Hemorrhagic Syndrome - a set of pathological conditions involving a single clinical symptom, manifested in increased bleeding and different polyetiology incurred.In terms of clinical and laboratory diagnosis and outlook for the patient's health is the fundamental difference between chronic and acute form of the flow of hemorrhagic syndrome.Clinical and laboratory manifestations of hemorrhagic syndrome can "start" at any age, from neonates to the elderly age group.
Causes of hemorrhagic syndrome
all pathogenic form of hemorrhagic syndrome can be divided into two broad categories (primary or hereditary and secondary or acquired), depending on the prevailing etiological factor, which is the cause of abnormal bleeding.
Hereditary hemorrhagic form of the syndrome can develop in several pathogenetic mechanisms, but for all of these states are characterized by genetic determination (the presence of the defective gene).Genetic determination can affect all parts of the coa
secondary or acquired hemorrhagic syndrome often develops as a result of vascular wall lesions of various calibers resulting inflammatory, mechanical, autoimmune or toxic effects.Secondary thrombocytopathy as one of etiopathogenetic variants hemorrhagic syndrome may be triggered by long-term use of certain drugs pharmacological groups (eg, antiplatelet agents), and as a result dismetabolic disorders in the body.
Reduced platelet blood cells also triggers the development of hemorrhagic syndrome and often develops as a result of violations of the processes of normal hematopoiesis in bone marrow or increased their destruction that occurs with DIC, thrombocytopenic purpura, and systemic lupus erythematosus.
coagulopathy as hypocoagulation manifest development of hemorrhagic syndrome and develops most often as a congenital disorder (hemophilia, von Willebrand disease).Pathogenetic mechanisms of this form of hemorrhagic syndrome is a significant reduction in the production of plasma factors and increased activity of the fibrinolytic system factors.
Primary vazopatii generally provoke a hemorrhagic syndrome in children during puberty and the basis for their development is necessary pathological increased permeability of the vessel wall and the formation of telangiectasia (limited extensions of the vascular wall).
Note that abnormal bleeding can occur not only on the skin, and mucous membranes, and serves as an example of hemorrhagic syndrome uterine tumors observed in the benign and malignant.
Symptoms of hemorrhagic syndrome
most common clinical variant pathological bleeding is a cutaneous hemorrhagic syndrome, which can vary greatly in intensity and morphological type of skin cells.So, the most difficult in relation to patient tolerance is gematomny option, which is observed in hemophilia and shows the development of the massive outpouring of blood and accumulation in intermuscular spaces and joint capsules, accompanied by severe pain.Pathognomonic symptom gematomnogo option hemorrhagic syndrome is a significant long-term restriction of mobility of the joints or the whole group.
emergence of bleeding occurs, usually after any traumatic exposure, and in severe hemophilia hematoma and hemarthrosis arise spontaneously on the background of well-being.Most pathognomonic sign gematomnogo hemorrhagic syndrome is a variant of the signs of intraarticular hemorrhage, and suffer disproportionately large group of the joints of the upper and lower extremities.Clinical criteria hemarthrosis in hemorrhagic syndrome is a sharp intense pain, swelling of the periarticular soft tissues and the inability to perform usual motor movements.In a situation where the joint capsule is collected in a large amount of fresh blood may be a positive symptom fluctuations.The consequences of such changes in hemorrhagic syndrome gematomnogo type is the development of chronic infection in the synovial membranes and destruction of cartilage, which inevitably leads to the development of deforming arthrosis.
When pathological change in the amount or form of platelet cells often develop petechial-spotty hemorrhagic syndrome.With this pathological condition in a patient with a disposition to develop Surface intradermal hemorrhage even with minimal traumatic effects on the skin (for example, mechanical compression of the skin cuff when measuring blood pressure).Petechial hemorrhage intradermal usually has a small size, not exceeding three millimeters of intense red color, which does not disappear on palpation.For this embodiment, the hemorrhagic syndrome characterized by the appearance of hemorrhages in the mucous membranes of different localization, especially in the conjunctiva of the eye.When petechial-spotted form of hemorrhagic syndrome observed in idiopathic thrombocytopenic purpura, there is a tendency to development of persistent nosebleeds, provoking anemisation body.
