Hemolytic-uremic syndrome: symptoms, diagnostics, treatment. Signs of the peculiarities of the treatment of hemolytic-uremic syndrome Atypical hemolytic uremic

Hemolithic-uremic syndrome is a clinical-hematologic symptom complex, which is responsible for the development of etiological officials and important breakdowns in the human organism. Qia pathology is characterized by hemolysis of erythrocytes, a decrease in the number of platelets in the blood, dysfunction of nirok... Thrombotic microangiopathy is superimposed on numerous thrombus and occlusion of other blood vessels. When thrombocytopenia develops, there are intermediate ischemia and necrosis in the cerebral tissue and internal organs.

Hemolithic-uremic syndrome is more likely to be called Gasser's ailment; Patients with this pathology have pain in the abdomen, blood-borne disease, sclera, icterus of the sclera, swelling of the face, petechial visip, anuria, symptoms of CNS damage, liver, heart. Diagnostics of illnesses primed on key symptoms and results laboratory dosages. Timely and competent therapy of HUS allows the prognosis of pathology to be developed by the friendly.

ailment Gasser to develop among old people, children of preschool age, schoolchildren, children, and even more rarely among older people. In women, when there are flat paths, the pathology is most important and often of a recurrent nature. Hemolytic-uremic syndrome is not of low seasonality. Acquisition of the maximum level in summer-autumn period: from worm to spring. The pathology of the intestinal ethology is reorganized in the late hour, and the disease of the viral mobility - in the rise and in the charge.

Atypical hemolytic-uremic syndrome (Agus)- there is a rare occurrence of pathology, as a result of an important interruption and a poor prognosis. The separation of capillaries, arterioles and venules poruses the function of internal organs, which manifests itself in clinical symptomatology.

Classification

Hemolithic-uremic syndrome according to the etiology and the type of disease is divided into two types:

Type, interconnection with diarrheal syndrome - D +,
Atypovy, no dressing with a direuyu - D-.

Diary forms are found among children younger than 5 years old, who live in endemic regions - Volga, Pidmoskov. Atypical HUS is developed in children of the school age and grown-ups.

HUS can be either light or heavy:

Easy form of two types. Type A manifests itself as a classic trait: anemia, thrombocytopenia and pathology nirok. Type B manifests itself as a triad, as well as in trials and hypertension.
An important form is divided into two types. Type A is manifested by three symptoms and three more symptoms. Type B may have analogous symptoms, to the point of being sufferer, anuria and hypertension.

Ethiological classification of atypical HUS:

post-vaccination,
postinfectious,
sloppy,
lykarsky,
idiopathic.

Ethiology

typical HUS

pathogenesis of bacterial diarrhea

The pupils of the HUS, tied with a diary, are enterohemorotic intestinal sticks, shigella, staphylococcus, and the larger ones are salmonella, campilobacterium, clostridia.

Yesherіkhіya produku shiga-podіbny verotoxin, which is the enemy of the clitini sudin nirok in children up to 3 years old. Endotheliocytes gin, winykus, hemolysis of erythrocytes, adhesion and aggregation of thrombocytes, developing disseminated intravascular coagulation syndrome. Produce microcirculatory discharges to hypoxia of internal organs. In ailments on aphids of GKI, there is an inflamed glomerulus of nirok, a deterioration of filtration function, development of ischemia, an affirmation of necrosis, a decrease in cerebration of nirok.

Gostra kishkov іnfektsіya - ailments of the hands. Microbes penetrate into the body of people when they come into contact with infected creatures or people. Infection is possible with implantation of raw milk, rotten fruits and vegetables. Insufficiently effective heat treatment of meat also becomes a cause of intestinal breakdowns.

atypical HUS

Theory of atypical HUS diagnosis:

Infectious diseases - pathogens of pathology є microbi: pneumococcus, virus of vitrianoisp, VIL, influenza, Epstein-Barr, Koksaki.
Lykarska - the development of pathology in the intake of certain medications: antibiotics, hormonal contraceptives, cytostatics.
Spadkova - symptoms of incidence of disease with autosomal dominant and autosomal recessive type of sedation.
Post-vaccination - the development of HUS will be introduced for the introduction of live vaccines.

Agus grows in individuals:

have suffered more surgical involvement,
May you get sick,
I am suffering systemic incapacitation- scleroderma, glomerulonephritis,
may have a history of malignant hypertension,
vagіtnykh women,
VIL-Infikovanih,
drug addict.

Atypical hemolytic-uremic syndrome (Agus) є the acceleration of the main incapacitation, the appearance of a typical form of ethology, clinical, histological and pathomorphological picture, unpleasant prognosis and severe summer. Pathology develops in 10% of cases.

pathogenesis

typical HUS

Pathogenetic lankas of typical hemolytic-uremic syndrome:

Bacterial toxins oppose intestinal cells, which can lead to the development of hemorrhagic colitis. The lungs have a distress syndrome. Nirkova tissue is even more sensitive to bacterial toxins. Disruption of the filter function is necessary to produce up to the accumulation of products in exchange, development of uremia and intoxication of the organism. Microcirculatory metamorphosis in the internal organs to summarize the symptoms of HUS.

atypical HUS

Pathogenetic lanka of atypical HUS:

hyperactivity and dysfunction of the system and complement,
the establishment of immune complexes and contributions to the Endotels of Sudin,
schooling of endothelial clientele and іх ruinuvannya,
ischemia fabric,
destruction of robots and internal organs.

Have healthy people system for complementing pathogenic biological agents - bacteria and viruses. In case of hemolytic-uremic syndrome, the immune activity of the immune system is set up, which “attack” the vital cells of the body - endothelial cells and the health of the body. Without interruption in the activation of platelets, it is necessary to produce thrombosis and pest in robotic organs.

symptomatology

Period of hemolytic-uremic syndrome - prodrome, raspal, renewal.

The prodromal period is trivial 2-7 dib and to recover from the appearance of nonspecific symptomatology - non-mutilation, weakness, catarrhal symptoms. intestinal symptoms presented with manifestations of gastroenterocolitis: blood-borne, dyspepsia, abdominal pain. Respiratory clinical signs - rhinitis, bile and perspiration in the throat, chhannya, cough.

At the end of the sickness, the zagalny camp of the patients is going to burn, the awkwardness is increased, and the ship is ready to change, apathetic, apathetic. Ailing children do not scream and do not cry, the stench reacts weakly to the calls of the teens. Symptoms of anemia, thrombocytopenia and dysfunction are detected. In organizmі it grows vodnennya, peripheral blood circulation collapses. A third of the patients develop postural thrombosis.

Shkira is old bled, scleri ikterichnost, worms pasty. In the world of the growth of hemolytic processes, the whiteness of the skin changes with yellowness.
Hemorrhagic syndrome is manifested by nasal bleeding, petechiae or ekhimosi on the skin and mucus, hemorrhage in the nasal hemorrhage or the eye.
Symptoms of nirkovy syndrome are oliguria abo anuria, hematuria, proteinuria.
Neurological damage - impairment of evidence, progressing malaise, nervous tic, Nistagm, ataxia, sudominal syndrome, Stuporous stun, coma.
Determination of heart and sudin - accelerated heart rate, muffled tones, systolic murmur, extrasystole, signs of cardiomyopathy, myocardial infarction, hypertension, diffusional vasculopathy, heart failure.
Defeat the broncho-legacy system - zadishka, zhorstke dikhannya, wheezing.
Symptoms of a reduction in the intestinal tract are abnormalities, stoves, gristle in the mouth, nausea, bloating, abdominal pain, flatulence, gurgling, unstable viporozhnenya, pathological houses in the feces.

As soon as pathogenetic therapy is started, the oligoanuric stage changes to polyuric. The organism of a sick person is almost catastrophically quick to drink water and electricity. Pislya rozpalu zvoryuvannya onset іnsha stadіya - renewal. The home camp of the ailments stabilizes, the damaged functions gradually normalize. Patients in their blood grow up instead of platelets, get more frequent, normalize hemoglobin levels and change intoxication.

Moderately severe hemolytic-uremic syndrome is characterized by rapid progress of non-toxic dysfunction: growths of intoxication, anuria, diagnoses of postural pathologies, fever. In neglected diseases, decompensation of nirkovo deficiency and a lethal result form.

Symptoms of atopic HUS are analogous to clinical manifestations of the th type form. Inquisitiveness grows up to be a boisterous interruption and development of serious acceleration. The diagnosis is made, as there is no association with the day and the day of the laboratory, the confirmation of the presence of the toxin in the organism.

diagnostics

Diagnostics of HUS to be primed on scargs of ailments, given to look around and the results of laboratory tests.

In the outward analysis of the blood of ailments, there is a decrease in the level of erythrocytes and thrombocytes. Chervoni blood tiltsya are fragmented and swelled into the shape of sticks or trikutniks.
In the biochemistry of pre-age blood, there are changes in the substitution of creatine, bilirubin, transaminases, calories, magnesium, excess nitrogen, low blood, chlorine and sodium. Also, the provision of microelements in case of damage to water electrolyte balanceі development of dehydration in organism.
In this case, there is a lot of blood and blood cells.
As a result of microbiological pre-development, feces appear as enterohemorrhagic intestinal sticks in the value of counts, in the coprogram - erythrocytes.

lykuvannya

The treatment of hemolytic-uremic syndrome in children is carried out in the minds of the hospital. We are sick of the indications of the post-treatment regime and the therapy. Children recognize a child that allows them to live only breast milk and lactic acid sum. The rate is expanded step by step. Doroslim recommend dotrimuvatisya children, scho between vicoristannya soli.

Ethiotropic therapy is anti-microbial. We are sick to carry out antibiotic therapy for additional drugs wide rangeдії. In the given hour, staphylococcus, salmonella, klebsiellezny and іnshі bacteriophages are widespread.
Pathogenetic therapy of polyagus in usuned and transient processes of thrombosis. We are averse to priznachayut anticoagulants and antiaggreganti - "Curantil", "Heparin", as well as drugs that paint microcirculation of blood - "Trental", "Cavinton", "Piracetam", "Vinpocetin".
Detoxification therapy - the introduction of colossal and crystalline breakdowns.
Antioxidant therapy with vitamin E.
Zamisna therapy - transfusion of fresh frozen plasma, erythrocyte mass.
From the hour of the oligouristic period, it is recognized that the sechogins are assigned - "Furosemid", "Lasix"; to carry out plasmapheresis, peritoneal dialysis or hemodialysis.
With tachycardia and hypertension - adrenergic blockers: "Atenolol", "Betaprolol" and іngіbіtori APF: "Kapoten", "Anapril".
With a lot of legends - "Euphilin", piece ventilation of legends.

