A comprehensive report of the clinical and mutational profiles of 30 Iranian malignant infantile osteopetrosis patients.

Osteopetrosis is a group of genetically and clinically diverse inherited disorders characterized by an increase in bone density. The main known cause is an abnormality in the development or function of osteoclasts. Hence, the process of bone resorption is impaired, resulting in: 1- a reduction in bone marrow volume and, subsequently, a decrement in the hematopoietic capacity of bone marrow, which leads to anemia and compromised immunological function; 2- improper bone development, which leads to pressure on peripheral nerves, causing auditory, visual, and movement impairments; and 3- disturbance in the formation of bone microstructure that leads to susceptibility to bone fracture.

Sleep stages classification based on feature extraction from music of brain.

Sleep stages classification one of the essential factors concerning sleep disorder diagnoses, which can contribute to many functional disease treatments or prevent the primary cognitive risks in daily activities. In this study, A novel method of mapping EEG signals to music is proposed to classify sleep stages. A total of 4.

Partial replacement of the tricuspid valve using cryopreserved tricuspid homograft: 20-year outcomes.

Objectives: There is limited consensus on the optimal strategy for surgical treatment of severe tricuspid valve pathology. At our institution, we have implemented a unique technique of using a tricuspid homograft with its supporting apparatus for partial replacement of the native tricuspid valve. We now present the long-term outcomes of these patients.

Evaluation of whey protein coating containing free and Pickering emulsion forms of . essential oil on quality of refrigerated beef.

The purpose of the present research was to prepare a novel biodegradable coating of whey protein (whey) and essential oil of (forms of free (EO) and Pickering emulsion (NEO)) to improve the shelf life of beef. In this study, various microbiological, chemical, and sensory analyses were performed. The results showed that after 18 days, the highest and lowest microbiological counts were related to control samples and whey-NEO treatments, respectively.

Mutation Analysis of PAH Gene in Phenylketonuria Patients from the North of Iran: Identification of Three Novel Pathogenic Variants.

Background: There are more than 1100 different pathogenic variants in the phenylalanine hydroxylase () gene that are responsible for phenylketonuria (PKU) diseases, and the spectrum of these mutations varies in different ethnic groups. The aim of the present study was to identify the frequency of pathogenic variants in all 13 exons of the gene among patients with PKU in Mazandaran and Golestan provinces in the north of Iran.

Methods: Forty unrelated PKU patients from Mazandaran and Golestan provinces were enrolled in the study.

Advances in Exosome-Based Therapies for the Repair of Peripheral Nerve Injuries.

Peripheral nerve injuries (PNIs) are the term used to describe injuries that occur to the nerve fibers of the peripheral nervous system (PNS). Such injuries may be caused by trauma, infection, or aberrant immunological response. Although the peripheral nervous system has a limited capacity for self-repair, in cases of severe damage, this process is either interrupted entirely or is only partially completed.

Fabrication and application of salicin-polycaprolactone 3D-printed scaffold in the healing of femur bone defects.

Polycaprolactone (PCL) is a suitable material for bone repair due to good biocompatibility and mechanical properties. However, low bioactivity and hydrophobicity pose major challenges for its biomedical applications. To overcome these limitations, PCL-based scaffolds loaded with bioactive agents have been developed.

Immune response evaluation after Sputnik V vaccination in Sari healthcare population.

Background And Objective: Generally, protection against SARS-CoV-2 was assessed by the measurement of antibody titers against spike protein and receptor binding domain "RBD". Many global efforts lead to development of several vaccines based on various strategies. The aim of the present study was to evaluate the efficacy of Sputnik V vaccine among Sari healthcare staff population, Iran.

Coinheritance of the c.-19 G > C and c.315 + 1 G > A Variants in the -Globin Gene Leads to Thalassemia Disease: A Report from the North of Iran.

Up to now, more than 300 pathogenic variants have been identified in the -globin gene, some of which are categorized as silent mutations that do not change the hematological indices. In the present study, our aim is to introduce the first report of a case with thalassemia intermedia with coinheritance of the c.315 + 1 G > A pathogenic variant and a silent variant (HBB: c.

Colloidal InAs Tetrapods: Impact of Surfactants on the Shape Control.

We have approached the synthesis of colloidal InAs nanocrystals (NCs) using amino-As and ligands that are different from the commonly employed oleylamine (OA). We found that carboxylic and phosphonic acids led only to oxides, whereas tri--octylphosphine, dioctylamine, or trioctylamine (TOA), when employed as the sole ligands, yielded InAs NCs with irregular sizes and a broad size distribution. Instead, various combinations of TOA and OA delivered InAs NCs with good control over the size distribution, and the TOA:OA volume ratio of 4:1 generated InAs tetrapods with arm length of 5-6 nm.

miR-155 and miR-92 levels in ALL, post-transplant aGVHD, and CMV: possible new treatment options.

Background: Acute lymphoblastic leukemia (ALL) is a malignancy that leads to altered blast cell proliferation, survival, and maturation and eventually to the lethal accumulation of leukemic cells. Recently, dysregulated expression of various micro-RNAs (miRNAs) has been reported in hematologic malignancies, especially ALL. Cytomegalovirus infection can induce ALL in otherwise healthy individuals, so a more detailed evaluation of its role in ALL-endemic areas like Iran is required.

Shielding design aspects of SR in 3 GeV ILSF.

