Syndrome of marshal likuvannya. Marshall's syndrome: short-sightedness, cataracts, and similar deafness. Marshall Syndrome. Epidemiology

Marshall's syndrome - it is necessary to bring about a rare autoimmune disorder, which occurs mainly in children of the preschool age. In the past, they started talking about this disease in the 80s, if the first description of the syndrome appeared, which attacks the body of a child periodically and for no apparent reason. Marshall's syndrome may be anonymously called: periodic syndrome, autoinflammation disease, PFAPA syndrome. The peculiarity of yoga is the reading of the periodicity and the presence of the day, whether it is a sign of being ill in the intermissions between the attacks.

Marshall's Syndrome

Marshall's Syndrome is important to call good old illnesses. The reasons for this guilt are unknown and it is important to avoid it.

The syndrome is characterized by a regular periodic rise in temperature (up to 40 degrees), fever, inflamed empty mouth, throat, larynx,.

Particularity of illness in yoga periodicity. Illness can be accurately identified, if an offensive attack begins, but without regard for it, it’s even more important to avoid it, the shards are more symptomatic. It helps to relieve symptoms, but does not prevent their appearance of an attack.

Marshall's syndrome is blamed on autoimmune disorders. Most often, the stench itself causes attacks, and non-infectious causes.

The provocateurs of the blame for the illness were not exactly identified, but three possible causes of Marshall's syndrome were seen:

Latent viral infection. Іsnuє tsiliy a number of dense and attached infections. The stench is consumed in the body, localized in the most sensitive tissues and does not show itself for a long time. However, such infections rarely appear periodically, and they provoke an unfriendly mind, dovkill and lower immunity. Before latent infections, herpes can be seen, which may not be the last hour of everyday symptoms, but it can also appear for singing minds (another infection, a decrease in immunity, hypothermia just)
Chronic inflammation. The tonsils of a child can be attacked by bacteria. Inflammation of the tonsils often transforms into a chronic form due to the presence of exuberance. In this case, the immune system of Viklikan has the products of the vitality of bacteria and their toxic influx on the mucous membranes and the organism in the whole. Periodically inflamed tonsils may protika even more importantly with purulent processes, stomatitis and fever.
Autoimmune sbіy. Nayimovіrnіsha cause blamed for Marshall's syndrome. Such ailments are often classified as blocking, but not ill. The human immune system gives rise to and starts to take in the air of cells like aliens and soothsayers, reacting to them with temperature rises and ignition processes. As a rule, there are no common reasons for such an immune reaction.

Signs of illness

Marshall's syndrome has a number of characteristic symptoms, it is not so easy to differentiate yoga from other autoimmune and infectious diseases.

Diagnosis is often made to help the history itself and the presence of symptoms in Marshall syndrome:

Attack fevers. The temperature shift is manifested by a clear periodicity. Sound the quiet period from 2 to 7 days. Start turbulence once a month or more often (once every 3 days). Fever can last 2-5 days, but it will show up without any sequelae. Vaughn blames for no apparent reason and learns also independently for various kinds of jubilation. Intervals may tend to zbіlshennya, so that with an overrun of ailments, intermissions between attacks become dowish.
Visoka. Marshall's syndrome is always accompanied by a high temperature (up to 405 degrees). In case of any ailments, it is a headache, fever, confusion of information, chills may appear. The temperature is dormant all 5 days in attack and does not decrease in case of antipyretics or decreases insignificantly and not for a long time.
. Marshall's syndrome is often accompanied by inflammation of the mucous membrane of the pharynx. With manifestations of pharyngitis, ailments are felt dryness in the mouth, strong, as if elated with washing and forging, there are visible breasts at the throat,. As a rule, signs of pharyngitis appear at once from an attack.
Stomatitis. In case of stomatitis, the ignition process of the empty mouth is guarded. By themselves, stomatitis is accompanied by a rise in body temperature, and induce vinification of the eyes and bulbs in the mouth, ailing sights. Often stomatitis is caused by damage to the immune system.
Improvement of lymph nodes. In Marshall's syndrome, subscleral nodes are more pronounced, you can visually treat them. On palpation, the nodes were sore and narrowed.
I hurt in my stomach. Abdominal syndrome does not affect all children with Marshall's syndrome, but it can be seen in approximately 50% of cases.
Suglobov and headaches. Qi symptoms accompany Marshall's syndrome, and fever okremo. In a strong heat, a strong bіl of the head, aches and bіl in the swamps are often predicted.

Not all ailments show the full range of symptoms. Sometimes the stench oozes with a vibrating and different intensity of the world. The most common signs of Marshall's syndrome are elevated temperature and increased lymph nodes.

Diagnosis of Marshall's syndrome is most problematic due to the confusion of symptoms and the presence of an infectious nature. Only after a few attacks can blame the suspected autoimmune disorder.

Ways to follow the syndrome:

Anamnesis. Anamnesis plays a great role in the correct diagnosis. The healer carefully collects information about those who have begun to attack, as if they were stinking for a long time, as they manifest themselves. If the fathers of the building turn to the beginning of an offensive attack, you can suspect the presence of autoimmune disorders. It is also necessary to guard the camp of a child between the attacks of a lihomania. As a rule, with Marshall's Syndrome, a child in between attacks feels good, she does not show the usual signs of infection, or she develops.
Look around. During the period of illness, the doctor looks at the child. When looking around, there are redness and inflammation in the empty mouth, on the mucous membrane of the throat and tonsils, the presence of ridges and erosions, an increase in the tonsils, an increase in lymph nodes, their palpation is carried out.
Hot blood test. If Marshall's syndrome is suspected, it is recommended to take a blood test, to check the number of leukocytes and SHOE. As a result, the number of leukocytes in the blood is increased, the number of erythrocytes is increased, and the ignition process occurs in the body.
Biochemical blood analysis. In biochemical analysis of blood, fibrinogen and C-reactive protein levels were observed, which is also an indicator of severe inflammation.