Hemolytic-uremic syndrome is an important pathology, and a very serious prognosis. Young children often have a fatal result, and older and older children develop a nircum deficiency and decrease in glomerular filtration. Typical HUS against nagato is lighter than non-arterial syndrome, which leads to frequent recurrence and a high rate of mortality.

Prevention of typical form of GUS polyagaє at the vikonanny deyakykh rules. Fakhivtsi recommend:

dotrimuvatisya rules of special hygienia,
homeless creatures in the summit waters,
drink only boiled water,
after trimming the processing technology and preparing meat strains,
do not live milk in sira,
good miti vegetables and fruits,
unique contact with people suffering from gosstro intestinal infection.

Video: presentation on hemolytic-uremic syndrome

Hemolytic-uremic syndrome (HUS) is a serious therapeutic problem in pediatric and child's nephrology, being one of the provincial causes of hospitable deficiency due to the potential transformation into thermal chronic deficiency.

Uninvolved on those who have expanded the typical form of HUS with a diarrheal prodrome, associated with the Shiga toxin (STEC), it is not necessary for the diagnosis of infectious etiology, pneumonia By all means, STEC-HUS is recommended to be adequate symptomatic therapy with free dial-up connections if necessary. Forecast in case of a large future of stagnation from the triviality of the anuric period and the support of the central earliest nervous system.

According to the recommendations of the Initiative for the improvement of global results in enrichment of nirok (Kidney Disease: Improving Global Outcomes, KDIGO) ...

Typical hemolytic uremic syndrome (HUS)

With the development of hospitality nirkova disadvantages in the period from 6 months to 3 years, there is a great value of GUS in the capacity of the reason.
An anamnestic reference to the previous episode of blood in the feces can serve as an argument for the HUS crimsonness.
Clinical signs of HUS, other symptoms of a thrombosis (oliguria, azotemia, hyperhydration and inn.)
It is necessary for a diagnostic test to be recommended until the detection of Shiga-toxin in the style of the polymerase lanceol reaction, for example, the determination of IgM-antibodies to lipopolysaccharides in Escherichia coli, as the production of Shiga-toxin is the main factor, which is
In case of development of a state of the art deficiency due to anuria, as well as uncorrected hyperhydration, electrolytic distribution and arterial hypertension It is recommended to recommend a negligible initiation of alternative therapy (peritoneal dialysis, hemodialysis, progression of venous-venous hemodynamics) in relation to the condition of the patient's hemodynamics (1B).
It is not recommended to prescribe antibiotics for the treatment of diarrhea in case of HUS due to the improvement of the youthful circulation of Shiga-toxin from the stimulated microbial cells (2B).
For the correction of severe anemia with symptoms of hypoxemia, transfusion of erythrocyte mass is recommended (1A).
Due to the blame of circulating bleeding, the introduction of platelet mass is not recommended, as it can lead to the strengthening of microthrombi (2B).
Children who have undergone HUS require trivial caution against the deterioration of the long-term inheritance of the chronic ailment.

Atypical hemolytic-uremic syndrome (Agus).

The appearance of an anterior diarrhea, a negative result of an admission to Shiga-toxin, a similar and recurrent nature of the disease, signs of the activation of an alternative to a complement, a multiorganism of an attack in the presence of a thrombotic Agus - complementopathic systemic pathology.
In case of aging on Agus, it is recommended to continue the activity of the ADAMTS13 factor for the onset of thrombotic thrombocytopenic purpura (1A).
Differential diagnostics of Agus slid is carried out with TMA in case of systemic cervonia in the presence of a number of drugs and methylmalonic aciduria (1B).
It is recommended that the level of antibodies to complement H (CFH) be continued for the prevention of the antibodies-mediated form of Agus (1B).
In the pathogenetic therapy of Agus, it is recommended to consume eculizumab, a monoclonal antibody to the C5 component of the complement, which blocks the distal part of the alternative pathway activation (1B).
If a high level of antibodies is detected to the official H, immunosuppressive therapy with rituximab can be administered (2B).
In case of uncomfortable cob drinking with ekulizumab, it is recommended to use plasma therapy in the presence of plasma exchange or transfusions of frozen plasma (2B).
For the purpose of triviality therapy with eculizumab, it is recommended to evaluate its effect on the basis of decile tests and to carry out molecular genetic monitoring for detecting mutations in the system's genes and complement: CFH, CFI, CFDB, MCP3, THB

Entry

Hemolithic-uremic syndrome is one of the most common reasons for the development of a hostile deficiency in children; characterized by a triad of symptoms: Coombs-negative hemolytic anemia with the manifestation of fragmented erythrocytes (schizocytes), thrombocytopenia and a guest nirkovy deficiency.

Appointments є warehouse thrombotic microangiopathy - generalized occlusion of sudinum with thrombus caliber, which was diagnosed as endothelium. As a result of the destruction of endothelial cells, there is a mechanical process of blood cells, activation of platelet aggregation from the formation of blood clots in the microcirculatory bed, especially in the cells.

Have children early vik in a large number of cases (90-95%), the so-called typical aboard postdiarrheal HUS (D + HUS), which is secondary in relation to Escherichia coli infection before infection, is the product of the so-called Shiga-coline toxin (Shigatox, E. STEC). Before the infectious stimuli to serve Shigel and pneumococci. The first form of HUS, called atypical, is developing on a higher level (5-10% of all types) and as a result of anomalies (partly genetic) of bins, which regulate the process of complement activation.

Typical post-arterial goose

D + HUS is the most common form of HUS in children. It is mainly used for up to 3 rockets and rarely - up to 6 months. Warehouse frequency ~ 2-3 drops per 10,000 children for up to 3 years.

pathogenesis

STEC-infection occurs in approximately 85% of D + HUS vials for an additional culture of a rectal smear in a sympathetic Mac Conkey with sorbitol. Most often it is detected with serotype є 0157: H7 (earlier O111, O103, 0121 and in.). The other variants of diagnostics of STEC-infection are the detection of the Shiga-toxin gene in the disease by the method of polymerase lanceyuga reaction, and, moreover, the detection of IgM-antibodies to lipopolysaccharides is often found in blood serum

The reservoir of infection is the intestines and feces of great thinness. Possible carriers can be also vivtsi, goats, horses, deer, kitties, dogs, birds and flies. Lyudin becomes infected with the implantation of rubbed yalovichin, unpasteurized unboiled milk, sir, fruits, juices, vegetables, contaminated water from a well and water, as well as with a malfunction in the water supply system. Direct contact of children with creatures or viporozhnennyy and transmission from humans to humans by the most serious dzherel infected.

D + HUS can be sporadic; in the case of infection from one and the same dzherel, manifestuvati occurs in a few days, or in siblings. Often, the members of the family may develop STEC-diarrhea without the development of the HUS.

Epidemiological diarrhea for hemorrhagic colitis as a result of STEC infection from one dzherel, which hunted hundreds of people, for example in the new lands, the frequency of HUS in the middle of them was 10-20%.

The pathogenetic link between intestinal contamination of STEC and HUS is generally clear. Microorganism to adhere to the villi of the mucous membrane of the intestine and vidilya Shiga-toxin. It is unclear, as the rank of Shiga-toxin is transferred from the intestine to its own name - endothelial clitin sudin. Transporters of Shiga-toxin can be polynuclear neutrophils, monocytes or thrombocytes. The toxin adheres to its receptor (globotriacylceramide, Gb3) on the endothelial cells of the nerves, the central nervous system (CNS) and other organs.

When the Gb3 link is active, a part of the Shiga-toxin penetrates into the clitin, which induces the synthesis of bilks, which, in its turn, produce clitin endothelium to death. Shiga toxin induction is also a cytokine product, which initiates a cascade of ignition and procoagulation pods.

key picture

In the prodromal phase D + HUS, diarrhea (in 90-95%), bloating (in 30-60%) and abdominal pain are indicated. In 70% of vipadkiv after 1-2 days, the ear will fall into shelter. Manifestation of the GUS will be repaired in the middle after 6 (2-14) days. Blindness of curvaceous curvatures, eccentricity, weakness, lethargy, changes in behavior, slight yellowness, a decrease in the number of chasms due to the "crooked" diarrhea of guilt of the drug's alertness.

The HUS is repaired with a rape and is characterized by a typical combination of signs.

Hemolytic anemia: the level of hemoglobin, as a rule,< 80 г/л, имеются шизоциты (2-10%); 70% пациентов нуждаются в трансфузии крови.
Thrombocytopenia (~ 50,000-70,000x10 * 9 / l) is not enough to develop, so that bleeding occurs during the daytime of surgical interventions, if some children have a hemoragic syndrome.
Leukocytosis is more than 20.0x10 * 9 / l in important HUS disorders, a common knowledge.
Gostra nirkova lack of supplementation of the level of syrovatous creatine and sechovine. Approximately half of the patients may have an important oliguria or anuria, 50-60% will be required by the state of the art.

With the manifestation of the sech, micro- or macrohematuria and proteinuria gradually begin. Oscillations of anuria are diagnosed due to a history, in patients with progressive hyperhydration, the first manifestations of HUS can be hyponatrial and hypervolemia with arterial hypertension. In some of the types of vials, water is indicated through diarrhea and blues. Rіven syrovatkovy kalіyu, which can be low through intestinal inflow, quickly move. Acidosis, hypocalcemia, hyperphosphatemia and hyperuricemia are often indicated.

extrarenally show

The lesion of the central nervous system, which is the main cause of death, is evident in about 20% of children: frequent symptoms are focal or generalized sudom, stridor, loss of svidomosty; Possibility of hemiparesthesia or hemiplegia, cortical slump, coma, inodi decerebration with brain stemburst lesions.

A collection of the results of a computer abnormal magnetic resonance scan can be normal in the absence of a decrease in proficiency. In the case of a contiguous and circulating ischemic lesion, it is more possible to renew the nervous system. Diffuse or localized necrotic changes in the brain can lead to death of any important neurological inheritance.

