The incidence of connective tissue diseases such as systemic lupus erythematous (SLE), in adult patients with sickle cell disease (SCD), appears to be increasing. The exact causes underlying this increased risk are still unknown, but a link with B regulatory (Breg) cells is possible as these cells suppress inflammatory responses, and maintain tolerance. Quantitative and qualitative analyses of circulating Breg cells were performed in a cohort of SCD patients with SLE, and their levels were correlated with key soluble mediators promoting autoreactive B cells.
View Article and Find Full Text PDFBackground: The literature on outcomes of patients requiring durable mechanical circulatory support (MCS) after extra-corporeal life support (ECLS) is limited. The aim of this study was to investigate the impact of preoperative ECLS cannulation on postoperative outcome after durable MCS implantation.
Methods: The durable MCS after ECLS registry is a multicenter retrospective study that gathered data on consecutive patients who underwent durable MCS implantation after ECLS between January 2010 and August 2018 in eleven high volume European centers.
Durable mechanical circulatory support (MCS) systems are established therapy option in patients with end-stage heart failure, with increasing importance during the last years due to donor organ shortage. Left ventricular assist devices (LVADs) are traditionally implanted through median sternotomy (MS). However, improvement in the pump designs during the last years led to evolvement of new surgical approaches that aim to reduce the invasiveness of the procedure.
View Article and Find Full Text PDFAcrodermatitis enteropathica is a rare autosomal recessive disease caused by a genetic mutation leading to zinc deficiency. Clinical manifestation includes skin lesions, diarrhea, and alopecia. We report the case of a two-month-old girl, admitted with erythematous scaly lesions in the neck and vesiculopustular lesions in the perioral region, associated with alopecia and diarrhea.
View Article and Find Full Text PDFBackground: Road traffic accidents are known to be the main cause of traumatic brain injury (TBI). TBI is also a leading cause of death and disability. This study, by means of the idiographic approach (single-case experimental designs using multiple-baseline designs), has examined whether methylphenidate (MPH - trade name Ritalin) had a differential effect on cognitive measures among patients with TBI with the sequel of acute and chronic post-concussion syndromes.
View Article and Find Full Text PDFDeletion of murine Thm1, an intraflagellar transport A (IFT-A) component that mediates ciliary protein trafficking, causes hyperphagia, obesity, and metabolic syndrome. The role of Thm1 or IFT-A in adipogenesis and insulin sensitivity is unknown. Here, we report that Thm1 knockdown in 3T3-L1 pre-adipocytes promotes adipogenesis and enhances insulin sensitivity in vitro.
View Article and Find Full Text PDFDue to their immunoregulatory properties, several specialized cell subsets, including regulatory T (Treg), invariant natural killer T (iNKT) and regulatory B (Breg) cells, are involved in the pathogenesis of non-Hodgkin lymphoma (NHL). However, the interaction between various cells remains to be elucidated. The aim of the present study was to evaluate the levels of Treg, iNKT and Breg cell subsets and their interrelationships in the peripheral blood (PB) and bone marrow (BM) of patients with B-cell NHL who received rituximab-based regimens and achieved a complete remission.
View Article and Find Full Text PDFA number of reports from different parts of the world have challenged the assumption that Dhat syndrome is confined to populations in and around the Indian subcontinent. This single case study reports an Omani with features typical of Dhat syndrome. Psychometric measures showed elevated scores on indices of hypochondriasis, psychasthenia, and gender role development as defined in the Minnesota Multiphasic Personality Inventory.
View Article and Find Full Text PDFDevelopment of inhibitors remains a major clinical complication in patients with hemophilia A receiving replacement therapy with factor VIII (FVIII). Understanding the immune mechanisms involved in the development of inhibitors can provide valuable information about pathways to human tolerance. Recent evidence indicates that B regulatory (Breg) cells play a pivotal role in controlling the production of antibodies (Abs) while promoting follicular T helper (Tfh) cells and monocytes, expressing the low-density lipoprotein receptor-related protein (LRP/CD91), which is involved in FVIII intake from the circulation.
View Article and Find Full Text PDFObjective: To determine the molecular basis of familial ichthyosis in three Omani families.
Subjects And Methods: Nine patients from three consanguineous families, A, B, and C, were born with typical features of lamellar ichthyosis subtype including collodion membrane and maintained ectropion, and epidermal scaling through their childhood. The 4 patients from family B had more severe symptoms requiring neonatal critical care and subsequent regular treatment with emollients, eye lubricants, and low-dose acitretin.
Sultan Qaboos Univ Med J
August 2007
Background: The rehabilitation of the traumatic brain injury (TBI) patient is especially challenging in non-western populations as the phenotypic indicators as well as the neurobehavioral assessments for the survivors of brain injury are limited.
Objective: The study screened for the prevalence of anxiety and depressive states among patients with TBI and examined the validity of the Hospital Anxiety and Depression Scale (HADS) to identify TBI patients with comorbid affective dysfunctions, specifically anxiety and depressive disorders, in an Omani population.
Methods: Sixty-eight survivors of TBI were screened with the semi-structured, Composite International Diagnostic Interview (CIDI) and the HADS.
Improving patients' knowledge has been suggested to improve their symptoms and prognosis. Very little is known about epileptic patient's knowledge of their illness in cross-cultural settings. This pilot study investigated what Omani patients know about their disorder.
View Article and Find Full Text PDFObjective: To describe the demographics, precipitating factors, substances and methods used for deliberate self-harm in Oman.
Methods: Data were extracted from the Accident and Emergency (A & E) records of patients treated at the A & E units in Muscat from 1993 to 1998. Data were obtained form the history, and clinical findings resulting form deliberate self-harm.
Purpose: To assess with a questionnaire the awareness and attitudes of the doctors in Oman toward epilepsy. Attitudes of society toward epilepsy have a wide-ranging influence, affecting issues as diverse as compliance with treatment and doctor--patient communication. Recent studies in both developing and developed countries suggest that within the medical profession, there is a lack of knowledge and negative attitudes toward people with epilepsy (PWE).
View Article and Find Full Text PDFObjective: This study reports the results of a questionnaire design to elicit doctors views about epilepsy.
Methods: Sixty-two percent of medical staff working in different regions of Oman responded. The questionnaire covered certain topics regarding the source of the knowledge of the doctors on seizure disorders and the personalities and behavior of people with epilepsy.