More information about Marshall syndrome can be found in the video:

The most effective child antibiotics for angina

Marshall's syndrome does not have any specific indications of markers, which can be diagnosed in less than an hour, if all the characteristic signs of illness are manifested.

So, for example, at the cob stages, Marshall's syndrome with chronic tonsillitis, which often develops in young children, is easy to tread. Vin also accompanies increased lymph nodes, sore throat, fever, redness and swelling in the area of the tonsils.

It is possible to distinguish between similar illnesses in an hour, if there is a clear cyclicity to Marshall's syndrome and the presence of external causes for the ignition process.

At times of difficulty, the doctor may require a genetic analysis. Recently, a disease gene has been identified that can be mutated.

Methods of likuvannya

Lіkuvannya lie down in the event of illness. Often it is directly directed to the symptomatic relief, and not to the danger of an offensive attack.

The use of antiseptic and antipyretic drugs often does not give results and in more and less half of the depressions lead to relapses.

Features of the lure:

. Treatment with antibiotics is prescribed at the cob stages of ailment, if there is a suspicion of a bacterial nature of infection. Treatment of Marshall's syndrome with antibiotics, as a rule, does not give positive results. The attack ends on its own on the 5th day, or earlier, but the attack will once again be blamed.
Antipyretic drugs based on paracetamol are also ineffective. The stench helps to lower the temperature for a short period of time, or it lowers the trochs, but does not give a likuval effect. Antipyretic drugs are included in symptomatic relief of illness, so they should be prescribed for Marshall's syndrome, in order to ease the sickness.
Glucocorticosteroids. Steroidal antiseptic drugs such as Prednisalone are considered to be effective in Marshall syndrome. Qi preparations with synthetic substitutes for hormones in supra-nipple ulcers. The stench can control all exchange processes in the body. With Marshall's syndrome, stench helps to relieve the symptoms of febrile fever, attack quickly up to 2-3 days. Prote є thought that I will take glucocorticosteroids in a short time of attacks, and the th hour of remissions. The skin offensive attack begins earlier for the front one.
. The visualization of the migdaliki was carried out in that moment, as the exultation of the attack was insufficient, or the overcoming of the disease was aggravated by the hypertrophy of the migdaliki. With a trivial and frequent swelling, the tonsils are lit up regularly, blocking the access through the nose. Even if the stench does not change, the child suffers from unsatisfactory sourness, suffers from the mind and physical development, doctors recommend seeing the tonsils. This operation does not lead to autoimmune disease, but it will help to get rid of unfortunate ailments. The operation for the removal of the tonsils is assigned only to the extreme depressions, and for the correct treatment, one can die.

As a rule, Marshall's syndrome does not affect the mind and physical development of the child. With regular watchfulness, the symptoms of exacerbation are also rare.

Ale, in case of a severe illness, ailments can be blamed for other illnesses that become ill to the body, caused by an immune reaction:

Diarrhea. Diarrhea is often blamed overnight on abdominal syndrome. A sick person develops stomach cramps, pain, everything is accompanied by a high temperature, frequent rare deformities. Can be blamed for the signs of flooding, which only help overcome the disease.
Neutropenia. With neutropenia in the blood, the number of neutrophils (a single type of leukocytes) decreases, which significantly reduces the body's functions. There are no usual symptoms in case of this illness, but through a decrease in immunity against the background of illness, new infections (bacterial, viral, fungal) are blamed. With a severe overshoot of neutropenia, there is a sharp rise in temperature, hand tremor, and heart rhythm disturbance.
Arthritis. Arthritis is blamed for Marshall's syndrome in that depression, as the igniting process expands to drift. Sooty whites with arthritis start to collapse normally. Occasionally, arthritis manifests itself in the appearance of reddening of the skins in the area of rotting of the sickly loam. Also, arthritis is often accompanied by changes in body temperature. The most severe form is rheumatoid arthritis; The ailment proceeds with remissions and relapses.
Neurological symptoms. Neurological manifestation may be strong. So, Marshall's syndrome can be accompanied by ailments of the whole body, migraines, confusion, and sometimes restlessness. Qi symptoms can be posed as in the period of remission, so before / before the hour of an illness.

Everyday prophylactic visits cannot be effective, but there are no reliable indications of the cause of Marshall's syndrome. Zapobіgti viniknennyu tsgogo syndrome is impossible, like і perebachiti. As a rule, the child is not afraid of daily rethinks, and it’s important to talk about risk factors.

Marshall's syndrome is a rare disease of an autoimmune nature, as it develops in children up to 6 years of age. Previously, such a pathology was seen only in the 80s of the last century. Until this hour, the first description of a sign of ailment is seen, which periodically attacks the child’s body, it would have been better, on aphids of total well-being. In Marshall's syndrome and є і kіlka Іnshih names - periodically vinikaє syndrome, auto-inflammation ailment, PFAPA. Especially given ailments are those that manifest themselves periodically and between attacks I do not know about myself.

Cause

Navit for the current development of medicine, Marshall's syndrome is not like a trace. The causes of this pathology are not exactly identified, it is practically impossible to prevent ailments.

With this ailment in a child, the temperature of the body periodically rises to 40 degrees, febrile fever and inflammation of the pharynx, tonsils and an empty mouth are observed. The ailment appears periodically and a person can tell for sure if there will be an offensive attack. However, it is impossible to get ahead of such an attack; Be it therapy, it is recognized in Marshall's syndrome, it is directed at the symptomatic relief of the disease, but it is not good enough to ward off the onset of attacks.

Є admission, which is blamed for illness through the songs of autoimmune damage.

Although the causes of this pathology are not known, doctors see a few factors that can help to cope with the development of the disease.