Serious striking shlunkovo-intestinal tract(~ 10%): important hemorrhagic colitis with persistent melenoy, blistering, bluetongue, low incidence rate; earlier toxic megacolon, invasion, perforation of the intestine, abnormal necrosis, secondary stenosis of the intestine.

Nabreak pidshlunkovaya zolozi during ultrasound pre-detection in the next period of amylase and lipase (~ 10% of patients). Ridko develop necrotizing pancreatitis. As a result of necrosis of acute cells, the development of transient abnormal permanent insulin red diabetes.

Liver damage (40%): appear hepatomegaly, due to the level of transaminases, and there is usually a good transition.

Heart failure (due to the blame of heart failure as a result of hypervolemia and hypertension) develop rarely (2%) and include myocardial ischemia with heart failure, arrhythmia, tamponitis and myocarditis a

forecast

Mortality in 2000 (In the main, as a result, the level of the central nervous system) was 1-5%.

Have a lot of vipadki in a stretch< 1-2 нед исчезают гемолитическая анемия и тромбоцитопения, нормализуется диурез. Несмотря на это, в среднем в течение 4 мес катамнестического наблюдения ~10% детей достигают терминальной хронической почечной недостаточности (ХПН). ХПН иногда развивается уже в острой стадии после транзиторного частичного улучшения функции почек. В дополнение у 25% детей отмечаются остаточные изменения почек: снижение клубочковой фильтрации (70-80 мл/1,73 м2 в мин), гипертензия или протеинурия.

The need for hemodialysis for more than 5 days, the triviality of oligoanuria is more than 10 days, the number of polynuclears is> 20.0 x 10 e / l, important> 20.0x10 intestinal accelerated... A large number of patients in the group will develop a terminal CHN in 20-30 years.

Vedennya ditini z D + GUS

In the region, it is necessary to promptly confirm the fact of STEC infection and the identification of the main signs of thrombotic microangiopathy, indicators of azotemia, electrolytes and basic vital parameters. When developing the oliguria, it is possible to transfer the possibility to the ear of dialogue.

Correction of water-electrolyte exchange

Necessary development of births from intercourse in case of hyperhydration and, navpaki, compensation for expenses due to stigma, blotching and sparing diuresis, as well as degradation of the body and deterioration of low-grade women. Signs of hyperhydration can be increased muscle mass, arterial hypertension, embryos, hyponatrієmiya.

Sprobi zastosuvannya temple furosemіdu doses (2-5 mg / kg) rіdko dozvolyayut dosyagti efekta so self yak i gіpotenzivna terapіya more peripheral vasodilators, to perevaga vіddaєtsya dіalіzu, Especially when nayavnostі virazhenoї gіperkalієmії i metabolіchnogo acidosis korektsіya yakih administration rozchinіv bіkarbonatu i Mauger glucose pogіrshiti hyperhydration.

liveliness

Harchuvannya as it is, like water and electricity, is more potent to be felt orally, if necessary - through a slurry probe. The number of calories and food must be 100% of the recommended additional consumption. The need for parenteral parenteral arthritis in the event of trivial vomiting and colitis symptoms.

Blood transfusion

Erythrocyte mass should be administered at a hemoglobin level lower than 70 g / l. For anti-HLA immunization, it is recommended to carry out transfusion through special filters (to detect leukocytes and thrombocytes). When there is an absence of serous bleeding and an indication before invasive visits (the installation of a central abnormal peritoneal catheter, abdominal surgery), there is no need for an injected platelet mass. Moreover, the introduction of thrombocytes can destroy the process of thrombosis.

dializ

The need for a visa to begin in the first place is evident because of oliguria. Dializ (call the peritoneal catheter for the help of the Tenckhoff catheter), it’s already possible to develop an accelerated state of disability.

accelerated therapy

Children with less neurological symptoms require careful and frequent pre-treatment, rarely in the case of intensive therapy: the dying may develop rapidly.

For baking your own prompt engagement in case of perforation / necrosis of the intestine or secondary stenosis in the patient, the brother is guilty of the fate of the surgeon. If a diabet is evident, insulin therapy is required. In children with cardiomegaly and heart failure, retreat monitoring of heart activity is recommended.

specific therapy

There is not a well-developed option of therapy, which is necessary for the transition of D + HUS. Heparin, thrombolytics and antiaggregants, steroids and frozen plasma (FFP) do not seem to be effective. In important cases, especially if the central nervous system is affected, carry out a piece of plasma transfusion (PPP). Methods є vividness of factors of throat, thrombosis and substitution for the help of FFP of potentially corny rhechovines, by the main antithrombin.

nirka transplantation

Rizik development of rotary D + HUS for transplantation of nirka from the day. It is necessary to discuss the possibility of transplantation from a living native donor. Cyclosporine is not contraindicated. At the end of the analysis, the problem is that of atypical HUS, if necessary, the path of molecular genetic diagnosis.

Zapobigannya STEC information and prevention of development of GUS

Slid know the fathers of young children with the rules of STEC contamination protection:

rubana yalovicina is guilty of buty good fried until it comes gray color on retail;
children up to 3 years are not guilty of living unpasteurized products (milk, sir, fruit juices);
before preparing a їzhі, especially when manipulating with a chopping yalovichin, it is necessary to wrinkle a hand;
children who hustled to the great horny thinness of those creatures who were guilty of turning their hands and hanging on to the letter, and also in front of the devil;
to prevent the contamination of meat by intestines, it is necessary to control the slaughter of thinness. An important, careful look and a look behind the water supply system;
antibiotics: numerical predictions have shown that antibiotic therapy during the period of development of HUS, possibly due to the development of Shiga-toxin as a result of bacterial lysis. Protein Rizik poki is not done. It is also necessary to clarify if antibiotics are indicated, as they do not undergo bacterial lysis, such as macrolide (azithromycin), patients with STEC-positive HUS.

HUS in the result of infection Shigella dysenteriae type 1

S.dysenteriae type 1, which is a product of Shiga-toxin, is the main cause of HUS in endemic regions, such as Bangladesh or Africa. The whole type of HUS is more important than STEC-HUS. In 20% of cases, bacteria are identified, often with the development of septic shock and internal vascular coagulation. The mortality rate is between 20-40%. In 40%, HNN develop, yak reaching a few rocks within the reach of the thermal stage. Early detection of antibiotics (cephalosporin of the 3rd generation or chinoloni) reduces the development of HUS in children infected with S. dysenteriae type 1.

HUS, secondary in relation to Streptococcus pneumoniae

They see a special form of HUS, which develops without the need for infection with S. pneumoniae (pneumonia and / or anemia and meningitis), mainly in children up to 2 years.

Mechanism of development of the center of the form of GUS specials. Neuraminidase S. pneumoniae attacks N-acetyl-neuraminic acid on the surface of clitin, which is heat resistant in cold weather T-antigen (criptantigen; Thomsen-denreich antigen) - a component of the cell membranes of the erythrocytes, thrombocytes, and clumps. In humans, natural antibodies to the T-antigen, which can lead to agglutination of the erythrocytes and start the processes, as a result, lead to the development of HUS. With pneumococcal infection positive test on T-activation, indicate about the advancement of the riser of the development of the GUS. The direct Coombs test is also positive. Mortality, in the main, is overwhelmed by meningitis, becoming ~ 10%. Інші 10% of patients quickly develop a thermal deficiency; 20% may be overwhelmed - impaired function, hypertension.

The introduction of plasmas and neotmitic erythrocytes is contraindicated, the odor of stench reveals anti-T IgM antibodies, which can provoke a relapse of HUS. Outside of robots to inform about the effectiveness of the ZPP with the albumen who have already been deputized.

atypical goose

Agus's name is not recognized. To be sure, Agus is a GUS without a concomitant ailment. Onset of ailments, hematological colitis, STEC infection, pneumococcal pneumonia, systemic chervonia, thrombotic thrombocytopenic purpura, recession of malnutrition in cobalamin metabolism, pathogenesis of cobalamin In the greater high school and traditional intelligence Agus - the HUS, the delay in the dysfunctional system and regulation of the complement with the uncontrolled activation of the alternative route.

Atypical option to become 5-10% of all types of HUS in children and in the main є weakened regulation of the complement system. Oremic changes in nemovlyat (methylmalonic acidemia) are the result of a decaying abnormality of the internal metabolism of cobalamin (Vitamin B12). According to the data of recent years, the breadth of Agus varies from 1 to 7 per 1,000,000 population.

pathogenesis

The complement system is the main factor in the formation of microorganisms. With normal regulation of activation, the complement is specifically straightened on the surface of the microbe, however, it is attached to the surface of the governor's cells. Upon activation of the complement, the C3bBb convertase is established, which can be produced before the conversion of C3 to C3b.

As a result, the deposition of C3b on the surface of microbes (opsonization) and the formation of a membrane-attacking complex (MAC, or C5b9) is seen, which can lead to the lysis of microbial cells. On the surface of the cell, the process is strictly controlled by regulators, including complementary factor H (CFH), factor I (CFI) and CD46, or membrane cofactor protein, non-circulating protein, and clotting (MCP) cellin. There are three officials, who are acting spiritedly, to secure the activation and deposit of C3b on the cells.

Mutations of cich bіlkіv produce up to the loss of endothelial clitins from the cinch products of activation of the complement, alternative to the way. In other words, the process of stifling the superfluous activity of the system and the complement will be disrupted, and it will lead to the realization of a great variety of Chinese products as an alternative route to the endothelium with the development of TMA.

Agus associates with CFH mutations in 20-25% of patients, MCP - 15% and CFI - 10%. Mutations of factor B (CFB) are detected in the edge (1%), at that time when the mutation of the C3 fraction is complementary - in 10% of patients. Mutations in the thrombomodulin gene (THBD). Approximately 10% of children may have mutations. For an additional 10% of children, there may be a functional deficiency of CFH due to the presence of anti-CFH antibodies. Only 30% sickness Agus does not know this year's proper explanation from the positions of molecular genetics.

diagnostics

Vyhodyachi from the name of Agus, for the statement of the diagnosis in the child of the guilty but also the reason for the development of TMA, in the first place D + GUS (postdirenal). In a number of vypadkov at the debut of Agus, diarrhea is also indicated, for that it is necessary to promptly receive STEC-infections, as well as S. pneumoniae infections.

