Congenital hyperinsulinusm is rare disease characterized high secretion of insulin by pancreatic beta cells leading to the development of hypoglycemia. Persistent and transient forms of hyperinsulinism are distinguished. Transient hyperinsulinism are the most common cause of severe hypoglycemia in newborns. The etiology of this disease is not known. There are risk factors for the development of transient hyperinsulinism: asphyxia at birth, prematurity, maternal diabetes, low or large weight by gestation. Hypoglycemia with hyperinsulinism is severe. Therefore, early diagnosis and therapy especially during the neonatal period, are necessary.The article describes 3 clinical cases of transient hyperinsulinism in children with different gestational age and concomitant pathology. All children recevied insulinostatic therapy with diazoxide with a positive effect: euglycemia without glucose requirement . In all children, therapy was completed subsequently. At the time of publication of the article, the physical and psychomotor development of children is normal.

The uncontrolled course of bronchial asthma (BA) in children and insufficient efficacy of standard therapy regimens may be due to underestimated infectious factors. The objective: to study specific parameters of the course and treatment of mycoplasma infection, improve monitoring over BA therapy in children of the tender and preschool age. Subjects and methods. 320 children with BA in the age from 1 to 7 years old were followed up. In this work, Mycoplasma pneumoniae (FH), Mycoplasma hominis (H-34), Ureaplasma urealyticum (serotype 8), Mycoplasma fermentans (PG18) and Mycoplasma arthritidis (PG6) were used, they were cultured on a liquid medium for cultivation of mycoplasmas and ureaplasmas. To isolate CIC from blood serum samples, we used the method of precipitation with 3.5% polyethylene glycol (PEG, 6000 Da), hemagglutination assays and IFA were used to identify mycoplasma antigens, mycoplasma DNA was detected by PCR with InterLabService diagnostic kits. The data of 47 patients with prolonged mycoplasma antigenemia were assessed at the baseline and in 1.5-3 months after the treatment course of azithromycin.Results. 320 blood serum samples from children with BA were tested, and the detection rate by hemagglutination assays of M. pneumoniae antigens was 60.9%, M. hominis – 43.4%, U. urealyticum – 44.8%, M. arthritidis – 29.7%, M. fermentrans – 45.3%. The assessment of relationship between of M. pneumoniae, M. hominis and asthma exacerbation showed that antigens of M. pneumoniae and M. hominis were found in 216 children (single or associated). After treatment with azithromycin, the frequency of BA exacerbations within 3 months decreased by 2.4 times, as well as there was a reduction in the number of samples positive for antigens and DNA of mycoplasma in a free state and within CIC. The persistence of antigens, DNA of M. pneumoniae and M. hominis before treatment of 47 children was 80.9 and 66.0% of cases, after treatment with azithromycin – 31.9 and 25.5% of cases, respectively (p < 0.001). Within CIC isolated from the blood serum of patients, antigens to M. pneumoniae and M. hominis before treatment were detected by IFA in 63.8 and 70.2% of children, after treatment – in 31.9 and 23.4%, respectively. p < 0.001. In blood samples, DNA of M. pneumoniae and M. hominis was detected by PCR before treatment in 8.5 and 34.0%; after treatment in 6.4% (p = 0.318) and 19.1% of cases, respectively (p = 0.009), and within CIC isolated from blood serum, in 27.7 and 48.9% of cases before treatment and 8.5 and 34.0% after it, respectively (p = 0.009).

Supplemental PEG tube feeding improves the nutritional status (BMI, body weight) of patients with very severe CF.