Latent infection

Prihovany viral infection. Here is a whole list of latent viral infections, which do not manifest themselves for a long time. Such viruses penetrate the body and accumulate in especially sensitive organs. However, these infections do not appear periodically, the shards of the development of an ailment provoke a decrease in immunity and an unacceptable mind dovkіllya.

Herpes is considered to be similar to viral infections. Carrying this virus, there are impersonal people, but the sickness is seen only for singing minds. Provoking factors may be hypothermia, lowered immunity or other infection.

Chronic tonsillitis

Small children often suffer from angina. In case of inadequate treatment, the disease quickly transforms into a chronic form. Bacteria that inhabit the tonsils of an ailing child see a lot of toxic speeches that lead to intoxication of the body. A high temperature, inflammation of the mucous membranes and a fever can cause the immune system to become inflamed.

Acute chronic tonsillitis in preschool children can sometimes be very important. There is a high temperature, stomatitis and purulent plaque on the tonsils.

Zbіy in the robotic autoimmune system

The most common cause of Marshall's syndrome in children is called autoimmune disorders. Ailments of this group can be blocked every hour, but it is impossible to vilіkuvati. In such a time, the immune system, for unknown reasons, begins to accept its own cells as strangers to fight against them. Tse to bring to a stable temperature rise and a strong inflammation. Well, there are no visible reasons for such a reaction to the body.

The frequency of blame for attacks can be different. In some children, attacks are blamed after 3 days, in others after two months.

clinical picture

Marshall's syndrome in children for the first time occurs with low characteristic symptoms. But knowing the peculiarities of overcoming ailments, it is important for healers sometimes to differentiate pathology from other illnesses or autoimmune disorders. To establish the diagnosis, the doctor carefully takes an anamnesis, seeing the characteristic signs of illness.

Fever. Є pevna periodicity the development of a feverish state. Periods of remission can last from 2 to 7 days. Attack about once a month, but sometimes the hourly interval is less. The heat can be close to 5 decibels, after which everything goes without a hitch. With the development of ailments, intervals between attacks of lihomania may tend to increase.
Temperature. Marshall's syndrome is characterized by a very high temperature. Temperature readings can reach 41 degrees. It is accompanied by an unbearable headache, impaired vision, madness and chills. Such a temperature is important to control with antipyretic drugs. If you start to beat the troch, then it’s a non-trivial hour.
Sore throat. For Marshal's ailments, he often gets pharyngitis. This disease is accompanied by a severe sore throat, which is significantly worsened by squeezing, dryness of the membranes of the oral cavity and coughing. The ailing vine has a noticeable sign of a third-party body in the throat. All symptoms of pharyngitis sound like absence of vision to pass overnight with an attack.
Stomatitis. Illness often occurs with stomatitis. When this happens, the mucous membranes of the company show sickness, and the temperature of the body rises strongly. Stomatitis can be the result of immune disorders.
Improvement of lymphatic nodes. In Marshall's syndrome, the subschelepny lymph nodes are strongly ignited. You can commemorate with an indefatigable eye. When matting the nodes, it is possible to notice their soreness and induration.
Strong pain in the abdomen. A similar symptom in this autoimmune disease develops in about half of sick children.
Bіl suglobakh that head. Qi signs are always present at high temperatures and hot.

Not all ailing children have these symptoms all the time. In most cases, it is less likely to be a sign of illness, and the stench can be of varying intensity. The most common symptoms of Marshall's syndrome in children are fever and angina.

Marshall's syndrome may be suspected in children of a lifetime. Sound that you are ill on your own to pass to the age of transition.

Diagnostics

It is important to diagnose the pathology, through the masking of the symptoms and the presence of the health worker. Less than a few attacks of the same type by the doctor can blame the suspicion on the pathology of an autoimmune nature. To establish the diagnosis of vicorist, the following diagnostic methods are used:

Take anamnesis. It plays a primary role in diagnosing ailments. The doctor respectfully vislukhovu batkіv ailing child, z'yasovuchi, how long ago the attack began and the character of their overrun. In times, if the fathers can accurately identify the ear of an offensive attack, Marshall's syndrome can be suspected. Krіm tsyogo, fakhіvets posterіgає child in the period between attacks. Ring out at this hour, the burning camp of the children is not broken, and the stench develops normally.
I'll take a look. During the period of acute illness, the doctor carefully looks at the child. Under an hour of looking around, the inflamed mucous membrane of the mouth, the spots on the tonsils, stomatitis and the increase in lymph nodes appear.
Clinical blood analysis. For clarity of the picture, the sickness of a child is directed to a blood test. If the number of leukocytes is strongly increased and the NCO is increased, then we can talk about the ignition process in the body.

Marshall's syndrome is not diagnosed according to any particular criteria. You can only talk about being ill during attacks, which are periodically repeated.

At the bud stage, auto-burning disease is often confused with chronic tonsillitis. This disease is also accompanied by increased temperature, increased lymph nodes and flares on the tonsils. Differentiation of pathology can be less than an hour later, if the cyclical attacks appear.

If the diagnosis is uncertain, then the doctor can perform a genetic analysis. We have recently discovered a gene for ailment and this gene can be mutated.

Celebration

Rejoicing Marshall's syndrome is always symptomatic. Whether it be the exaltation of pathology, the elimination of all symptoms, and the prevention of an offensive attack. Treatment with antipyretic and antipyretic drugs rarely gives an effect, more often such therapy leads to relapse of the disease. When treated, you can use the following medicinal preparations:

Antibiotics. Often prescribed on the cob of ailment, if there is a suspicion of the infectious nature of the pathology. With Marshall's ailment, antibiotics do not give the same effect.
Antipyretic preparations. Faces based on paracetamol should not be given an effect. The stink of the building troch beat the temperature for a couple of years. In case of autoimmune illness, give children preparations based on ibuprofen.
Hormonal drugs Prednisolone is considered to be an effective antiseptic drug for periodic syndrome. The cei hormone is a synthetic analogue of the hormone of supra-nural ulcers and takes part in various metabolic processes that occur in the body. Prednisolone itself helps to speed up the hour of attacks up to 2-3 days.

