the skin, palpation, compression of the hand meter cuff (casing), the formation of bruises around the injection places, bleeding from mucous membranes, menorrhagia. Horn hemorrhages in the brain, the risk of which can testify hemorrhage on the skin of the face and neck. Hemorrhages in the retina and ovaries are also possible.

When examining patients with idiopathic thrombocytopenic purple (Verlgood disease), a significant decrease in blood platelet levels (less than 100,09 liters) and a megacariocytic sprout hyperplasia in a myelogram are detected. Characterized by cacilocytosis of platelets, shortening the time of their life, disruption of the retraction of the bunch and an increase in bleeding time. Samples on the breakdown of vessels positive.

Secondary (symptomatic) thrombocytopenia develop under a number of diseases and conditions. V.M. Forecode propose to allocate:

1. Autoimmune thrombocytopenia with systemic diseases of connective tissue and other diseases of immune genesis.

2. Heteroimmune thrombocytopenia due to the formation of antibodies to the surface tombocyte antigen (drug immune thrombocytopenia and thrombocytopenia in viral diseases).

3. Thrombocytopenic syndrome as a result of hypersplenism.

4. Thrombocytopenia due to the influence of physical and chemical factors (ionizing irradiation, electromagnetic waves, ex- and endogenous intoxication).

5. Thrombocytopenia with DVS syndrome.

6. Thrombocytopenia for diseases of blood system (acute and chronic leukemia, hypoplastic anemia, B12 deficient, immune hemolytic anemia).

The clinical manifestations of secondary thrombocytopenia do not differ from those in idiopathic thrombocytopenia, therefore, it is particularly important to eliminate the secondary nature of its nature and the elimination of the advent of hemorrhagic syndrome with drug receptions, professional factors, infectious and other diseases that may be accompanied by thrombocytopenia. In secondary thrombocytopenias due to the lesion of the megacariocyte sprout, assistance to clarify the diagnosis can provide data from sternal puncture (during leukemia, hypoplastic anemia).

The presence of a functional defectiveness of platelets must be suspected if there is a patient typical clinical signs of a petechial-spotted type of bleeding in the normal amount of platelets in peripheral blood. The thrombaster of Glanzmann is hereditary thrombastenes inherited by an autosomal-recessive type, which is based on a lack or deficiency of the glycoprotein complex IIB-IIIA platelet shell, leading to a violation of fibrinogen and platelets. More often a woman is sick, the disease is manifested in childhood. Characteristics of adhesion and platelet aggregation, blood clot retraction and a significant increase in bleeding duration with a normal number of platelets.

Among the hereditary DG associated with the violations of the blood coagulation system are most often the hemophilia A and B and Willebrand's disease, and in connection with the presence of a patient with a hematomic type of bleeding, diagnostic measures should be directed primarily to the recognition of these diseases. All other hereditary disorders of blood clotting (deficiency V, VII, X and XI and other blood coagulation factors) are rare, and cases of deficiency and anomalies II, XII, XIII blood coagulation factors, precasallitin, high molecular weight KININOGEN - extremely rarely, in connection with which This article does not consider.

Hemophilia A and B are due to genetically determined synthesis impairment (less often anomalies) VIII and IX blood coagulation factors, whose genes are localized in different parts of the X-chromosome and are recessive. In this regard, hemophilia A and B are inherited by the type linked with the floor, and cause a disease in male people who have received pathologically modified X-chromosomes from mothers. In the female line, the disease can be transmitted in latent form throughout many generations, and therefore, according to the history of the history, it is not always possible to trace the inheritance of the disease. In addition, the hemophilia gene refers to frequently mutating genes.

In most cases, hemophilia A and B are easily recognized due to the presence of a typical hematomic type of bleeding. Repeating hemorrhages in joints and bones in individuals with these diseases can lead to the development of severe destructive arthrosis, contractures, fibrous ankyloses. For patients with hemophilia, abundant and long-term delayed (after 2-6 h) post-traumatic and postoperative, gastrointestinal, nasal and renal (often with the colic and the outstanding of blood clots) bleeding are characterized. The severity of bleeding symptoms in patients with hemophilia A and B corresponds to the degree of deficiency of VIII and IX blood coagulation factors. If their blood content is below 1%, the disease is very severe, and when the content is more than 5% - light.

The diagnosis of hemophilia is based on the results of genetic history (inheritance, adhered to the male floor), the data of the clinical picture (hematomic type of bleeding) and laboratory studies (an increase in blood coagulation time, signs of hypocoagulation according to the auto-adaging test and an increase in the activated partial thromboplastin time - ACTV). Differential diagnosis of hemophilia A and B are carried out using correctional tests, which use the principle of breeding and correction of impaired blood coagulation of the patient with the components of normal blood. The diagnosis verifies the quantitative definition of the VIII and IX coagulation factors.

