Progressive multifocal leukoencephalopathy (PML) is one of the most severe opportunistic diseases of the central nervous system, which leads to multiple demyelination of brain structures, neurological symptoms and frequent death or disability of the patient. The etiological factor of this disease is Human polyomavirus 2 (JCPyV).This pathogen is widespread – antibodies are found in 80% of the world›s population. However, the clinical symptoms of this infection appear only in people with a pronounced decline in cellular immunity. Until 1980 progressive multifocal leukoencephalopathy was extremely rarely diagnosed. Now days the main cause of the clinical symptoms of PML is immunodeficiency caused by HIV infection. Clinical manifestations of PML are characterized by various non-specific neurological symptoms, similar to other lesions of the central nervous system, the symptoms progress slowly over several months, and usually lead to death. Diagnosis of PML is based on laboratory and instrumental methods, such as DNA JCPyV detection in the cerebrospinal fluid, brain biopsy, and radiation diagnostic methods. There is no effective prevention and etiotropic therapy for PML. Improved parameters of cellular immunity and antiretroviral treatment in HIV positive patients significantly increase the life expectancy of patients with PML. Despite the ability of drugs to prevent the progression of the disease, pathological changes in the brain are irreversible and lead to persistent disability of patients, therefore, it is necessary to diagnose PML in the early stages of the disease.

Generalized forms of meningococcal infection are characterized by high risk of complication and fatal outcomes. In Russian Federation, 2016, meningococcal infection was the second most common cause of pediatric deaths (25%) due to infectious diseases after community associated-pneumonia. Mandatory vaccination against meninococcal infection is not regulated, and immunization coverage according to epidemic indications is insufficient. During first 6–8 hours of illness clinical signs of this infection may be non-specific, because of that it is difficult to make correct diagnosis at outpatient department.
Aim of the study was to analyze mistakes of diagnostics of meningococcal infection and to assess factors influencing the disease outcome.
Material and methods. Retrospective cohort study was performed including 113 pediatric cases of generalized meningococcal infection occurred in Arkhangelsk region (46 cases with fatal outcome and 67 patients, who recovered). Factors influencing the outcome were recognized using Cox regression.
Results. Median age of patients was 11 months. Clinical forms were meningitis (13,3%), meningococcemia (40,7%), mixed form (46%). The diagnosis of meningococcal infection was made at outpatient department in 36,9% of cases. The main causes of diagnostic mistakes were the lack of experience among primary care physicians and non-specificic symptoms during the first hours of the disease. Age younger 2 years, septic shock and time between onset of the diseases and admission to the hospital were associated with fatal outcome.
Conclusion. To manage meningococcal disease we need to have good compliance with clinical guidelines for outpatient departments and hospitals; to optimize medical students education for prevention of diagnostic mistakes. Vaccination is the most effective method of prevention of deaths associated with meningococcal infection.

Aim. In this study we retrospectively evaluated the clinical effectiveness and safety of nucleoside analogues (Triazavirin© and ribavirin) with that of oseltamivir for treating moderate severe influenza in adults.
Materials and methods. We have used data from 191 health records of patients with moderate severe PCR confirmed influenza A and B. Control group included 57 patients treated with oseltamivir, comparison groups – 53 and 81 patients, who received Triazavirin© and ribavirin accordingly. We compared infectious intoxication syndrome duration, fever duration, duration of acute rhinitis, acute pharyngitis, acute laryngitis, acute tracheitis, acute bronchitis, cough duration as well as pneumonia occurrence.
Results. No statistically significant difference in the duration of developed syndromes in Triazavirin©-treated group and oseltamivir-treated group were observed. We have noticed that fever duration had been significantly longer in ribavirin group compared to control group (4,1±2,22 days vs. 3,1±1,94 days, p<0,05) as well as more frequent pneumonia occurrence (1,2% vs. 0,0%, p>0,05).
Conclusion. New nucleoside analogue Triazavirin© showed good efficacy and safety profile in adult patients with influenza. This fact provides the opportunity to recommend it for treatment of influenza along with neuraminidase inhibitors.

