Гипофосфатаземия у детей: анализ результатов лабораторных исследований за 10 лет

The analysis of fatal outcomes of myasthenic crisis in patients with myasthenia. The data on 19 patients with myastheniccrisis admitted at the Moscow Regional Research and Clinical Institute («MONIKI») over 12 years (1997-2009) have been summarized. There are more than 600 patients with myasthenia in the Moscow region, including 13% patients with onset after 60 years. Seventeen patients of 19 were in the intensive care unit. Total death occurred in 7 cases (36.6%). Myocardial infarction, bilateral confluent pneumonia and hemorrhagic pulmonary edema caused death. All patients had concomitant diseases: hypertension, myocardial changes, hyperglycemia and others. Steroids and plasmapheresis were used for treatment of myasthenic crisis. The authors believe that methods of treatment of myasthenic crisis that does not lead to complications in elderly age and are suitable for patients with concomitant diseases, who can't be treated with glucocorticoids and plasmapheresis, should be used in clinical neurology. Normal human intravenous immunoglobulin is recommended in these cases.

Diagnosis of neoplasms, especially malignant, of the larynx and pharynx and their differential diagnosis with non-tumor lesions is one of the most urgent and socially significant problems in modern oncology. Over 3000 patients with suspected cancer of the larynx and pharynx were examined in the Moscow Regional Research and Clinical Institute over the last 10 years. In most cases a CT scan was performed. Rare non-tumor diseases were confirmed in 0,5 % of the cases. In all the cases further clinical laboratory tests were carried out after the radiation examination, confirming the non-tumor nature of the identified changes, which fundamentally changed the tactics of treatment.

BackgroundBiologic disease-modifying antirheumatic drugs (bDMARDs) are increasingly used for treating patients with different rheumatic diseases. The safety and effectiveness of using biologics therefore become important issues of the rheumatologists' practice.ObjectivesTo...

Background. Currently, the incidence of complications and perioperative mortality in cardiac surgery is still higher than in general surgery. This is partly associated with the development of oxidative stress, which is regarded as excessive accumulation of reactive oxygen species and nitrogen species. There are nowadays few studies demonstrating an increase of nitrotyrosine plasma level in patients during cardiac surgery. The relationship of these changes with post-operative complications and adverse outcomes has not been studied yet. Aim. Investigation the dynamics of the nitrotyrosine plasma level in patients after cardiac surgery and the assessment of its prognostic significance in terms of the development of early postoperative complications.Methods. The prospective observational cohort study involved 28 adult patients admitted at the cardiac intensive care unit of Moscow Regional Research and Clinical Institute (Moscow, Russian Federation). The relationship of the oxidative stress severity measured by intraoperative nitrotyrosine plasma levels and the development of acute kidney injury, cardiac failure and systemic inflammatory response syndrome was studied. Results. There was no significant perioperative dynamics of nitrotyrosine plasma levels when studying this oxidative stress marker in patients undergoing cardiac surgery. The level of nitrotyrosine plasma by the end of surgery was 12.1 [9.9; 13.0] nmol/mg of protein, which was 5% higher (p>0.5) than the initial level of 11.6 [9.3; 12.2] nmol/mg of protein, and returned to 11.5 [10.9; 12.4] after the 1st postoperative day. The same perioperative dynamics occurred in the subgroups with different surgery techniques: valve surgery, сoronary artery bypass grafting with cardiopulmonary bypass and off-pump сoronary artery bypass grafting.Conclusion. The negative result achieved in the given study might suggest that there are no significant changes of the perioperative nitrotyrosine plasma level in patients undergoing cardiac surgery, as well as that there is no significant correlation between the nitrotyrosine plasma level of and the incidence of postoperative complications.Received 26 December 2016. Accepted 4 May 2017.Funding: The study was carried out within the state-funded research project “Oxidant stress in cardiac surgery: new markers-predictors of complication development and pathogenetically substantiated therapy” granted to Moscow Regional Research and Clinical Institute.Conflict of interest: The authors declare no conflict of interest.Author contributionsConceptualization, study design: V.V. Likhvantsev.Data collection and analysis: T.S. Zabelina, Yu.V. Skripkin, O.N. Ulitkina. Drafting the article: O.A. Grebenchikov, Zh.S. Philippovskaya.Critical revision of the article: O.A. Grebenchikov.Final approval of the version to be published: V.V. Likhvantsev.