In mixed-sinyachno gematomnom embodiment the patient is noted as the development of intradermal hemorrhage and intermuscular hematomas.The principal difference between this form of hemorrhagic syndrome gematomnogo option is the absence of intra-articular hemorrhage.
When hemorrhagic vasculitis, hemorrhagic syndrome as a variant, there is development of skin hemorrhages in the form of purpura.Hemorrhagic rash elements are formed in the inflammatory phase change of the skin, so they are always a few rise above the surface of the skin and have a clearly defined pigmented contours.The principal difference between hemorrhagic rash vasculitis is resistant pigmentation of the skin, even after leveling acute manifestations of hemorrhagic syndrome.
most specific for the clinical manifestations is angiomatous hemorrhagic syndrome, which is caused by the appearance of anatomical changes in the vascular wall.For this form of hemorrhagic syndrome is characterized by bleeding a certain location, such as the nasal cavity with the syndrome Rendu-Osler.When angiomatous variant of hemorrhagic syndrome never seen intradermal and subcutaneous hemorrhage.
Since acquired hemorrhagic syndrome develops in the background of any diseases and pathological conditions can be supplemented with a classical picture of nonspecific symptoms typical of background pathology.
Qualitative diagnosis of hemorrhagic syndrome involves the assessment of clinical, laboratory and instrumental data.A fundamental element in carrying out diagnostic research is a thorough collection of anamnestic data and carrying out a careful examination of the patient with the definition of localization, the nature of cutaneous manifestations.
hemorrhagic syndrome in newborns
Note that a child's birth is celebrated imperfection of almost all organs and systems in the body, including the mechanisms of hematopoiesis.All newborns with a disposition to increased vascular permeability of the capillary network, as well as the immaturity of processes and clotting factors, which are purely functional and not provoke the development of classical hemorrhagic syndrome.In a situation where a child after birth in the short term develop acute hemorrhagic syndrome, one must first assume the genetic nature of the disease.Indirect predisposing factors in the development of hemorrhagic syndrome is a long-term prescription drugs group salicylates mother during pregnancy, intrauterine fetal hypoxia and prematurity.
In the last decade in neonatology practice widely used prophylactic use of vitamin K in the minimum dosage of 1 mg, significantly reduces the incidence of hemorrhagic syndrome among children newborn period.Notable is the fact that the clinical picture of hemorrhagic syndrome does not develop immediately after delivery, and after the seventh day, when the child is discharged from the hospital, so the main task of local pediatrician, watching the baby after birth is to adequately assess the severity of the child's condition and in a timely manner to determine the cause of thethis pathological condition.
classical clinical criteria of hemorrhagic syndrome in the newborn child is the appearance of blood in the stool or vaginal bleeding, prolonged healing of the umbilical wound and discharge of blood from it, discoloration of urine, which becomes brownish-red, and the selection of fresh blood from the nasal passages.
In the long term (a few weeks after birth) hemorrhagic syndrome can develop as a result of severe dysbiosis and manifest symptoms of intracerebral hemorrhage, which has a very severe course.
treatment of hemorrhagic syndrome
volume and methods of treatment for every form of hemorrhagic syndrome can vary greatly, so in determining the tactics of the patient is the determinant of pathogenic variants of the disease.
Considering hemophilia, it should be borne in mind that the only pathogenetically substantiated treatment is transfusion of blood components is a variant of substitution therapy.Due to the fact that most of the clotting factors are very labile and retain their properties in the preservation of blood and manufacture of dry plasma, the priority is a direct blood transfusion from a donor patient.
Factor VIII has a short period of excretion, therefore adequate replacement therapy scheme is transfusion and plasma cryoprecipitate at least three times a day in a single dose of 10 ml calculated for 1 kg of the recipient.It is inadmissible to mix with any antihemophilic means whatsoever infusion solutions, the drug should be administered only intravenously as drip infusion does not allow to raise the level of factor VIII in plasma.