Willebrand disease (angiohemophilia) is due to an inherited violation of the synthesis or anomalies of a protein cofactor VIII of blood coagulation factor (Willebrand factor). Faces of both sexes are sick, but in women the disease occurs harder. The shortage of Willebrand factor leads to a change in not only the coagulation activity of the VIII of the blood coagulation factor, but also the vascular-thrombocyte hemostasis (reduced platelet adhesion to subendotel and collagen and their aggregation under the influence of ristomycin). Therefore, for patients with this disease, a mixed bias is characterized, and in the blood, along with a violation of coagulation, an increase in bleeding time is revealed, a decrease in platelet adhesiveness and their ristomycin agglutination. The diagnosis is established on the basis of a decrease in the content of the Willebrand factor in blood plasma and (or) in platelets.

The deficiency of K-vitaminosen-dependent coagulation factors (II, VII, IX and X) can develop when lesion of the liver in patients with cirrhosis, acute damage to toxic and other genesis (due to their insufficient synthesis), with mechanical jaundice, severe enteropathy and intestinal dysbacteriosis (due to disorders of suction in the intestine of fat-soluble vitamins, including vitamin K), the hemorrhagic disease of the newborn (due to the temporary depression of the production of these factors in the first 4-7 days after birth), as well as with an excessive reception of non-accumulant anticoagulants (due to competition with vitamin K and Displacing the latter from the metabolism of K-vitamin-dependent blood coagulation factors with a violation of their carboxylation). Bloodfaction with DG of this group is mixed fiddly hematomic character. Indicators of laboratory studies indicate a pronounced decrease in the prothrombin index and a significant lengthening of blood coagulation time according to the ABTT data under normal indicators of thrombin time and in the blood of fibrinogen and platelets, with negative paracoagulation tests (ethanol, protamin sulfate).

DG due to the overdose of direct anticoagulants and fibrinolytic drugs may cause the bleeding of mixed type (phetechial-spot-hematomic), characterized by nose, renal and gastrointestinal bleeding, as well as the high risk of profuse gastrointestinal bleeding in patients with peptic ulcers, or stroke - In patients with arterial hypertension. Causes of bleeding in patients receiving drugs of these groups are clear and usually do not require a differential diagnosis.

Thus, the differential diagnosis in the presence of signs of the hemostasis disorder should include the following steps:

1. The patient's survey, which will determine the hereditary or acquired nature of the disease, acute or chronic its course, the severity of hemostasis disorders and provoking factors.

2. Physical examination of the patient, allowing to determine the type of bleeding, which is highly likely indicated by the damage to a certain hemostasal link (vascular - with a vasculitically purple type, plateletone - with a petechial-spot or coagulation - with hematomic and blue-hematomic type).

3. The use of laboratory tests indicating the defeat of various hemostasis links, including a targeted quantitative determination of coagulation factors in the blood for a differential diagnosis within various groups of DG.

"

The hemorrhagic diatelas include diseases based on the violation of the vascular wall and the various links of the hemostasis system, which determine the increased bleeding or a tendency to its occurrence.

Pathogenesis

The pathogenesis of hereditary hemorrhagic states is determined by the violation of normal hemostatic processes: the anomalies of megakaryocytes and platelets, a deficiency or defect of plasma factors of blood coagulation, the inferiority of small blood vessels. Acquired hemorrhagic diathesis are due to DVS-syndrome, immune damage to the vascular wall and platelets, toxicoinfection lesions of blood vessels, liver diseases, and the effects of drugs.

Epidemiology

On the globe about 5 million people suffer from primary hemorrhagic manifestations. Considering that secondary hemorrhages, such as the DVS-syndrome in a distant agreement, are not always fixed, you can imagine a wide prevalence of hemorrhagic diathesis.

Clinic

Classification 1. Hemorrhagic diathesis due to a platelet defect - insufficiency of platelets - functional defectiveness of platelets - a combination of a quantitative and high-quality pathology of platelets 2. Hemorrhagic diathesis caused by the defect of proagulant (hemophilia) - the insufficient amount needed for the formation of fibrin - the lack of functional activity of individual Promoagulantov - the presence of inhibitors of individual proaagulants 3.

Hemorrhagic diathesis caused by a vascular wall defect - congenital - acquired 4. Hemorrhagic diathesis, due to excess fibrinolysis - endogenous (primary and secondary) - exogenous 5.

