Marshal Syndrome: description, causes, diagnosis, symptoms and treatment. Features of the treatment of chick syndrome in children Disease Marshall

Clinical observations

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

The case of difficult diagnosis of the patient with Marshall syndrome

Asia Ildusna Safina1 *, Ildus Yaudatovich Lutfullyn1, Camille Ziaevich Zakirov2,

Valery Yuryevich Shapiro2.

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

A clinical observation of the patient with the first Marshall syndrome was given. Describes the diagnostic algorithm for the patient and possible options for therapeutic tactics. Keywords: periodic syndromes, Marshall 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 No. 1, Kazan City, Russia

Conducted Was A Clinical Observation of A Patient With Newly Diagnosed Marshall Syndrome. Described Was An Algorithm Of The Diagnosis of this Patient and Possible Therapeutic Tactics. Key Words: Periodic Syndromes, Marshall Syndrome, Diagnosis, Children.

Periodic syndromes are a group of auto-fixed diseases (Human AutoInflammatory Diseases - Haids) with periodically emerging non-infectious fever and signs of systemic inflammation. According to the classification of the European Society for Immunodeficiency, these diseases relate to primary immunodeficiency. Periodic diseases are developing against the background of a genetic defect in the system of inflammation regulators, which ultimately leads to an increase in the level of proteins of the acute phase, such as C-re-re-protein and serum amyloid protein A (SAA). A long-term increase in the latter can lead to the formation of the amyloi-dose of organs. Inflammation at periodic syndromes is primary, non-infectious (viruses, bacteria, fungi, simplest, etc.) or non-infectious (AU tantaitel, autosensitized T-lympho-quits, tissue decay products, etc.) agents.

The relevance of the problem of periodic syndromes for a pediatrician's doctor is related to the fact that in most cases they debut in childhood. Thus, the first manifestations of the family Mediterranean fever in 90% of cases fall on children's and adolescence, and Marshall syndrome is at age under 5 years. The possession of semiotics of periodic syndromes allows you to diagnose a timely diagnosis and avoid unnecessary medical manipulations, such as the appointment of antibiotics due to an angina during Marshall syndrome or diagnostic

laparotomy to a child with a family Mediterranean fever due to the clinical picture of the "acute abdomen".

Currently, several periodic syndromes are isolated: family Mediterranean fever, a periodic fever syndrome associated with a tumor necrosis factor receptor mutation I, periodic fever syndrome with a hyperimmunoglobuline-mia D and Marshall syndrome.

Periodic fever syndrome with thomatite, pharyngitis and lymphadenitis, Marshall Syndrome (Periodic Fever with Aphthous Stomatitis, Pharyngitis and Adenitis - Pfapa-Syndrome). Genetic and molecular mechanisms of pathogenesis of the disease are unknown, hereditary predisposition is not traced. Diagnostic criteria: periodic feral fever, the beginning of the disease at an early age (under 5 years), the symptoms of the lesion of the upper respiratory tract during the absence of infection, as well as at least one of the following clinical symptoms - aphodose stomatitis, cervical lymphadenitis, pharyngitis / Acute tonsillitis, lack of cyclic-trail, presence of asymptomatic intervals, normal growth and development.

The disease has distinct frequency, which can also serve as a diagnostic criterion. The attack of Marshall syndrome usually flows under the mask of purulent angina, aphthous stomatitis or cervical lymphadenitis. Therefore, the diagnosis of "Angina" in a child of 1-3 years should pay attention to the pediatrician, as it is extremely rare pathology at this age. It is noteworthy that the appointment of antibiotics and antipyretic means is not

Hematological indicators of the patient Y. at the age of one year and 6 months.