It is clear that hormonal preparations do not help to shorten the hour of attacks, but also significantly change the period of remission. In times of skin, an offensive attack begins earlier.

If the ailment is aggravated by hypertrophy of the tonsils, then the doctor can make them visible.. It is necessary in order to normalize nasal breathing in children. Tonsilectomy does not change the autoimmune pathology, but it can change the complication. Until the end of the mygdaliki, they go into an extreme depression, with the right choice of therapy, such an operation can be lost.

Constantly enlarged tonsils lead to the destruction of the nasal breath, with which the child feels the lack of sourness. Such a camp can be brought up to the age of rosum and physical development.

complication

Marshall's syndrome in children does not affect the mind and physical development. If a child is regularly guarded by a doctor and appears in a state of exuberance, then it is very rare to become aggravated. Alecia autoimmune diseases are often accompanied by other pathologies, which can also destroy the child's body:

Diarrhea. In young children, fever often occurs with severe diarrhea and abdominal pain. Tse mozhe quickly bring the body to the dawn, with which the camp of the little one can only grow stronger.
Damage to the warehouse of blood. Neutropenia is most likely to occur, and the number of neutrophils in the blood changes significantly, which leads to a permanent decrease in immunity. It does not show the usual symptoms, but such aphids often develop fungal and bacterial infections. With a severe overshoot of neutropenia, the temperature rises significantly, the work of the heart is disrupted, and there are three terminations.
Arthritis. Tse illness may break out at that fall, as if the igniting process had torn into the snow. The sick child is importantly collapsing, she is becoming drastic and whiny. The snowdrifts of a child can be blackened, become swollen and deformed. Arthritis occurs with periods of acute remission.
Neurological disorders. Marshall's syndrome often occurs with severe confusion and fatigue. Such a break can be like in the period of congestion, and in the period of remission.

With often repeated attacks, the immunity is greatly reduced, which is manifested by partial ailments. Children with Marshall's syndrome may sound blue and look unhealthy.

Forecast

With early rozpochatym lіkuvannі prognosis ¾ favorable. A lot of children are dressed up after the death of the tonsils, in the case of all the symptoms of the disease go away in the child's eye. In a short period of time, after dressing, there may be a slight neurological disorder.

Even among close relatives were affected by Marshall's syndrome, it is necessary to consult a doctor. For such an approach, diagnosing pathology is much easier.

Daily prophylactic visits with Marshall syndrome are not effective. The reasons for this illness are definitely not explained, therefore, it’s impossible to save the blame for the attacks, just like that. The ailment begins to sound and the natomist is completely well-being, without any rethinking.

CLINICAL WARNINGS

UDC 616-616-003.821-021.3-039.13-039.42-039.52

VIPADKOV DIFFICULT DIAGNOSIS OF ILLNESS WITH MARSHAL SYNDROME

Asiya Ildusivna Safina1*, Ildus Yaudatovich Lutfullin1, Kamil Ziyaevich Zakirov2,

Valeriy Yuriyovich Shapiro2

1 Kazan State Medical Academy, 2 Children's Hospital No. 1, Kazan

Induced clinical care for ailments from the previously established Marshall's syndrome. An algorithm for diagnosing this disease and possible options for therapeutic tactics are described. Key words: periodic syndromes, Marshall's syndrome, diagnosis, children.

A CASE OF DIFFICULT DIAGNOSIS OF A PATIENT WITH MARSHALL SYNDROME

A.I. Safina1*, I.Ya. Lutfullin1, K.Z. Zakirov2, V.Yu. Shapiro2

1 Kazan State Medical Academy,2Pediatric City Hospital № 1, Kazan city, Russia

Virobnik buv clinical complication of the patient was newly diagnosed with Marshall syndrome. Guidance letter algoritm of diagnosis of this patient and possible therapeutic tactics. Key words: periodic syndromes, Marshall syndrome, diagnosis, children.

Periodic syndromes - a group of autoinflammatory diseases (Human autoinflammatory diseases - HAIDS) with periodic non-infectious fever and signs of systemic inflammation. Vіdpovіdno to klasifіkatsії Єvropeyskogo comradeship z іmunodefіtsіtіv, tsі zahvoryuvannya v_dnosyatsya to primal іmunоdefіtsіtіv. Periodic ailments develop on aphids of a genetic defect in the system of regulators of inflammation, which in the end case leads to an increase in the level of proteins in the acute phase, such as C-reactive protein and gray amyloid protein A (SAA). Trivale promotion of the rest can lead to the formation of amyloidose of organs. Inflammation in periodic syndromes is the first, not associated with infectious (viruses, bacteria, fungi, for example, and in) or non-infectious (autoantibody, autosensitization of T-lymphocytes, products of tissue breakdown and other) agents.

The urgency of the problem of periodic syndromes for a pediatrician is due to the fact that the stench of life makes its debut in a child's wife. Thus, the first manifestations of family mediterranean fever in 90% of cases fall on a child and adolescence, and Marshall's syndrome - on a century of up to 5 years. The study of the semiotics of periodic syndromes allows one to make a diagnosis and eliminate unnecessary manipulations, such as the recognition of antibiotics in angina with Marshall's syndrome or diagnostic

laparotomy of a child with family mediterranean lihomania associated with the clinical picture of "acute abdomen".

In Denmark, a small number of periodic syndromes have been seen: febrile mediterranean lihomania, febrile febrile syndrome, association with tumor necrosis factor I receptor mutation, febrile necrosis factor I syndrome with hypermunoglobulinemia D, and Marshall's syndrome.