Hemorrhagic diathesis, due to a combination of violations of various components of the hemostasis system (Willebrand disease, DVS syndrome, etc.) This classification does not include all known hemorrhagic diathesis.

There are more than 300 of them. It is a scheme of the principles of classifying hemorrhagic states, which can be refreed to refrund not only any of the known hemorrhagic states, but also every newly detected.

Thrombocytopathy is a second group of hemorrhagic states caused by the inferiority of the thrombocyte component of hemostasis. It combines diseases that are manifested by the qualitative inferiority of platelets while maintaining their quantity.

She got the name of thrombocytopathops. In recent years, serious changes have occurred in the classification of thrombocytropathy.

Their essence lies in the fact that many nosological forms, the characteristic feature of which was bleeding, turned out to be inhomogeneous. Attempts to link one or another feature of platelet functional disorders with damage or the features of the development of other organs or systems (Hermansky syndrome - Prudlak, Cedyaga - Higashi, etc.

) In this regard, a certain polymorphism also demonstrate. All this forced doctors to concentrate attention on the specific pathology of platelet functions, which was based on the basis.

The following types of thrombocytopathops distinguish: 1) thrombocytopathy with a violation of platelet adhesion; 2) thrombocytopathy with a violation of platelet aggregation: a) to ADP, b) to collagen, c) to ristomycin, d) thrombin, e) adrenaline; 3) thrombocytopathy with impaired reaction reaction; 4) thrombocytopathy with a defect "pool of accumulation" of released factors; 5) thrombocytopathy with a defect retraction; 6) thrombocytopathy with a combination of the foregoing defects. In addition to the concentration of platelet defects, it is necessary to supplement the diagnosis of the disease with a mandatory indication of the quantitative side of the platelet (hypotombocytosis, hypertromability, normal number of platelets), as well as a statement of concomitant pathology.

The classification of vascular diseases occurring with hemorrhagic manifestations involves their division depending on the localization of the lesion of the morphological structures of the vessel. There are distinguished diseases with the defeat of the endothelium itself and the disease with the defeat of the subendothelium.

Endothelium lesions are divided into congenital and acquired. The representative of the congenital damage to the endothelium is hereditary hemorrhagic teleangectasia (Randu-Ozeri disease).

Among the acquired defeats, the endothelium distinguishes diseases of inflammatory and immune character, damage due to mechanical factors. Inflammatory and immune acquired hemorrhagic states are Shenlayin - Genochi disease, nodule arteritis, allergic granulomatosis, vasculitis in infectious diseases and influences of drugs.

In the same subgroup, chronic inflammatory infiltrates are combined, such as venener granulosis, temporal arteritis, an arteritis of such. Among the mechanical damage, the endothelium distinguishes orthostatic purpura and sarcoma caps.

Hemorrhagic diseases caused by disorders of subendothelial structures are also divided into congenital and acquired. Among the congenital, Eilee - Danlos syndrome, elastic pseudochast age, marfan syndrome, and a disease of imperfect osteogenesis.

In the acquired defects of subendothelia, hemorrhagic states with amyloidosis, seenile purple, corticosteroid purple, simple purple and hemorrhagic conditions during diabetes mellitus are combined. Approximate diagnosis formulation: 1.

Immune thrombocytopenic purple flowing with hemorrhages on the skin and on visible mucous membranes, gums, nose, intestinal bleeding. 2.

Hemophilia A (classical hemophilia), due to a deficiency of VIII factor with hemorrhages in muscles and joints, nasal, gantry, intestinal, uterine bleeding. 3.

Diessemined intravascular coagulation syndrome with skin petechs, bleeding of mucous membranes, hematuria, hemochkump. The bleeding caused by a quantitative or high-quality platelet defect has certain features.

Most often, it is characterized by lesions of the skin - the appearance of small, sizes from the point to the pin head, skin hemorrhages arising from the minimum bruises, or as if spontaneously carrying the name of Petechius. But along with them, bruises and bruises of large sizes can appear - ekkimosis arising from the impregnation of blood and mucous membranes.

They easily arise in places of pressure on the skin of the rubber, belts, etc.

Winds at different times, Petchiy and Ekchimoza, undergoing the stage of natural development and changing its painting with a bug-blue on blue, blue-green, green-yellow, etc.

They lead to the formation of the so-called "leopard skins" in the patient. Multiple surface petechs and ecchimosis are not disappeared when pressing and more often are formed in places with elevated capillary pressure.

Surface cuts and scratches are accompanied by long bleeding. Along with the skin manifestations for the thrombocyte defect, hemorrhage on the mucous membranes are characteristic.