Objective: to study the clinical and epidemiological features of tularemia to identify its differences from other diseases. flowing with lymphadenopathy.
Materials and methods The study included patients living in the Kirov region undergoing tularemia from 2012 to 2018. For the laboratory confirmation of the diagnosis of tularemia used agglutination with tularemia antigen and a method of solid phase ELISA.
The results Among the patients were dominated by citizens (63.4%). All cases were recorded in the summer. Infection usually occurred during the bites of flying insects. The most common manifestations of the disease were fever and lymphadenitis. Among the clinical forms prevailed ulcero – glandular (71,7%). The correct diagnosis at the prehospital stage is exhibited only in 28.6% of patients.
Conclusion Tularemia remains an actual natural focal infection. To reduce the incidence of disease, it is necessary to increase the volume of vaccination of the population and regularly improve the knowledge of physicians with regard to endemic infections in the region.

Objective: to establish clinical and epidemiological patterns of chickenpox in adults in the Primorsky Territory to improve treatment and prevention work.
Materials and methods: The study used statistics on the incidence of chicken pox in the Primorsky Territory from 2009 to 2017 and demographic data on the population for the specified period. A clinical characteristic of 102 cases of varicella in patients hospitalized in the infectious disease ward of Primorsky Regional Clinical Hospital No. 2 (Vladivostok) in 2015-2017 is given.
Results: From 2009 to 2017, the incidence of chickenpox in the Primorsky Territory was consistently high, with an average of 636.2 per 100,000 population. Analysis of the structure of patients showed that the maximum proportion of cases of chicken pox occur in organized children 3-6 years old (55.9%), schoolchildren (19.1%) and children 1–2 years old (11.6%). The proportion of 15–17 year olds was 3.0%. Adults, from 18 years old – 4.9%. The proportion of unorganized children 3–6 years old (2.9%) and children in the first year of life (2.6%) is not high. High risk of infection was detected in Vladivostok (57.8 per 100.000), Yakovlevsky (52.1% ooo), Olginsky (45.9% ooo), Lazovsky (42.8% ooo) and Shkotovsky
(40, 2% ooo) areas. In the annual dynamics, a pronounced spring-summer seasonal rise was formed. The clinical course of varicella in the group of adults was characterized by an average duration of the initial period of the disease of 1.4 ± 0.7 days. For clinical diagnosis, an important time was the appearance of the rash, which varied within four days. The phenomenon of “spillage” was noted in 97% of patients. The rash was polymorphic, with the formation of crusts. The period of height was characterized by the presence of catarrhal manifestations. The granularity of the posterior pharyngeal wall was observed in all patients without exception. In 84.3% of patients there was a bright hyperemia of the pharynx. In 14.7% of cases, enantema was detected on the soft palate, palatine arches, and posterior pharyngeal wall. Dry cough was detected in 28 of 103 patients, which probably increased their epidemiological significance.
Findings: The clinical picture of varicella was typical and was represented by a combination of intoxication, exanthema, and catarrhal syndrome.
The epidemic process of varicella in adults in Primorsky Krai was characterized by relative autonomy of manifestations in adults, children up to one year old, and unorganized children. When implementing chickenpox epidemiological surveillance, priority should be given to interventions in epidemic foci.

The aim: to study the etiological structure of acute respiratory infections in children aged 3 to 12 hospitalized in the early stages of the disease in the department of respiratory infections of the children’s hospital, and to reveal the features of their clinical course and the timing of DNA / RNA elimination of respiratory viruses from nasal secretions, depending on the method of therapy.
Materials and methods: 100 children with acute respiratory infections aged 3 to 12 years were monitored. The nasal secrets on the DNA / RNA of respiratory viruses were studied by PCR. Depending on the method of therapy, patients were divided into 2 groups: patients of group 1 (comparison) received basic treatment (without the use of antiviral drugs), in patients of the 2nd group (main), along with basal therapy, the drug was used umifenovir in a 5-day course at the ageappropriate dosage.
Results: In the etiologic structure of ARVI in children from 3 to 12 years, the leading place was taken by rhinovirus, influenza and metapneumovirus infections (isolated – 18%, 19% and 20% respectively, in the form of a mixed infection – 11%). The main syndromic diagnosis at the height of the disease was rhinopharyngitis. Complications were observed in 42% of cases, as often as possible with flu – 53% of cases. Features of metapneumovirus infection in children of this age group were: predominance of non-severe forms of the disease in the form of acute fever with symptoms of rhinopharyngitis, as well as a small incidence of lower respiratory tract infections. The use of the drug umiphenovir in children with acute respiratory viral infections of various etiologies contributed to significantly faster elimination of viral DNA / RNA from the nasal secretion, which was accompanied by a ecrease in the duration of the main clinical and hematological symptoms of the disease, a decrease in the incidence of complications, and reduced the duration of stay in hospital.
Conclusion: application of modern molecular genetic methods of diagnostics made it possible to identify the leading role of influenza, metapneumovirus and rhinovirus infections in the etiology of acute respiratory viral infection in patients aged 3 to 12 years, and to determine a number of clinical features characteristic of this age group. The results of the study testify to the effectiveness of umiphenovir in the treatment of children with acute respiratory viral infections of various etiologies and allow us to recommend this drug as an effective and safe etiotropic agent.

