Background: Cystinosis is a rare autosomal recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. It is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. Elevated white blood cell cystine content is the cornerstone of the diagnosis. Since chitotriosidase (CHIT1 or chitinase-1) is mainly produced by activated macrophages both in normal and inflammator

Abstract: Recently, there is increasing interest in using mode-division multipelexing (MDM) technique  to enhace  data rate transmission over multimode fibers.  In this technique, each fiber mode is treated as a separate optical carrier to transfer its own data.  This paper presents a broadband, compact, and low loss three-mode (de)multiplexer designed for C+L band using subwavelength grating (SWG) technology and built-in silicon-on-insulator SOI platform. SWG offers refractive index engineering for wider operating bandwidth and compact devices compared to conventional ones. The designed (de)multiplex deals with three modes (TE0, TE1, and TE2) and has a loss > -1 dB and crosstalk < −15 dB, and its operation c

In recent years, there has been a significant increase in research demonstrating the new and diverse uses of non-thermal food processing technologies, including more efficient mixing and blending processes, faster energy and mass transfer, lower temperature and selective extraction, reduced thermal and concentration gradients, reduced equipment size, faster response to extraction control, faster start-up, increased production, and a reduction in the number of steps in preparation and processing. Applications of ultrasound technology have indicated that this technology has a promising and significant future in the food industry and preservation, and there is a wide scope for its use due to the higher purity of final products and the

In addition to the primary treatment, biological treatment is used to reduce inorganic and organic components in the wastewater. The separation of biomass from treated wastewater is usually important to meet the effluent disposal requirements, so the MBBR system has been one of the most important modern technologies that use plastic tankers to transport biomass with wastewater, which works in pure biofilm, at low concentrations of suspended solids. However, biological treatment has been developed using the active sludge mixing process with MBBR. Turbo4bio was established as a sustainable and cost-effective solution for wastewater treatment plants in the early 1990s and ran on minimal sludge, and is easy to maintain. This

To create a highly efficient photovoltaic-thermal (PV-T) system and maximise the energy and exergy efficiency, this study aims to propose an innovative configuration of a PV-T system comprising wavy tubes with twisted-tape inserts. Following the validation of a numerical model, a parametric study has been conducted to assess the geometrical effects of twisted tape and wavy tubes, as well as the coolant fluid type and velocity, on the overall performance of a PV-T system, located in Shiraz, Iran. It is found that employing twisted tape improves the energy and exergy efficiency by approx. 6.3%. The best configuration yields 12.4% and 16.8% increase in energy and exergy efficiency compared to conventional PV systems. This is achieved at 15% vo

ABSTRACT : Alzheimer’s disease (AD) is one of the most common inflammatory neurodegenerative diseases linked with dementia, it is characterized by the deposition of amyloid beta-peptide (Ab) in the brain. The present study aims to innovate a biochemical relationship between AD and interleukin 38 (IL-38) as an anti-inflammatory cytokine, expose novel mechanisms and concepts regarding other biochemical parameters studied previously or recently in AD patients and also examine the biochemical action of memantine (10 mg daily) on AD patients. Sixty (60) diagnosed AD patients participated in the present study and classified into four (4) groups: G3 were composed of (15) newly diagnosed males (52-78) years / without treatment, G4 composed of (15

To assess the biochemical, mechanical and structural characteristics of retained dentin after applying three novel bromelain‑contained chemomechanical caries removal (CMCR) formulations in comparison to the conventional excavation methods (hand and rotary) and a commercial papain‑contained gel (Brix 3000). Seventy‑two extracted permanent molars with natural occlusal carious lesions (score > 4 following the International Caries Detection and Assessment System (ICDAS‑II)) were randomly allocated into six groups (n = 12) according to the excavation methods: hand excavation, rotary excavation, Brix 3000, bromelain‑contained gel (F1), bromelain‑chloramine‑T (F2), and bromelain chlorhexidine gel (F3). The superficial and deepe

The discourse surrounding lingual sovereignty within the African postcolonial context is profoundly intertwined with the fabric of cultural identity and self-determination. Language serves not merely as a conduit for communication but as a repository for a people's collective consciousness, encapsulating their traditions, thoughts, and perspectives. In the realm of postcolonial literature, this dialogue often grapples with the paradox of expressing indigenous narratives through the linguistic tools of former colonizers. Chinua Achebe's seminal work, "Things Fall Apart," exemplifies this conundrum, artfully weaving the orature and culture of Umuofia within the English language. Achebe's choice to write in English—a language imposed upon hi

Background: Unlike normal EEG patterns, the epileptiform abnormal pattern is characterized by different mor phologies such as the high-frequency oscillations (HFOs) of ripples on spikes, spikes and waves, continuous and sporadic spikes, and ploy2 spikes. Several studies have reported that HFOs can be novel biomarkers in human epilepsy study. S) Method: To regenerate and investigate these patterns, we have proposed three large scale brain network models (BNM by linking the neural mass model (NMM) of Stefanescu-Jirsa 2D (S-J 2D) with our own structural con nectivity derived from the realistic biological data, so called, large-scale connectivity connectome. These models include multiple network connectivity of brain regions at different

Solubility problem of many of effective pharmaceutical molecules are still one of the major obstacle in theformulation of such molecules. Candesartan cilexetil (CC) is angiotensin II receptor antagonist with very low water solubility and this result in low and variable bioavailability. Self- emulsifying drug delivery system (SEDDS) showed promising result in overcoming solubility problem of many drug molecules. CC was prepared as SEDDS by using novel combination of two surfactants (tween 80 and cremophore EL) and tetraglycol as cosurfactant, in addition to the use of triacetin as oil. Different tests were performed in order to confirm the stability of the final product which includes thermodynamic study, determination of self-emulsificat