The nasal bleeding from the Kisselbach plot, the gurisy bleeding, provoked by the use of a toothbrush. On the mucous membranes, petechs and hemorrhagic bubbles often occur, reaching large sizes when traumating the lump mucous membranes during the chewing process.

Surgical interventions on the oral organs and in the nasophaling area are very dangerous. Extraction of teeth and removal of almonds can lead to massive bleeding, threatening the life of the patient.

It is curious that the lengthy operations by these patients are moved significantly easier. In the pathology of platelet level of hemostasis, hemorrhages are described in the abdominal cavity, pleura, cavity, retina, brain hemorrhage.

Pulmonary, intestinal and renal bleeding are also not rare. In women, the main manifestations are often menorragia and metrragia - long-term abundant menstruation and non-cyclic uterine bleeding.

Cases of hemorrhage into ovaries simulating the development of ectopic pregnancy are described. For the defects of the thrombocyte level of hemostasis, hemorrhage in joints and muscles is not peculiar.

Most often, similar hemorrhagic diathesis develop due to a decrease in the number of platelets. It is customary to consider a critical state when the platelet level will be below 30 * 109 / l, although some authors believe that the bleeding is mandatory only to reduce platelets to even lower digits - 7 * 109 / l.

With the explanation of the documented thrombocytopenia, it is always necessary to always remember that each healthy person is to 1/3 of all platelets deposited in the spleen. With an increase in the sizes of the spleen, the number of deposited platelets can significantly increase and lead to a decrease in their number in peripheral blood.

In such cases, it is often necessary to solve the question of the need for splenectomy, in most cases returning platelet levels to normal. When using large doses of alcohol, thrombocytopenia occurs both by the destruction of platelets and by reducing their formation.

Like anemia, thrombocytopenia may have a dilution character, i.e.

Appeal due to blood breeding. It must be recognized that such situations are not rare, but they do not have a practical significance, since they do not cause hemorrhagic manifestations and are in advanced character.

Practical importance has thrombocytopenia, arising from the replenishment of large blood loss "old" blood. This should be taken into account and alternate the transfusion of the "old" blood with fresh.

In addition to the thrombocytopenia, due to insufficient reproduction, shortening the timing of life, deposit and dilution of platelets, it is necessary to keep in mind the possibility of their development due to the elimination of platelets from blood flow during sedimentation on the surfaces of artificial blood circulation devices and sedimentation in the fragments formed, especially when the DVS syndrome. In addition to the quantitative defect of platelets, their qualitative disorders - thrombocytopathy are also known.

The existence of thrombocytopathy became known after the publication of the observations of the Swiss doctor E. Glantsman, in 1918

The resulting patient, hemorrhagic manifestation of which corresponded to the thrombocytopenic purple, although the number of platelets was within the normal range. The clinical picture of hemophilia is characterized by bleeding, which is usually associated with some injury, both household and surgical.

More often, the bleeding is developing some time after damage to the tissues and is characterized by difficulty in its stop. Bleeding can be outer, subcutaneous, intramuscular, intra-articular and parenchymal.

The most traumatic are hemorrhages in muscles and joints. Hemorrhage into the cavity of the skull often ends fadingly.

The nasal and gantry bleeding, hemorrhage into mucous membranes, naughty bleeding of newborns, metrragia, bleeding from the gastrointestinal tract and urinary tract may occur with any version of hemophilia. In individual patients, bleeding can be provoked by the infections causing local inflammation (angina, cystitis, acute respiratory diseases, etc.

It is important to keep in mind that the severity of hemorrhagic manifestations only in hemophilia A and B has a certain correlation with the level of defect proguagulant. With other hemophilia, this is not clearly traced.

Differences in the clinic have only two diseases caused by the defect of fibrin formation. They are the defect of the XIII factor, which is characterized by the formation of coarse keloid scars on places of damage to tissues, as well as the defect of the antigen antigen VIII (Willebrand disease).

This disease is described by the Finnish physician Erich von Willebrand in the residents of the Aland Islands in 1926. In the study of these patients, it was shown that normal platelets were not capable of performing their hemostatic function without an antigen factor VIII attaching them to a damaged segment of the vascular wall.

This was the cause of the "thrombocytopenic" clinical picture - phetechial bleeding in the skin and mucous membranes - nasal and oral bleeding, ekkimosis, menorrhagia, long bleeding from cuts, with extremely rare deep hematomas and hemarthrosis. Willebrand's disease inherited by autosomal dominant type, which is sick both men and women, in the frequency of occurrence, stands in third place among hemorrhagic states caused by a defect of procoagulant, accounting for about 10% of their number.