Background: The expected ageing of the population and the increase in the number of patients with osteoporosis require a correct approach to identification of individuals with a high fracture risk, to their rational routing for the work-up and timely prescription of therapy. A preliminary analysis of characteristics of patients referred to specialized osteoporosis centers may help to optimize medical care for these patients.
 Aim: To analyze the profile of patients referred for densitometry and/or to an osteoporosis specialist at the Moscow Regional Osteoporosis Centre in order to optimize care for this patient population in the region.
 Materials and methods: The study was performed in the Moscow Regional Osteoporosis Centre located in Moscow Regional Research and Clinical Institute (MONIKI). We retrospectively analyzed medical files of all consecutive outpatients attending the center at their own or with a referral for densitometry and/or specialist consultation January 1 to December 31, 2019.
 Results: The study included 1940 patients aged 42 to 90 years, with 96.5% (n = 1873) being female, with the mean age of 65.4 8.5 years; males accounted for 3.5% (n = 67), their mean age was 65.6 9.0 years. Patients who had not previously been diagnosed with osteoporosis accounted for 63.8% (n = 1238), whereas 36.2% (n = 702) of the patients have been diagnosed earlier. 27.8% (n = 540) of all patients, were remarkable for the history of low-energy fractures. 88.1% of the patients were referred by endocrinologists or rheumatologists. The main purpose of the referral was to perform densitometry and get the prescription of treatment for osteoporosis (61%).
 The median FRAX-based risk of major fractures in the total group was 8.2 [6.9; 13.0] % (n = 1277), with the risk in women being significantly higher than in men (8.3 [7; 13] % vs. 4.6 [4.2; 7.7] %, respectively, p 0.001). When bone mineral density was assessed with the T-score (n = 1928), the result of +0.1 SD was found in 6.6% (n = 127), whereas 11.6% (n = 224), 44.4% (n = 856), 37.4% (n = 721) were judged as normal values, osteopenia, and osteoporosis, respectively.
 According to the specialist consultation in the Centre, a clinical diagnosis of osteoporosis was made in 53.2% of the cases (n = 1032), while 0.3% (n = 5) had another bone disease, 19.2% (n = 373) had no bone disease, and 27.3% (n = 530) had some other problems. The proportion the patients with indications for treatment for osteoporosis was 56% (n = 1089); 45% (n = 489) of them were recommended to initiate therapy, 36.7% (n = 400) continued with their previous medication and 18.3% (n = 200) needed a modification of their management.
 Conclusion: The study results have shown that patients are frequently referred for densitometry without enough grounds and with a low risk of fracture. This makes highly relevant the use of the FRAX algorithm as a screening tool for diagnosis and timely administration of treatment for osteoporosis not only by specialists, but also by primary care physicians.

The article is devoted to the differential diagnosis of hypertrophic form of lichen planus (CPL) with acrokeratosis verruciformis of Hopf (AVH). It is very rare hyperkeratotic genodermatosis usually inherited in an autosomal dominant manner. Due to the marked clinical similarity of these two dermatoses crucial for the correct diagnosis is histological study of the lesion with the identification of histological feature unique for AVH - papillomatosis as a “church steeples”. Two clinical cases diagnosed last year in the department of dermatology and dermatooncology in Moscow regional research and clinical institute are described in the article. The effectiveness of the treatment AVH with acitretin was described.

The laser “in vivo” autofluorescence diagnostics is now widely studied and applied in different areas of medicine, such as an oncology, dermatology, etc. Recently we have reported of created new professional multiwave laser diagnostic system (MLDS) for this purpose under the international scientific research and development project #1001 supported by the International Scientific and Technology Center. This presentation lights some results of application of the MLDS in a real clinical practice at Moscow Regional Research and Clinical Institute “MONIKI”, Department of Radiology. With the use of MLDS we investigated a skin and oral cavity cancer endogenous fluorescence before, during and after standard radiotherapy treatment. A statistical analysis showed that the best radiotherapy result was achieved for the patients with a small initial porfirines’ autofluorescence and a great initial flavines’ one from irradiated tumor tissues. It was shown that each radiotherapy procedure has an influence on a tissues’ autofluorescence intensity. The tendencies in porfirines’ fluorescence during a treatment course can be an additional prognostic factor for the prediction of the efficacy of a radiotherapy treatment. Moreover, it was estimated that a number of non-cancerous skin disease has a typical “cancer” initial autofluorescence, that makes it difficult to distinguish them one from another with the use of only the fluorescence diagnostics, but opens the way to investigate the non-cancerous tissues diseases with the help of tissues endogenous fluorescence phenomenon.