Periodic Fever with Aphthous stomatitis, Pharyngitis and Adenitis - PFAPA-syndrome. The genetic and molecular mechanisms of the pathogenesis of illness are unknown, the fall-off is not easy to catch. Критерії діагностики: періодична фебрильна лихоманка, початок захворювання в ранньому віці (молодше 5 років), симптоми ураження верхніх відділів респіраторного тракту за відсутності інфекції, а також хоча б один з наступних клінічних симптомів - афтозний стоматит, шийний лімфаденіт, фарингіт гострий тонзиліт, відсутність циклічної neutropenia, presence of asymptomatic intervals, normal growth and development.

Illness may be a clear periodicity, which can also be a diagnostic criterion. An attack on Marshall's syndrome sounds like a mask of purulent tonsillitis, aphthous stomatitis, cervical lymphadenitis. For this, the diagnosis of "angina" in children of 1-3 years of age is to blame for the respect of the pediatrician, the shards out of it in the region of a rare pathology in this person. It is noteworthy that the recognition of antibiotics and antipyretic effects is not

Hematological indications of Yu.'s ailments in the same year and 6 months.

Leuk., x 10 9/l Leukocyte formula SHOE, mm/year

Date of p.i.n., s.i.n., limf., mon., eoz., diagnosis

10.11.08 p. 7.5 1 15.5 63 13 7.5 4 healthy

19.12.08 p. 6.8 0 28 55 6 11 2 healthy

29.01.08 p. 8.5 1 28 55 6 10 3 dorov

14.04.09 p. 12.7 6 39 51 4 - 6 aphthous stomatitis

06/03/09 p. 10.6 0 20 67 12 1 2 healthy

07.06.09 p. 10.8 8 41 46 5 0 6 GRVI

09.09.09 p. 14.4 4 41 38 15 2 10 pyelonephritis, vulvitis

02.11.09 p. 27.9 24 45 27 4 - 26 follicular angina

follicular

16.02.09 p. 11 3 39 39 17 2 19 angina, aphthous stomatitis

pours into the trivality of the episode, which sound lasts 4-6 days and is allowed spontaneously. There are no specific laboratory criteria for the diagnosis of Marshall's syndrome. Global blood analysis shows a decrease in leukocytosis due to neutrophilic damage to the left, an increase in SOE. Infection should not be brought before the formation of an amyloid dose of either liver or liver.

A one-time and two-time distribution of prednisolone at a dose of 1-2 mg/kg in the middle of the day is a fast way to cope with attacks from a different clinical picture for a long period of time. However, the recognition of glucocorticoids cannot be defeated by offensive attacks. In addition, it is believed that prednisolone can disrupt the internal rhythm of contagion and increase the frequency of fever attacks. To this day, the most effective method of curing illness is tonsillectomy, which brings about 68-90% of illnesses to cure. Illness is allowed spontaneously, less than 3% of ailments at the reach of povnolittya save the attack of fever.

At the pediatric department of the child's medical clinic No. 1 of the city of Kazan from 10.03.2010. watch out for sickness in the south at vіtsi 1.5 roku. Periodically recurrent short-hour episodes of febrile febrile fever in accompanying angina and aphthous stomatitis became the reason for going to the doctor. The first episode of febrile fever with manifestations of aphthous stomatitis was indicated in a 6-month-old child. With the diagnosis of aphthous stomatitis, the patient was hospitalized for 9 days before an infectious drug treatment, she received antibiotic therapy (cefazolin, ceftriaxone, amikacin), and was treated for stomatitis. There were no significant effects observed with antibiotics. Roasted bula was bought on the 6th day of likuvannya. Over the course of 11 months, the child was diagnosed with 6 episodes of febrile fever s 288

increase in body temperature up to 40.0 ° C with intervals of 6-8 days.

With a graphic display of episodes of fever, the lihomania turned out to be respectful of their periodicity and fixed trivality. Mati ditini could finish off exactly the cob of an offensive attack of illness. Conducted at the time of feverish episodes, hematological investigations showed that the attacks of illness were not accompanied by neutropenia, but, on the other hand, were characterized by an adequate camp of flare changes in the presence of leukocytosis and neutrophilic disease "to the left" of different virulence (div. table).

Bacteriological culture of a smear from the pharynx on 11.09.2009 r. - ß-hemolytic streptococcus 104 KUO. For the rest of the episode of lacunar angina (16.02.2010), the level of procalcitonin in the blood was 0.61 ng/ml. Investigated markers of infectious diseases: Chl. trachomatis, cytomegalovirus (PLR) – neg. dated 10.08.2009, Chl. trachomatis, Micoplasma hominis and Ureaplasma urealyticum IgG and IgM (IFA) - negative, Micoplasma hominis IgG - weak. dated 11.10.2009

Upon entering the hospital (March 10, 2010), the child's camp was stable, overwhelmed. On the hairy part of the head - a hemangioma with a size of 2x2 cm; Ziv is not hyperemic, tonsils are not zbіlshenі, puhkі. Heart tones are rhythmic, clear. In legenia, they are vesicular, carried out in all fields, there is no wheezing. Lives soft, painless. The liver is palpable along the edge of the costal arch, the spleen is not palpable. Smear from throat - hanging Strp. Pyogenes. Echocardioscopy - open the oval window, the chambers of the heart are formed correctly. An ENT-drug review: chronic tonsillitis, which is forming, nasal Strp.

pyogenes. The girl was discharged with a diagnosis of Marshall's syndrome; hemangioma of the scalp; Open the oval window. It is recommended to consult an immunologist, sanitation of the oropharynx, as well as repeated hospitalization at the hour of the onset episode of fever.

12 days after discharge (approximately 6.5 days after the last attack of illness), the girl again developed febrile fever with an increase in body temperature to 39.3°C.

