Mutational Landscape of Patients with Wiskott Aldrich Syndrome: Update from India.

Purpose: Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder characterized by distinctive features including microthrombocytopenia, eczema and recurrent infections. In the present study we report clinical, immunological and molecular spectrum of 41 WAS patients diagnosed over last five years.

Methods: Clinical and family history was collected from case records.

First Cu-nanostar as a sustainable catalyst realized through synergistic effects of bowl-shaped features and surface activation of sporopollenin exine.

Recently, nanostar-shaped structures, including gold nanostars (NS), have drawn much attention for their potential use in surface-enhanced Raman spectroscopy (SERS) and catalysis. Yet, very few studies have been conducted on Cu-Au hybrid NS, and there are none for Cu-based NS. Herein, we describe an effective method for controlling copper-oxide nanostar (ESP-PEI-CuO-NS) growth using sporopollenin as a sustainable template material.

Outcome of pregnancy in sickle cell anemia patients with COVID-19 infection.

Sickle cell anemia (SCA) is a multisystem disease, associated with increased risk for infection and thromboembolic disease, and pregnancy is a stressor for patients with SCA. In general, coronavirus disease 2019 (COVID-19) infection in SCA is associated with a favorable outcome. Literature of pregnancy in SCA with COVID is scarce.

Is there a difference in the clinical profile and outcome of women using levonorgestrel IUD for abnormal uterine bleeding and those using it for contraception?: A comparative cross-sectional study.

Objective: The most common indications for Levonorgestrel intrauterine device (LNG-IUD) are contraception and management of abnormal uterine bleeding (AUB). This study was conducted with the aim of exploring the differences in the clinical profile and outcome of women using LNG-IUD for contraception and AUB.

Materials And Methods: This was a retrospective comparative cross-sectional study of women who underwent LNG-IUD (52 mg) between 2012 and 2017.

Parental consanguinity and ovarian reserve: A retrospective cohort study.

Background: Infertility affects around 10-15% of couples worldwide and is both a social and medical problem. Parental consanguinity is considered to reduce fertility reserve. Consanguineous marriages, especially first cousin marriages, are very common in Oman according to the Oman National Health Survey data.

Smart Nanofibrous Hydrogel Wound Dressings for Dynamic Infection Diagnosis and Control: Soft but Functionally Rigid.

Chronic wounds, such as burns and diabetic foot ulcers, pose significant challenges to global healthcare systems due to prolonged hospitalization and increased costs attributed to susceptibility to bacterial infections. The conventional use of antibiotic-loaded and metal-impregnated dressings exacerbates concerns related to multidrug resistance and skin argyrosis. In response to these challenges, our research introduces a unique approach utilizing antibiotic-free smart hydrogel wound dressings with integrated infection eradication and diagnostic capabilities.

Acquired preferences for a novel food odor do not become stronger or stable after multiple generations of odor feeding in Bicyclus anynana butterfly larvae.

Many herbivorous insects have specific host-plant preferences, and it is unclear how these preferences evolved. Previously, we found that Bicyclus anynana larvae can learn to prefer novel food odors from eating leaves with those odors and transmit those learned preferences to the next generation. It is uncertain whether such acquired odor preferences can increase across generations of repeated odor feeding and be maintained even in the absence of odor.

Superior Photophysical and Photosensitizing Properties of Nanoaggregates of Weakly Emissive Dyes for Use in Bioimaging and Photodynamic Therapy.

The development of luminescent dyes based on 1,1,4,4-tetracyanobuta-1,3-dienes (TCBDs) is an active research area, and a quantum yield (Φ) of 7.8% has been achieved so far in cyclohexane by appending a fluorophore. Our novel method radically refines weakly emissive 2,3-disubstituted TCBD (phenyl-TCBD ) (Φ = 2.

Normative data for paediatric lymphocyte subsets: A pilot study from western India.

Background & Objectives: Accurate diagnosis of immunodeficiencies requires a critical comparison of values with age-matched controls. In India, the existing reference values for rare lymphocyte subsets are currently not available and we rely on the data originating from other countries for the interpretation of the results. Furthermore, there is limited information on normal variation for these rare-subset parameters in Indian children.

Maternal, Perinatal, and Neonatal Outcomes of COVID-19 Severity in Pregnant Women: A Retrospective Study from Oman.

Objectives: To evaluate the effect of COVID-19 severity on maternal, perinatal, and neonatal outcomes in pregnant women infected with SARS-CoV-2. We also aimed to assess the effect of medical comorbidities on the severity of COVID-19.