If a patient has a combination of hemarthrosis with severe accumulation of blood in the intermuscular spaces should be used more intensive therapy Antihemophilic means.With existing external bleeding enough to use topical treatments where (thromboplastin solution).Signs of acute hemarthrosis is the basis for the application of the joint capsule puncture and aspiration of blood, followed by intra-articular injection of hydrocortisone.In remission necessarily need to perform physical therapy exercises that target the restoration of motor function of the joint and prevent the development of atrophic changes of muscle array.Surgery is used only as a last resort in the formation and ankylosis at the stage of osteoarthritis.In this situation, apply such operating benefits as a synovectomy and distraction of bone tissue).
In hemorrhagic syndrome disease with coagulopathy, the fundamental method of treatment is a parenteral administration of menadione in a daily dose of 30 mg.In a situation where a hemorrhagic syndrome develops in the long-term use of anticoagulants of indirect action in a high dosage, it is imperative they cancel.In severe coagulopathy use fresh frozen plasma replacement therapy intravenously at a dose of 20 ml calculated per 1 kg of body weight with growth hormone at 4 IU per day, the effect of which is aimed at improving the synthesis of coagulation factors.
DIC is the most severe and difficult to remedy forms of hemorrhagic syndrome and the mortality rate in acute DIC is more than 60%.The drug of choice in hemorrhagic syndrome caused DIC, heparin is in a stage of hypercoagulable initial dose of 10 to 000 units, and the subsequent transition to subcutaneous administration of a single dose of 5000 IU.When a massive hemorrhage was observed in patients hypocoagulation stage shows the introduction of high doses of intravenously kontrikala to 10,000 units.
When thrombocytopenic purpura pathogenetically justified is the appointment of a number of glucocorticosteroid medications, and surgery as a splenectomy.In the complete absence of a positive effect of the applied treatment necessary is the appointment of cytostatics immunosuppressive action.Prednizologa daily dose is 60 mg, and provided ongoing hemorrhagic syndrome in the form of new hemorrhages, a dose of prednisolone should be increased twice.The duration of corticosteroid therapy depends on the speed of the relief of clinical manifestations of hemorrhagic syndrome and the degree of normalization of the number of platelet blood cells.In a situation where tapering prednisolone provokes recurrent hemorrhagic syndrome, it is necessary to increase the dose again.
Adequate dose immunosuppressant is 5 mg per 1 kg of body weight (Cyclosporin A).The absolute indication for the use of drugs of this group is continuing during the hemorrhagic syndrome and progressive thrombocytopenia even after splenectomy.
When hemorrhagic telangiectasia as an embodiment of the hemorrhagic syndrome, the use of common therapeutic interventions are not having a positive effect.However, estrogen or testosterone, in some situations, decreases bleeding.Recently, widely used method of operational and laser correction of this disease with good long-term results and a low percentage of recurrence.
Since different versions of hemorrhagic syndrome accompanied by the development of anemia in the body to some extent, in most cases, patients with bleeding disorders should be further prescribe iron supplements (Tardiferon a daily dose of 600 mg).
prevention of hemorrhagic syndrome
as primary and most effective methods of prevention of hemorrhagic syndrome among children newborn period should be considered routine subcutaneous administration of vitamin K to all preterm infants, as well as holding of early initiation of the child to her breast.
in respect of preventive measures aimed at preventing the development of hemophilia, as the most severe form of hemorrhagic syndrome should be considered a genetic screening, the most accurate method to determine the degree of risk of having a child with symptoms of hemophilia.People suffering from hemorrhagic syndrome, should inform all treating professionals about if they have this disease, as even banal tooth extraction in these patients should be accompanied by pre-medical training.
in the prevention of recurrent episodes of hemorrhagic syndrome, particularly gematomnogo option, a good positive effect have special exercises of physiotherapy, subject to regular performance.