bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Pharmaco-Economic Analyzis of Treating Severe Uncontrolled Child Asthma with Omalizumab — Actual Russian Clinical Practice Data
Authors: Kolbin A. S. , Namazova-Baranova L. S. , Vishneva E. A. , Frolov M. Y. , Galankin T. L. , Alekseeva A. A. , Dobrynina E. A. 
Published: January 2017

Background:Omalizumab is the first and yet the only biopreparation for asthma which combines high efficiency and high cost. The clinical-economic expediency of using omalizumab in asthmatic children has not been previously studied in Russia.Objective:Our aim was to evaluate the clinical and economic expediency of using omalizumab as additive treatment (to basic or supporting therapy) in children with severe uncontrolled atopic asthma under the Russian economic conditions.Methods:We conducted a mathematical simulation of asthma treatment in children with an increased frequency of hospitalization (9 times per year) with an average monthly omalizumab requirement of 558 mg. The model is based on the Markov chain. The model includes direct and non-direct costs. The planning horizons were 2 and 5 years. We analyzed the efficiency and utility of the costs and their influence on the budget. The stability of received data is proven by sensitivity analyzis.Results:Over a 5-year planning horizon the cost of an additional year of quality life (due to using omalizumab) was 1,259,185 roubles, while the “society’s solvency” is 1 341 308 roubles (cost utility analizis). It takes 39,820 rubles to prevent one hospitalization with omalizumab over a 5-year planning horizon (cost efficiency analizis), which is comparable to the cost of hospitalization (43,141 rubles). Total costs for treating 100 children with asthma, 7 of which would be treated with omalizumab, were equal to the amount of money which is enough to treat 105 children without omalizumab (analysis of budgetary influence).Conclusion:The analyses of cost efficiency and utility have shown that the strategy of using omalizumab together with standard treatment is economically expedient. Budgetary influence analysis has not detected a significant burden on the budget.
Demographic, Clinical and Genetic Characteristics of Child Gaucher Disease Patients in Russia: Pediatric Register Data
Authors: Movsisyan G. B. , Gundobina O. S. , Namazova-Baranova L. S. , Savostyanov K. V. , Pushkov A. N. , Chernikov V. V. , Mazanova N. N. , Romanyuk A. M. , Smirnov V. I. 
Published: January 2017

Background: Registers are an effective tool for tracing the dynamics of patients with rare pathologies.Objective: Our aim was to examine the demographic, clinical and genetic features of child Gaucher disease patients in Russia.Methods: We held a retrospective survey of the pediatric register data with regard to children suffering from Gaucher disease. The period of data accounting was from 2006 to 2016.Results: 115 children with Gaucher disease aged from 3 months to 17 years (the median age of diagnosis is 5 years) were registered; 62 them (53.9%) are girls. The prevalence of the disease was 0.32 cases for 100,000 children. 95 (82.6%) children had 1st type of Gaucher disease, 6 (5.2%) — 2nd, and 1 (12.2%) — 3rd. Maximum morbidity was in Central (27; 23.5%) and Volga (27; 23.5%) Federal Districts; minimal — in the Far East (3; 2.6%). By the time of diagnosis all the patients were suffering from splengomegaly. The genotype and phenotype correlations in 90 children with Gaucher disease were as follows: in case of 1st type (n = 77), in 21 (27.3%) cases, the p.N370S/р.L444P genotype was set, in 12 (15.6%) — the р.N370S/other mutation; in case of 2nd and 3rd types, in 13 children with neuropathic forms, in 9 (62.9%) cases — the p.L444P/p.L444P, in 3 (231%) — the p.L444P/p. D409H. The rest of genotypes were presented by other mutations, 13 of which were revealed for the first time. The p.W223R (p.W184R) mutation is specific for Russian patients. Enzyme replacement therapy was carried out for 109 patients (94.8%): in 105 (96.3%) children (1st and 3rd types of Gaucher disease) with imiglucerase, in 4 (3.7%) children with 1st type — with velaglucerase alfa. Pathogenetic treatment stops the main symptoms in most patients.Conclusion: The pediatric Gaucher disease register allows to systemize the data concerning the disease course in children and optimizing the approaches to its monitoring in Russia.
Evaluating the Physical Development of Early Age Schoolchildren (7–10 Years): Cohort Study Results
Authors: Khodzhieva M. V. , Skvortsova V. A. , Borovik T. E. , Namazova-Baranova L. S. , Margieva T. V. , Bushueva T. V. , Melnichuk O. S. , Nekrasova S. V. 
Published: January 2017