At the same time, the characteristic of the clinical picture often cause objections to the unconditional assignment of Willebrand's disease to the category of hemophilia. The work of recent years give reason to talk about the heterogeneity of this disease and the possibility of the allocation of six subtypes.

Deciphering pathogenesis of Willebrand's disease has shown the possibility of developing the disease, both with an absolute decrease in the factor and in the presence of its functional inferiority. Hemorrhagic states caused by a combination of violations of various components of the hemostasis system are very often found.

The most typical representative of this group is acute and subacute variants of disseminated intravascular coagulation syndrome (DVS syndrome). DVS syndrome, also called thrombo-hemorrhagic, or consumption coagulopathy, has its own features of development.

Hemorrhage with it secondary. They are caused by multiple microtrombs that consume fibrin and platelets and thereby causing hypocoagulation.

Hypocoagulation is usually amplified by hyperfibrinolysis that has a jet. Chronic types of DVS syndrome are practically no bleeding and will not be considered in this section.

Clinically disseminated intravascular blood microwave is extremely varied. This diversity is determined by the unpredictable and most bizarre combination of the prevalence and severity of thrombosis of microcirculatory paths of certain internal organs with activation of the kinin system, complement and secondary fibrinolysis.

Not only the intensity of the thrombosis in its quantitative terms is essential, but also the rate of thrombosis, as well as the localization of the predominant obturation of the microcirculatory channel, which determines the violations of those or other organs. Hemorrhage, reduction in blood pressure and a decrease in the functionality of the internal organs are determined by the severity of intravascular microsinking blood.

Bloodstock and hemorrhage in the skin are often the most noticeable symptoms of the DVS, especially in people of young age. They are determined by such changes in blood properties as a reduction in the level of proagulatants, a decrease in the number of platelets, changes in their functional properties caused by the action of thrombin, as well as activation of reactive fibrinolysis and the effect of certain fibrin degradation products - fibrinogen.

Bloodstock is most often manifested by skin petechs, ekchimozamos, bleeding of mucous membranes, bleeding from injections, hematuria, hemochkami. Sometimes bleeding and microtromombing can be limited to the framework of one or more organs, such as brain, kidney, lungs.

At the same time, the phenomena of the functional insufficiency of a particular organ, which determine the clinical symptoms. The flow of the economy can also have reverse development, but it depends on both treatment and the abilities of the organism itself to neutralize the active factors of blood coagulation, fibrin.

The clinical picture of intravascular microsinking blood may be complicated by the phenomena of acute achemia of organs. These cases can not be considered rare.

Quite often, this phenomenon is observed in patients with neoplastic diseases, in which the signs of the DVS syndrome can be combined with the symptoms of the surface phleet, thrombosis of deep veins, legs, arterial thrombosis and abacterial thrombotic endocarditis. Although the clinical manifestations of the disseminated intravascular microsinking blood are very diverse, it is necessary to keep in mind that all of which are primarily caused by impaired blood flow in the microcirculatory stream of certain bodies due to microtrombosition, which determine this or that violation of their functions, and in second-severity and prevalence Hemorrhagic diathesis, which in turn can affect the functions of certain organs and systems, in its own way modifying the clinical picture of the disease.

The possibility of the development of the DVS syndrome should be remembered when the patient has circulatory disorders, infections, malignant neoplasms, hemolytic anemia. A clinical picture, quite clearly can help diagnose intravascular microsinking blood in the acute version.

To detect a chronic type of engine, clinical indicators give significantly less information. Hemorrhagic states caused by the pathology of the vascular wall are a very heterogeneous group, which is characterized by a very wide range of clinical manifestations.

The most frequent manifestations of these diseases are skin petechia and hemorrhagic rash, easily caused or spontaneous bleeding from the mucous membranes of various localization. On vascular lesions, as the cause of hemorrhagic syndrome, it is possible to speak only in the absence of pathology from platelets and the fibrinization process.

At most commonly found from the above, we will stop more. The most frequent congenital vascular pathology, inherited by autosomal dominant type, is hemorrhagic teleangectasia (Randu - Ozerlee).

The diagnosis becomes in the presence of a classic triad - skin teleangectasis, the hereditary nature of the disease and frequent bleeding. Capillaries are affected and postcase venules.

Teleangectases can be sizes from pinchs to large angioms with a diameter of 3-4 mm. They are located on the mucous membranes, the skin of the face, torso and the upper limbs.

Their recognition is not difficult. Up to 20% of patients with this pathology have arteriovenous shunts in the lungs.