Lake, x 10 9 / l Leukocyte Formula EE, mm / h

Date P.Ya.N., S.Ya.n, Lymph., Mont., Eos., Diagnosis

10.11.08 7.5 1 15.5 63 13 7.5 4 Healthy

12/19/08 6.8 0 28 55 6 11 2 Healthy

01/29/08 8.5 1 28 55 6 10 3 Dorova

04/14/09 12.7 6 39 51 4 - 6 Aphtheasic Stomatitis

06/03/09 10.6 0 20 67 12 1 2 Healthy

07.06.09 10.8 8 41 46 5 0 6 ORVI

09.09.09 14.4 4 41 38 15 2 10 Pyelonephritis, vulvit

02.11.09, 27,9 24 45 27 4 - 26 Follicular angina

follicular

02/16/09 11 3 39 39 17 2 19 Angry, Aphtose Stomatitis

it affects the duration of the episode, which usually lasts 4-6 days and is resolved spontaneously. There are no specific laboratory criteria for diagnosing Marshall syndrome. A general blood test shows moderate leukocytosis with a neutrophilic shift to the left, an increase in ESR. The disease never leads to the formation of the amyloidosis of the kidneys or liver.

A single and twofold prescription of prednisolone in a dose of 1-2 mg / kg inside quickly stops attacks with a clinical picture resolution within a few hours. However, the purpose of glucocorticoids cannot prevent subsequent attacks. In addition, it is reported that prednisone can disrupt the internal rhythm of the disease and participating the attacks of fever. To date, the most effective method of treating the disease is tonsille-mia, which leads to curable 68-90% of patients. The disease is resolved spontaneously, only 3% of patients in achieving majority are preserved fever attacks.

In the pediatric department of the Children's City Hospital No. 1 of Kazan, from 10.03.2010, sick Yu is observed at the age of 1.5 years. The reason for appealing to the doctor was periodically recurring short-term episodes of febrile fever accompanied by an angina and aphthose stomatitis. The first episode of the febrile fever with the phenomena of the aphtheasian stomatitis was celebrated at 6 months of age. With a diagnosis of thomatitis, the patient was hospitalized for 9 days into an infectious hospital, where he received antibiotic therapy (cefassoline, ceftriaxone, amikacin), local treatment of stomatitis. Not a distinct effect from treatment with antibiotics. Fever was bought on the 6th day of treatment. In the future, within 11 months, the child had 6 episodes of febrile fever with 288

increase body temperature up to 40.0 ° C at intervals of 6-8 weeks.

With a graphic mapping of episodes of fever, their clear frequency and fixed duration have drawn attention. The mother of the child could definitely predict the beginning of the next attack of the disease. The hematological studies carried out at the time of feverish episodes showed that the attacks of the disease were not accompanied by neutropenia, but, on the contrary, were characterized by adequate state with inflammatory changes in the form of leukocytosis and neutrophilic shift "left" of various severities (see Table).

Bacteriological sowing smear from the 7/11/2009: Flora is not highlighted, dated 02/16/2010 - ß-hemolytic streptococcus 104 Come. During the last episode of the lacunar angina (02/16/2010), the level of procalcitonin in the blood was 0.61 ng / ml. Investigated markers of infectious diseases: CHL. trachomatis, cyto megalovirus (PCR) - OTP. from 10.08.2009, chl. Trachomatis, Micoplasma Hominis and Ureaplasma Urealyticum IgG and IgM (ELISA) - otf., Micoplasma Hominis IgG - weak. from 11/10/2009

When entering the hospital (10.03.2010), the child's condition is stable, satisfactory. On the scalp - hemangioma size 2x2 cm, the rest of the skin clean, physiological color. Zev is not hyper-mynted, almonds are not increased, loose. Rhythm heart tones, clear. In the lungs, the breath is vesicular, is carried out in all fields, there are no wheezes. The belly is soft, painless. The liver is palpable along the edge of the rib arc, the spleen is not palpable. A stroke from the mouth - the STRP is expelled. Pyogenes. Echocardioscopy - Open Oval Window, Heart Cameras are formed correctly. Inspection of the ENT doctor: forming chronic tonsillitis, STRP media.

pyogenes. The girl was discharged with a diagnosis: Marshall syndrome; Hemangiomas of the scalp; Open oval window. Recommended observation in the immunologist, the rehabilitation sanitation, as well as re-hospitalization at the next episode of fever.