Background:Misbalance between energy intake and consumption is considered the main reason of obesity. However, over the recent years there has been a lot of emerging data concerning early origins of obesity that forms during intrauterine development and/or early age periods.Objective:Our aim was to study how physically developed are the children of early school ages.Methods:The study included children aged 7 to 10 years. Their physical development was assessed with the WHO AnthroPlus (2009) software.Results:652 children were examined. Of them, balanced development was found in: according to the WAZ index (body mass/age) — 466/530 (87,9%) schoolchildren; HAZ index (height/age) — 620/652 (95,1%); BAZ (body mass index/age) — only 438/652 (67,2%) children. Excessive body weight was found in 61 (18,8%) of the 324 girls and 65 (19,8%) of the 328 boys (р = 0,891), obesity — in 24 (7,4%) and 52 (15,9%) children correspondingly (р = 0,038).Conclusion:Having evaluated the physical development of early aged schoolchildren between the ages of 7 and 10 years, it is possible to state that there is evidence in favour of higher readings of mass-weight indexes as compared to the standard WHO population. Every third early age schoolchild can have physical development deviations, at the same time boys are obese twice as often as girls.
TISSUE ENGINEERING AND REGENERATIVE MEDICINE TECHNOLOGIES IN THE TREATMENT OF ARTICULAR CARTILAGE DEFECTS
Authors: Basok Yu. B. , Sevastianov V. I. 
Published: January 2017

Some of the most pressing health problems of the industrial society are the damage and degeneration of articular cartilage associated with the limited capacity of tissues to regenerate. The review describes the existing and developing technologies for the recovery and replacement of damaged joint cartilage tissue. The results obtained are analyzed covering two major areas: the stimulation of regeneration of damaged cartilage tissue and the growing of cartilage tissue elements in bioreactors.
BIOBANKS ARE AN ESSENTIAL TOOL FOR TRANSPLANTATION. HISTORY, CURRENT STATE, PERSPECTIVES
Authors: Reznik O. N. , Kuzmin D. O. , Skvortsov A. E. , Reznik A. O. 
Published: January 2017

Organ shortage remains to be a crucial issue in transplantation. However, it’s not the only challenge that modern transplantation is facing. There are critical issues to be solved, such as, choosing optimal patient-specific immunosuppressive regimen, preventing or coping effectively with graft rejection and improving graft and patient survival rates. There are still a number of ongoing studies on ischemia-reperfusion injury mechanisms. It is also unknown what the true limitations of organ viability are and what mechanisms actually regulate it. The upcoming era of personalized medicine dictates the necessity for individualized approach to these issues. The creation of transplant biobanks followed by different studies on their base may be the key option to resolve problems of modern transplantation. Biobanks are an essential basis of personalized medicine, which allows largescale population studies, discovery of new biomarkers and therapy targets as well as new drugs development. The importance of this relatively new fi eld has increased over last decades making its way from small collections of samples to large national and international biorepositories. There are biobanks of different types and purposes. In this article a systematic review is given covering historical reference, term definition, and classifi cation variants. Furthermore, the current state of the industry is highlighted along with current issues and perspectives with respect to transplantation.
PROSPECTS OF APPLICATION OF TISSUE-ENGINEERED PANCREATIC CONSTRUCTS IN THE TREATMENT OF TYPE 1 DIABETES
Authors: Skaletskaya G. N. , Skaletskiy N. N. , Sevastianov V. I. 
Published: January 2017

Allotransplantation of pancreatic islets remains the most effective method of treatment of diabetes mellitus type 1 being capable under combination of favorable conditions (suffi cient number of isolated islets, effective combination of immunosuppressive drugs) to reach the recipients’ insulin independence for several years. However, the overwhelming shortage of donor pancreas and limited post-transplantation islet survival do not allow increasing the number of such transplants and their effectiveness. This review presents a critical analysis of the work done by Russian and foreign authors onto creation of tissue-engineered pancreatic constructs that may lead to the resolution of the three main pancreatic islet transplantation issues: 1) lack of donor material; 2) necessity of immunosuppressive therapy; 3) limited survival and functional activity of the islet.
NONCOMPACTION CARDIOMYOPATHY
Authors: Iljinsky I. M. , Mozheyko N. P. , Shevchenko A. O. 
Published: January 2017