Typically, the disease proceeds benign with timely events at stopping bleeding and treating anemia. The presence of teleconctases is determined by the absence of elastic membrane and muscle fibers in certain sections of the vessels.

The wall consists of only endothelium. In other areas there are formation of arteriovenous aneurysms.

The disturbed structure does not allow vessels to decline when damaged, which determines the bleeding.

Differential diagnosis

The diagnosis of hemorrhage based on the analysis of the data of the anamnesis and the clinical picture can only be made approximately. The use of laboratory methods is always required. This usually scares practical doctors, although a set of diagnostic tests that allows you to put the qualitative diagnosis of hemorrhagic states, very small and easy to perform. This set should be performed in any hospital and polyclinic laboratory. It includes the definition of the number of platelets, prothrombin time, partially activated thromboplastine time, as well as bleeding time.

The elongation of the bleeding time with normal indicators of prothrombin and partially activated thromboplastin time makes thinking about the pathology of the platelet. Calculation of the number of platelets allows to separate thrombocytopathy from thrombocytopenia. The elongation of partially activated thromboplastin time with normal prothrombin and bleeding time gives the base to assume the most common hemophilia. The elongation of prothrombin time with normal bleeding time and partially activated thromboplastine time make it possible to diagnose the factor VII defect (hypoproconvertinemia).

Adding to the listed tests of a challenge study with the use of barium plasma allows to differentiate hemophilia A from hemophilia in, which matters to selection of therapeutic measures. Naturally, this diagnosis is only qualitative. The determination of the quantitative severity of the defect of the progguulant requires tests using plasma, which is carried out in specialized laboratories. There are also tests, deciphering defects of platelet functions - adhesion, aggregation, release reactions, retractions.

For practical doctors, quite high-quality diagnosis, in cases where the clinical situation requires urgent medical measures.

Treatment

Treatment of hemophilia. It is recommended to avoid the use of preparations containing aspirin. Treatment reduces in the blood of patients with the level of antihemophilic globulin in quantities providing hemostasis.

AGG concentrates, freshly frozen plasma, concentrates containing IX factor are used. In recent years, the effectiveness of the application in these patients has been shown, which is capable of rapidly raising the plasma levels of the complex of the VIII factor due to its release from vascular endothelial cells.

The drug (0.3 mg / kg) is introduced intravenously drip for 15-30 minutes. Willebrand disease treatment is recommended for the use of cryoprecipitate.

With the I Type of Willebrand Diseases, desmopressin may be effective. Treatment of immune thrombocytopenic purpura.

Splenectomy is recommended for patients with a disease duration of more than 1 year in the presence of 2-3 exacerbations after corticosteroid therapy. This method is also shown to all patients with severe purples in the absence of an effect from the use of corticosteroids.

With the ineffectiveness of corticosteroid therapy and splenectomy, the use of cytostatic preparations is possible (vincristine of 1.4 mg / m2 or vinblastine of 7.5 mg / m) intravenously once a week for 4-6 weeks. Treatment of teleangectas (Randy - Osler's disease).

No specific therapy. With a daily blood loss of 50-100 ml, it is recommended to use iron preparations, hemotransphus.

When bleeding, due to defects of the factors of the prothrombin complex, it is recommended to use vitamin K (synthetic water-soluble preparation of 5 mg daily). Treatment of DVS syndrome.

Due to the pronounced heterogeneity of this group, as well as the absence of a generally accepted classification of such states, claiming the possibility of exhaustive recommendations for each specific case is not possible. Nevertheless, we believe that the principles of treatment of such patients should be known to each doctor.

1. Removal or active therapy of the main cause of the DVS-syndrome - antibiotics, cytostatics, active treatment of shock, normalization of the volume of circulating plasma, delivery, the extirpation of the uterus, etc.

Stopping of intravascular blood coagulation - the introduction of heparin subcutaneously or intravenously, antiagregative preparations (kuranyl, tiklopens, acetylsalicylic acid, etc.); Intrombin III concentrate administration; The introduction of protein concentrate "C".

3. Introduction of blood components according to indications - thrombocytic mass, washed erythrocytes, cryoprecipitate, prothrombin complex; Freshly frozen plasma.

4. Suppression of excess fibrinolysis - M-aminocaproic acid, para-aminobenzoic acid.

Naturally, this treatment should be carried out with constant control of the level of fibrinogen, platelet numbers, as well as fibrinolysis. Extracorporeal gravyrurgic correction of the aggregate state of blood with hematological and non-hematological diseases.

