Growth Hormone and Insulin-like Growth Factor-1 in Children with Cholestatic Diseases and Pediatric Liver Transplantation

The growth hormone/insulin-like growth factor 1 (GH/IGF-1) system is the most important regulator of growth, regeneration, and metabolism in children and adults. Children with congenital cholestatic diseases have elevated GH blood levels, which is combined with growth failure and body mass deficit. Congenital cholestatic diseases lead to end-stage liver disease (ESLD), where GH bioavailability, mediated through IGF-1, is impaired. Blood IGF-1 levels are decreased due to impaired production by the liver. This study included 148 children up to 5 years (60 months) old with congenital cholestatic diseases. The patients underwent liver transplantation (LT) at a leading transplant center in Russia. The clinical significance of the GH/IGF-1 axis in pediatric liver recipients was investigated. Relationship between the patients’ GH/IGF-1 levels and anthropometric parameters was analyzed before and after LT. It was shown that LT leads to renewal/recovery of GH-IGF-1 regulation and improved anthropometric parameters (body height and body mass) in pediatric recipients

Assessment of heart transplant recipients survival based on ultrasound diagnostic methods and immunological screening of antibodies to leukocyte donor antigens

Aim. To create a mathematical model for predicting an assessment of the risk of antibody-mediated rejection (AMR) and analyze the survival of recipients with antibodies to leukocyte donor antigens.Material and methods. A single-center study was conducted on the basis ofS.V.OchapovskyRegionalClinicalHospital № 1. During the 7 years 181 heart transplant recipients were observed. Based on the AMR crisis and detected antibodies to leukocyte donor antigens (HLA), 5 groups were identified: group 1 (n=10) — donor-specific antibodies (DSA) and AMR crisis, group 2 (n=7) — patients without DSA and AMR crisis, group 3 (n=17) — patients with antibodies to HLA, without AMR crisis, group 4 (n=11) — with AMR crisis, without identified antibodies to HLA, group 5 (n=87) — patients, not having antibodies to HLA and signs of both AMR and cell-mediated rejection (according to endomyocardial biopsy). The recipients underwent immunological tests, 2D-speckletracking echocardiography (2D-STE) and transthoracic echocardiography (TTEchoCG). Statistical methods were used to assess the results.Results. Predictors of the severe form of AMR in TTEchoCG are: left ventricle enddiastolic diameter, interventricular septum thickness, ejection fraction, right ventricle volume. Predictors were determined using the 2D-STE method: global longitudinal peak strain, sensitivity (SE) — 86,2%, specificity (SP) — 90,4%; radial strain, SE — 75,8%, SP — 84,5%; circular strain, SE — 78,6%, SP — 84,4%. When taking into account the indicators of the global longitudinal peak strain of the left ventricle and the longitudinal peak strain of the right ventricle, SE increases to 91,9%, SP — 94,6%, with p<0,001. The survival rate of patients with identified post-transplant (de novo) donor-specific antibodies of the late period is 40%, without identified donor-specific antibodies — 68%. Dedicated predictors are used for mathematical prediction of AMR risk.Conclusion. The relationship between immunological changes and data of TTEchoCG, deformation parameters and mechanics of a heart transplant was revealed. The presence of de novo DSA decline the survival, increases the risk of AMR, and contributes to the development of coronary artery disease. The proposed AMR risk prediction model will improve the long-term results of heart transplantation

The State of Dark Coniferous Forests on the East European Plain Due to Climate Change

As a result of global climate changes, negative processes have been recorded in the coniferous forests of the Northern Hemisphere. Similar processes are observed in the Urals, including in Udmurtia. In the course of this research, archival analysis methods were used, as well as field research methods. In the process of analyzing archival materials in the Urals, a reduction of spruce forests was observed. If in the 20th century the share of spruce forests in the region was 50%, then in the 21th century it decreased to 35%. As a result of this research, it was revealed that the most unfavorable sanitary condition was recorded in the boreal–subboreal zone of Udmurtia, with a sanitary condition index of 3.2 (from 2.62 to 3.73). The main reason for the unfavorable sanitary condition of spruce forests was the vital activity of Ips typographus L. According to our research, in 11 sample plots out of 18, a high score for sanitary condition was associated with the vital activity of bark beetles. The correlation coefficient of the index of the sanitary condition of plantings and the number of individuals of Picea obovata Ledeb. affected by Ips typographus L. was0.93. Bark beetle activity has increased in the 21th century, which is associated with changing climatic factors. Unstable precipitation over recent years (differences of more than 100 mm) and an average temperature increase of 1.2 °C were observed in the region. The most significant increase in temperature over the past 10 years was observed in winter, which in turn affected the high survival rate of insect pests

Impact of the European bark beetle Ips typographus on biochemical and growth properties of wood and needles in Siberian spruce Picea obovata

European spruce bark beetle Ips typographus is an important driver of ecological processes in spruce stands, with severe effects on forestry economies. To prevent bark beetle outbreaks, early detection of infestations is a crucial step in forest management. It is expected that bark beetle infestation modifies biochemical composition of wood and needles, alters physiological responses in the early stage of infestation, which results in the reduction of tree growth and ultimately a tree death. Therefore, we studied the differences in biochemical composition of wood, content of photosynthesis-related pigments, shoot morphology, and growth between the healthy Siberian spruce trees (Picea obovata) and trees which were infested by I. typographus. The study was performed in five experimental plots established in the south of the Udmurt Republic in the European part of the Russian Federation. Three infested and three non-infested trees were chosen on each plot. Our results showed no significant effect of bark beetle infestation on the content of main structural components of wood – holocellulose (cellulose and hemicellulose) and lignin. On the other hand, we found differences in the content of extractive substances in the wood. Specifically, we found a higher tannin content in the non-infested trees. The content of photosynthesis-related pigments differed between the non-infested and infested trees as well. Unexpectedly, bark beetle infestation caused the overproduction of both pigment types rather than their degradation. Moreover, we observed that a higher amount of total extractive substances positively affected the incremental growth, whereas tannins supported the growth of shoots and needles

Клинические рекомендации по ведению детей с дефицитом лизосомной кислой липазы

Lysosomal acid lipase deficiency is s a rare hereditary enzymopathy. The article presents epidemiological data and features of etiopathogenesis of two phenotypic forms of lysosomal acid lipase deficiency — Wolman disease and cholesterol ester storage disease. Special attention has been given to the key issues of differential diagnostic search, clinical guidelines based on the principles of evidence-based medicine have been given.Дефицит лизосомной кислой липазы — редкая наследственная ферментопатия. В статье представлены эпидемиологические данные и особенности этиопатогенеза двух фенотипических форм дефицита лизосомной кислой липазы — болезни Вольмана и болезни накопления эфиров холестерина. Подробно описаны клинические характеристики быстропрогрессирующей формы и медленно развивающейся болезни накопления эфиров холестерина. Особое внимание уделено ключевым вопросам дифференциально-диагностического поиска, приведены рекомендации по лечению, основанные на принципах доказательной медицины