In this article, the review of the literature on various aspects of the noncompaction cardiomyopathy is presented. Non-compaction cardiomyopathy is a relatively rare congenital heart disease characterized by an excessive prominence of trabecular meshwork, and deep recesses of the ventricular myocardium. The most frequent is left ventricular noncompaction (LVNC). LVNC can occur in isolation or coexist with other cardiac and systemic anomalies. Noncompaction can be detected with an echocardiography or cardiac magnetic resonance imaging. The clinical presentation varies ranging from asymptomatic patients to patients who develop ventricular arrhythmias, heart failure, thromboembolism.
REPEAT KIDNEY TRANSPLANTATION
Authors: Sushkov A. I. 
Published: January 2017

Nowadays, kidney transplantation is the best approach of renal replacement therapy for the majority of patients with end-stage renal disease that significantly improves the quality and length of life. Advances in the field of organ donation, immunosuppression, transplant surgery and immunology have improved short-term graft and patient survival. But the long-term graft survival remains static over last two decades. The disparity between low graft and high patient long-term survival led to increasing number of transplant recipients with failed grafts. Repeat renal transplant is presumed to be a good option for many patients losing their grafts, but it is associated with higher complication rates. Unfortunately, there are no evidence-based recommendations or guidelines for renal retransplantation procedure. This review is based on 100 scientifi c publications related to various aspects of the kidney retransplantation and provides the recent data on this matter.
A 6-YEAR EXPERIENCE OF HEART TRANSPLANTATION IN FEDERAL ALMAZOV NORTH-WEST MEDICAL RESEARCH CENTRE
Authors: Shlyakhto E. V. , Gordeev M. L. , Karpenko M. A. , Nikolaev G. V. , Gnevashev A. S. , Grebennik V. K. , Malaya E. Ya. , Naymushin A. V. , Rubinchik V. E. , Sukhova I. V. , Sitnikova M. Yu. , Moiseeva O. M. , Mitrofanova L. B. , Zverev D. A. , Fedotov P. A. , Sazonova Yu. V. , Stepanov S. S. 
Published: January 2017

Aim. To estimate the results of 6-year experience of heart transplantation (HT) in Federal Almazov North-West Medical Research Centre. Methods. From 2010 to 2015 we have performed 65 HT. Mean age was 44.3 ± 14 years old (from 10 to 64 years old). We used biventricular assist device (BIVAD, Berlin Heart Excor) support in 7 heart transplant candidates before HT. 19 patients (29%) received thymoglobulin, whereas 46 patients (71%) had basiliximab to induce immunosuppression.Results.Extracorporeal membrane oxygenation machines were implanted in 5 patients (7.7%) after HT due to acute right ventricular failure. Suture annuloplasty (the Batista procedure) for tricuspid valve repair was carried out in 3 cases (4.6%). Venovenous hemodiafi ltration was used in 11 patients (16.9%). A total of 598 endomyocardial biopsies (EMB) were performed after HT. Evidence of cellular rejection (R1 and R2) was presented in 286 biopsies (48%). The 30-day in-hospital mortality rate was 3.1%. The 6-month survival rate after HT was 92%, 1-year – 91% and overall survival for the 6-year period of observation – 89.2%. Maximum observation period was 71 months.Conclusions.The 6-year experience of HT in our Center has shown a high level of survival. BIVAD Excor support can be effectively used as a «bridge» to HT. Prevention of graft loss due to acute rejection in heart transplant recipients can be achieved only through regular EMB monitoring. The rate of viral infection increased in 2 months after HT.
LAPAROSCOPIC LEFT LATERAL SECTIONECTOMY IN LIVING LIVER DONOR
Authors: Gautier S. V. , Gallyamov E. A. , Monakhov A. R. , Zagainov E. V. 
Published: January 2017