Aim. To estimate the distribution of genotypes and subgenotypes of the hepatitis B virus among military personnel with chronic viral hepatitis B.
Materials and methods. The work used samples of blood plasma and biopsy material obtained from 90 active or retired military personnel with chronic viral hepatitis B with various degrees of fibrosis undergoing treatment in St. Petersburg. Primary detection of HBV was carried out by isolating nucleic acids (NK) from the blood plasma using the «AmplePrime Ribo-prep» commercial kit (FBIS CRIE, Moscow). Specific primers were used for the amplification and sequencing reaction. Overlapping primer pairs were used, jointly flanking 1475 base pairs (bp) fragment, including the recommended for HBV genotyping the 1169 bp Pre-S1/Pre-S2/S.
Results. Among 90 samples from patients with chronic viral hepatitis B from different regions of the Russian Federation, HBV subgenotypes are represented in the following ratios: D2 = 45.6% (n=41), D1 = 32.2% (n=29), D3 = 13.3% (n=12), A2 = 6.7% (n=6), D4 and A1 by 1.1%, respectively. The distribution of HBV subgenotypes from the North Caucasian federal district (D1 – 63.6%, D2, D3, D4, A2 – by 9.1%) was significantly different from the distribution among patients from the Central and North-Western federal districts (D1-20, 9%, D2 – 58%, D3 – 16.3%, A2 – 4.8%) (χ2=11,9 при p=0,0076, df=3). Uncharacteristic for the Russian Federation subgenotypes D4 and A1, representing single imported cases. The tendency to shift the distribution of genovariants due to imports of the corresponding HBV subgenotypes from other countries, including the Central Asian countries, is discussed.
Conclusion. A systematic study of the HBV isolates phylogeny provides new information about the HBV subgenotypes distribution among certain population groups, including military personnel.

The acute intestinal infections (AII) with a haemocolitis syndrome represent group of a serious illness at children that defines relevance of studying of their etiology and pathogenesis.
The purpose of the work is to study the clinical and laboratory features of AII with hemocolitis syndrome depending on the etiology and complications.
Materials and methods. Observed 77 patients of AII aged from 3 months up to 16 years. The etiological diagnosis verified using bacteriological and PCR studies of feces with AmpliSens® OKA screen-FL reagents and serological methods.
Hemocolitis syndrome was observed in all patients. Patients with AII of a clarified etiology formed group 1 (n = 59), with AII of unclarified etiology – group 2 (n = 18). Intercurrent diseases were detected as non-specific complications of acute intestinal infections: respiratory (RI) and urinary infections (UI). AII severity was determined by the Clarke-index, the severity of dehydration – according to the WHO clinical scale. To evaluate the data of a research, the Mann-Whitney U-test and Pearson correlation coefficients were used.
Results. There was a difference in the age of children in group 1 (3.9 ± 4.2 g) and group 2 (2.2 ± 2.6 g; p = 0.03) and in the frequency of complications: RI is diagnosed in group 1 for 54.5% of children, in group 2 for 83.3% (p=0.001); UI – at 45.5% and 16.7% respectively (p=0.03). In complicated course of AII, macroscopic signs of hemocolitis and inflammatory changes in the hemogram with the inclusion of platelet hemostasis in group 1 observed significantly more often than in group 2. In children of group 1, with uncomplicated acute intestinal infections, there was a significant correlation of signs of inflammation with dehydration and a higher severity of the disease according to the Clarke-index than with a complicated course.
Conclusion. The age of children with AII of a clarified etiology was 3.9 ± 0.6 years, with AII of an unclarified etiology – 2.2 ± 0.6 years. AII of a clarified etiology preceded more hard, than AII of unclarified etiology. Complicated course of AII with hemocolitis syndrome characterized by expressiveness of signs of the local and system inflammatory answer.