12 days after the discharge (approximately 6.5 weeks after the last attack of the disease), a girl had a febrile fever with an increase in body temperature to 39.3 ° C.

Surveys were conducted: a general blood test - leukocytosis (12x109 / l), neutrophilic shift to the left (P.Ya.N. - 10%, S.Y.N. - 45%), acceleration of ESO (22 mm / hour); General urine analysis - without pathological changes. Biochemical blood test - hepatic, renal markers, nitrogen slags, mineral exchange indicators, bilirubin exchange, blood proteins - within normal values. The level of proteins of the acute phase (C-RB - 30 mg / dl, fibrinogen - 4.7 g / l), prokalcitonin - 0.2 ng / ml.

With the differential-diagnostic purpose, the girl was once assigned to the pre-zone at a dose of 10 mg inward (at the rate of 1 mg / kg). Antibiotics and antiboding drugs were not used. After the appointment of prednisy-leave, there was a quick relief of the main manifestations of the disease. The body temperature returned to normal after 3 hours after receiving a pre-nodulo, the fever did not recur in the future. Pump on the almonds disappeared after 5-6 hours, and Zea hyperemia - 12 hours after the prednisone destination.

Based on the distinct periodicity and fixed duration of the attacks of the disease, the ineffectiveness of antipirectrics and antibiotics in the control of the main manifestations, a characteristic clinical picture (short-term episodes of fever, 4 episodes of sharp tonsillitis and 2 - aphthous stomatitis), lack of a clear connection with infections, the efficiency of small doses of prednisolone in the relief Angina without antibiotics and antipirectrics

a refined diagnosis was set: Marshall syndrome; Hemangiomas of the scalp; Open oval window.

In order to determine the further tactics of conducting and clarifying the possibility of holding tonsilctomy, the patient was sent to a consultation to the Polyclinical Department of the RDKB (Moscow).

The above clinical example illustrates the possibility of diagnosing periodic syndromes based, primarily on a thorough analysis of the clinical picture and anamnesis of the disease. Periodic syndromes as diseases are very rare, nevertheless are not casual, and basic information on the characteristic manifestations of these syndromes should be in the "Luggage of Knowledge" a pediatrician.

LITERATURE

1. Harutyunyan V.M., Akopyan G.S. Periodic disease (etiopathogenetic and clinical aspects). - M.: Mia, 2000. - 304 p.

2. Barabanova O.V., Konoploya E.A., Produce A.P., Shcherbina A.Yu. Periodic syndromes // difficult. a patient. - 2007. - №2 - p.46-52.

3. Salikhov I. G. and others. Fever of unclear origin / Educational. benefit. - Kazan: Publishing House of KGMI, 1993. - 94 p.

4. BERLUCCHI M, MEINI A, PLEBANI A, ET AL. Update On Treatment of Pfapa-Syndrome: Report of the Literature // Ann Report of the Literature. Otol. Rhinol. Laryngol. -

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

5. DREWE E, HUGGINS M.L., MORGAN AG., ET AL. Treatment of Renal Amyloidosis WITH ETANERCEPT IN TUMOR NECROSIS FACTOR RECEPTOR-ASSOCIATED PERIODIC SYNDROME // RHEUMATOLOGY. -

2004. - Vol. 43 (11). - P.1405-1408.

6. Feder H.M. Periodic Fever, Aphthous Stomatitis, Pharyngitis, AdeNitis: A 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. Child. - 2002. - Vol.86. - P.434-435.

Marshal syndrome, experience in the use of classical homeopathy.

PFAPA-syndrome (Periodic Fever, Aphtousis Stomattis, Pharingitis, Cervical Adenitis) - Periodic Fever, Aphthosal Stomatitis, Faringitis, Adenit. The disease is also called Marshall syndrome (G. S. Marshall).

