Molecular Analysis of Vietnamese Patients with Mucopolysaccharidosis Type I

Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disorder caused by deleterious mutations in the α‑L‑iduronidase (IDUA) gene. Until now, MPS I in Vietnamese has been poorly addressed. Five MPS I patients were studied with direct DNA sequencing using Illumina technology confirming pathogenic variants in the IDUA gene. Clinical characteristics, additional laboratory results, and family history were collected. All patients have presented with the classical characteristic of MPS I, and α‑L‑iduronidase activity was low with the accumulation of glycosaminoglycans. Three variants in the IDUA gene (c.1190‑10C>A (Intronic), c.1046A>G (p.Asp349Gly), c.1862G>C (p.Arg621Pro) were identified. The c.1190‑10C>A variant represents six of the ten disease alleles, indicating a founder effect for MPS I in the Vietnamese population. Using biochemical and genetic analyses, the precise incidence of MPS I in this population should accelerate early diagnosis, newborn screening, prognosis, and optimal treatment

Pilot identification of the Live-1/Prox-1 expressing lymphatic vessels and lymphatic elements in the unaffected and affected human brain

We report here a pilot identification of the presence of the lumenized Lyve-1/Prox-1-expressing vessels with distinct walls composed of a single endothelial layer in the unaffected brain and with intraventricular hemorrhages. These lymphatic vessels (LVs) have valves and an undulating shape in the distal region that is the classical characteristic of lymphatic precollectors. Furthermore, we identified Lyve-1/Prox-1-expressing lymphatic elements in the enlarged perivascular spaces. These pioneering results might be a revolutionary step in our understanding of anatomy and physiology of the cerebral lymphatics and stimulate a reassessment of basic assumptions in the aetiology of brain diseases associated with lymphatic dysfunction. The discovery of the cerebral LVs is a crucial step in the development of breakthrough technologies of modulations of lymphatic removing of toxins and blood from the brain.

Duchenne muscular dystrophy in a female with x-autosome translocation

Duchenne’s muscular dystrophy is the most common hereditary neuromuscular disease, which affects all races. Its classical characteristic clinical features being progressive muscular weakness, intellectual impairment and hypertrophy of the calves with proliferation of connective tissue and progressive fibrosis in muscles. As the disease is inherited as an X-linked recessive trait, thus females not manifesting the disease and acting as carriers only, as second X chromosome prevents the manifestation of disease. We report a case of classical Duchenne muscular dystrophy in 10 year old female with no intellectual deficit and no family history of similar type of muscular dystrophy.

Updating the implementation of lean logistics in a changing environment

The article is devoted to the analysis of the possibilities of implementing Lean Logistics in an unstable competitive environment. The article sets out the theoretical and practical aspects of the lean enterprise. The concept of Lean Production and its main goals are considered. The classical characteristic of the Lean Production concept to the main types of losses is highlighted. The concept of Lean Logistics is proposed as a Lean Production tool. The content of the concept of Lean Logistics is disclosed. The main reasons leading to the occurrence of logistic losses are considered. The analysis of basic tools and methods that can be applied in Lean Logistics. The possibilities of obtaining benefits from Lean Production tools, when applied in a logistics strategy, are considered. Lean Production methods used in logistics are proposed. The economic benefits of implementing Lean Logistics tools are presented. Recommendations for further research are provided.

Averaged time-optimal control problem in the space of positive Borel measures

We introduce a time-optimal control theory in the space ℳ+ (ℝd) of positive and finite Borel measures. We prove some natural results, such as a dynamic programming principle, the existence of optimal trajectories, regularity results and an HJB equation for the value function in this infinite-dimensional setting. The main tool used is the superposition principle (by Ambrosio–Gigli–Savaré) which allows to represent the trajectory in the space of measures as weighted superposition of classical characteristic curves in ℝd.

THE BERNSTEIN CENTER OF THE CATEGORY OF SMOOTH -MODULES

We consider the category of smooth $W(k)[\text{GL}_{n}(F)]$-modules, where $F$ is a $p$-adic field and $k$ is an algebraically closed field of characteristic $\ell$ different from $p$. We describe a factorization of this category into blocks, and show that the center of each such block is a reduced, $\ell$-torsion free, finite type $W(k)$-algebra. Moreover, the $k$-points of the center of a such a block are in bijection with the possible ‘supercuspidal supports’ of the smooth $k[\text{GL}_{n}(F)]$-modules that lie in the block. Finally, we describe a large explicit subalgebra of the center of each block and give a description of the action of this algebra on the simple objects of the block, in terms of the description of the classical ‘characteristic zero’ Bernstein center of Bernstein and Deligne [Le ‘centre’ de Bernstein, in Representations des groups redutifs sur un corps local, Traveaux en cours (ed. P. Deligne) (Hermann, Paris), 1–32].

