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1.
Congenital factor VII deficiency is an autosomal recessive serious disorder of blood coagulation with wide genotypic and phenotypic variations. The clinical presentation can vary from asymptomatic patients to patients with major bleedings in severe deficiency (factor VII <1%). Investigations show prolonged PT and low factor VII. Treatment modalities include FFP and repeated recombinant factor VII infusions. We hereby report the first successful LRLT for factor VII deficiency in an infant, the first‐ever youngest baby reported worldwide. A six‐month‐old male child presented with easy bruisability, ecchymotic patches, hematuria, and convulsions. CT of the head showed subdural hemorrhage, which was treated conservatively. He had markedly increased PT (120 s) with normal platelets, and aPTT with factor VII level <1%. Despite the treatment by rFVIIa administration weekly, which was very expensive, he still had repeated life‐threatening bleeding episodes. LRLT was performed with mother as the donor, whose factor VII level was 57%. A factor VII infusion plan for pre‐, intra‐ and postoperative periods was formulated and TEG followed. Postoperatively, his factor VII started increasing from third day and was 38% on 24th day with PT <14 s. He had uneventful intraoperative and postoperative courses. LT is a safe and definite cure for factor VII deficiency.  相似文献   
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Proceedings of the National Academy of Sciences, India Section B: Biological Sciences - Lepidopteran insects are the most dominant and diversified group among phytophagous pests and economically...  相似文献   
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Clinical Rheumatology - To present single centre experience on the efficacy and safety of similar biologic of rituximab in patients with granulomatosis with polyangiitis (GPA). This was a...  相似文献   
4.
Inflammation Research - Isoproterenol (ISO) is widely used agent to study the effects of interventions which could prevent or attenuate the development of myocardial infarction. The sequence of...  相似文献   
5.
Clinical and Experimental Medicine - Activating mutations in the KRAS gene (Kirsten rat sarcoma 2 viral oncogene homolog gene) are commonly seen across the various solid organ and hematolymphoid...  相似文献   
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Individualism and collectivism are cultural syndromes that have been associated with adolescent problem behavior in studies conducted in the U.S. and Southeast Asia. However, research investigating the mechanisms of how cultural orientation impacts health risk behaviors has been limited. This study tested a new model explaining the relationship between cultural orientation (i.e., individualism, collectivism) and adolescent problem behavior (i.e., substance use and risky sex) in terms of interpersonal self-regulation (i.e., social self-control). As such, the study is rooted in theories of the role of culture in developing self-regulation. Participants were high school students (N = 716) from the Bashkirtostan Republic of the Russian Federation. Adolescents from the Russian Federation tend to show high prevalence of cigarette smoking and binge drinking. People of the Russian Federation in general are traditionally collectivist in orientation, although increased globalization and post-Soviet capitalism may indicate high individualist values in younger generation Russians. Using path analysis we found that in addition to having direct effects, higher individualism indirectly affected substance use and risky sexual behavior through social self-control and negative life events. Higher collectivism was found to have a direct protective effect on risky sexual behavior and a direct effect on social self-control. However, collectivism was not found to have indirect effects on substance use or risky sexual behavior. Higher individualism appears to function as a risk factor for adolescent problem behavior and this relationship may be mediated by lower social self-control. Culturally-tailored prevention programs utilizing the individualism-collectivism framework may benefit from addressing social self-control.  相似文献   
8.
Today, research in biomedicine often requires the knowledge and technologies in diverse fields. Therefore, there is an increasing need for collaborative team science that crosses traditional disciplines. Here, we discuss our own lessons from both interdisciplinary and transdisciplinary teams, which ultimately ushered us to expand our research realm beyond bone biology.  相似文献   
9.
The structure-function correlation of membrane proteins have been a difficult task, particularly in context to transient protein complexes. The molecular simulation of ternary complex of Rab7::REP1::GGTase-II was carried out to understand the basic structural events occurring during the prenylation event of Rab proteins, using the software YASARA. The study suggested that the C-terminus of Rab7 has to be in completely extended conformation during prenylation to reach the active site of RabGGTase-II. Also, attempt was made to find putative drug binding sites on the ternary complex of Rab7::REP1::GGTase-II using Q-SiteFinder programme. The comprehensive consensus probe generated by the program revealed a total of 10 major pockets as putative drug binding sites on Rab7::REP:: GGTase-II ternary complex. These pockets were found on REP protein and GGTase protein subunits. The Rab7 was found to be devoid of any putative drug binding sites in the ternary complex. The phylogenetic analysis of 60 Rab proteins of human was carried out using PHYLIP and study indicated the close phylogenetic relationship between Rab7 and Rab9 proteins of human and hence with further in silico study, the present observations can be extrapolated to Rab9 proteins. The study paves a good platform for further experimental verifications of the findings and other in silico studies like identifying the potential drug targets by searching the putative drug binding sites, generating pharmacophoric pattern, searching or constructing suitable ligand and docking studies.  相似文献   
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