The PFAPA syndrome is formally not included in the group of hereditary periodic syndromes, since it has not yet detected the causal genetic factor (a specific mutant gene), but due to the similarity of the clinical picture, it is covered among these states.

Hereditary predisposition has not been proven. Describes sporadic cases. More often this syndrome is found in boys.

Marshal syndrome refers to a group of auto-fixed diseases caused by violations of the interaction of inflammation regulators.

Clinical signs

Febrile attacks with a duration of 3-5 days (a sharp start with chills and raising the Teotherapy of LP 40 and higher), which have a clear frequency

Aphtoxic stomatitis and / or pharyngitis + adenit

Interval 4-6 weeks (varies)

Early childhood

In the blood of leukocytosis, an accelerated ESP, an increase in the CRH,

According to observations of a number of researchers, antibiotics and antibiotic drugs are traditionally used in the treatment of this syndrome, although their effectiveness is minimal. Keeping the attack helps the use of hormone therapy (prednisone 1-2 mg / kg / day), but the latter does not prevent the appearance of the following attacks. Now a positive prednisone test in the attack of an unmotivated fever is one of the diagnostic criteria in the diagnosis of marchala syndrome.

Timely diagnosis makes it possible to abandon ineffective therapy with antibiotics and tonsilectomy. However, it was not found for effective treatment. Parents are advised to just wait for Pubertata (this, on average, from 4DU8 years), when the attacks will probably stop themselves without consequences.

The report presents 4 cases of successful marshal syndrome with the help of classical homeopathy. Experience shows that this disease representing the major difficulties for diagnosis and treatment is quite easy to treat classical homeopathy. It also deserves the causal factor that provoked the development of periodic fever: it is more often a strong fright or vaccination. For colleagues, homeopaths can be interesting to the fact that in all of the treated cases, the preparations of tuberculin miasm were effective. Most often it is a carbonics calcaret and tuberculin as an anti-amateur destination.

Pediatrician's doctor, Gomeopath Sidorenko E. V.

Clinic classic homeopathy named after S. Ganemann G. Moskva

Marshall syndrome It is a rare autosomal dominant genetic disease, which is caused by mutations in the COL11A1 gene. The main symptoms and manifestations of this syndrome include: a distinctive face with a flattened bridge, widely separated eyes, myopia, cataract and hearing loss.

Marshall Syndrome. Epidemiology

Marshall syndrome develops from both sexes in equal ratios.

Marshall Syndrome. The reasons

Marshall Syndrome is a rare autosomal dominant genetic disease, which is caused by mutations in the COL11A1 gene. This gene is located on chromosome 1P21.1. Most often, mutations causing Marshall syndrome are located on the splicing site.

Congenital spondylophyphizar dysplasia is a rare genetic disease, it is characterized by a growth deficit before birth (prenatal), spinal defects and / or anomalies affecting the eyes. Growth deficit, ultimately, leads to shortness (dwarfs). In most cases, children may have a reduced muscle tone (hypotension), kifoscoliosis, lumbar lordosis and / or an unusual shedding of the sternum.
Congenital syphilis is a chronic infectious disease, it is caused by spirochetes (pale spirochet), which are in the uterus of a pregnant woman. The symptoms of this disease can debut after a few weeks or months after birth, and in some cases they can manifest themselves only years later. Congenital syphilis is transmitted to a child from a mother who has acquired a disease before or during pregnancy. The symptoms of congenital syphilis include: fever, skin problems and low baby weight at birth. Late symptoms and manifestations include: Bone pain, vision pain, eyes in eyes and insensitivity to light, bony forehead, short upper jaw and deafness.
Sticar syndrome. This syndrome refers to a group of connective tissue disorders that affect several organs and organism systems (eyes, skeleton, inner ear, head and face). The sticker syndrome is particularly often affected by the connecting fabric of the eyes (very often in the vitreous body).
Wagner Syndrome is a very rare hereditary genetic disease. It is caused by mutations in the CSPG2 gene located on chromosome 5q13-14. Eye problems include: vitreoretinal degeneration, cataracts, retinal detachment (rarely).