Methods: A retrospective cohort study was conducted on women who became infected with SARS-CoV-2 during pregnancy and delivered at Sultan Qaboos University Hospital, Oman, from 1 March 2020 to 31 December 2021.

Cleavage-resistant RIPK1-induced autoinflammatory syndrome-A report of three generations with periodic fever and clinical response to colchicine.

The clinical syndrome caused by cleavage-resistant RIPK1 is known as CRIA (Cleavage-resistant RIPK1-induced autoinflammatory) syndrome. We present a family with three generations affected by CRIA syndrome. Our index patient (P1), a boy born of a non-consanguineous marriage, developed recurrent episodes of fever after 5 months of age, with variable periodicity.

Monogenic inborn errors of immunity in autoimmune disorders.

To estimate the prevalence of monogenic inborn errors of immunity in patients with autoimmune diseases (AID), the study included 56 subjects (male:female ratio: 1.07) with mean age of onset of autoimmunity 7 years (4 months-46 years). 21/56 had polyautoimmunity.

Clinical, immunological and molecular findings of patients with DOCK-8 deficiency from India.

DOCK8 deficiency affects various cell subsets belonging to both the innate and adaptive immune systems. Clinical diagnosis is challenging, as many cases present with severe atopic dermatitis as the only initial manifestation. Though flow cytometry helps in the presumptive diagnosis of DOCK8-deficient patients by evaluating their DOCK8 protein expression, it requires subsequent confirmation by molecular genetic analysis.

A study of [2 + 2] cycloaddition-retroelectrocyclization in water: observation of substrate-driven transient-nanoreactor-induced new reactivity.

Organic solvents limit [2 + 2] cycloaddition-retroelectrocyclization (CA-RE) in biological fields. We examined the formation of 1,1,4,4-tetracyanobuta-1,3-dienes (TCBDs) through CA-RE reactions and their unusual reactivity to produce N-heterocyclic compounds when the nature of the surfactant and the concentrations were varied in the aqueous phase. An environment in which transient self-assemblies (vesicles) were induced by the substrate and surfactant molecules initiated new reactivity through HO addition on the TCBD, generating the enol form of the intermediate, which results in the formation of the 6,6-dicyano-heteropentafulvene (amidofulvene) compound, while lamellar sheets at higher concentrations favored TCBD generation.

Are There Predictors of Early Diagnosis of Endometriosis Based on Clinical Profile? A Retrospective Study.

Objectives: Significant delays occur in referring cases for care of endometriosis, despite women suffering for years. This study was conducted to assess whether there is a specific symptom profile characteristic of endometriosis to alert physicians for earlier referrals.

Methods: In this retrospective observational cohort study, patient data of the women who attended Sultan Qaboos University Hospital from January 2011 to December 2019 with a diagnosis of endometriosis was collected from the hospital's electronic data archive and analyzed.

Performance analysis of autonomous UV disinfecting robot (UV bot) using Taguchi method.

As a result of the COVID-19 epidemic, there is a growing demand for robots to perform various operations which include service bots, cleaning, and disinfection bots. Viral contamination has been one of the major causes of human fatality which has abruptly increased in this situation. Availability of existing technologies is always surpassed by an effective one so as is the UV-Bot developed in this project.

The Success of Ovulation Induction with Letrozole and Gonadotropins in Obese and Nonobese Women: A Study from a Tertiary Center.

Background: Letrozole, an aromatase inhibitor, is suggested as a first-line drug for ovulation induction in women with polycystic ovary syndrome (PCOS) especially in obese women. Letrozole has also been used in women with unexplained infertility with similar rates of success to clomiphene. However, literature on letrozole and gonadotropins in obese and nonobese women is sparse.

Cellulose-Conjugated Copper-Oxide Nanoparticles for the Treatment of Ethanol-Induced Gastric Ulcers in Wistar Rats.

Gastric ulcer (GU) is the most common and chronic inflammatory condition mediated by multiple immune cells like neutrophils, macrophages, and lymphocytes with multiple pro-inflammatory cytokine interleukins such as IL-8, IL-10, IL-β, and interferon-γ (IFN-γ). Copper (Cu) is one of the essential micronutrients mainly found in the liver and brain. It plays a major role in metabolism, enzyme conversion, free radical scavenging, trafficking agents, and many others.

Thiol-Functionalized Cellulose-Grafted Copper Oxide Nanoparticles for the Therapy of Experimental Colitis in Swiss Albino Mice.

Ulcerative colitis (UC) is a chronic inflammatory disease, which deleteriously affects the lower end of the gastrointestinal tract, i.e., the colon and the rectum.