On top of that, it turns out that systemic chervonia Vovchak, VIL-infection, infection with the H1N1 virus, due to overturned hypertension, HELLP-syndrome in the breed, intake of a drug

I especially respect those who have become ill with thrombotic thrombocytopenic purpura (TTP). It is necessary to monitor the level of the ADAMTS13 factor, which is indicative of the inactivation of the Willebrand factor in all patients with the TMA picture, the deficiency of rotations (ADAMTS13< 5% нормы) приводит к определенной форме ТТП. Клинически ТТП и ГУС имеют много сходного. У детей ТТП чаще носит врожденный характер и ассоциируется с наследственным полным дефицитом ADAMTS13. Приобретенные формы в результате наличия анти-ADAMTS антител у детей встречаются исключительно редко. Для ТТП характерно превалирование неврологической симптоматики при умеренном нарушении функции почек.

Oscillations of abundant mutations are more likely to lead to the destruction of the function of the protein systems and complement, not to the change in plasma concentration, the level of CFH, CFI, C3 and CFB may become less normal in the presence of mutations. The very same molecular-genetic analysis of the significant hour, and rejection of its results in the state of the phase of ill-health is practically unrealistic. At the same time, we are in the region in the process of guarding for ailments for the purpose of establishing a strategy for pre-city therapy.

Klinichny show

An even earlier ear (naval in the period of new birth) is typical for Agus, linked with CFH and CFI mutations (middle age 6 and 2 m / s, apparently).

Nawpaki, with the MCP mutation, you should be ready to repent for 1 fate of life. Options Agus with unidentified mutations can be seen in any business. Anti-CFH antibodies are often indicated closer to the mid-life period.

Deyakі іnfektsії (upper dikhalnyh nobles, Likhomanka, gastroenteritis) start the first episode of HUS and relapse, directly from the genetic variant, in 2/3 patients. Diarrhea of provocation Agus in 1/3 of the patients, who in one stage of accelerated differentiation from D + HUS (typical).

For 1/4 of the children, Agus has a family character (sibs, dads, grandmothers and didus may be ill). An uncomplicated family history does not include the possibility of genetic transmission of illness. Only half of the noses of mutation in the seven are extended by the manifestation of becoming ill.

forecast

In general, the prognosis of Agus is unpleasant. The mortality rate in the hospital stage becomes 5-10%. Approximately 50% of patients develop a terminal HNN, often with a stretch of 1 rock as a cob of manifestation. Extrarenaline manifestation, more often of the central nervous system (sudomi, coma, ischemic symptoms with magnetic resonance tomography), is more likely to occur in 20% of patients.

Relapses of Agus are seen in the first time, often in patients with the MCP mutation. To provoke infection in case of a mutation, to be guided by hemolysis, thrombocytopenia and severe deficiency as a result of hemoglobinuria. In a large number of these types of functions, there will be more and more innovations. For about an hour, there are recurrences from one time to another from deciduous age to bagatech rocky.

The most agreeable prognosis is indicated for MCP, the most unpleasant - for CFH and some mutations. In French pre-admission deaths are either terminal CHNI in terms of less than 1 year as an ear of disease in 60% with CFH mutation, in 37% with CFI mutation, in 33% with C3 mutation, in 60% with combined mutations, in 60% with combined uncommon ethology and 0% with MCP mutation. In ailments with anti-CFH antibodies, in the early stages of plasma exchange, the disease is highly susceptible.

lykuvannya

plasma

The introduction of FFP has been overwhelmed by the first line of therapy for a long time. For the sake of efficiency, according to the data of retrospective reports, it is not possible to develop a thermal HNN. FFP є a range of normal CFH, CFI, C3 and CFB, as well as a great number of other functional blocks. For additional plasma exchange, mutant CFH, CFI, C3, CFB and anti-CFH antibodies are seen. The early visualization of plasmas in STD caused hypervolemia and risk of heart failure as a result of the introduction of a great amount of FFP.

Plasma therapy is effective in most cases with CFH mutations. In case of MCP mutations, the efficiency is practically visible, as the blocks are expressed on cell membranes, so that in the solid phase, and not in the circulation.

We reduce є membrane plasmapheresis with a volume of substitution of 50-60 ml per kg of mass. An alternative option is the infusion of FFP in a total of 10-20 ml / kg.

Ekulizumab

Therapeutic approach to Agus bouv key practice ekulizumab - humanized monoclonal antibody to the C5-fraction of the thermal stage to the complement cascade. The drug is capable of splitting C5, until the pro-combustion C5a and prothrombotic C5b-9 components are approved, and thus assimilates the pathogenic effect. Until now, the results of the treatment with ekulizumab have been published for 189 patients from Agus; preparation of congestions in bagatokh regions, including in Russia.

Before the introduction of the drug, it is recommended for one-hour consumption of plasmapheresis, so that the last part of the drug was seen in the circulation. In case of individual concerns about the development of a meningococcal meningitis on aphids of administration of ekulizumab, it is recommended to pre-vaccinate and / or prevent the intake of antibiotics.

In the published results of the control of the disease, a rapid response to the activity of hemolysis was demonstrated (in the middle for 7-14 days) and a sign of active TMA in 88% of patients with hematological remission during the long course of the disease. The increase in the glomerular filtration rate in the middle by 32 ml / 1.73 m2 in the first half of the variety is indicated for the greatest variation in the case of an early cob of addiction. In children, the growth of the glomerular filtration rate will be larger (64 ml / 1.73 m2 in hv). In a number of vipadkіv they promoted more polіnshennya nirkovoі funktsії (stretching 2 rockіv). Most of the children took up the consumer in the dialogue.

In case of small groups of illnesses, who have been discarded or not discarded eculizumab, it seems that a significant difference in a certain period of time reached the thermal stage of HNN up to 1 period of caution (25 to 63%).

In case of pathogenetic priming of pre-treatment therapy in noses of mutations in gene systems and complement, the triviality of therapy with ekulizumab becomes controversial. In a single case, about the use of ekulizumab in 10 grown-up ailments in three cases of recurrent TMA with progressive decrease in nirk function.

adaptive therapy

With Agus options, mediated by antibodies to CFH, except for therapy with eculizumab, the effectiveness of such a patient, possibly immunosuppressive therapy. Described are the positive results of hematological changes and indicators of the function of niqs against the background of the pulse rate intake of cyclophosphamide in a dose of 0.5 g / 1.73 m2, corticosteroids and rituximab. A positive clinical dynamic is supportive of a decrease in the titer of antibodies to CFH. For additional therapy, the order with corticosteroids is taken into account mycophenolate mofetil.

prevention of infections

Most of the Agus epizodes are launched for additional infections, so that the need for the treatment of chronic diseases of adenoid, tonsillar and dental infections is explained. In some people with ailments, relapses occur due to vaccination. Protect the cinnamon immunization but rather prevail over the rizik. It is recommended to carry out all vaccinations, including prothyroid.

Nirka transplantation with aHUS

In the middle of children with the need for transplantation of nirka, as a result of the postponed HUS, a part of the ailing atypical option you can reach half. Rizik turned Agus, immediately after transplantation, is extremely high in patients with CFH mutations (~ 80%), CFI and C3 (> 50%). Transplantation was carried out in all 3 patients with CFB mutation: all of them lost nirks as a result of the revitalizing Agus.

Oscillations of the transplanted nirka do not take revenge on the muddled MCP-block, the degree of turnover of Agus with cych mutations is even small. Most of the ailments when turning Agus will consume nirki mensh nіzh after 1 rіk. Another reason for the loss of nirka in children is thrombosis.

It is only in the distance of the lonely patients that the Agus Shlyakhom turned away from them to carry out ZPP sessions before the operation and in the post-transplant period. Greater optimistic prospects of tying with the vicars of ekulizumab in the pre-and during the operational period transplantation. Є Often about 13 patients with the second anterior transplant, for those who received eculizumab a few years before transplantation and for 24 years, there was a step-by-step transition to the standard mode of treatment.

Oskilki CFH, so itself, such as CFI, CFB and C3, are synthesized in the liver, combined liver transplantation and liver transplantation, in order to preserve the function of the therapy, it is possible to see such options. However, the previous results showed that Danish method to sacrifice for the effectiveness of the storage of ekulizumab.

visnovok

Hemolithic-uremic syndrome is a serious therapeutic problem in pediatric and childish nephrology, being one of the provincial causes of hospitable deficiency with a potential transformation into thermal chronicity of deficiency

Unimportant for those who have expanded the STEC-associative form of HUS with a typical prodrome diarrhea, it is necessary to confirm an infectious etiology in order to maximize the early term of pneumonia.

Incidentally, STEC-HUS (typical) is recommended to provide adequate symptomatic therapy for free dialysis if necessary. The prognosis is mainly due to the triviality of the anuric period and the supportive ears of the central nervous system.

Atypical HUS is most often in the basis of gene mutation, which can lead to dysfunction of the complement cascade with uncontrolled activation of the alternative path. In case of an unpleasant, unpleasant prognosis of the schoolchildren before relapse, the form is promising є treatment with ekulizumab, which blocks the thermal components of the complement cascade.

A.N. Tsigin, T.V. Vashurin, T.V. Margina, P.V. Ananyin,

A.M. Mazo, A.A. Pushkov, K.V. Savostyanov

>> Sergiy Baiko, Associate Professor of the 1st Department of Children's Ailments BDMU, Candidate of Medical Sciences sciences.

Hemolytic-uremic syndrome (HUS) is the most common cause of gastrointestinal disability (GNN) in children of early age. It is common for the Republican Center for Child Nephrology and Nirkovy Nephrology and Nephrology to come from 20 to 30 patients with such a pathology, 75% of them will require some kind of Nirk therapy (RPT).

HUS - clinical and laboratory symptom complex, including microangiopathic hemolytic anemia, thrombocytopenia and gostre nirkovy poshkojennya(OPP).

The trigger factor for the development of sickness is most often Escherichia coli, which produces a shig-like toxin (Stx), a typical manifestation of sickness - diarrhea (HUS D +), often bloody in nature. In 10-15% of cases, HUS can occur without diare (HUS D-). ARF is observed in 55-70% of cases. Dzherela infected people shigatoxin-producing E. coli (STEC) - milk, meat, water; also not safe contacts with the infected creatures, people and their visions.

