Literature survey

About: Literature survey is a research topic. Over the lifetime, 15372 publications have been published within this topic receiving 459196 citations.

Zellweger syndrome: Diagnostic assays, syndrome delineation, and potential therapy

Abstract: Patients with the cerebrohepatorenal syndrome of Zellweger lack peroxisomes and certain peroxisomal enzymes such as dihydroxyacetone phosphate acyltransferase in their tissues. Deficiency of this enzyme, which is necessary for glycerol ether lipid synthesis, provides a biochemical method for recognizing patients with subtle manifestations of Zellweger syndrome and suggests the utility of exogenous ether lipid precursors as a therapeutic strategy for these children. We describe the results of glycerol ether lipid supplementation to two children, one with classic Zellweger syndrome and 9% of control fibroblast dihydroxyacetone phosphate acyltransferase activity, and one with mild facial manifestations, wide sutures, hypotonia, developmental delay, hepatomegaly, peripheral retinal pigmentation, and 50% of control fibroblast dihydroxyacetone phosphate acyltransferase activity. An increase in erythrocyte plasmalogen levels following therapy was clearly demonstrated in the milder patient, and neither patient showed evidence of toxicity. Evaluation of therapy by comparison to the usual clinical course of Zellweger syndrome was not helpful because of the variability and incomplete documentation of 90 previously reported cases. The literature survey did provide criteria for classic Zellweger syndrome, which include hypotonia with or without deformation of limbs, large fontanels and split sutures, prominent forehead, flattened facial profile with hypoplastic supraorbital ridges, anteverted nares, highly arched palate, cryptorchidism or labial hypoplasia, hepatomegaly or elevated liver enzymes, peripheral pigmentation of the retina, renal cortical cysts, and characteristic neuropathology involving decreased myelinization, abnormal neuronal migration, and sudanophilic macrophages. Less severe patients, as exemplified by our case 2 and others from the literature, will not have all the classic features and can be recognized only by a growing panel of biochemical indicators. Our patient studies illustrate the complexity of designing comprehensive therapy for Zellweger-like conditions, suggest other diseases that may involve peroxisomal alterations, and emphasize the need for multicenter, collaborative studies to evaluate biochemical heterogeneity and therapy of peroxisomal disorders.

Systematic review on wearable lower-limb exoskeletons for gait training in neuromuscular impairments.

Abstract: Gait disorders can reduce the quality of life for people with neuromuscular impairments. Therefore, walking recovery is one of the main priorities for counteracting sedentary lifestyle, reducing secondary health conditions and restoring legged mobility. At present, wearable powered lower-limb exoskeletons are emerging as a revolutionary technology for robotic gait rehabilitation. This systematic review provides a comprehensive overview on wearable lower-limb exoskeletons for people with neuromuscular impairments, addressing the following three questions: (1) what is the current technological status of wearable lower-limb exoskeletons for gait rehabilitation?, (2) what is the methodology used in the clinical validations of wearable lower-limb exoskeletons?, and (3) what are the benefits and current evidence on clinical efficacy of wearable lower-limb exoskeletons? We analyzed 87 clinical studies focusing on both device technology (e.g., actuators, sensors, structure) and clinical aspects (e.g., training protocol, outcome measures, patient impairments), and make available the database with all the compiled information. The results of the literature survey reveal that wearable exoskeletons have potential for a number of applications including early rehabilitation, promoting physical exercise, and carrying out daily living activities both at home and the community. Likewise, wearable exoskeletons may improve mobility and independence in non-ambulatory people, and may reduce secondary health conditions related to sedentariness, with all the advantages that this entails. However, the use of this technology is still limited by heavy and bulky devices, which require supervision and the use of walking aids. In addition, evidence supporting their benefits is still limited to short-intervention trials with few participants and diversity among their clinical protocols. Wearable lower-limb exoskeletons for gait rehabilitation are still in their early stages of development and randomized control trials are needed to demonstrate their clinical efficacy.

Supplier Selection: A Fuzzy-ANP Approach

Abstract: The main goal of this paper is to develop a framework to support the supplier selection process in an Iranian automotive industry. Although numerous criteria are being used for the selection of suitable supplier, selection of the critical factors in conformance to the specification of the automotive industries is less investigated. In order to fill this gap, this research was carried out to systematically propose a framework comprising of the most critical factors for the aim of supplier selection. A literature survey was conducted and measures for assessing the suppliers were extracted. Nominated Group Technique (NGT) was deployed to extract the most critical performance measures from the initial list. Seven measures were found to be proper for the supplier selection process. A Fuzzy Analytical Network Process (FANP) was then proposed to weight the extracted measures and determine their importance level. The model was then implemented to assist an automotive company for the aim of its supplier selection.

Toward Future Green Maritime Transportation: An Overview of Seaport Microgrids and All-Electric Ships

Abstract: Nowadays, extensive electrification of maritime transportation, represented by the seaport microgrids and all-electric ships (AESs), has been viewed as a feasible route to enhance the overall system flexibility as well as to mitigate the resulted in growing environmental issues. However, with the trend of electrification, the connections between the seaport and ships are no longer limited in the logistic-side, but also expanded to the electric-side, which makes the future maritime transportation management as a complex transportation-power multi-microgrid coordination problem. In land-based applications, multi-microgrid coordination is a relatively mature technology and already brings enormous economic and environmental benefits, but there still exists some gaps before those land-based technologies being integrated into maritime applications. In this perspective, this overview study emphasizes the characteristic of seaport microgrid and AESs, then several emerging technical challenges and the future research prospects are raised after a comprehensive literature survey.