Introduction.Living donor liver transplantation has proved to be an effective, safe and radical treatment modality for patients with end-stage liver diseases. Left lateral section (LLS) of donor’s liver is used in pediatric recipients. Laparoscopic LLS procurement was fi rst described by D. Cherqui in 2002. At present, this technique is routinely used only by 5 hospitals in the world. However, up to date, some authors consider it to be a new standard to perform such a surgery in living donors.Aim.To analyze the fi rst Russian experience in laparoscopic left lateral sectionectomy (LapLLS) in living related donor.Materials and methods.From May to September 2016 fi ve LapLLS were performed in living donors. In all cases donors were women (mothers in 4 cases and aunt in 1 case). Recipients were children aged from 6 months through 3 years with body mass from 5.6 to 12.5 kg. Liver transection was carried out under conditions of maintained blood supply. Parenchymal transection was performed using a harmonic scalpel, bipolar coagulation and ultrasound dissection.Results. Average donor age was 32 ± 5 years. Average operation time was 287 ± 16 min. Average LLS graft weight was 220 ± 16 g. Intraoperative blood loss did not exceed 100 ml (95 ± 5 ml). Donors were discharged on the 3rd–4th post-op day. There were no postoperative complications in donors. Recipients were also characterized by a standard course of the postoperative period.Conclusion.LapLLS is an effective and safe method with several advantages. Primarily, these are early rehabilitation of the donors and shortening of the hospital stay, which are important for the quick return to normal way of live. Excellent visualization of anatomical structures, including vascular and biliary ones, allows performing precise selection. In addition, good cosmetic effect is also an important aspect.
EARLY POSTOPERATIVE PERIOD AFTER ORTHOTOPIC HEART TRANSPLANTATION IN RECIPIENTS OF 60 YEARS AND OLDER
Authors: Poptsov V. N. , Spirina E. A. , Ukhrenkov S. G. , Ustin S. Yu. , Aliev E. Z. , Masyutin S. A. , Voronkov V. Yu. , Chibisov N. S. , Bondarenko D. M. , Mebonija N. Z. , Mozheyko N. P. , Shevchenko A. O. 
Published: January 2017

Introduction. Increase in the number of older patients with terminal CHF results in increase in their proportion among potential recipients requiring mechanical circulatory support and/or heart transplantation (HT) [Abecassis M., Bridges N.D. et al., 2012].Aim.To analyze our own experience of HT in recipients of older 60 years.Materials and methods. The study included 63 patients (56 men (93.3% ) and 4 (6.7%) women) aged 60–74 years (63.0 ± 0.8) with body weight of 76.6 ± 1.4 kg, body surface area of 1.7 ± 0.02 m2, and body mass index of 23.4 ± 0.6. Indications for heart transplantation: dilated cardiomyopathy – 24 (38.1%), coronary heart disease – 34 (54.0%), hypertrophic cardiomyopathy – 2 (3.2%), irreversible cardiac graft dysfunction – 3 (4.7%). 46 (73.0%) patients had NYHA functional Class III and 17 (23.0%) patients – Class IV. Transpulmonary pressure gradient was 11.2 ± 2.7 mmHg, pulmonary vascular resistance – 3.5 ± 1.3 Wood units. According to UNOS algorithm 10 (15.9%) patients were listed as Status 1A of urgency of orthotopic cardiac transplantation (VA ECMO, n = 8, and implantable left ventricular assist device, n = 2), 21 (33.3%) patients as Status 1B, and 32 (50.8%) patients as Status 2. Recipients had the following comorbidities: arterial hypertension (n = 51; 81.0%), diabetes mellitus (n = 6; 9.5%), cerebrovascular disease (n = 13; 20.6%), history of stroke (n = 9; 14.3%), dialysisindependent renal dysfunction (n = 21; 33.3%). Before cardiac transplantation 9 (14.2%) recipients underwent various thoracic surgeries, 2 (3.2%) recipients – brain surgery. Heart donors (49 (77.8%) men and 14 (22.2%) women) were aged 18–59 (34.3 ± 10.4) years.Results. ICU hospitalization lasted for 4–15 (8.2 ± 0.5) days. In 61.9% of cases (n = 39) early postoperative and hospital periods were uncomplicated. Early on-table postoperative activation («early» tracheal extubation) was performed in 32 (50.8%) patients in 48 ± 6 minutes after surgery completion. 2 (3.2%) recipients demonstrated early graft dysfunction and required VA ECMO. Complications other than graft dysfunction were in 24 (38.1%) recipients: renal dysfunction (n = 8; 12.7%), renal and hepatic dysfunction (n = 4; 6.3%), infections (bacterial pneumonia, n = 3, [4.8%]), dyscirculatory encephalopathy (n = 9; 14.3%). 12 (19.0%) patients required continuous renal replacement therapy, 10 of them (15.9%) demonstrated renal function recovery. In 2 (3.2%) cases long-term hemodialysis was used. Hospital lethality (n = 6; 9.5%) was due to multiple organ failure syndrome and sepsis.Conclusion.Our own experience demonstrates satisfactory short-term and long-term survival after heart transplantation in recipients of 60 years and older.
EFFICIENCY OF ENDOVASCULAR MYOCARDIAL REVASCULARIZATION AS A «BRIDGE» TO HEART TRANSPLANTATION IN PATIENTS WITH ISCHEMIC HEART FAILURE
Authors: Mironkov A. B. , Sakhovsky S. A. , Ryadovoy I. G. , Mironkov B. L. 
Published: January 2017