HUS is considered to be thrombotic microangiopathy, which is characterized by thrombosis of sudin nirok. The current classification (div. Table 1) viklyucha the witness GUS D + і D-, and to avenge the options in the fallowness of the cause of the disease: typical (tGUS), atypical (Agus), viklicanii Streptococcus pneumoniae) (SPA-HUS).

When a child enters the hospital and before the etiological cause of HUS is revealed, it is possible to use the terms GUS D + and D-. However, in the falsified need, a more precise version of the GUS is required: STEC-GUS, SPA-GUS, etc.

The most common form of the middle of all variants of HUS (90-95% of the HUS) is tGUS, in the association with diarrhea and shigatoxin Enterohemorrhagic strains of E. coli (STEC-HUS), later type - zeigella

HUS, but not associations with diarrhea and shigatoxin, including a heterogeneous group of patients, in some ethiologically unassigned infections, vicious bacteria, but eliminating toxicity. Prepare for options:

SPA-HUS - to respond to Streptococcus pneumoniae, which produces neuraminidase;
atypical HUS - accumulation of genetic defects in the protein systems and complement (factor H (CFH), I (CFI), B (CFB), membrane cofactor protein (MCP), thrombomodulin (THBD), fraction C3 to complement) or even up to the antibody H (CFHR 1/3));
Secondary HUS - may supervodzhuvati systemic chervonia vovchak, scleroderma, antiphospholipid syndrome; to develop in the presence of anti-inflammatory, antiplatelet drugs, immunodepressants;
cobalamin Z deficient HUS (methylmalonic aciduria).

The CLINICAL CLASSIFICATION OF THE GUS is based on the significance of the severity of the disease:

easy feet - three symptoms (anemia, thrombocytopenia, AKI) without any deterioration in the severity;

middle feet - the same class, accelerated by convulsive syndrome and (abo) arterial hypertension, without deterioration of the severity;
the importance of steps is a triad in the case of an oligo-anuric (or without it), if you need some kind of therapy; triad on aphids of oligoanuria with arterial hypertension and (abo) convulsive syndrome, as a result of the diagnosis.

Manifestation of a typical HUS is mainly used from 6 months to 5 years. With atypical malaise early ear(You can navigate in the period of new pregnancy), linked to mutations in the CFH and CFI genes (the average for the first time I develop - 6 months and 2 months, respectively). In case of gene mutation, MCP code, GUS start the debut of rock.

In the provinces of Pivnichnaya America and Western Europe, STEC-HUS in 50-70% of vypadkiv - inheritance of infection E. coli, serotype O157: H7.

Volodya is a unique biochemical power (the fermentation rate of sor-Bitol), which allows easy identification of this species of fecal E. coli. Large-scale serotypes of E. coli (O111: H8; O103: H2; O121; O145; O104: H4; O26 and O113) also use STEC-HUS. In the powers of Asia and Africa, the main cause of HUS is Shigella dysenteriae, serotype I.

For the last 10 years in Bilorus, there were no types of HUS, shigella dysenteriae, serotype I.

For contact with enterohemorrhagic E. coli, 38-61% of patients develop hemorrhagic colitis and 10-15% inferior HUS. Indigenous incidence of STEC-GUS in the regions of Europe: 1.71 drop per 100,000 children up to 5 years old and 0.71 - younger than 15 years old in Nimechchyna; 2 and 0.7 inc. In the Netherlands; 4.3 and 1.8 in Belgium; 0.75 і 0.28 in Italy.

The prevalence of HUS in Bilorus is one of the most popular in Europe: in the middle of the year there are 4 drops (from 2.7 to 5.3) per 100,000 of the child population in those up to 5 years and 1.5 (1-2) younger than 15 years. Naybilsha number of ailments to reestablish in the Vitebsk, Grodno regions and Minsk; the least - in Brest and Gomel. The peak is meant in the warm season of the rock (grass - serpen).

KLINICHNA PICTURE

STEC-HUS is characterized by the presence of a prodromal period in viglyadi diarea. The average period of an hour between E. coli infections and illnesses is three add-ons (from one to eight). To fix, as a rule, pains in the abdomen and non-bloody diarrhea. Stretching for 1-2 days in 45-60% of cases the style becomes bloody. Bloating occurs in 30-60% of cases, fever in 30%, leukocytosis occurs in the blood. X-ray dosage from the bar of the clasm allows you to paint a picture of "finger beats", as well as on the embankment and blood in the pid-mucous ball, especially in the area of the upper and lateral and lateral colon... Arterial hypertension in the state of the HUS period (occurs in 72% of cases) is linked to the hyperhydration and activation of the renin-angiotensin-aldosterone system, the

Factor of increased risk of development of HUS due to infection, vicious E. Coli: bloody diarrhea, fever, bloating, leukocytosis, as well as extreme diseases of the group, gut, intestinal fever, antibiotics. STEC-HUS is not tolerated to good illnesses - 50-75% of patients develop oligoanuria, dialis is required, in 95% of cases the erythrocytic mass is overflowed, and in 25% of cases it is considered nervous. Oscillations are dia-liz available and in the center of intensive therapy, the mortality rate of the middle and young children has decreased. However, up to 5% of patients die in the GUS state phase.

In Bilorus, over the last ten years, the mortality rate from STEC-GUS has changed from 29.1 (1994-2003) to 2.3% (2005-2014). HUS, when S. dysenteriae starts up, may even accelerate bacteremia and septic shock, systemic internal vascular laryngeal larynx and host necrosis of the cortical ball. In such situations, the mortality of the temporalis (up to 30%).

Infectious diseases that cause Streptococcus pneumoniae are associated with 40% of HUS types that are not associated with shigatoxin, and 4.7% of all HUS infections in children in the United States. Neyramіnіdazu, utvorena bakterіyami S. pneumoniae, vidalyayuchi sіalovі acid of klіtinnih membrane antigen ogolyuє Thomsen - Frіdenrayha, pіddayuchi Yogo vplivu tsirkulyuyuchih іmunoglobulіnіv M. Away zv'yazuvannya ostannіh of CIM novim antigen on platelets i endotelіalnih klіtinah generally produces up agregatsії trombotsitіv i poshkodzhennya endotelіyu. To become ill with a serious problem, supervise respiratory distress syndrome, neurological impairment and coma; lethality reachє 50%.

The most frequent remedies for the disease are secondary HUS antiuchlinic (mitomycin, cisplatin, bleomycin and hemcitabin), immunotherapy (cyclosporine, tacrolimus, Zhovtarnitin) and antithrombocyte Rizik the development of the GUS was made to become 2-10%. An ear of cob is swallowed up, through the rik of a cob of therapy. The prognosis of unpleasantness, mortality within 4 months reaches 75%.

The literature describes the types of post-transplant HUS. Possibly yogo belly in patients, nicholas previously could not tolerate the disease (de novo), for some wines were the primary cause of the thermal nircum deficiency (recurrent post-transplant HUS). Launch a post-transplant HUS, which is de novo vinik, can induce calcineurin in or out of a humoral type (C4b positive). Qia form of HUS for transplantation of nirka vinikє in 5-15% of patients receiving cyclosporine A, and approximately 1% of patients receiving tacrolimus.

HUS on the basis of vaginosti inodi develop yak accelerated preclamps. In the case of children who are not threatening, they suffer from severe thrombocytopenia, microangiopathic hemolytic anemia, nyrcological deficiency and liver damage (HELLP syndrome). In such situations, it is shown that there is an extraordinary development - followed by a follow up of remission.

Pislyarodovy HUS is mainly manifested in a stretch of 3 months during the canopy. Viciously unpleasant, mortality becomes 50-60%.

For atypical HUS, mumbled with genetic defects in the system and complement, there are three main signs, which are superfluous and recurrent. The qi form can be sporadically chi family (the disease is denoted more by one member of the family and the Stx injection is enabled). The prognosis for Agus is unpleasant: 50% of cases against the development of a thermal deficiency or a non-vascular brain disorder, mortality in the hospital phase reaches 25%.

LABORATORY DIAGNOSTICS I CRITERIA microangiopathic hemolysis in HUS is characterized by:

decrease in hemoglobin and haptoglobin values;
the development of lactate dehydrogenase (LDH), viral to hemoglobin plasma and bilirubin (importantly indirect), reticulocytes;
the appearance of schizocytosis in the peripheral blood (more than 1%),
negative reaction of Coombs (the presence of anti-arthrocytic antibodies).

Thrombocytopenia is diagnosed when the number of platelets in the peripheral blood is less than 150109 / l. Decrease in platelet level by 25% more as a result of the outgoing (to navigate in the boundaries of the current norms) to inform about the changes in the current and the display of the HUS development.

The level of syrovatkovogo creatinine, rosrahunkovy, the glomerular filtration rate allows for the presence of the stage of AKI (div. Table 2).

* For calculating the rosrahunkovy SCF, the Schwartz formula is used.

** If there is a daily amount of creatine for the assessment of the child's adjustment, it is possible to set the upper limit for the normal child.

*** In children under 1 year of age, oliguria starts with a decrease in fluidity of less than 1 ml / kg / year.

To detect the transition of prerenal AKI to the renal or first stage in the other, start with equal neutrophilic gelatinized lipokaline (NGAL) in the blood and (abo) section. The NGAL adjustment step reflects the severity of the OPP.

An early marker of decreased glomerular filtration rate - cystatina Z in blood.

Diagnosis of "STEC-HUS" is confirmed by the presence of E. coli in cultures of kalu ditini (for diagnostics of E. coli O157, it is determined in the middle of sor-Bitola). Antigens of E. coli O157 and shigatoxin are revealed by the method of polymerase lanceol reaction in vials.

For approval infectious nature HUS should be tested for serological tests for antibodies to shigatoxin or to lipopolysaccharide Enterohemorrhagic E. coli strains. Early diagnosis of the transmission of express tests for the detection of E. coli O157: H7 antigens and shigatoxin in the style.

For viklyuchennya sepsis, C-reactive block, procalcitonin, blood presepsin are used.

All patients need to read the C3 and C4-fraction to complement the blood for the assessment of the turnover and the path of activation, and in some cases - to confirm the atypical interruption of the HUS.

As a child with HUS, there is no diarrhea in the prodromal period;

To get sick in reality, for earlier transferring a disease, as most often

S. pneumoniae: pneumonia, otitis media, meningitis. For identification of the originator, cultural pre-detection of blood, liquor and (abo) express diagnostics of S. pneumoniae antigens in the section is carried out.