Goal. To estimate a possibility of correction laboratory abnormalities (increased level of cholesterol and/or triglycerides at blood of HIV-positive children) by switch the boosted PI on HIV integrase inhibitor (Raltegravir).
Methods. The retrospective study was conducted in group of 58 HIV-positive children (less 18 yo). Inclusion criteria were increasing level of cholesterol in blood (more than 5,0 mmol/l) and/or the triglyceride (more than 2,3 mmol/l) ART with boosted PI. The ART regime was changed for all children (n=58). Boosted PI was replaced to integrase inhibitor (RAL). RAL formulation (chewable tablets, 25mg and 100mg) used accordingly weight. Time horizon of observation and laboratory control after boosted PI switch was 24 months.
Results. Lab abnormalities in study group (n=58) after switch to RAL were changed: reliable decrease in level of cholesterol (p <0,01), triglycerides (p <0,001), viral load (p <0,001) and growth CD4 count (p <0,05).
Conclusion. Boosted PI switch to integrase inhibitor is providing long-term and effective HIV management and improvement of lipid abnormalities among children.

Background: Assessment of the informativeness of using various parameters characterizing the epidemic process during rotavirus infection to analyze the effects of the RotaTeq (MSD, USA) pentavalent rotavirus vaccine’s using at low (<20%) level of vaccination coverage of the target cohort.
Materials and methods: Were analyzed the correlation links between the vaccination coverage rates and the number of reported cases of rotavirus infection, incidence rates, the number of rotavirus-positive laboratory tests and their shares among the examined children for the territories of Moscow and the Moscow Region in 2014-2018, using the database of the laboratory information system and data of the Federal statistical monitoring,
Results: The presence of a strong reliable inverse correlation between the coverage of vaccination and the only of the analyzed indicators – the share of positive results of laboratory studies in the age group of children 6-24 months was revealed. There was a one and a half to two-fold decrease in the share of positive laboratory tests for rotavirus infection in the years of reaching 18–20% vaccination coverage.
Conclusion: The obtained data indicate the high informative value of laboratory information system data and the perspective of their use for a comprehensive assessment of the activity of the epidemic process.

The aim of the study is to determine the clinical and epidemiological features of measles in the Altai territory.
Materials and methods: a retrospective analysis of 92 medical histories of inpatient patients with a confirmed diagnosis of «Measles» at the age of 17 to 54 years, who were treated in the Infectious Departments of City Hospitals № 5 and №11 in Barnaul in 2015–2018, was made.
Results: the epidemic situation of measles in the Altai territory is characterized by an increase of morbidity rate in 2015, as well as isolated cases in 2017–2018. The peculiarity of the outbreak of the disease was in-hospital infection of patients upon admission to medical hospitals and late diagnosis of measles, as well as the importation of infection from adjacent territories (regions of Russia and the Republic of Kazakhstan), which is confirmed by genotyping of the virus in patients. The development of the disease in 23,9% of those,who were vaccinated, was routinely observed in the age group older than 30 years. In the clinical picture of measles at the present stage, along with the classical manifestations, in 15.2% of patients in the first days of the disease gastroenteritis was marked, in 40,2% of patients the signs of hepatitis (cytolysis syndrome) were observed.
Conclusion: the prevalence among the patientspersons, older than 30 years, may indicate the extinction of post-vaccination immunity. Timely immunization against measles in adulthood (after 30 years), will increase the effectiveness of measures to implement the program of elimination of measles in the Altai territory. The development of gastroenteritis up to the 5 days of the disease (on average 2,5±0.9 days), hepatitis in the period of height of the disease (in 15,2% and 40,2% of patients, respectively), determined the erroneous diagnosis of «Enterovirus infection», pseudotuberculosis, etc. and deserved the attention of practitioners.