Marshall Syndrome. Photo

Marshall Syndrome. Symptoms and manifestations

Patients have a distinctive flat sunken middle area of \u200b\u200bthe face with a flattened nose and a wide space between the eyes (hypertelorism). The skull arch is thicker than usual. Many patients have calcium deposits in the skull. The frontal sinuses may be absent. Eye defects that are found in patients with Marshall syndrome include: myopia, cataracts, wide space between eyes. Hearing loss can vary from light to severe, sound distortion is a consequence of nerve damage. Other symptoms and manifestations, in some patients with Marshall syndrome include: squinting (Esotropy), retinal detachment, glaucoma, and the absence of some nose bones.

Marshall Syndrome. Treatment

Plastic surgery can be corrected an abnormal shape of a nose in patients with Marshall syndrome. Other surgical procedures can also be used to remove cataracts with subsequent lenses implantation. Subsequently, contact lenses can help improve clarity. Using the auditory can be useful in some cases. Other treatment methods are only symptomatic and supporting.

Marshall Syndrome. Forecast

Forecast for patients is excellent, over time there will be a gradual decrease in the frequency of development of periodic fever and some complications.

Marshal syndrome is a rather rare autoimmune disease, which is found in children under 6 years. For the first time, such pathology was allocated separately in the 1980s of the last century. It is by this time that the first description of the signs of the disease, which periodically affects the body of the child, it would seem, against the background of complete well-being. Marshall Syndrome has several other names - periodically occurring syndrome, auto-surround disease, PFAPA. A feature of this ailment is that it manifests periodically and between the attacks is completely not reminded.

The reasons

Even with the modern development of medicine, Marshall syndrome is not studied as follows. The reasons for this pathology are still not defined, so it is practically impossible to prevent the ailment.

In this case, the child periodically increases the body temperature of up to 40 degrees periodically increases, fever and inflammation of the pharynx, almonds and oral cavity are observed. The disease is manifested periodically and a person can say for sure when the next attack will be. However, it is not possible to prevent such attacks possible, since doctors resort only to symptomatic treatment. Any therapy appointed with Marshal syndrome is aimed at relieving the symptoms of the disease, but is not able to prevent subsequent attacks.

There is an assumption that the disease arises due to certain autoimmune violations.

Although the causes of this pathology are still not known, doctors identify several factors that can contribute to the development of the disease.

Latent infection

Hidden viral infection. There is a whole list of virus latent infections, which for a long time do not show anywhere. Such viruses penetrate the body and accumulate in particularly sensitive organs. But infections are usually not manifested periodically, since the development of the disease provokes reduced immunity or unfavorable environmental conditions.

Similar viral infections include herpes. The carrier of this virus is a lot of people, but the disease is manifested only under certain conditions. Conducting factors can be overcooling, reducing immunity or other infection.

Chronic tonsillitis

Little kids often suffer an angina. In improper treatment, the disease quickly goes into a chronic form. Bacteria, which inhabit the almonds of the patient of the child, distinguish many toxic substances that lead to intoxication of the body. High temperature, inflammation of mucous membranes and fever can be the response of the immune system to inflammation.

The exacerbations of chronic tonsillitis in children of preschoolers sometimes proceed very hard. There is a high temperature, stomatitis and purulent raids on the glands.

Failure in the work of the autoimmune system

The most likely cause of Marshall syndrome in children is called autoimmune failures. Diseases of this group can be blocked for a while, but it is impossible to heal. In this case, the immune system for unknown reasons begins to perceive his cells as other people and fights them. This leads to a resistant increase in temperature and strong inflammation. What is interesting, there are no visible reasons for such a reaction.

The frequency of attacks may be different. In some children, attacks arise after 3 weeks, others in two months.

Clinical picture

Marshal syndrome in children proceeds with a number of characteristic symptoms. But even knowing the features of the course of the disease, physicians are sometimes very difficult to differentiate pathology with other diseases or autoimmune disorders. To make a diagnosis, the doctor carefully collects history, with the allocation of characteristic signs of the disease.