In patients with HUS, who may have neurological symptoms (sudominal syndrome, diagnosis of symptoms, coma), for the prevention of thrombotic thrombocytopenic purpura (TTP), assess the activity of blood metal, ADF. TTP is characterized by neurological symptoms, low platelet count (30x109 / l), daytime or azotemia (blood creatine not more than 150-200 micromol / l), fever, decreased activity of 13% ADAMTS-before

Development of the symptom complex of HUS in nonmovlya up to 6 months in the absence of methylmalonic aciduria. When a pathology is suspected, an analysis of amino acids is carried out - isoleucine, valin, methionin and threonin; the change in acylcarnitine and homocysteine in the patient's blood, nircysteine homocysteine and organic acids - methylmalonic, 3-hydroxypropionic, 3-hydroxy-n-valeric, propionic, propionic. Molecular genetic prediction of a susceptible diagnosis, when mutations appear in the genes MUT, MMAA, MMAB, MMAASNS, MMADHC, MCEE.

A change in diagnostic procedures for setting up a diagnosis of "HUS", including the basic procedures, which in most cases should be completed for verification of the diagnosis, and additional information, is necessary in case of urgent problems.

Main news:

zagalny blood analysis (number of thrombocytes, leukocyte formula, SHOE - if possible with a podrakhunkom vіdsotka shіzotsіti);
acid-lining mill;
biochemistry of blood (it starts with equal amount of blood, albumin, creatine, sechovine, alanine transferase, aspartate aminotransferase, LDH, blood calcium direct, glucose, calcium, sodium
zagalny analysis of the slaughter (if it is obvious);
coagulogram;
the value of the blood group (according to AB0 systems) and the Rh factor;
direct Coombs' test (anti-rithrocytic antibodies);
detection of shigatoxin (first and other type) and antigens of E. coli O157 and (abo) detection of shigatoxin cultures, not to replace E. coli, on special mediums (with coli in O157 for E. coli) їх DNA in samples viporozhnen;
analysis of feces for pathogenic intestinal flora;
Ultrasound nirok i sectional mikhura.

Dodatkovі doslіdzhennya:

in case of biochemical analysis - pre-development of cystatin C, haptoglobin, procalcitonin, presepsin;
for coagulograms - detection of rodent fibrin-monomer complexes, D-dimers;
the value of the blood complement system and blood complement - C3 and C4;
pre-detection of factors H, I, MCP (CD46) in the blood;
enumeration of blood homocysteine levels, methylmalonic acid (blood and blood) ± molecular genetic predictions for detecting mutations in the MMACHC gene;
control of NGAL blood and blood vessels;
test for vaginism (next to the robustness of all women-nurses with a GUS or TTP class);
the value of the activity of ADAMTS-13 and antibodies to ADAMTS-13 in the blood;
antibodies to shigatoxinu and (abo) lipopolysaccharide STEC in syrovatz blood after 7-14 days from the ear of diarrhea (again - after 7-10 days);
the value of autoantitils to factor H in the blood;
molecular-genetic dosage for the detection of mutations of genes, for the code of the complex and complement systems;
Ultrasonic examination of a nirok with an assessment of a nirk blood flow and a section of a slit microorganism.

Indicators that are allowed to conduct differential diagnostics, Reinsured in tables 3.

The lock of the successful management of children with HUS is an early diagnosis of sickness and an early ear of a child's liking.

lykuvannya

Not a simple therapy of TGUS with an improved efficiency. Pid hour of the guest phase is required on a weekly basis. The complex of drug treatments includes ethiotropic, syndromic, pathogenetic and alternate nirk therapy.

The lock of the successful management of children from HUS is an early diagnosis of sickness and an early ear of a child's liking. Vono symptomatic, straightened on zapobіgannya accelerated from the side of the shlunkovo-intestinal tract (bends pain syndrome, Colitis), blood systems and hemostasis (anemia, thrombocytopenia, rhizic bleeding), court system(Hypervolemia, arterial hypertension, increased permeability / filling) and renal (disruption of the water-electrolyte balance and acid-water balance, intoxication with nitrogen metabolism products). In order to be respected, it is necessary to come to the other organs, as well as to eat, psychological education of the child.

Children with HUS without a decrease in dyuresis can be promoted in nephrological or pediatric education. In case of a decrease in the degree of malignancy to oligoanuria - in the form of intensive therapy and reanimation.

Infusion therapy (not important physiological breakdown) When STEC-infection is confirmed for the first 4 days, a cob of diarrhea decreases the development of oligoanuria, rather than the HUS itself. Infusion therapy is useful and necessary for the preparation of the patient. The water balance is closely monitored: vimiryuvati vagu 1-2 times a day, control of the proper and vivedennya of the skin organism 6-12 years, the frequency of the heart and arterial tension of the skin 1-3 years. The re-engagement of the life of critical patients is an independent factor in the risk of death.

Bile in the stomach is often a superfood GUS D +, especially when it is against colitis. Bazhano unique signs of antiperistaltic and non-steroidal anti-pyretic agents - acetaminophen (paracetamol). In all vipads stubborn belly with HUS, it is necessary to diagnose surgical pathology.

Indicated before transfusion of the erythrocytic mass, or if the erythrocytes appear - hemoglobin up to 70 g / l, or even more, with the most important manifestations of anemia (tachycardia, orthostatic hypotension, congestive heart disease) Platelet oil is infused with trivial bleeding. It is indicated before the transfusion of the thromboconcentrate. Most of the authors are unique in the designation of such transfusions, allowing the stench to supplement the aggregation and thrombotic treatment, and by themselves, they were overwhelmed by the disease. For the prevention of bleeding, thrombocyte infusion with a decrease in blood levels of less than 20109 / l, or before surgical interventions. In the current hour, there is no transfusion of fresh frozen plasmas in the context of additional therapy with STEC-HUS, however, it cannot be done without it if a child is needed to the hospital due to the development of uremic coagulopathy.

Plasma of an older person is contraindicated in patients with HUS, S. pneumoniae viclikanim, fragments of plasma to avenge the antigen against the Thomsen-Fridenreich antigen, good health was lost.

According to Cochrane's tribute, there was no evidence of a rejection of glucocorticosteroids, heparin, dopamine in nirkovy doses for HUS - the names of drugs were included in the HUS treatment protocols.

For the drive of the one who needs antibiotics in the fight against infection, the STEC, is still mute. Wong and the authors have shown that at the stage of gastrointestinal infection STEC, antibiotic therapy increases (approximately 17 times) the development of HUS. І we made a breakdown: the membrane of bacteria was reduced, induced by an antibiotic, it was possible to drink welcome toxin in great quantities. According to the international protocols for the treatment of intestinal infections, it is indicated before antibacterial therapy is prescribed - invasive diarea (hemcolite and in.). When developing HUS, practically all patients require antibiotics for treatment and prophylaxis.

The majority of children with HUS have arterial hypertension of a low degree of severity - a correction is required.

It is indicated for the ear of a substitute nirkovy therapy in children with HUS as well as with other options for a nirkovoi deficiency (GNN). Absolute - anuria stretching for 12-24 years, oliguria more dobi.

For children with a nirkovo deficiency and adequate diuresis on the first plan, this is shown before OST:

1) development of life-threatening stanіv, but conservative therapy is not supported:
hyperhydration with a lot of legends, the brain and is resistant to the introduction of furosemide;
hypercalcium (> 6.0 mmol / l with EKG signs);
uremic encephalopathy;
malignant arterial hypertension;

2) metabolic diseases, which are not absorbed by conservative therapy:
important metabolic acidosis (pH<7,2; ВЕ<-10);
hypo- and hypernatrium (<120 ммоль/л и >160 mmol / L);
sečovina rіven> 40 mmol / l (in newborns> 30 mmol / l);
the increase in blood creatine in 3 levels of the species rivnya (upper range of normal norms) or more than 353.6 μmol / l.

The prognosis of life and normal vision of the patient's head is early dialogue: there is a direct fallowness between the term and the ear and the result. In most centers, the most frequent method of RRT in children with HUS is peritoneal dialysis.

Briefly about HUS therapy - in table 4.

Practice vip

Patient K., 2 rocky. Sickness began to fall from the day: the first day - up to 10 times, the other - up to 20. Poor style, with mucus, strong pain in the belly. Disposable bloat, the temperature did not move. Mati gave baby stopdiar (nifuroxazide) 1 tsp 4 times a day.

Hospitalization to the district hospital.

Zagalny blood analysis: hemoglobin 100 g / l, leukocyte 13.6109 / l, zsuv leukocyte formula alone, thrombocyte 48109 / l. Biochemical blood analysis: creatinine 144 μmol / l, sechovina 11.8 mmol / l. Zagalny analysis of the section: block 3.8 g / l, glucose nemaє, erythrocytes 15-20 / 1.

They carried out infusion therapy, prescribed sodium etamzylate, metoclopramide, cefotaxime, Enterohermina, stopdiar. The frequency of the style changed up to 5 times per day, the bluetongue did not recur, the temperature was normal.

Mom washed the baby's diaper from the night dry, the boy became mlyavim, before the temperature the temperature had moved to subfebrile and streaks of blood appeared in the style. I will look at the development of oligoanuria, as well as the growth of the level of age and creatine for the prevention of anemia and thrombocytopenia, children with the diagnosis "HUS D +, OPN, oligoanuristic stage" of translations in the course of the day.

Stan with proper importance. In witness. Shkіrni pokrivi blіdі, clean, light pasty povіk. AT 132/86. Style 1 time, rare without pathological houses. When catheterization of the cuticle with a Foley catheter 8F, the cut is not felt.

Hemoglobin 70 g / l, leukocyte 18109 / l, zsuv leukocyte formula vlovo, thrombocytes 69109 / l, anisocytosis 2+, amount 2+, hypochromia 2+, important metabolic acidosis, extracellular blood 40 g / l, albumin g / L, creatinine 333.6 mmol / L, Secovina 23.6 mmol / L. Advancing ALAT - 1.8 times, AsAT - 2 times, lactate dehydrogenase - 5 times higher than normal. Electrolytes in the norm, C-reactive block 24 mg / l, in the coagulogram of increased levels of fibrin-monomial complexes in 3.4 times. The test for antierythrocytic antibodies (direct Coombs' test) is negative. The level of the C3 fraction for complement is reduced - 0.56 g / l (the norm is 0.9-1.8 g / l) with a normal level of C4 - 0.18 g / l (0.1-0.4 g / l).