Gravitational blood surgery (HCC) is a complex of extracorporeal methods for correction of the composition of peripheral blood using special devices in which gravity forces are used to separate the fluid on the fraction. In these devices called by the hemoprocessors-fa -ctionators, under the influence of centrifugal forces, the blood is divided into components of various molecular weight.

As a result, it is possible to remove cells, plasma, toxic substances, immune and other blood components, as well as replacing them with drugs that make it easier by its morphological composition, aggregate state and rheological properties. Depending on the composition of the morphological substrate to be removed (afeisu), the methods of HCC are divided into several types: 1) Plasmapheres - removal of plasma from peripheral blood; 2) granulocitafferesis - removal of granulocytes; 3) lymphocitheresis - removal of lymphocytes; 4) thrombocitherase - removal of platelets; 5) Blast-cytapheresis - removal of blast cells; 6) Lymphapfersez - removal of lymphs of breast lymphatic duct; 7) Myelocarocyteneres - separation of bone marrow suspension into cellular elements and extracting its elements from the suspension.

The HCC methods are restored by the normal composition of the blood not only by means of a combined addition (transfusion), but also by removing (aezeresis) of individual components, during this operation, the doctor has the ability to program the composition of the patient's blood in advance, to carry out the purposeful correction of the cellular and protein composition of the blood, disturbed when various pathological conditions. For the operation of the HCC, special devices are designed.

In the devices with continuous blood flow, the fractionation process is carried out continuously, during the operation the blood is divided into components, the necessary fraction is withdrawn, and the remaining of the blood is continuously returned to the patient. The devices have a centrifuge rotor, a system of highways, roller pumps, engine, control system.

Under the action of peristaltic pumps, blood from the patient through the catheter is fed to the system of highways, where it is mixed with the anticoagulant and enters the rotor of the device. In the rotor, blood fractionates into components under the influence of centrifugal forces (gravity forces), the layers of various fractions are summarized to special holes and with the help of peristaltic pumps is seized by the required blood component.

Blood is continuously reinfused. In the devices with intermittent blood flow in the rotor, its fractionation occurs.

As the blood accumulates in the rotor, the plasma is consistently displaced, then platelets and leukocyte suspension. After that, blood flow from the patient and the reverse move of the rotor, the erythrocyte suspension is supplied to the reinfusion tank.

Following the reinfusion of the erythrocytic mass, the cycle is repeated. The equipment provides a series of methodological techniques: 1) the degliserinization of the frostbed erythrocytes; 2) laundering canned erythrocytes; 3) erythrocytafferes; 4) plasmapheres; 5) lymphocitheresis; 6) granulocitafferes; 7) thrombocitherapieres; 8) PlasmaSorption on biological sorbents (hepatocytes, spleen cells, beta cells of the pancreas); 9) plasmaSorption on chemical sorbents; 10) Erythrocyte oxygenation against the background of erythrocytacherresis.

Gravyrurgical operations are applied in the following cases associated with impaired morphological and biochemical composition of blood: 1) the correction of the protein composition of the plasma is the removal of ex- and endogenous pathological proteins; 2) correction of immune disorders - removal from the blood of circulating immunoglobulins, antigen - antigen complexes, complement components, T-lymphocyte components, breast lymphatic fluid lymphs; 3) Correction of the cell composition of blood - removal of platelets, erythrocytes, blast cells, etc. In most cases, gravyururgical operations are carried out with TAGA, when patients are resistant to generally accepted therapy and need events that reduce this resistance and increase treatment with conventional conservative therapy methods.

The gravyururgical correction of plasma protein composition is used in pathological conditions and diseases specified in the table. Gravitational plasmapheresis can be combined with other methods for removing pathological proteins from plasma.

More often to Plasmapheresis connect immunosorption, plasmaSorption. The most successful operation of the gravyrurgical correction of the plasma composition is, if necessary, to relieve long-term squeezing syndrome, increased viscosity, disseminated intravascular blood coagulation.

The methods of gravyrurgical correction are promising and are also effective if necessary, actively regulate the aggregate state of the blood, impaired as a result of various diseases or due to surgical intervention using the artificial circulation apparatus. Control of the feed rate of infusion solutions in the highway, the return of the erythrocytic mass of the patient on the machines of PF-0.5 or RK-0.5 allows you to adjust the filling of the vascular channel, maintain at the required level of blood pressure, at the same time remove cholesterol »fibrinogen and other substances significantly improve rheological Properties of blood and microcirculatory blood flow, including in thromboembolism of small branches of the pulmonary artery.