Conducted obstezhennya: deep blood test - leukocytosis (12x109/l), neutrophilic acid vlivo (p.i.n. - 10%, s.i.n. - 45%), accelerated SOE (22 mm/year); deep analysis of the section - without pathological changes. Biochemical analysis of blood - liver, nirk markers, nitrogenous slags, indicators of mineral exchange, exchange of bilirubin, blood proteins - at the boundaries of normal values. Increased rhubarb in the acute phase (C-RP - 30 mg/dl, fibrinogen - 4.7 g/l), procalcitonin - 0.2 ng/ml.

With a differential diagnostic method for girls, one-time administration of prednisolone at a dose of 10 mg intravenously (with a dose of 1 mg/kg). Antibiotics and antipyretic drugs were not tested. After the recognition of prednisolone, it was indicated that the symptoms of the main manifestations of illness were diagnosed. The body temperature returned to normal after 3 years after taking pre-nisolone, fever did not recur. Rot on the tonsils znik after 5-6 years, and hyperemia of the throat - 12 years after prednisolone was prescribed.

На підставі чіткої періодичності та фіксованої тривалості атак захворювання, неефективності антипіретиків та антибіотиків у контролі основних проявів, характерної клінічної картини (короткочасні епізоди лихоманки, 4 епізоди гострих тонзилітів та 2 – афтозних стоматитів), відсутності чіткого зв'язку з інфекцією ангіни без антибіотиків та антипіретиків

clarifications of the diagnosis were made: Marshall's syndrome; hemangioma of the scalp; open the oval window.

Due to the method of further management tactics and the possibility of performing tonsillectomy, the disease was referred for a consultation at the RDKL polyclinic (Moscow).

Guidance clinical application illustrating the possibility of diagnosing periodic syndromes, basing us on the relay analysis of the clinical picture and anamnesis of illness. Periodic syndromes like illness are more rare, proteo are not casuistic, and the main manifestations of these syndromes may be in the “baggage of knowledge” of a pediatrician.

LITERATURE

1. Arutyunyan V.M., Akopyan G.S. Periodic disease (etiopathogenetic and clinical aspects). – M.: МІА, 2000. – 304 p.

2. Barabanova O.V., Konopl'ova E.A., Prodeus O.P., Shcherbina O.Yu. Periodic syndromes// Difficult. patient. - 2007. - No. 2 - P. 46-52.

3. Salikhov I. G. ta in. Likhomanka obscure travel / Navch. help. - Kazan: View of KDMI, 1993. - 94 p.

4. Berlucchi M, Meini A, Plebani A, et al. Update on treatment of PFAPA-syndrome: report of five cases with review of the literature // Ann. Otol. Rhinol. Laryngol. -

2003. - Vol.112. - P. 365-369.

5. Drewe E, Huggins ML, Morgan AG, et al. Ailment of renal amyloidosis with tanercept in tumors of the necrotic factor receptor-associated periodic syndrome // Rheumatology. -

2004. - Vol. 43(11). – P.1405-1408.

6 Feder H.M. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: Clinical review of a new syndrome // Curr. Opin. Pediatr. - 2000. - Vol.12. – P.253-256.

6. Galanakis E., Papadakis C.E., Giannoussi E., et al. PFAPA syndrome in children evaluated for tonsillectomy // Arch. Dis. Children. - 2002. - Vol.86. – P.434-435.

In the midst of illness, which are characterized, it was given b, without provoking attacks of lihomania, there is Marshall's syndrome, which is manifested in children for a few years (overall from 4.5 to 8 years).

Pathology, described in the article The Journal of Pediatrics more than 30 years ago by American pediatricians, took the name of the first of the spivauthors - the doctor of the child's clinic in Philadelphia Gary Marshall.

In English medical terminology, Marshall's syndrome is called PFAPA syndrome - periodic fever (periodic fever) with aphthous stomatitis (aphthous stomatitis), pharyngitis (pharyngitis) and lymphadenitis (adenitis), which is inflamed lymph.

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ICD-10 code

D89.9 Impairment by immune mechanism, unspecified

Epidemiology

The exact extent of this syndrome in the global population is unknown; in boys, Marshall's syndrome occurs less often, less often in girls (in 55-70% of cases).

The first manifestation sounds at the beginning of two to five years (approximately three and a half years), if possible earlier. Manifest the syndrome (attacks) in most patients try to stretch five to seven years and spontaneously attach themselves after reaching the decimal age to the next one.

Causes of Marshall Syndrome

For quite a long time, febrile lihomania was caused in children with symptoms of inflammation in the throat area, an empty mouth and shii were treated with an idiopathic state. Later, the causes of Marshall's syndrome began to appear due to recessive genetic mutations, but a specific gene remained largely unaffected. However, pediatricians have a history of family history and stubbornness of blood relatives to the fuse of given localization and fever: for some kind of tribute, a positive family history is observed in 45-62% of patients. And so skillfully the real officials of the risk show the PFAPA syndrome.

In modern clinical pediatrics, the genetic causes of Marshall's syndrome are attributed to an uncharacteristic activation of both forms of immune response during infections - congenital and adaptive, as well as changes in the nature of either the kinetics of immune response. Tim is not less, the pathogenesis of Marshall's syndrome is so up to the end and not z'yasovany, but two versions are considered: activation of the immune system in recurrent infections and damage to the mechanisms of the immune response themselves. The first version is clearly implausible, because the rest of the microbiological investigations showed superbly clear serological results and the daily response to antibiotic treatment.

And if there are problems with the mechanism of the immune reaction, then there is a simple connection with defects in the proteins of the innate immunity. The number of activated T-clitin or antibodies (immunoglobulins) in the blood does not rise in the blood, and the level of eosinophils and lymphocytes often decreases. On the other side, in the qi period, activation of the production of interleukin IL-1β (which plays an important role in the initiation of fever and inflammation), as well as inflammatory cytokines (gamma-interferon, tumor necrosis factor TNF-α, interleukin IL-6) . I tse may have a slightly supraworld expression of genes CXCL9 and CXCL10 on the 4th chromosome.