An express test of feces for first and other types of verotoxin is positive, for E. coli O157: H7 antigen - negative.

The ultrasound scan revealed an increase in both nirok, diffuse changes in parenchyma, and an increase in intrarenal blood flow. Beruchs to respect the visualization of indicators, displaying the diagnosis "STEC-HUS, an important step V. OPN, anuric stage." I will look at anuria more than dobi, a substitute nirkova therapy is shown, a vikoristovuvati hemodializ is shown.

After an hour of hospitalization, the child has acquired arterial hypertension, which is why antihypertensive drugs (amlodipin, enalapril, metoprolol) have been detected. Three were transfused with erythrocytic mass, filled with leukocytes, and three - albumin. At the moment of the hopefulness and stretching of the offensive 4 days, infusion therapy, Scho included a part of parenteral feeding(Glucose, amino acids). Antibacterial therapy was also carried out for the first time to the center - cefotaxime in 50% of the standard dose (12 days).

Until the moment of vipiska, the patient had normalized anemia (hemoglobin - 105 g / l), there were few changes in the zebra test (1-8 / l) - 0.16 / l - 0.161 and arterial hypertension, for the control of what is required by taking three antihypertensive drugs: amlodipin, enalapril and metoprolol.

Pomilka fell in the fact that the dads became lіkuvati child with a stopdіar (Nifuroxazide), if there was no show for them.

Before the medicine became ruined by E. coli O157, there was a large amount of verotoxin, which inoculated to necrosis of enterocytes (blood in the feces), and if soaked into the blood - before the thrombosis of the endothelium

Only in 10% of children infected with Enterohemorrhagic E. coli, HUS develops, and the factor that takes them away is viciousness of antibacterial and antiperistaltic diseases.

Patient D., 6.5 rock. 16 of the fierce dad turned into a child of infectious disease with scars for the weakness of the child, a decrease in appetite, and an increase in stamina by a stretch of a child. Remained 3 days in vrance, the blues were pushed back, the cheeks appeared. Behind the words of the grown-ups, stretching out 2 types, D. did not have symptoms of GRI or GKI. Pislya otrimannya laboratory tribute to hepatitis.

Zagalny blood analysis: hemoglobin 73 g / l, leukocyte 5.2109 / l, thrombocyte 30109 / l, anisocytosis 2+, number 1+. Biochemical blood analysis: creatinine 109 μmol / l, sechovina 24 mmol / l, ALT, AST in between the norms, bilirubin zagalny 36 μmol / l, direct 8.5 μmol / l. Zagalny analysis of the section: the number of storms, block 2.8 g / l, leukocytes 2-3 / 1, erithrocytes 2-3 / 1.

Ditin demanding stimulation of duresis with furosemide (titration up to 5 mg / kg / dob) to provide an adequate dose. With the diagnosis “Hemolytic anemia. GUS? »Patient of transfers in the Republican Scientific and Practical Center for Children's Oncology, Hematology and Immunology.

Zberigalasya an important stan, dobovy dyuresis on a few lower doses of furosemide up to 10 mg / kg / dobu step by step lowering.
Zagalny blood analysis: hemoglobin 59 g / l, reticulocytes 28 ‰, leukocytes 6.4109 / l, thrombocytes 21109 / l, mild poikilocytosis for shizocytosis (close to 10%). Biochemical blood analysis: creatinine 188 mmol / l, sechovina 26 mmol / l.

Direct and indirect Coombs probes are negative. Zagalny analysis of the section: bilk 1.82 g / l, leukocytes 2-4 / 1, erythrocytes 10-15 / 1. For carrying out a different kind of therapy with the diagnosis "HUS", the boy of transfers to the children's diaryzny center.

In case of a proper stance, it is important, of anemia, intoxication through azotemia, impaired water (oliguria) and electrolyte balance, arterial hypertension (AT 165/110).

Hemoglobin 58 g / l, leukocyte 9.7109 / l, platelets 62109 / l, creatinine 205 μmol / l, sechovina 39 mmol / l, LDH 7.3 times higher than the norm, calories 5.4 mmol / l , block 1.94 g / l, erythrocytes 4-6 / 1. Test for anti-rithrocyte antibodies negative. Express test of feces for verotoxin of the first and other types, for E. coli O157 antigen: H7 negative. Diagnosis "HUS D - severe stage V. OPN, oligoanuric stage". Razpochato zasna nirkova therapy - hemodialis. The punctuation of nephrobiopsy was carried out: a picture typical for HUS, signs of mesangiocapilary glomerulonephritis.

Hemodialysis trivial until 22 birch, if there was a positive dynamics - normalization of duresis, decreased azotemia indicators (creatinine 121 μmol / l, sechovina 18 mmol / l). Let us take into account the significant increase in azotemia and the decrease in the child's deiurez from the transfer to hemodialysis. A low level of C3-fraction for complement (0.65 g / l) is saved, with a full C4 in the norm - 0.39 g / l (after 2 months after the debut becomes ill). Re-adjustment of LDH was indicated (25 birch - 826 U / l, 13 April - 1 332.2; norm - less than 764). Mayzhe misyats having trimmed glucocorticoids - 0.5 mg / kg prednisolone.

Diagnosis of "atypical HUS, an important stage V. OPN, oligoanuric stage." Plasma therapy was assigned for the donation of defective complement factors, deficiency of such sprias, microthrombosis of nirkovy sudins. Oskilki child having undergone hemodialysis and an excess of half-life of plasma, it is decided to switch to infusion of fresh frozen plasmas (1st day - 20 ml / kg, 2-14th day - 10 ml / kg) and not exchange of plasma. The result with such therapy did not reach into the distance, the child and earlier became dializozavisimim.

16 herbal diagnosed with a thermal nirkov deficiency as a result of Agus. 27 peritoneal catheter implantations, 3 worm transfers to automatic peritoneal dialysis.

Vrantzi 2 worms the patient doesn’t go out of the way, skarzhivya for weakness in livіy rutsі and nosі, ruining the zoru. A neurologist's consultation was urgently organized, a CT scan of the brain was performed. A neurologist's note: encephalopathy of a decreased genesis with the manifestation of cerebral neurological symptoms (right-handed ptosis, anisocoria, right-handed paresis of the facial nerve, loosened hemiparesis) in the presence of severe cerebral palsy The emergence of neurological symptoms is characteristic of Agus.

In a frivolous child of writing, outpatiently removing a low automatic peritoneal dialysis.

Diagnosis "Agus" transferring a high risk of turning sickness into the graft (from 30 to 100%). Placing a nirk graft on a sheet is not worthwhile without a molecular genetic diagnosis, which is called to identify defective genes, which code a number of complex systems and complement. When defective genes are detected, the factor H is encoded or the factor is synthesized in the furnace (div. C9) complement, - eculizumab).
Even though such a possibility cannot be transferred, turning the disease into the next month for transplantation, the transplant is not practical.

Likars made a number of efforts, organizing molecular genetic analysis at the Institute of Pharmacological Dosages of Mario Negri (Bergamo, Italy), and they were rather hard at work on the Agus problem. It took 3 months to complete all complement factors.

Mutations of genes that encode the main cells of the system and complement, which are not revealed, but are not confirmed by low levels of C3-fraction for complement. The eyes of the patient's blood are shown to be victorious for those who have given up on a joke, for those who are not homeless factors, but take a part in the pathogenesis of Agus.

Tsey visnovok allowed 11 zhovtnya to put the patient on the list of nirkovo transplant, and 8 breast of the offensive rock yom successfully viconan transplantation of donor nirka. Already on the verge of 2.5, the graft function is becoming more important.

- tse superstitiously ridkisne (Orfa) fell ill, tied z. Behind the MKB-10 system, yak D 59.3

Atypical hemolytic-uremic syndrome (Agus). Forecast. Reasons.

Unfortunately, the prognosis of becoming ill, most often, unpleasant. Atypical hemolytic-uremic syndrome (Agus) - tse I will load my life chronic ailment, It is characterized by multisystem lesions of organs and tissues. Poisoning is approximately the same for children and for grown-ups. Thrombotic microangiopathy (TMA) is at the heart of the disease, with thrombi in small blood vessels. Through the price of bloodstending, and the organization of suffering.

The cause of the ailment is that it is in the complement system, and the activity is still active in order to seize the organism, to start ruinuvati. Control the level of activity of a particular child, with atypical hemolytic-uremic syndrome (Agus), the function is significantly impaired, and the process goes under control.

Chim is more trivially toxic and aggressive on organisms, with larger teeth there are traces: nirki grows, develop an insult or an infarction.

Apparently, when early diagnosis In case of malignancy at the cob stages, it is possible to reduce the dental infusion of atypical hemolytic-uremic syndrome (Agus), and by itself give people a chance for a normal life.

Atypical hemolytic-uremic syndrome (Agus). Decline.

Atypical hemolytic-uremic syndrome (Agus) lethargic only in 20% of cases, with an autosomal recessive or dominant type of transmission. In approximately half of the cases, the genetic mutation does not occur. To that, DNA - analysis in atypical hemolytic-uremic syndrome (Agus) is not a very useful diagnostic method;

Atypical hemolytic-uremic syndrome (Agus) is, however, widening all over the planet, there is no evidence of fallowness from the stati, but to racial patients. Through the afflictions of ailment, it is easy to talk about the exact number of patients, є about those who develop ailments from about 1 to 9 per million people.

Atypical hemolytic-uremic syndrome (Agus). Show clear.

Symptoms of atopic hemolytic-uremic syndrome (Agus) occur all at once, as well as okremo. Alert maє be-yak z key manifestations ailments.