Using the gravyosurgical correction of the aggregate blood state, you can form controlled hemodilution, hypo- and normophemia, control the concentration of any biochemical factors in the blood and the number of its cellular elements. A new approach to counter-tomb therapy is to use to reduce coagulation processes and reduce the blood viscosity of gravyrurgic fractionation of blood in a continuous flow on the devices of the PF-0.5 apparatus with the removal of the excess number of cell and plasma hemostasis factors from the circulating blood.

Simultaneously in the vascular channel, rheologically active solutions (REOPOLIGLUKIN, ALBUMIN) and ANTITROOMBINI III are introduced as part of a freshly frozen donor plasma. The use of this method does not exclude traditional anti-lumen funds, but, on the contrary, increases their therapeutic effect, expands the capabilities of modern cardiology, resuscitation, surgery.

In the syndrome of a long compression, plasmapheresis is shown to all patients having signs of intoxication, the duration of the compression of more than 4 hours, expressed local changes in the damaged limb. The correction of the immunological status is effective by plasmapheresis, removal from the blood flow of antibodies and immune complexes, immunogenic plasma proteins and mediators of inflammation - kinines, complement factors that determine the inflammatory response and tissue damage in autoimmune processes.

The traditional method of reducing the immunological reactivity of the pharmacological means of immunosuppressive action (cyclophosphane, azathioprine, corticosteroids) for diseases that were called "diseases of immune complexes" is not always effective, and the drugs themselves are rather toxic. Clinical observations indicate a significant therapeutic efficacy of gravitational plasmapheresis with such a kind of diseases.

Pathological conditions Diseases alloimmunization Hemolytic disease of the newborn rejection in renal transplant Autoimmunity Autoimmune hemolytic anemia, autoimmune thrombocytopenic purpura, myasthenia syndrome Goodpasture Pemphigus Aggression immune complexes Systemic lupus erythematosus Tolerability cytapheresis overwhelmingly good, which allows this operation in an outpatient setting, delaying the patients after the treatment supervised The doctor is 2-4 hours. With chronic lympholesis during one session of leukocytaphelinis from the patient's body, up to 4 * 1012 leukocytes are removed.

The number of remote leukocytes is directly dependent on the initial leukocytosis of peripheral blood. The intervals between sessions and courses of cytopheresis, their number is determined by the peculiarities of the patient's response to this operation and the clinical and hematological picture of the disease.

The clinical effect of gravitational cytopheresis provides the possibility of subsequent cytostatic treatment and improving the response to it, which is important in cases of development of chemotherapy resistance in tumor diseases of the blood system. Methods for correction of the cellular composition of blood based on transfusion of missing cellular elements are also used in clinical practice.

Transfusions single cell concentrates obtained from the donor by cytapheresis occupy sometimes leading position Pathological conditions Diseases Hemoblastosis Acute Leukemia Chronic Lymphocytic Leukemia Chronic myelogenous Erythremia thrombocytosis haemorrhagic trobotsitemiya Thrombocytosis Hemoglobin Sickle cell anemia, beta-thalassemia chronic nonspecific pathology of chronic pneumonia, bronchial asthma (infectious-allergic form) Other states of rheumatoid arthritis glomerulonephritis vasculitis Malignant neoplasms Inflammatory bowel disease (Crohn's disease) To obtain a resistant positive effect, it is necessary to repeatedly use intensive plasmapheresis in a complex with immunosuppression and cytostatic therapy. The result in each case will depend on the timing of the origin of the treatment, frequency and volume of plasmapheresis and plasma exchange, a clear establishment of patient selection criteria.

Methods for correction of the cell composition of blood based on cytopherase are used in therapy of gemoblastosis, thrombocytosis, hemoglobinopathy and other diseases in the complex therapy of a number of diseases. Granulocyte transfusion is used in patient therapy with burns, sepsis, neutropenia.

Platelet transfusion is used in thrombocytopenic hemorrhages. The erythrocytic weight free of leukocyte impurities and platelets is overflowing with patients with anti-gositic antibodies, during kidney transplantation, long-term compression syndrome and surgical interventions.

When establishing contraindications to carry out the operation of the HCC, it is important to determine the degree of somatic compensation of patients, the state of the cardiovascular system, the system of regulating the unit of blood, hemograms, liver, kidney functions. For peptic ulcer of the stomach and duodenal intestine in the stage of exacerbation, mental diseases of the GKC operation is not recommended.

Mandatory is to determine the possible presence of surface antigen and antibodies to the human immunodeficiency virus (HIV). In case of finding HASAG or antibodies to HIV for a gravyrurgical operation, it is necessary to use specially selected blood fractionation devices.

Attention! Describing treatment does not guarantee a positive result. For more reliable information, be sure to consult a specialist.