Fever. There are certain frequency of development of a feverish state. Periods of remission can continue from 2 to 7 weeks. The attacks are approximately once a month, but sometimes this time interval is less. Fever can last about 5 days, after which everything goes without a trace. As the disease develops, the intervals between the attacks of fever tend to increase.
Temperature. Marshall syndrome is characterized by a very high temperature. Temperature indicators can reach 41 degrees. This is accompanied by unbearable headache, violation of consciousness, delirium and chills. Such a temperature is severely shot by antipyretic drugs. If it is possible to shoot down a little, then for a short time.
Sore throat. In the sickness of Marshal often there is a pharyngitis. This disease is accompanied by severe throat pain, which is significantly enhanced by swallowing, dry oral cavity shells and cadia. The patient has a persistent feeling of finding a foreign body in the throat. All the symptoms of pharyngitis usually take place simultaneously with the attack.
Stomatitis. The disease often proceeds with stomatitis. In this case, painful ulcers appear on the mucosa of the mouth, and the body temperature rises greatly. Stomatitis may be a consequence of immune disorders.
Increase lymph nodes. In terms of Marshall syndrome, submandibular lymph nodes are strongly inflated. Their increase can be seen even with a naked eye. When attaching nodes, their pain and compacon can be noted.
Strong abdominal pain. A similar symptom with an autoimmune disease occurs approximately half of the sick children.
Pain in the joints and head. These signs are always present at high temperatures and fever.

Not all sick children have such symptoms manifest themselves completely. Most often only a few signs of the disease are observed, and they can all be of different intensity. The most visual symptoms of Marshall syndrome at the child are high temperature and manifestations of angina.

Marshall syndrome can be observed in children for several years. Usually this disease independently passes to adolescence.

Diagnostics

Diagnose this pathology is quite difficult, due to the lubricated symptoms and the absence of the causative agent. Only after several of the same type attacks, the doctor may have a suspicion of an autoimmune pathology. For diagnosis, such diagnostic methods use:

Collect anamnesis. It plays a paramount role in diagnosing the disease. The doctor carefully listens to the parents of the sick child, finding out how longcoming the attacks and the nature of their current flow began. In the event that parents can accurately determine the beginning of the subsequent attack, you can suspect Marshall syndrome. In addition, the specialist observes a child during periods between attacks. Usually at this time the general condition of children is not violated and they are developing normally.
Data inspection. During the exacerbation of the disease, the doctor carefully examines the child. During the inspection, inflammation of the mucous membrane, ulcers on almonds, stomatitis and an increase in lymph nodes are revealed.
Clinical blood test. For clarity, the painting of the patient is directed to the blood test. If the number of leukocytes is strongly increased and ESP increased, then we can talk about the inflammatory process in the body.

Marshall syndrome is not diagnosed for some particular criteria. It is possible to talk about this disease only with periodically repeated attacks.

At the initial stage, auto-pumping disease is often confused with chronic tonsillitis. This disease is also accompanied by an increased temperature, an increase in lymph nodes and ulcers on almonds. These pathologies can be differentiated only through time when the cyclicality of the attacks is manifested.

If the diagnosis is doubtful, the doctor may recommend a genetic analysis. Most recently, the disease gene was identified and this gene can mutate.

Treatment

Marshall syndrome is always symptomatic. Any treatment of this pathology is aimed at eliminating all symptoms, but not to prevent the next attack. Treatment with anti-inflammatory and antipyretic drugs rarely gives effect, more often such therapy leads to the recurrence of the disease. During treatment, these drugs can be used:

Antibiotics. Often are appointed at the very beginning of the disease, when there is a suspicion of the infectious nature of pathology. With Marshall's disease, antibiotic treats no effect.
Antipyretic drugs. Medicines based on paracetamol effect do not give. They are able to hit the temperature just a couple of hours. With this autoimmune disease, it is advisable to give the child drugs based on ibuprofen.
Hormonal preparations. Prednisolone is considered a fairly effective anti-inflammatory drug with a periodically arising syndrome. This hormone is a synthetic analogue of adrenal hormone and participates in many metabolic processes that occur in the body. It is prednisone that helps to reduce the seizures of up to 2-3 days.