Background: Research into the pathogenesis and morphogenesis of new coronavirus infection (COVID-19) is currently a hot topic that requires the accumulation, management and analysis of the data. Understanding the mechanisms of the disease and their characteristics would help to improve diagnostic quality, treatment efficacy and reduce mortality. Aim: To identify morphological and immunohistochemical characteristics of the bronchopulmonary lymph node tissues in autopsied patients with COVID-19. Materials and methods: The study was carried out on 44 autopsy samples from patients who were hospitalized and died from the new coronavirus infection COVID-19 in the Moscow Regional Research and Clinical Institute. Macroscopic, histological, and immunohistochemical assessment methods were used. Results: We found stereotypical abnormalities in the morphology and cellular composition of the lymphoid tissue depending on the duration of the disease. These included signs of B-cell-mediated immunity suppression manifested by a decrease in the number and size of lymphoid follicles and occurs at latest after 7 days from the onset of the disease. In the patients with the disease duration of up to 14 days, the T-cell pool of the lymph nodes is represented mainly by CD4 + T-lymphocytes. With longer duration of the disease, there is a gradual increase in the number of lymphoid follicles and their sizes, with changes of the CD4 + to CD8 + T-cell ratio towards higher CD8 + counts. After 21 days of the disease, the CD4 + to CD8 + ratio levels off. Conclusion: The results obtained indicate a direct damaging effect of SARS-CoV-2 on the lymph node tissues of the bronchopulmonary group. The possibility of secondary transient immune deficiency and infectious complications in patients with a new coronavirus infection is discussed.

To describe the methodology of laparothoracoscopic Ivor Lewis esophagectomy in surgical treatment of esophageal cancer and compare early outcomes of this procedure with conventional Ivor Lewis surgery. There were 30 laparothoracoscopic Ivor Lewis esophagectomies followed by non-hardware esophageal-gastric intrapleural anastomosis for esophageal cancer. All procedures have been performed for the period 2016-2019 at the Moscow Regional Research and Clinical Institute (suturing of anastomosis was based on the method of professor A.S. Allakhverdyan). Laparothoracoscopic esophagectomy is characterized by higher surgery time by 136.57 min (p=0.012), less duration of anesthesia and mechanical ventilation by 77.5 min (p=0.042), postoperative ICU-stay by 2.25 hours (p=0.021), blood loss by 550 ml (p=0,000), duration of postoperative fasting by 2 days (p=0.034), hospital-stay by 8 days (p=0.021) compared to open esophagectomy. There were no significant between-group differences in the number of resected lymph nodes (p=0.142). Incidence of esophageal-gastric anastomosis failure is insignificantly higher in the OE group (χ2=1.89; p=0.075). Incidence of pulmonary complications (pneumonia, chylothorax, paresis of the vocal cords, pleural empyema) is less in the LTSE group (p<0.05). Cardiovascular morbidity is significantly lower in the LTSE group (p<0.05). A 30-day mortality rate was similar in both groups (χ2=2.56; p=0.0253). Early results of laparothoracoscopic Ivor Lewis esophagectomy are superior to the results of conventional Ivor Lewis surgery in surgical treatment of esophageal cancer.

