Clinical and Para clinical characteristics of non-classic Phenylketonuria

Abstract Objective Phenylketonuria (PKU) is one of the most common inherited metabolic diseases, classified as classic and non-classic forms.2% of children with PKU develop a severe and progressive neurologic disease called non classic (malignant) PKU. We aimed to demonstrate the clinical features and laboratory findings and the diagnostic and therapeutic properties of non-classic PKU patients referred to a tertiary level referral center of children in Tehran. Material and Method  In this study, we evaluate background information such as gender and age, clinical manifestations, laboratory findings, and response rates to conventional treatment of patients with non-classic PKU who referred to Mofid Children's' Hospital through neonatal screening.   Result Twenty patients with diagnosis of non-classic PKU were included. The mean age of them was 6.00 ± 2.81 years ranging from 2 to 12 years, and 45.0% were male. In patients with late diagnosis, the most common presentations were motor developmental delay (15.0%), skin and cutaneous manifestations (15.0%), seizure (5.0%), and restlessness (5.0%). An overall response to treatment was 85.0%. Factors that predict good response to treatment were female gender, higher neopterin level, and lower age at diagnosis and management. Conclusion In conclusion, about half of patients with non-classic PKU remain asymptomatic, which is due to early diagnosis via neonatal screening. Also, higher age at diagnosis and treatment, besides low neopterin levels, may be useful as prognostic factors. &nbsp

Scolicidal effects of biogenic selenium nanoparticles against protoscolices of hydatid cysts

Cystic echinococcosis (hydatid cyst, CE) as a zoonotic parasitic infection caused by the larval stage of the dog tapeworm Echinococcus granulosus is still an important economic and public health concern in the world. One of the treatment options for CE is surgical removal of the cysts combined with chemotherapy using albendazole and/or mebendazole before and after surgery. Currently, many scolicidal agents, which have some complications, have been used for inactivation of the cyst contents. Therefore the development of new scolicidal agents with low side effects and more efficacies is an urgent need for surgeons. The present study was aimed to investigate the in vitro scolicidal effect of selenium nanoparticles biosynthesized by a newly isolated marine bacterial strain Bacillus sp. MSh-1 against protoscoleces of E. granulosus. Protoscolices were aseptically aspirated from sheep livers having hydatid cysts. Various concentrations (50e500 mg/ml) of Se NPs (in size range of about 80e220 nm) were used for 10e60 min. Viability of protoscoleces was confirmed by 0.1% eosin staining. The results indicated that biogenic Se NPs at all concentrations have potent scolicidal effects especially at concentrations 500 and 250 mg/ml after 10 and 20 min of application, respectively. In conclusion, the findings of present study proven that Se NPs have potent scolicidal effects, therefore may be used in CE surgery. However, the in vivo efficacy of these NPs remains to be explored

Indoor environment assessment of special wards of educational hospitals for the detection of fungal contamination sources: A multi-center study (2019-2021)

Background and Purpose: The hospital environment was reported as a real habitat for different microorganisms, especially mold fungi. On the other hand, these opportunistic fungi were considered hospital-acquired mold infections in patients with weak immune status. Therefore, this multi-center study aimed to evaluate 23 hospitals in 18 provinces of Iran for fungal contamination sources.Materials and Methods: In total, 43 opened Petri plates and 213 surface samples were collected throughout different wards of 23 hospitals. All collected samples were inoculated into Sabouraud Dextrose Agar containing Chloramphenicol (SC), and the plates were then incubated at 27-30ºC for 7-14 days.Results: A total of 210 fungal colonies from equipment (162, 77.1%) and air (48,22.9%) were identified. The most predominant isolated genus was Aspergillus (47.5%),followed by Rhizopus (14.2%), Mucor (11.7%), and Cladosporium (9.2%). Aspergillus(39.5%), Cladosporium (16.6%), as well as Penicillium and Sterile hyphae (10.4% each), were the most isolates from the air samples. Moreover, intensive care units (38.5%) and operating rooms (21.9%) had the highest number of isolated fungal colonies. Out of 256 collected samples from equipment and air, 163 (63.7%) were positive for fungal growth.The rate of fungal contamination in instrument and air samples was 128/213 (60.1%) and 35/43 (81.2%), respectively. Among the isolated species of Aspergillus, A. flavus complex (38/96, 39.6%), A. niger complex (31/96, 32.3%), and A. fumigatus complex (15/96, 15.6%) were the commonest species.Conclusion: According to our findings, in addition to air, equipment and instrument should be considered among the significant sources of fungal contamination in the indoor environment of hospitals. Airborne fungi, Hospital, Indoor air, Equipment, Sources of fungal contamination in the indoor environment of hospitals

Reducing the operational energy demand in buildings using building information modeling tools and sustainability approaches

A sustainable building is constructed of materials that could decrease environmental impacts, such as energy usage, during the lifecycle of the building. Building Information Modeling (BIM) has been identified as an effective tool for building performance analysis virtually in the design stage. The main aims of this study were to assess various combinations of materials using BIM and identify alternative, sustainable solutions to reduce operational energy consumption. The amount of energy consumed by a double story bungalow house in Johor, Malaysia, and assessments of alternative material configurations to determine the best energy performance were evaluated by using Revit Architecture 2012 and Autodesk Ecotect Analysis software to show which of the materials helped in reducing the operational energy use of the building to the greatest extent throughout its annual life cycle. At the end, some alternative, sustainable designs in terms of energy savings have been suggested

Curing Kinetics Modeling of Epoxy Modified by Fully Vulcanized Elastomer Nanoparticles Using Rheometry Method

In this study, the curing kinetics of epoxy nanocomposites containing ultra-fine full-vulcanized acrylonitrile butadiene rubber nanoparticles (UFNBRP) at different concentrations of 0, 0.5, 1 and 1.5 wt.% was investigated. In addition, the effect of curing temperatures was studied based on the rheological method under isothermal conditions. The epoxy resin/UFNBRP nanocomposites were characterized via Fourier transform infrared spectroscopy (FTIR). FTIR analysis exhibited the successful preparation of epoxy resin/UFNBRP, due to the existence of the UFNBRP characteristic peaks in the final product spectrum. The morphological structure of the epoxy resin/UFNBRP nanocomposites was investigated by both field emission scanning electron microscopy (FESEM) and transmission electron microscopy (TEM) studies. The FESEM and TEM studies showed UFNBRP had a spherical structure and was well dispersed in epoxy resin. The chemorheological analysis showed that due to the interactions between UFNBRP and epoxy resin, by increasing UFNBRP concentration at a constant temperature (65, 70 and 75 °C), the curing rate decreases at the gel point. Furthermore, both the curing kinetics modeling and chemorheological analysis demonstrated that the incorporation of 0.5% UFNBRP in epoxy resin matrix reduces the activation energy. The curing kinetic of epoxy resin/UFNBRP nanocomposite was best fitted with the Sestak–Berggren autocatalytic model.Applied Science, Faculty ofNon UBCChemical and Biological Engineering, Department ofReviewedFacultyResearche