Otzhe, symptoms of atypical hemolytic-uremic syndrome (Agus) є:

Post-vtoma and nezduzhannya;
Urazhennya nirok right up to the need for hemodialysis through a thermal nirkova deficiency;
Nabryaki, importance at the feet;
Decrease in dyuresu;
Blood creatine adjustment;
Decreased glomerular filtration rate;
Arterial hypertension;
Post-burial thrombosis;
proteinuria;
encephalopathy;
Integrity of property;
sudomi;
insult;
infarction;
Cardiomyopathy with the development of heart failure;
High arterial vise;
Defeat the full-time judges;
Exercise Legend;
Defeat the shkiri. visip;
Pain in the abdomen;
diarrhea;
blues;
colitis;
Pancreatitis

Viclikati show atopic hemolytic-uremic syndrome (Agus) for a number of reasons, most often the stench will become a trigger for the cob to become ill. Stink axis:

diarrhea;
gastroenteritis;
Infections of the upper dichally nobles;
Viability and canopy;
glomerulopathy;
Scleroderma;
Systemic chervona vovchanka;
Malicious arterial hypertension;
Malicious new solutions;
Transplantation of nirka and cystic marrow.

All the stench podsiluyut in the system of complement. Only one factor is enough for the manifestation of the congestion, but the smell can be a little bit in the end.

Atypical hemolytic-uremic syndrome (Agus). Diagnostics.

Thrombotic microangiopathy (TMA) is the result of atypical hemolytic-uremic syndrome (Agus), which may not only be Agus, but rather ill. It is so important for that to carry out a differential diagnosis, for the qualification of the atypical hemolytic-uremic syndrome (Agus).

Perse, atypical hemolytic-uremic syndrome (Agus) is not on the very cob of becoming ill with hematocolitis, in other words, diarrhea can be sick by itself, and not being a symptom. At all stages, STEC and Streptococcus pneumoniae - infections are required.

By the method of switching the patient over to the systemic chervonia vovchak, SNID, malignant hypertension, thrombotic thrombocytopenic purpura, HELLP is a syndrome in women who live and a number of those who are ill.

However, genetic analysis is not less than 100% certainty, in some patients with diagnosed atypical hemolytic-uremic syndrome (Agus), impaired gene was not established.

Atypical hemolytic-uremic syndrome (Agus). Likuvannya.

Lіkuvannya by plasma infusions, flame exchange. At the present moment, the method is inconsistently ineffective, for some patients there is little improvement in indicators, and for those of them it is practically marnim.

Hemodialis. The procedure for the individual cleansing of the organism is less assimilated by the inheritance of the animal in the system of complement, and even if it does not infuse into the process of oversubscription of the bottle. Wonderful for a bit of rock in the life of a patient. It is possible to develop lateral thrombotic microangiopathy.

Transplantation. Nirka, the yaka has lost its function, may be replaced by a new, more progressively enlightened one, may be able to start working on the organization and fabric. 90% of patients are aware of symptoms of atopic hemolytic-uremic syndrome (Agus). Є a great risk of relapse after transplantation. Sometimes it is possible to go through transplantation, and liver, and nyrka, but at the same time there is great folding in a joke, ideally two donor organs are suitable. Moreover, such a foldable manipulation may not give a guaranteed positive result. Thrombotic microangiopathy develops in other organs.

Ekulizumab. A single preparation, which is infused into the mechanism of development of ailments, and not on the inheritance of ailments. Ekulizumab tied the component to the complement and by itself accumulated negative factors. The drug has been registered in Russia and has improved its effectiveness. People with atypical hemolytic-uremic syndrome (Agus) do not see it permanently, they do not develop a damaged organ and tissue. Introduced into the clinical practice of ekulizumab, the humanized monoclonal antibody to C5 is a fraction of the thermal stage to the complement cascade, meaningfully the triviality of the life of the patients, and the life itself is growing.

Hemolytic-uremic syndrome is characterized by lesions of nirok, the appearance of anemia and thrombocytopenia.

The pathology is very important, especially in older children.

Rizik seize the soul of a human being.

For a reason for the skin potential of patients, especially the fathers of small children, the mother needs to be aware of the ethological factors, symptomatology and methods of prevention of this disease.

General information

Hemolithic-uremic syndrome, for Gasser's ailment, in most cases, enemies are younger than 5 years old.

Acquisition is mainly diagnosed in the summer period throughout the earth.

Hemolithic-uremic syndrome is characterized by 3 symptoms - thrombocytopenia in the back blood test, nirkov deficiency, hemolytic anemia.

Causes of the blame

Gastrointestinal infections are the most common reasons for the development of ill health, especially in children. ethiological factor There may be infections of the organisms of the disease, but it is not possible to diagnose it well enough.

In elderly patients, the disease develops on the basis of the pathology of non-infectious nature: for example, the use of certain drugs (immunodepressants), during the period of vaginosis, as a result.

Pathology is associated with glomerulonephritis, rheumatic diseases.

The most frequent hemolytic-uremic syndrome of mummification is verotoxin, which is a particular species intestinal stick... Danium toxin is a building block for the clines of sudin nirok. Winikє is an ignition process, the cells are fired up, they are brought to the bend of thrombocytes and erythrocytes.

On the walls of the sudinum, there is an attachment to the fibrin, which is the cause of microthrombosis.

The bend of the sudin nirok should be brought to the ischemia of the organ and the destruction of its function.

The very pathophysiological mechanism of Gasser's ailment can be seen.

It is important to note that verotoxin is the most common cause of illness, but not the only one. Krim, Gasser's ailment may be provoked by toxin bacteria from the genus Shigel, streptococcus. Be an agent, building a ruinuvati to a nirkov tissue, a building viclikati to a pathology.

Classification of disease

Hemolithic-uremic syndrome is classified for a few signs. We will see the offensive rank:

It is important to note that the hemolytic-uremic syndrome can be idiopathic, so that the reason is not clear. Likewise, the ability to a given pathology is transmitted in recessions.

Features of the grown-ups

Patients with an old age of ailment Gasser rarely get gut infections. Expect to develop as a result of the manifestation of the virus, immunodeficiency, the reception of certain lykarsky diseases, on the systemic and chubby ailments. The prognosis is in such a drop of unpleasantness.

Have children

Children of a child's age become ill in large numbers of children as a result of intestinal infections.

Infection is caused by the introduction of insufficiently fried meat, which has not passed the pasteurization of dairy products, unrefined water.

It is evident that in children the hemolytic-uremic syndrome is seen as a nosological one in children, at that time the pathology develops in older adults as if they have developed ailments.

Vagitnykh

Aggressiveness is a factor before the diagnosis of a given ailment. It is clinically to manifest itself as an eclamation and a threat of Wikipedia. The problem is perceptible to the conduct of the development. The pathology can be manifested by severe nirk deficiency, embolism, sepsis.

Developing a cool picture

Symptoms of hemolytic-uremic syndrome of ma characteristic rice... Viglyadaє with the offensive rank:

The average triviality of ailments Hesserit becomes 10-14 days. Indicator of stagnation due to heavy burden. The renewal of the organism to be increased more often, with the help of the author of the month.

diagnostics come in

To the patient, it is important to turn up an hour before the lykar. Tse can be a therapist abo pediatrician, gastroenterologist, infectious disease specialist. Be-like a drug can be a sign of illness and refer a sick person to hospitalization. Diagnostic algorithm:

a back-to-back examination of the patient with the analysis of scarg and anamnesis is carried out;
in the blood analysis, anemia is diagnosed, erythrocytes of the developmental form and in their own development, the indicator of platelets is lower than the norms;
in the biochemical analysis of blood, it means a change in the amount of bilirubin, a deterioration in the water-electrolyte balance;
, In them there may be houses in the viglyad blood, a large vmist is diagnosed;
obov'yazkovo behavior of feces with the mark of viznachennya zbudnik.

It is important to note that the instrumental diagnostics is based on the nature of the acceleration.

Patients can know about the electrocardiography, ultrasound dosage heart abo black empty, computer abo magnetic resonance tomography.

methods of therapy

lykuvannya g emolitic-uremic syndrome lie in the nature of the pathology. It is important to turn up to the doctor before, so as to ensure the success of the business and in the preservation of the development of important acceleration.

medication methods

Before the hour of therapy, hematocrit is controlled. For many, it is not often necessary to transfuse erythrocyte mass. Likuvannya is also carried out with antiplatelet drugs (heparin). For the improvement of peripheral blood circulation, patients are prescribed pentoxifylline.

For lіkuvannya vikoristoyutsya razchini, as normalizuyut water-electrolyte balance to the body. Vitamin A and E. are also introduced.

Bagato patients will require plasmepheresis, as they allow the use of toxic products in the exchange of rechovins.

For usunennya nabryak_vikoristovuyut sechogіnnі preparations, accelerated sercebitty - beta-blockers. With the help of lowering the vice, drugs are used to avenge nitropruside sodium.

Obov'yazkovy post_yny control showing arterial vise.

As a rule, doctors should be given the benefit of an educator, to be carried out for antibiotic sensitivity, on the basis of the results of which the appropriate therapy is indicated.

Lіkuvannya kids and vіtnykh

The principles of children’s liking do not conceal the exact characteristics of such children. Riznytsya polyagaє in the wake of victorious lykarsky zasobіv.

There are many people who live, food is more foldable. In most cases, it is necessary to carry out growth, some conservative therapy in some cases appears to be effective.

Folk medicine

Not known methods of folk medicine, which are struggling with hemolytic-uremic syndrome. Patients need to turn themselves into a drug and not engage in self-abuse.

It is possible to take courses of urologic fitosbory, which can be used as an antiseptic action, only for normalization.

dietician attribution

In the period of lіkuvannya and renewal to the patients, it is important to see special activities. It is necessary to switch to the power ratio.

Harchuvatsya is required for products with a high blenka - smoked and yaloviche meat, milk virobi, Greek groats, pebble varieties of ribi. The reception of the uzhi is guilty of small portions, 5 times a day.

Forecast and prevention

Zakvoryuvannya duzhe seriozne, zdatne lead to a lethal result. Chim earlier the patient turned up to the likar, it is more beautiful for the new one. Among children, the mortality rate becomes close to 5%, the children increase the deteriorated function of the child.

In older adults, the prognosis is worse, some hemolytic-uremic syndrome is a manifestation of their illnesses, and they are susceptible to interruption (often a severe form is diagnosed).

Prevention of pathology is carried out until all the rules of special hygiene are met, and the wrist is bound in front of the patient. The products of the blame undergo a retinal thermal processing, vegetables and fruits are necessary for good mitity.

Hemolithic-uremic syndrome is an even more severe pathology, which leads not only to nirkovoi, ala and polyorganic deficiencies. Therefore, it is important to vikonuvati all the recommendations from the hygiene of the food and in the case of a sickness to be innocently beaten for medical assistance.