The main mystery of the PFAPA-syndrome is that the ignition reaction has no infectious triggers, and it is not possible to express genes. Officially Marshall's syndrome є illness of unknown etiology and unidentified pathogenesis (for ICC-10 class 18 - not classified in other classes of symptoms and recovery from normal; code - R50-R610). I, as before, yoga was brought to sporadic illnesses, now it is confirmed by recent reports to consider yoga recurrent, tobto periodic.

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Symptoms of Marshall Syndrome

The first signs of attacks in Marshall's syndrome are yak, after clinical warnings, they trap through the skin.

Also, there may be prodromal symptoms of Marshall's syndrome, which appear approximately before the rise in temperature indications in what looks like severe illness and headache. Then there is inflammation of the mucous membrane in the mouth with small, slightly painful aphthous lesions (on average, in 55% of patients). Pain at the throat (alone with exudation) may look like a pharyngitis - inflammation of the mucous membrane of the pharynx. Swelling of lymphatic nodes on the neck is indicated, as if from lymphadenitis. If the mother is on the verge, the whole complex of symptoms is observed in 43-48% of cases.

Daily other symptoms, for example, rhinitis, cough, severe pain in the abdomen or diarrhea, do not occur with Marshall's syndrome. The fever can last three or three days until Monday, after which the temperature is so sharply normalized, and everything manifests itself.

In case of any child in the period between attacks, the lihomania is absolutely healthy, and she does not have an opportunity to live with a wild rose. According to the data of clinical studies, the incidence of this aggravation of the PFAPA-syndrome is daily (otherwise, it has not been revealed at the moment).

Diagnosis of Marshall syndrome

Today, the diagnosis of Marshall's syndrome is carried out depending on the typical clinical picture. The analyzes are intermingled with the buildings of the global blood analysis.

In order to remove the anxiety of the fathers, to escape the inconvenient and expensive ones, and to avoid the potentially unsafe methods of extortion, to establish the criteria for diagnosing the PFAPA-syndrome:

the occurrence of over three fixed regular fluctuations in febrile fever;
the presence of pharyngitis with slight lymphadenopathy in the area of the neck and / or aphthous lesions on the mucous membrane of the empty mouth;
the presence of anomalies in development and the normal state of health between episodes of illness;
promptly caused symptoms after a one-time infusion of corticosteroids.

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Differential diagnostics

Differential diagnosis of this syndrome includes other illnesses with periodic febrile febrile illnesses: co-morbid mediterranean febrile illness, Behçet's autoimmune ailment, cyclic neutropenia (with a three-term cycle and great tissue lesions), juvenile rheumatoid arthritis (ailing disease). The following include infections of the upper dichal pathways and pharyngitis, tonsillitis, lymphadenitis, aphthous stomatitis, which are caused by bacteria.

Важливо диференціювати синдром Маршалла і вроджений синдром гіпер-імуноглобуліну D (синдром дефіциту мевалонаткінази) у дітей першого року життя, при якому періодичні напади лихоманки - крім симптомів, властивих PFAPA-синдрому - супроводжуються болями в області живота, збільшенням селезінки, блювотою, і набрякання суглобів ; From the earliest age, such children show a slow development and a worsening of the dawn.

Treat Marshall Syndrome

Pediatricians have not yet come up with a single idea about how Marshall's syndrome can be treated.

The main drug therapy may be symptomatic and is based on single doses of glucocorticoids. So, for relief of pain, betamethasone or prednisolone is prescribed for Marshall's syndrome. Prednisolone in tablets is taken internally once on the cob of fever fever - 1-2 mg per kilogram of body weight of the child (the maximum dose is 60 mg); betamethasone - 0.1-0.2 mg / kg.

GCS, which may have an immunosuppressive effect, is contraindicated in cases of severe circulatory diabetes, hypercortisolism, gastritis, inflamed nitrogen, in the period after vaccination, weakened children. The most widespread side effect of treatment with prednisolone is restlessness and sleep disturbance, which should be taken as a sprat a year before bedtime. On the third or fourth day of the attack, the dose can be changed to 0.3-0.5 mg / kg (day).

Clinical evidence shows that antipyretic drugs, zocrema, non-steroidal anti-inflammatory drugs, help to reduce fever even more, and fight against other symptoms is not effective. In case of symptomatic treatment of this syndrome, it is important to evaluate risks associated with possible side effects. From the same time, first of all, stop taking lozenges for a sore throat, then be glad to see a doctor. Zocrema, it is recommended that you choose not to use antibiotics, but antibacterial agents do not give the same result in Marshall's syndrome.

Children who suffer from Marshall's syndrome need vitamins, especially calciferol (vitamin D), which - to supplement their role in calcium homeostasis and bone metabolism - can act as an immunoregulatory factor.

It is also called the Marshall's syndrome, one of the most common and least developed child diseases. About those who are the Marshall's syndrome in children and how they rejoice, it is mentioned in these articles.

Coils of ailment

Formerly, spalls in Marshall's syndrome were documented in 1987 roci. At that time, medicine had little record of twelve such precedents. All attacks were similar to a similar illness: as a rule, there were periodic attacks of fever, when patients had stomatitis and swelling of the lymph nodes in the neck. In the English version, this syndrome is named after the capital letters of the main symptoms. In France, yoga was named after Marshall. Syndrome otrimav analogous to the meaning of the vіtchiznânіy medicine.

Symptoms

Under the hour of the cure of this illness, the French elders established that most of the disease affects children for a total of three to five years.
The main symptoms of ailments are regular, ale rіdkіsnі, sound periodicity once or twice a month, temperature cuts. With this, the child suffers from such cold symptoms, such as swelling of the lymph nodes on the neck and under the lower cleft, as well as fusing processes at the empty mouth and throat. It was established that the appearance of sickness in children is in no way related to their national, gender, or any other belonging. Manifest the syndrome also may have a clearly defined geographic areola.