Congenital cytomegalovirus infection contributes substantially to the incidence of sensorineural hearing loss, which may be late-onset, progressive or fluctuating. It leads to delayed diagnostics of hearing impairments in children.
The aim is to assess the frequency and time of hearing loss occurrence in children with congenital cytomegalovirus infection and to develop the audiological follow-up algorithm adapted for those children.
Materials and methods: 60 children with verified congenital cytomegalovirus infection have been involved into research as the main group. 61 children, with other sensorineural hearing loss risk factors, but excluding congenital cytomegalovirus, were included into the comparison group. The age of children ranged from 2 months to 7 years old. The follow-up duration was up to 4 years. Audiological assessment included: auditory brainstem response, auditory steady state response, otoacoustic emissions, impedancometry, pure tone audiometry (its type depended on the children’s age and development level) and speech tests.
Results: Hearing loss was revealed in 17% of the main group, it varied from mild to severe degree. Among these children 30% developed late-onset hearing loss. 5% of children of the comparison group were identified with congenital hearing loss. Assessment of central auditory pathways function has been performed in children elder than 4 years old: 70% of children of the main group and 10% of children of the comparison one were identified with auditory processing disorders.
The received data may be used by otolaryngologists, audiologists, pediatricians and infectious diseases physicians. The developed follow-up admits timely diagnostics of sensorineural hearing loss in children with congenital cytomegalovirus.

Objective: to offer additional criteria for assessing registration quality of congenital infections and neonates’ pyoseptic infections.
Material and methods: a retrospective epidemiological analysis of congenital infections incidence, pyoseptic infections of puerperas and neonates during 2007–2017 was conducted in this study. All subjects of the Russian Federation were divided into some groups according to registered incidence rate and ratio among congenital, pyoseptic infections of puerperas and neonates with using the method of ranging. Comparative correlation analysis was conducted between puerperas’ pyoseptic infections and neonates’ congenital infections in accordance with registered rate. The sample of the date was taken from Statistical Reporting Form No 2 «Report on infectious and parasitic diseases» for the Russian Federation during 2007–2017, Unified interdepartmental information and statistical system.
Results: in most regions of the Russian Federation the registration of healthcare associated infections puerperas and neonates is at a low. More than a half of subjects have low incidence rates (less than 1,0) for one or more of the considered infections. Until now, there are several subjects that do not register healthcare associated infections at all. The absence of correlation relation between the morbidity of the puerperas’ pyoseptic infections and neonates’ congenital infections on the whole in the country confirms this fact. At the same time, the correlation analysis of separate regions with the registration close to the real figures has shown a strong positive relationship between puerperas’ pyoseptic infections and congenital infections.
Conclusion: thus, it can be concluded that the presence of relation between rates of incidence of puerperas’ healthcare associated infections and neonates’ congenital infections can be used as one of the epidemiological criteria for assessing the completeness of the registration of healthcare associated infections.

Aim: Analysis of the morbidity of vaccine-associated paralytic poliomyelitis and acute flaccid paralysis and the results of virological investigation of the patients on 14 territories of the Russian Federation in 1998-2017.
Materials and methods: We investigated nearly 3000 stool samples from paralytic patients and contact persons. Isolation and identification of polioviruses were performed according to WHO recommendations with the help of cell lines RD and L20B. We conducted the sequencing of the genome fragments VP3-VP1, VP1-2A and full sequencing of genome region VP1 of 45 poliovirus strains.
Results: From 1998 till 2017 1257 cases of acute flaccid paralysis were registered on 14 territories of Russia, 15 cases of which (1,2%) were classified as vaccine-associated paralytic poliomyelitis. From these patients 9 children were non vaccinated and 6 children received from one to four doses of oral poliomyelitis vaccine. The percentage of the detection of polioviruses from the patients and contact persons in different years was not equal and constituted from 3, 4±0,89% to 9, 5±0,79%. All in all from the patients with acute flaccid paralysis and contact persons we isolated 191 polioviruses, 60 of them belonged to type 1, 55 polioviruses were identified as types 2 and 76 as type 3. Some cases of vaccine-associated paralytic poliomyelitis are described in the article; polioviruses were isolated from all these patients. The sequencing of the genome fragments of 45 poliovirus strains showed that the majority of them had the nucleotide substitutions including neurovirulent substitutions.
Conclusion: In order to prevent the risk of the appearance of vaccine-associated paralytic poliomyelitis it is necessary to maintain the high quality of surveillance of poliomyelitis and acute flaccid paralysis, to ensure the 95% coverage of children with poliomyelitis vaccine, to minimize the cases of groundless delays of vaccination according to medical recommendations and parents’ refusals to vaccinate children against poliomyelitis and to respect strictly the National calendar of vaccination.