It is believed that hormonal drugs help not only reduce the time of attacks, but also significantly reduce the period of remission. In this case, each subsequent attack begins earlier.

If the disease is complicated by hypertrophy of the almonds, then the doctor may recommend removing them. This is necessary in order to normalize the nasal breathing from the child. Tonsilloectomy is not able to heal autoimmune pathology, but it can reduce the likelihood of complications. To remove almonds, they are resorted only as a last resort, with properly selected therapy, such an operation can be avoided.

Constantly enlarged glands lead to a violation of nasal respiration, while the child feels the lack of oxygen. Such a state may lead to mental and physical development.

Complications

Marshal syndrome in children usually does not affect mental and physical development. If the child is regularly observed by the doctor and the appropriate treatment passes, then complications are extremely rare. But this autoimmune disease is often accompanied by other pathologies, which also disturb the state of the child:

Diarrhea. Little children fever often flows with strong diarrhea and stomach pain. This can quickly lead to dehydration of the body, in which the child's condition is only aggravated.
Blood violations. Neutrophenia is most often observed, while in the blood the number of neutrophils is significantly reduced, which leads to a resistant decrease in immunity. This is not manifested by any symptoms, but against the background of such a state, fungal and bacterial infections are often developing. In case of a heavy flow of neutropenia, the temperature is significantly increased, the work of the heart is disturbed and the limb trembling is observed.
Arthritis. This disease can develop if the inflammatory process affected the joints. The sick child is hard to move, it becomes irritable and fodder. The joints of the child can redden, become wound and deformed. Arthritis proceeds with periods of exacerbation and remission.
Neurological disorders. Marshal syndrome often flows with strong dizziness and fainting. Such disorders can be both in the period of exacerbation and during the remission.

With frequently repeated attacks, immunity strongly decreases, which is manifested by frequent diseases. Children with Marshal syndrome usually have a pale and unhealthy look.

Forecast

With early treatment, the prognosis is favorable. Many children are recovering after removing almonds, the rest of all the symptoms of the disease pass in adolescence. In rare cases, light neurological disorders may remain after recovery.

If among close relatives there were cases of Marshall syndrome, this worth reporting to the doctor. With this approach, diagnose pathology is much easier.

None prophylactic measures in the Marshall syndrome are ineffective. The reasons for this disease are still not defined, it means that it is impossible to prevent the occurrence of attacks, as well as to predict them. The disease usually begins against the background of complete well-being, without any prerequisites.

(PFAPA syndrome) is a disease of predominantly children's age, including periodic fever, aphthous stomatitis, pharyngitis, cervical lymphadenopathy. Symptoms are regularly repeated episodes of temperatures above 39 ° C, throat pain, ulcerative damage to the mucous membrane, an increase in cervical lymph nodes. The diagnosis is established on the basis of the clinic, blood tests, crops separated from the language, the exclusion of other possible causes of recurrent fever. Treatment is limited by glucocorticoids or antipyretic drugs, as antibiotics and antiviral agents are ineffective. In rare cases, tonsillectomy is carried out.

MKB-10.

D89.9 Violation involving the immune mechanism, unspecified

General

Complications

The complications of Marshall syndrome was not described. There were no long-term studies in patients. However, against the background of the aphtomatic stomatitis, pharyngitis is possible to attach a secondary infection, which can lead to the development of tonsillitis, cap abscesses, otitis, purulent mediastine. A long-term inflammatory process increases the risk of amyloidosis. In addition, the recurrent episodes of fever have an exhausting effect, forcing the child to miss school classes, can lead to failure.