Forecast of specialists

Most of the symptoms can be repeated for a period of several to eight years, which are periodically repeated in their typical manifestations of Marshall's syndrome. Signs of illness after the end of a severe break in the ailment sound like a trace. The development of the child under the hour of the break of the ailment does not stick and does not succumb.
Doctors mean that the prognosis for children, if they have transferred their diagnosis, may be positive. After a new relapse, there will be a recurrence of relapses and further normal physical, mental and neurological development of the child.

Relief of symptoms

One of the most common signs of the syndrome is an attack at a high temperature. Sound out there kolivaetsya at thirty-nine degrees and more. Sometimes the value of the thermometer can reach the sign of thirty-nine and five, or, more likely, the value of over forty.
Sound zastosuvannya be-any zasobіv zanizhenny spec no significant effect pіd hіkuvannya ailments Marshal. Syndrome can be used up only in case of complex lіkuvannі. As a rule, therapy with hormonal drugs.

Side effects

Krіm already knew before the lihomania, a scorching humiliation camp, typical for overcoming any serious illnesses, it can also be evidence of such an illness, like Marshall's syndrome in children. Diagnosis in children cannot be associated with a large number of scientific symptoms similar to other colds. Patients may show weakness, increased aggression. More than that, even more often in children, high temperatures in the Crimea, tremtinnya, white in m'yazakh, brushes and swamps. A lot of ailments also sting for a strong bіl head with Marshal's ailment. The syndrome can also be vibrating in the abdomen, more likely to be the presence of vomiting.

Regardless of those that the symptoms of Marshall's syndrome are already similar to those of colds, there are no more signs of infection. Sometimes in some children there may be teasing of the redness of the mucous membrane of the eye, as well as sleazy, cough, nasal congestion and vision of the new. Nerve disorders and allergic reactions, as well as other symptoms, were not noted.

Perebіg zagostrennya

The temperature has risen to disturb the child for about three to five days. However, the hour of lihomania is far from being a skin child, seeing the whole complex of symptoms that are considered typical for Marshal's ailment. The syndrome mostly affects the lymphatic system in the area of the neck. With this, the knots swell up to a couple of five centimeters, the stench becomes sharp and painful. In the majority of depressions, the swollen vuzleys become remembered by an unbroken eye, which is the most common reason for going to the doctor. Sound the lymph nodes, rotting in other parts of the body, they do not change in case of illness.

Accompanying symptoms

As a rule, a cream reaction from the side of the lymphatic system, in a child, causes irritation in the throat, sounds like pharyngitis or tonsillitis. Wines can pass in a milder form, fluctuations are prone, if the ailment is manifested as a clear infusion on one or both of the tonsils. In medical practice, it is necessary to bring up depressions in connection with ailments. Data from Greek scientists talk about thirty thousand children with symptoms of Marshall's syndrome in the middle, they recognized the procedure. At the same time, our American colleagues declare about twenty-two and one hundred and seventeen children, as if they knew about the operation, with post-onset tonsillitis and the onset of other Marshall's syndromes. Five of them were small for all known scientific symptoms of ailment. All the children, the cream of the inflamed tonsils, suffered from the blackness of the throat, and the axis of the feet developed tonsillitis in all of them. As a rule, after the passage of the sore throat, the tonsils change in the rosemary and no longer turbulate the child. Ignition is also known by itself.

Rіdshe, krіm inflamed lymph nodes and tonsils, in children the mucous membrane of the mouth is irritated. It will be guarded from three to seven out of ten vibrations.

Folding diagnostics

The problem with the diagnosis is due to such a factor that in young children it is quite easy to show all the signs of a difficult disease, such as Marshall's syndrome, that are necessary for diagnosis. Diagnosis is often made difficult because a child in three or five years is unlikely to be skarzhitisya fathers on the head or unacceptable in the region of the tonsils. On top of those, sometimes the signs of illness appear in full obsyazu chi through the singing interval of an hour.

Laboratory investigations show the progression of red blood cells in the patient's blood, as well as the possibility of fermentation of ignition processes as an increase in the blood count of leukocytes. It is also possible to change further changes in the volume of proteins at the plasma warehouse. As a rule, such haircuts with a few elements of the blood quickly turn to the norm. There were no more significant changes in the plasma warehouse than any of the typical signs for this syndrome.

Celebration

Science does not have a single idea about the treatment of children who have been diagnosed with Marshall's syndrome. There are a lot of symptoms, such as fever, undead, which cannot have any desired effect. Consider taking antipyretic drugs for the use of such symptoms, which are common for the disease, as the temperature rises, headache and chills appear insufficiently. The statistics are strong for their own line, which is sufficient for dressing. Analysis of the post-operative period to talk about those who, in seven out of ten ectomies, succumbed to the ailment. However, not all successors are happy with the fact that similar therapy can have such a strong effect on healing

Another way to treat the syndrome is to use such a drug as cimetidine. As practice shows, vin can restore the balance between T-helpers, as well as block receptors on T-suppressors. Three-quarters of the patients were similarly treated with a drug, but such a therapy did not come up with a wide spread.
In another way, exuberance is encouraged by the stosuvannya of steroids. Such an exaltation may have an effect in any case, if Marshall's syndrome had not been revealed. In children, likuvannya polagaє at zastosuvanni shock dose or a course of stretching decades. Call such procedures to help relieve the heat, but do not include repeated attacks. Irrespective of the past thought, that the very steriodial zdatnі shorten the period of remіsіy, such therapy can be the most wide-spread among fahіvtsіv. As a rule, vibir falls most often on the drug prednisolin, which is administered to a child in a dose of 2 milligrams per kilogram of body. Varto mother on uvazі, scho pіdboro steroid that recognized yоgo dozuvannya is guilty to take up only the doctor!