Rationale: Liver transplantation is the only curative treatment for diffuse end-stage liver disease and some liver neoplasms. The amount of these interventions in the Moscow Region is very low.Aim: To analyze the results of the first series of liver transplantations done in the Moscow Regional Research and Clinical Institute (MONIKI), to compare it with those done currently in Russia and worldwide, and to establish the optimal volume and trend of development for this new regional center.Materials and methods: More than 200 patients with liver cirrhosis, polycystosis and alveococcus invasion have been examined from May 2016 to August 2018; 70 of them were eligible for liver transplantation and were put on the waiting list. From October 2016 to July 2018, 29 liver transplantations from deceased donors (including 2 retransplantations) and one living related transplantation of the right lobe have been performed. Among the indications to the transplantation, the leading one was viral (HCV or HBV-related) cirrhosis. Four patients were diagnosed with hepatocellular carcinoma.Results: The waitlist mortality was 19%. Median waiting time was 5.5 [3; 9] (0 to 27) months. Until now, the results were followed till 22 months, with median follow-up of 7 [2; 13] months. The survival rate of the recipients was 96.4%, of the grafts 93.3%. In-patient mortality was 3.6%. Early allograft dysfunction was seen in 33% of cases. Median length of the in-hospital stay was 22 [19; 25] days.Conclusion: The successful implementation of the liver transplant program at its initial stage demonstrates the results that meet current efficacy criteria. Achieved level of organ procurement from deceased donors in the Moscow Region could ensure at least 30 liver transplantations annually, with current facilities and a potential for further growth. An increase in the transplantation number would depend on the improvement of transplantation service facilities in MONIKI and on the stable financial support of the program. Finally, it would promote increased availability of this transplantation technology in the region, lower waitlist mortality and shorter waiting times.

Atherosclerosis is a chronic disease in which lipids, cells and various proteins accumulate in the walls of the arteries, forming atherosclerotic plaques. The growth of plaques leads narrowing of the lumen of the blood vessels. Atherosclerosis is accompanied by local inflammation, while the number of hematogenous macrophages are derived from monocytes increases in the vascular wall. The reasons why the inflammatory reaction cannot be completed and becomes chronic are not clear. To resolve inflammation and protect tissues from high concentrations of cytokines that can cause apoptosis, there is a mechanism of immune tolerance of innate immunity. Lipopolysaccharide (LPS) tolerance of monocyte-macrophages is a phenomenon in which cells reduce their sensitivity to repeated exposure to LPS. This condition is characterized by a decrease in the ability of macrophages to produce proinflammatory cytokines and promotes resolution of inflammation. We hypothesized that in atherosclerosis, tolerance violations in monocyte-macrophages are possible. The study included patients who were admitted to the department of cardiac surgery, Moscow Regional Research and Clinical Institute (MONIKI). Patients were divided into patients with coronary atherosclerosis (CAD) with detected stenosis in 2 or more arteries and healthy controls without stenosis in the arteries according to the results of coronary angiography. In the present study, we examined the ability of macrophages from 13 patients with CAD and 11 patients without CAD to develop tolerance to LPS. To do this, we isolated CD14+ monocytes from the blood by positive selection using immunomagnetic separation and subjected them to two sequential LPS stimulations, immediately after cell isolation and after 6 days of culture. The secretion of cytokines TNFα, IL-1b, IL-6, IL-10, IL-8, and CCL2 was measured in cell culture supernatants using by ELISA. Our results showed impaired macrophage tolerance for CCL2 secretion and improved tolerance for IL-8 secretion in macrophages from patients with CAD compared with patients without. Since IL-8 and CCL2 are chemoattractants for other immune cells, it can be assumed that the observed impairment of macrophage tolerance to LPS in atherosclerosis increases the infiltration of other monocytes into the inflammatory site, contributing to the chronicity of inflammation.

Rationale: Lichen planus (LP) is an important problem for the healthcare due to its increasing prevalence, as well as typical, atypical and prone to malignization types of the disease that can be both severe and poorly responding to treatment. Accurate data on the prevalence of LP are lacking. Aim: To assess the prevalence of various LP types in the patient population of the Moscow Region. Materials and methods: We analyzed the results of examination of 338 patients with LP, who were getting standard treatment in the Department of Dermatovenereology and Dermato-oncology of the Moscow Regional Research and Clinical Institute (MONIKI) in 1999 to 2017. Results: Among 338 LP patients, atypical LP of the skin was diagnosed in 81 (23.96%): hypertrophic type, in 31 (38.27%), pigmented, in 23 (28.4%), atrophic, in 11 (13.58%), follicular, in 8 (9.88%), lichen obtusus, in 3 (3.7%), bullous, in 1 (1.23%), erythematous, in 1 (1.23%), vulvovaginal-gingival syndrome, in 2 (2.47%), and Grinspan's syndrome, in 1 (1.23%). In 35 (43.21%) of patients, cutaneous LP was associated with atypical oral LP (OLP): in 17 (48.57%) of cases with hyperkeratotic, in 11 (31.43%), with erosive, and in 7 (20%), with exudative-hyperemic type. In 19 (23.46%) cases, atypical cutaneous LP was associated with LP of the genital mucosae, including in 12 (63.16%) cases with a typical reticular type, in 6 (31.58%), with atypical erosive, and in 1 (5.26%), with hypertrophic type. In 16 (4.73%) of the patients there was atypical OLP only; in 6 (37.5%) of them it had the exudative-hyperemic type, in 5 (31.25%), erosive, and in 5 (31.25%), hyperkeratotic. The most severe course of the dermatosis was observed for follicular, atrophic, hypertrophic types of cutaneous LP, vulvovaginal-gingival syndrome, and erosive OLP. Conclusion: Atypical LP was diagnosed in 81 (23.96%) cases, and in 45 (55.6%) cases it was associated with the OLP. The latter in 35 (43.21%) of cases was characterized by atypical lesions of OLP, including 11 (31.43%) cases of obligate precancerous erosive LP, which necessitates that this patient category should be managed with high cancer vigilance.