Background. In the Russian Federation the number of a parvovirus В19 infection cases has increased significantly with the introduction of rubella into the measles elimination program and laboratory examination of patients with maculopapular rash and fever.
The aim of study was the examination of the distribution of the parvovirus В19 infection in the North-Western Federal District.
Materials and methods. In 2014–2017, 1044 blood sera of patients with exanthema diseases from different territories of the North-Western Federal District have been investigated with «Anti-Parvovirus B19 ELISA IgM» kit (EUROIMMUN, Germany) to detect IgM antibody, and 733 blood sera of clinically healthy men and women 18 to 60 years old have been tested for IgG antibodies using the «Anti-Parvovirus B19 ELISA IgG» kit (EUROIMMUN, Germany).
Results. Parvovirus В19 infection is contentiously detected in 10 of the 11 territories of the district. Typical is the winterspring seasonality; in terms of the age structure – prevalence of children 3–6 (25,3% of cases) and 7–14 (33,3% of cases) years old was detected. A high proportion of seropositivity was established among the examined donors from the higher educational institutions of Saint Petersburg (75,4–88,9%%). Low rate of seropositivity (56,7%) was detected among pregnant women. A high proportion of false negative results in the primary diagnosis of parvovirus infection has been revealed.
Conclusion. Parvovirus В19 infection is widespread in the territories of the North-Western of the Russia, mainly among children. The results indicate the feasibility of conducting a laboratory examination of pregnant women who are contact for exanthemous diseases, for markers of parvovirus infection; on the importance of screening donor blood for PV B19 DNA with “culling” pools characterized by high viral load; on the need for differential laboratory diagnosis between rubella and parvovirus infection.

The case of diagnostics and differential diagnostics of a case of a visceral leushmaniosis at the child of early age with development an infectionassociated hemophagocytosis is provided. This complication demands carrying out broad diagnostic search, and therapy in our country represents considerable difficulties as drugs for treatment of a visceral leushmaniosis in Russia are unregistered.

Metal constructions can cause fever even after a few years post implantation. This paper describes a case of 27 year old female with a fever associated with titanium construction implanted for kyphoscoliosis. 3 years post-operatively she developed afever (up to 39°С) and associated back pain. Examination revealed a hepatomegaly and lymphadenopathy (cervical and inguinal groups). Other investigations showed: neutrophilic leukocytosis (16×109\L), thrombocytosis (620×109\L), elevation of the C-reactive protein (76 ng/ml), ESR (62 mm/h) and ferritin (1615 ng/ml). There were no features of infection, connective tissue disease or hemoblastosis. A short course of therapy with methylprednisolone led to transient improvement. The metal device was surgically removed which caused an immediate elimination of fever and subsequent resolution of leukocytosis, thrombocytosis, lymphadenopathy and hepatomegaly.

Objectives. Actinomycosis in children has not been studied enough, the number of publications is limited. Actinomycosis of the knee joint is very rare, the disease mimics tuberculosis or malignant neoplasms, which can lead to late diagnosis and inadequate treatment.
Materials and methods. We report on the first case of an 8-year-girl with actinomycosis knee. Diagnosis is based on histological examination.
Results. Surgical resection of infected tissue and longterm antibiotic therapy for 4.5 months was effective in the treatment of pediatric patient with knee actinomycosis. In the scientific literature no such clinical case was found.
Сonclusion. Case of successful treatment of pediatric patient with knee actinomycosis is presented for the first time.

Family hemophagocytic lymphohistiocytosis (hemophagocytic syndrome) is a rare hereditary disease, which is based on a disturbance of the regulation of the immune response, leading to proliferation and activation of histiocytes, phagocytosis of peripheral blood cells. The most common mutations include – PRF1, UNC13D, STX11. Two cases of familial hemophagocytic lymphogystyocytosis in children of an early age from a single family, features of the course are described.

The article describes a case of the spontaneous esophagus rupture (the Boerhaave syndrome) in a 4-year-old child developed simultaneously with an acute intestinal infectious disease. The main reasons of prolonged diagnostic process are poor awareness of most of the medical specialists about the Boerhaave syndrome, the rarity of the disease and the diversity of clinical implications. Any case complicated with this orphan disease needs an emergent surgery operation. The development of the Boerhaave syndrome ends fatally in most cases.