Start of the CFTR modulating therapy is an important and promising step towards better treatments for all cystic fibrosis (CF) patients. Therapy with CFTR potentiator Ivacaftor resulted in dramatic improvements in CFTR function in patients with G551D mutation, later it was prescribed for patients with another 9 gating mutations. We present a case of successful treatment of a CF patient with G461E/N1303К genotype. We have suggested that due to gating mutation G461E, the patient may profit from the treatment with ivacaftor. After six months of treatment, sweat chlorides concentration decreased from 93 mmol/l to normal 50 mmol/l, respiratory function (FEV1) increased from1.44 L(48%pred) to2.0 L(67%pred). The clinical course of the disease has changed significantly. The patient became stable; symptoms of chronic bronchitis diminished. There are other gating mutations in the Russian National Registry that may profit from treatment with ivacaftor.

The major risk factor for death in cystic fibrosis (CF) is a progressive lung injury; however, low nutritional status (NS) remains an important and underestimated problem in the management of these patients. The NS of a patient with CF is impaired by many factors, such as chronic malabsorption, pancreatic failure, chronic inflammation, and recurrent pulmonary infection - they all lead to higher energy demand in the presence of lower intake of nutrients. The NS of those with CF plays an important role in maintaining lung function. The patients with higher NS have longer life expectancies. According to the Russian National Registry, both pediatric and adult patients with CF have shorter life expectancies. The article discusses various nutritional support regimens and their impact on lung function parameters.

Background. Improvement of control methods of treatment of mycoplasma infection in children with bronchial asthma. Methods. 52 children from 1 to 4 years old with bronchial asthma who had mycoplasma antigens in the blood were examined before treatment of mycoplasma infection, 15 patients were examined in 1,5 months after treatment. Reaction of aggregate-hemagglutination, immunofluorescence assay and the reaction of the polymerase chain reaction (PCR) were used for detection of mycoplasmas’ antigens and DNA. Results. In reaction of aggregate-hemagglutination Mycoplasma pneumoniae (Mpn) was detected in 65,4% of patients, Mycoplasma hominis (Mh) — in 32,7%, Ureaplasma urealyticum — in 50%, Mycoplasma arthritidis — in 46,2%, Mycoplasma fermentrans — in 46,2%. In more detail, we investigated Mpn and Mh. Antigens of Mpn and Mh were detected in serum significantly frequently than DNA by PCR. DNA in structure of circulating immune complexes (CIC) was detected more often than in free state. After treatment by azithromycin the number of positive tests on antigens and DNA in free state and in structure of CIC decreased. In most cases DNA found in serum of blood in free state and in structure of CIC belongs to persistent living cells of mycoplasma. Conclusion. Empowering diagnosis of mycoplasma infections in asthma in children allows to base a comprehensive approach to laboratory diagnosis, monitor treatment, predict disease course.

In the presented case report, 36-year-old female patient, who was hospitalized in the rheumatology department, developed thrombotic microangiopathy and sepsis-related coronavirus disease 2019 (COVID-19). In the department, hereditary thrombophilia was revealed: heterozygous polymorphism in the coagulation factor 5 gene (Factor V Leiden). Livedo reticularis. It is known that in October 2020, the patient had COVID-19 with bilateral pneumonitis on chest computed tomography. On examination, dry gangrene of 1, 2, 4, 5 fingers of the right hand, 2 finger of the left hand, as well as 2, 3, 4, 5 fingers of the right and left feet were diagnosed. Necrosis of the nasal tip. Surgery was performed with amputation of the affected fingers and distal feet with autodermoplasty.

In the presented case report, 36-year-old female patient, who was hospitalized in the rheumatology department, developed thrombotic microangiopathy and sepsis-related coronavirus disease 2019 (COVID-19). In the department, hereditary thrombophilia was revealed: heterozygous polymorphism in the coagulation factor 5 gene (Factor V Leiden). Livedo reticularis. It is known that in October 2020, the patient had COVID-19 with bilateral pneumonitis on chest computed tomography. On examination, dry gangrene of 1, 2, 4, 5 fingers of the right hand, 2 finger of the left hand, as well as 2, 3, 4, 5 fingers of the right and left feet were diagnosed. Necrosis of the nasal tip. Surgery was performed with amputation of the affected fingers and distal feet with autodermoplasty.