Background: Follicular thyroid nodules or suspected follicular thyroid nodules are classified as Bethesda IV, being found in 10% of fine needle aspiration biopsy samples. Standard ultrasound examination in patients with thyroid follicular neoplasia is insufficient to give a definitive answer about the risk of the nodule malignancy, which affects the treatment tactics. Definitive tumor verification requires a pathological assessment of the macropreparation obtained during surgical treatment. Purpose: To develop additional ultrasound criteria for the differential diagnosis of follicular thyroid neoplasia. Material and methods: The study included 62 patients who underwent treatment at the Moscow Regional Research and Clinical Institute named after M.F. Vladimirsky from March 1 to November 11, 2020 The patients had Bethesda 4 cytology report result at admission, the mean age was 52,5±13,7 years, the ratio of women to men was 53:9. Based on the set of ultrasound criteria, the nodular formations were classified according to TI-RADS 2020 Additionally, the quantitative characteristics of blood flow in intra-nodular vessels were assessed by spectral Doppler ultrasound. After the surgical treatment and the pathological verification, the ultrasound data obtained before surgery were compared with the morphological data. The analysis of digital data was carried out by methods of variation statistics with the calculation of the mean values, standard deviation, and 95% confidence interval. Sensitivity, specificity, and other criteria were calculated to quantify the performance of the ultrasound criteria in assessing the malignancy risk of thyroid nodules. T-test for independent groups was used when comparing spectral indices in the intra-nodular vessels between the groups of benign and malignant tumors. The results were considered statistically significant at p-value of less than 0.01. Results: Bethesda 4 nodules were categorized by TI-RADS as follows: TI-RADS 3 – 7 (12%) nodules, of which only 1 (14%) was cancer; TI-RADS 4 – 41 (66%), of which 5 (12%) were malignant; TI-RADS 5 – 14 (23%) nodules, of which 10 were cancers (71%). The diagnostic performance assessment showed low sensitivity values (less than 75%) of all the “major” and “minor” signs of malignancy, with sufficient specificity values (more than 80%). An additional comparison of the spectral characteristics of blood flow in the intra-nodular vessels of the nodules with the pathological examination data was carried out: the mean vascular resistance index was statistically significantly different (p=0,0001) between the intra-nodular arteries of the benign (0,54±0,13) and malignant (0,78±0,17) tumors. Conclusion: When describing thyroid nodules and categorizing them according to TI-RADS, the “major” and “minor” signs of malignancy need to be considered. An increase in the vascular resistance index in intra-nodular arteries can serve as an additional malignancy criterion.

A congenital liver cyst is a rather rare pathological condition in most cases failing to manifest itself clinically. True liver cysts are an indication for surgical treatment. A newborn baby with a congenital true liver cyst admitted the Department of Pediatric Surgery of the M.F. Vladimirsky Moscow Regional Clinical and Research Institute for the treatment. Lack of knowledge about this pathology and diagnostic errors can lead to incorrect surgical tactics.

Abstract. In March 2016 the urological clinic of the Moscow Regional Research and Clinical Institute named after M.F. Vladimirskiy is 85 years old. The article presents a biography of the head Clinics (1951-1975), Abramyan Aram Yakovlevich (Akopovich).