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Clinic and diagnosis of the IDCS - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Remission and recurrence of an acute leukosis
Inborn leukosis
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

The clinical picture of the IDCS consists of the symptoms of a basic disease and symptoms caused by disturbance of hemocoagulation. Allocate several typical clinical syndromes which emergence against the main pathological process can authentically demonstrate the developed hemocoagulative disturbances: 1. Disturbance of a blood-groove and development of a decompensation of peripheric circulation. 2. Hemocoagulative shock as extreme extent of frustration of a hemodynamics. 3. Disturbance of microcirculation in different bodies: a) in kidneys — leads to development of an acute renal failure. One of options is the gemolitiko-uraemic syndrome of Gasser which is most often noted at acute hemolitic processes. At the same time the acute renal failure, hemolitic anemia, a Werlhof's disease are observed: b) in lungs — leads to development of the acute pulmonary insufficiency which is characterized by cyanosis, expressed by an asthma the possibility of a fluid lungs is not excluded; c) in a liver — leads to development of the liver failure which is followed by parenchymatous jaundice; d) in adrenal glands — leads to development of acute adrenal insufficiency (Waterhouse's syndrome — Frideriksena), most often meets at a meningokokktsemiya. It is characterized by a fulminant aggravation of symptoms, the heavy neurologic status (a coma, spasms), a hyperthermia, falling of arterial pressure, vomiting, a diarrhea, development of dehydration, the progressing respiratory and cardiovascular insufficiency; e) in TsNS — it is characterized by neurologic symptoms: headache, spasms, loss of consciousness, decrease in visual acuity, hearing etc. 4. Hemorrhagic syndrome. Hemorrhages, bleedings from mucous membranes of nasal and oral cavities, a digestive tract, a hemoperitoneum and internals are noted extensive spotty petekhialnye; in the presence of operational and traumatic wounds — bleedings from these places. Bleedings from places of injections, blood, testimonial of a deep depression of coagulant system, are considered as an adverse predictive sign. Bleeding develops against almost full, and quite often absolutely full incoagulability of blood.
In diagnosis the main pathology (sepsis, hemolitic processes, pathological childbirth etc.) which has to orient the doctor in respect of threat of emergence of the IDCS is of great importance. Emergence against a basic disease of symptoms of disturbance of hemocoagulation svidetelstvut about development of the IDCS.
Diagnosis of various phases of the IDCS is possible by the elementary low-sensitive tests. However, as mark out 3. S. Barkagan and coauthors (1982), are easier to define initial hypercoagulation, using modern standard tests. Especially they are important at control of a geparinoterapiya.
In a phase of hypercoagulation shortening of a blood clotting time, plasma recalcification, increase of consumption of a prothrombin, shortening of prothrombin and thrombin time is noted. The same information is given also by the standardized tests — a kaolin-kefalinovoye time, the autokoagulyatsionny test, etc. Adhesion of thrombocytes is also increased.
At the end of a phase of hypercoagulation and an initial stage of hypocoagulation find the following typical shifts (3. S. Barkagan, 1980):
a) existence in a smear of peripheral blood of fragments of erythrocytes (fragmentation phenomenon); b) the progressing thrombocytopenia; c) lengthening of a prothrombin time; d) lengthening of thrombin time; e) decrease in level of fibrinogen in plasma; e) increase of content in plasma of products of degradation of fibrinogen and fibrin (PDF); g) increase of content in the plasma deprived of thrombocytes, an anti-heparin factor (a factor 4); h) in some cases saving positive paracoagulative tests (ethanol, protamin - sulfate) which are usually noted at early stages.
The phase of hypocoagulation is characterized by the expressed blood coagulation disturbance that is reflected by all low - and highly sensitive coagulative tests. Research in dynamics of parameters of an autokoagulogramma, antithrombin III definition, and also plasminogen is of great importance for an assessment of a geparinoterapiya. Besides, careful control of functions of bodies which defeat is possible at the IDCS is necessary.

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