Diagnostics

To formulate the diagnosis of PFAPA-syndrome, the diagnostic criteria proposed by Marshall (1987) are used: regularly repeated fevers from an early age (beginning of 2-5 years); Presence of one of the following clinical signs: Aphtose Stomatitis, cervical lymphadenitis, pharyngitis; Fully asymptomatic interval between fever episodes; normal physical and nervous psychic development of the child; Lack of cyclic neutropenia. There are currently no specific analyzes for the establishment of Marshall syndrome. Diagnostic search includes:

Consultation pediatrician, rheumatologist. Detailed collection of the patient's history is made: the history of the course of pregnancy and childbirth in mothers, heredity, features of nutrition, growth, child development, transferred diseases, information on vaccination, presence or absence of contact with infectious patients. The mucous membranes of the brush, pharynx, almonds are examined; Auscultation of the heart, lungs, measuring blood pressure, pulse; Palpation of belly, lymph nodes.
Clinical Biochemical Analyzes. During the fever, a general blood test identifies leukocytosis with increasing neutrophils, acceleration of ESO. In periods between attacks, all inflammatory parameters are normalized. Also during attacks, the level of C-reactive protein increases, the hepatic enzymes are not changed. Blood analysis by 25-OH may detect the deficiency of vitamin D3-cholecaliforol. IgG, IgA, IgM, IgD, procalcitonine, antinuclear antibodies, rheumatoid factor even with increasing body temperature remain normal.
Additional research. Sowing from the upper respiratory tract on the microflora and sensitivity to antibiotics, sowing urine, blood for sterility (at the height of the fever), the radiography of the chest organs, the apparent sinuses of the nose are performed to eliminate the infectious nature of the fever. With Marshall syndrome, pathological changes in these studies are not detected.

Differential diagnosis of Marshall syndrome is carried out with the following nosologies: return tonsillitis, infectious diseases, juvenile idiopathic arthritis, cyclic neutropenia, family Mediterranean fever (FMF), hyperglobulinemia syndrome D, Behchet's disease.

Treatment of Marshall syndrome

Treatment methods are still subject to disputes. Antibiotic therapy, the use of antiviral, antihistamine drugs do not have efficacy; NSAIDs have only a short-term antipyretic effect. To date, PFAPA-syndrome is successfully used for therapy:

Corticosteroids. One or two doses of prednisone (1-2 mg / kg), betamethazone (0.1-0.2 mg / kg) can dramatically stop the fever attacks within a few hours. Other accompanying symptoms require more time to resolve. Steroids are applied only during the attacks, the specified dosages do not cause toxic effects. Glucocorticoid therapy is able to reduce the interval between the attacks, but does not prevent recurrences.
Colchicine. It can be effective to prevent frequent fever episodes, it does not affect the course of the fever. The side effect are gastrointestinal disorders (in 20% of cases). Several studies of this drug were conducted, most of them - in Israel, where a large proportion of patients carry pathogenic variants of MEFV.
Cimetidine. In studies in small groups, about a quarter of patients (24-27%) had a complete permission of fever episodes at its reception, and 24-32% reported partial efficiency with a decrease in frequency or severity of attacks.
Anakinra. Currently, recombinant antagonists of the Interleukin-1β receptor (Anakinra) receptor antagonists are considered, studies of this group of drugs are carried out. All patients demonstrated clinical improvement, reducing the level of cytokines in the blood.
Tonsillectomy. It is a radical method that leads to complete cure. The operation should be performed only in the case of intolerance or inefficiency of standard drug therapy due to certain risks of invasive intervention (bleeding, anesthesia complications).

Prediction and prevention

All manifestations of Marshall syndrome are usually independently resolved before adolescence. Fatal consequences and serious complications are not described in the literature. Despite the favorable forecast, when similar complaints, it is necessary to refer to children's experts (pediatrician, otolaryngologist, dentist) for examination, the diagnosis and selection of adequate therapy. Specific prevention methods do not exist. Patients suffering from these syndrome, it is recommended to use vitamin D3 at a dose of 400 meters in winter.