Aim: to estimate effi ciency of endovascular myocardial revascularization in patients with ischemic chronic heart failure, potential candidates for heart transplantation.Materials and methods.Survival of 108 patients with ischemic heart disease complicated by chronic heart failure (CHF) after performance of endovascular myocardial revascularization by percutaneous coronary intervention (PCI) is presented. The observation period composed 32.79 ± 6.2 (from 3 to 126) months, age from 33 to 72 (58.9 ± 0.64) years, 102 men, 6 women. Left ventricular (LV) ejection fraction was 34.9 ± 0.6%, EDV 249.75 ± 4.9 ml, ESV 163.27 ± 3.7 ml, mitral regurgitation 1.51 ± 0.07 points. 73% of patients had NYHA Class III CHF, 27% had NYHA Class IV CHF. Duration and quality of life after revascularization were determined. In 2–3 days after PCI dynamics of LV ultrasound parameters were estimated.Results.At the time of the end of our research 88 from 108 patients (81%) were alive, including 18 patients who underwent heart transplantation (HT). Repeated revascularization was carried out to 19 (17%) patients. 20 patients died: 16 patients with cardiovascular disorders (15%, including 4% of stroke), 3 with pulmonary embolism and 1 with oncological disease. Survival of 90% of the patients composed 4.5 years, 50% survival composed 9 years. At the time of the end of our research the maximum observation period was equal to 126 months. The duration from the fi rst PCI to HT composed from 7.5 to 105 months, mean value – 37 ± 7.5 months. Average life expectancy after HT composed 54.9 ± 24.4 months. Life expectancy from the fi rst PCI composed 87.5 ± 36.9 months. All patients of this group were alive by the time of the end of this research.Conclusion. Endovascular revascularization in patients with ischemic heart failure in 20% of cases can serve as «bridge» to HT, in 50% of cases it can be considered as an alternative to heart transplantation.
THE DIAGNOSTIC VALUE OF PLATELET-DERIVED FACTOR PDGF-BB AND ST2 IN HEART REJECTION
Authors: Shevchenko O. P. , Ulybysheva A. A. , Aksyonova A. V. , Mozhejko N. P. , Stakhanova E. A. , Dontsov V. V. , Nikitina E. A. , Shevchenko A. O. 
Published: January 2017

Aim: to determine the association between plasma concentrations of biomarkers (sCD40L, PDGF-BB, PlGF-1, ST2) with histochemical and immunohistochemical signs of heart rejection.Materials and methods.The study included 98 heart recipients aged from 12 to 69 (mean age 43 ± 14) years, of which 78 men. In 68 patients dilated cardiomyopathy was diagnosed, 30 recipients were diagnosed with coronary heart disease. The concentrations of placental growth factor (PlGF-1), platelet-derived growth factor (PDGF-BB), soluble CD40 ligand (sCD40L) were measured using xMAP technology. The concentrations of ST2 cardiac biomarker were measured by ELISA.Results.No correlation was found between the levels of biomarkers (sCD40L, PDGF-BB, PlGF-1, ST2) and gender, age and diagnosis. The rejection was diagnosed via biopsy in 49 biopsies taken from 37 recipients. 1A rejection was found in 25 patients (34 biopsies), 1B rejection was identifi ed in 2 patients (3 biopsies), 3A rejection was diagnosed in 4 patients. Immunohistochemical signs of humoral rejection were identifi ed in 3 patients. The combination of acute cellular and humoral rejection was found in 4 patients (5 biopsies). The PDGFBB level was measured at the same day as the biopsy was taken, and it was shown to be signifi cantly higher in patients with rejection (p = 0.02). Rejection frequency was signifi cantly higher in patients with high PDGF-BB level (≥2473.7 pg/ml, RR = 1.64 ± 0.23; 95% CI [1.03–2.61]). Rejection frequency increased to 2.11 ± 0.34 [95% CI [1.08–4.11]] in recipients with ST2 and PDGF-BB concentration higher than the median value. The highest predictive value for heart rejection can be reached by a panel of three biomarkers: sCD40L, PlGF-1 and ST2 (RR = 2.51 ± 0.38; 95% CI [1.18–5.3]).Conclusion. PDGF-BB has moderate predictive value for heart rejection. The highest predictive value for heart rejection was reached by a panel of three biomarkers: sCD40L, PlGF-1 and ST2.