Iranian Light Source Facility (ILSF) is an under-construction synchrotron radiation accelerator consisting of a 150 MeV linac, a booster synchrotron operating from 150 MeV to 3 GeV, and a 3 GeV storage ring that stores a maximum of 400 mA current of electrons. As the stored beam circulates, a fraction of the beam is lost due to interactions with gas molecules, interactions among beam particles, and orbital bending, which produce radiation. The bulk shielding calculation for the ILSF and the input parameters used for this analysis are discussed in this paper.

Beyond steric selectivity of ions using ångström-scale capillaries.

Ion-selective channels play a key role in physiological processes and are used in many technologies. Although biological channels can efficiently separate same-charge ions with similar hydration shells, it remains a challenge to mimic such exquisite selectivity using artificial solid-state channels. Although there are several nanoporous membranes that show high selectivity with respect to certain ions, the underlying mechanisms are based on the hydrated ion size and/or charge.

The effect of preparation design on the fracture resistance and adaptation of the CEREC ceramic endocrowns.

Objective: The purpose of this experimental in vitro study was to assess the effect of having one or two intact axial walls on the improvement of the fracture resistance, and marginal and internal adaptation of computer-aided design/computer-aided manufacturer fabricated ceramic endocrowns.

Materials And Methods: Thirty-six endodontically treated mandibular molars were divided into three groups (n = 12). Group A, represented teeth that all of their axial walls were reduced till they all had 3 mm height.

First report of novel mutation (c.790del) on gene on a family with childhood onset of progressive cerebellar ataxia with vertical gaze palsy.

SQSTM1 gene encodes a protein called p62 that acts as an autophagy receptor in the degradation of protein molecules. A homozygous deletion variant that changes the frame shift in the SQSTM1 gene named c.790 Del A .

Enhance VR: A Multisensory Approach to Cognitive Training and Monitoring.

Cognitive training systems aim to improve specific domains or global cognition by engaging users in cognitively demanding tasks. While screen-based applications can improve performance in the trained cognitive abilities, they are often criticized for their poor transferability to activities of daily living. These systems, however, exclude the user's body and motor skills, which invariably serves to restrict the user experience.

Ecology of sand flies (Diptera: Psychodidae, Phlebotominae) in Jajarm County, an area with high risk of cutaneous leishmaniasis, in North Khorasan, Iran.

The present study was conducted to investigate the ecological aspects of sand flies in southwestern North Khorasan, in which cutaneous leishmaniasis caused by Leishmania major has been reported with the highest annual incidence in Iran. Sampling was carried out in four localities including: Khorasha (natural), Ghamiteh (natural), Jorbat (semi urban) and Brick kilns (urban), twice a month using 105 sticky paper traps from indoors and outdoors dwellings during May-December 2017. Specimens were removed from sticky papers, washed in acetone, preserved in 80% ethanol, mounted on microscopic slides by Puri's medium, and identified using valid morphological keys.

Comparison of homografts and bovine jugular vein conduits in the pulmonary position in patients <20 years of age.

Objective: To compare the performance of homografts and bovine jugular vein (BJV) conduits in the pulmonary position.

Methods: All patients with congenital heart disease up to age 20 years who underwent pulmonary valve replacement with homografts or BJV at 3 centers in Australia were evaluated. There were 674 conduits, with 305 (45%) pulmonary homografts (PHs), 303 (45%) BJV conduits, and 66 (10%) aortic homografts (AHs).

Cloning of phytase gene construct in .

Background And Objectives: Phytase has a hydrolysis function of phytic acid, which yields inorganic phosphate. species can produce thermostable alkaline phytase. The aim of this study was to isolate and clone a gene () from in

Materials And Methods: In this study, the extracellular gene was isolated from .

Selenium enhances the expression of miR-9, miR-124 and miR-29a during neural differentiation of bone marrow mesenchymal stem cells.

Background: Selenium (Se) is a trace element that plays important role in antioxidant defense in the brain. Sodium selenite (NaSeO) is an inorganic salt of Se which has an antioxidant function. In the present study, we investigated the effect of Sodium selenite on the expression of important neuronal microRNAs during neural differentiation of bone marrow-derived stem cells (BMSCs).

Switchable Anion Exchange in Polymer-Encapsulated APbX Nanocrystals Delivers Stable All-Perovskite White Emitters.

We report a one-step synthesis of halide perovskite nanocrystals embedded in amphiphilic polymer (poly(acrylic acid)--poly(styrene), PAA--PS) micelles, based on injecting a dimethylformamide solution of PAA--PS, PbBr, ABr (A = Cs, formamidinium, or both) and "additive" molecules in toluene. These bifunctional or trifunctional short chain organic molecules improve the nanocrystal-polymer compatibility, increasing the nanocrystal stability against polar solvents and high flux irradiation (the nanocrystals retain almost 80% of their photoluminescence after 1 h of 3.2 w/cm irradiation).

The synergistic effects of zinc oxide nanoparticles and fennel essential oil on physicochemical, mechanical, and antibacterial properties of potato starch films.

The purpose of this study was to evaluate the effects of a combination of zinc oxide (ZnO-N) nanoparticles and fennel essential oil (FEO) on the functional and antimicrobial properties of potato starch films. Films based on potato starch containing a combination of ZnO-N (1, 3, and 5%(w/w)) and FEO (1, 2, and 3% (w/w)) produced by casting method and water solubility, water absorption capacity (WAC), barrier properties, mechanical properties, color indexes, and antimicrobial activity of the films against , and were studied. The combination of ZnO-N and FEO had a significant decreasing effect on solubility, WAC, water vapor and oxygen permeability, elongation, and * index.