Otherworldliness and Otherscopeness as Metaphors of Social and Cultural Development of a Contemporary

A borderline state of the culture is reflected today in human social practices, revealing some new meanings. They are connected not only with the external reality but also with the spiritual state of society; they go into a special sphere of otherness in relation to the previous stages of human development. The transition beyond the bounds of actual existence into the space of consciousness objects creates a new type of otherworldly (average) man. The otherwordliness is, first of all, a classical characteristic, often forgotten and unclaimed by the young generation of Russians. When we say classics, we mean not only the art but also the manners, perceptions and, especially, ethics. The otherscopeness is a goal of the innovational development; it is reflected in the new ideal transmitted and created by the modern education, whose specifics the article is devoted to.

The Laminated Composite Thermosetting Pipe

The thermosetting pipes are eligible to be used in oil transportation where resistance to crude oil, paraffin build-up as well as ability to withstand relatively high pressures is required. Lamination design of such system could provide better strength and stability for internal and external loadings in both the circumferential and longitudinal directions. To this aim, a straightforward design and modeling of thermosetting pipe is developed via using first order shell theories. Apart from possessing an accurate operational condition, on account of its simplicity the proposed simulation seems also very suitable for further developing and prototyping purposes. Finally, it has been shown that the proposed thermosetting pipe, which partially attains some classical characteristic of both offshore and onshore pipeline by a different line of reasoning, is may serve as a reference in designing thermosetting pipe.

Flexible Thermosetting Pipe

The typified as flexible thermosetting pipe, is a new product, developed initially in response to the need for a non-metallic replacement for steel pipe used for oil and gas productions. This product may use for high pressure down-hole applications in which the pipe could repeatedly transported onto a drum. The proposed pipe comprise an internal thermoplastic layer play as liner and a fluid-tight cover, and one or more dual-helical wounding tape stacks applied to the internal liner for absorbing axial and bending loads. The tape stacks are wrapped helically onto the pipe structure with adjacent gaps between wrappings tape to reduce the pressure integrity of the tubular pipe structure. The composite tape stacks are formed from a plurality of thin tape strips and are bonded to each other within a stack. All the layers are manufactured from composite material consisting of highly noncorrosive epoxied matrix reinforced by long continuous fibers. Thus, the proposed flexible pipe designed to be stand alone with thermoplastic liner over-wound with one or more of an epoxy-based structural thermosetting laminate. To this aim, a straightforward simulations of thermosetting pipe is developed via using ANSYS software. Apart from possessing an accurate operational condition, on account of its simplicity the proposed simulation seems also very suitable for further developing and prototyping purposes. Finally, it has been shown that the proposed thermosetting pipe, which partially attains some classical characteristic of both offshore and onshore pipeline by a different line of reasoning, is may serve as a reference in designing flexible pipe.

Minimal spectral functions of an ordinary differential operator

Letl[y]be a formally self-adjoint differential expression of an even order on the interval [0,b〉(b ≤ ∞) and letL0be the corresponding minimal operator. By using the concept of a decomposing boundary triplet, we consider the boundary problem formed by the equationl[y] − λy = f,f ∈ L2[0, b〉, and the Nevanlinna λ-dependent boundary conditions with constant values at the regular endpoint 0. For such a problem we introduce the concept of them-function, which in the case of self-adjoint separated boundary conditions coincides with the classical characteristic (Titchmarsh–Weyl) function. Our method allows one to describe all minimal spectral functions of the boundary problem, i.e. all spectral functions of the minimally possible dimension. We also improve (in the case of intermediate deficiency indicesn±(L0)and non-separated boundary conditions) the known estimate of the spectral multiplicity of the (exit space) self-adjoint extensionà ⊃ L0. Results are obtained for expressionsl[y]with operator-valued coefficients and arbitrary (equal or unequal) deficiency indicesn±(L0).