Rheumatologic rehabilitation: the great expectation for rheumatic patients

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VULVAR HISTOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL CHANGES IN PATIENTS WITH PRIMARY SJÖGREN SYNDROME

Background: Primary Sjogren Syndrome (pSS) is an autoimmune disease mostly affecting women, characterized by a lymphocyte-mediated infiltration and destruction of several exocrine glands, which causes mucosal dryness. Genital involvement is frequent and characterized by vulvar and vaginal dryness, dyspareunia and pruritus, that significantly impairs sexual function. However, despite the high frequency of genital involvement, few data were published about the histopathology of external genitalia in pSS. The studies performed until now show that vaginal and vulvar dryness are due to the presence of a vulvar inflammatory infiltrate and to the atrophy of minor and major vestibular glands, whose secretions are important for the sexual function. Objectives: To evaluate the presence and the characteristics of histopathological and immunohistochemical changes in vulvar tissues in women with pSS. Methods: Women with pSS (21 patients) underwent vulvar biopsies that have been evaluated for histopathological and immunohistochemical changes and finally compared with those obtained from 26 patients with lichen sclerosus. Results: An inflammatory infiltrate (composed predominantly by T lymphocytes (CD3+), sparse CD20+ B cells and mean CD4:CD8 T-cell ratio of 1.5) was evidenced in all 21 biopsies and classified in mild (10), moderate (11) and severe (0). No correlation was shown between vulvar inflammatory infiltrate score and salivary Chisholm e Mason score. No differences were found neither in gynecological symptoms neither in clinical and demographical characteristics between patients with mild and those with moderate vulvar inflammatory score. A higher prevalence of moderate inflammatory infiltrate was observed in biopsies from women with lichen sclerosus than in pSS

A Rare Pathology Masquerading as Myocardial Infarction

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Troubleshooting During Temporary Epicardial Lead Implantation in a Child with an Erosive Twiddler’s Syndrome and Multiple Sternotomies: A case report

Temporary epicardial cardiac pacing in patients with bradyarrhythmias may be used as a bridge to implantation of a permanent pacemaker. The temporary epicardial lead placement may sometimes necessitate a sternotomy that may pose a challenge in patients who have had multiple earlier sternotomies. The difficulty in accessing the epicardium for urgent implantation of temporary epicardial pacing leads depends on the extent of adhesions in such patients. We report an 8-year-8-month-old girl with a pacemaker with an extruded pulse generator and difficult myocardial access due to 5 prior transsternal procedures. The child presented to a tertiary care hospital in Muscat, Oman, in 2021. A trouble-shooting technique was adopted to achieve temporary epicardial pacing to provide time for a course of antibiotic therapy administration. A permanent transvenous pulse generator system was implanted after 7 days of temporary pacing. Keywords: Heart Block; Artificial Pacemaker; Implanted Electrodes; Case Report; Oman

Spatial Orientation in Cardiac Ultrasound Images Using Mixed Reality: Design and Evaluation

Spatial orientation is an important skill in structural cardiac imaging. Until recently, 3D cardiac ultrasound has been visualized on a flat screen by using volume rendering. Mixed reality devices enhance depth perception, spatial awareness, interaction, and integration in the physical world, which can prove advantageous with 3D cardiac ultrasound images. In this work, we describe the design of a system for rendering 4D (3D + time) cardiac ultrasound data as virtual objects and evaluate it for ease of spatial orientation by comparing it with a standard clinical viewing platform in a user study. The user study required eight participants to do timed tasks and rate their experience. The results showed that virtual objects in mixed reality provided easier spatial orientation and morphological understanding despite lower perceived image quality. Participants familiar with mixed reality were quicker to orient in the tasks. This suggests that familiarity with the environment plays an important role, and with improved image quality and increased use, mixed reality applications may perform better than conventional 3D echocardiography viewing systems.publishedVersio

Propofol and Kearns-Sayre Syndrome: An idiographic approach

With the focus on an idiographic approach whereby the observations incorporated the various dimensions of individual functioning ‘top-down’ to ‘bottom-up’, this case report describes the successful management of a 14-year-old girl with Kearns-Sayre syndrome and Dyggve-Melchior-Clausen disease requiring a transvenous permanent pacemaker implantation for complete heart block. The patient presented to a tertiary care centre in Muscat, Oman, in 2023 seeking consultation. The current idiographic approach appears to have a heuristic value for 2 interrelated reasons. Firstly, it is unlikely that even tertiary care units can accrue such rare presentations and scrutinise them under nomothetic approach. Secondly, by employing the idiographic approach that is capable of examining each case in-depth, the aspiration for good health and well-being may come to the forefront. To the best of the authors’ knowledge this is the first published idiographic report in anaesthesia care. Keywords: Intravenous Anaesthetics; Artificial Pacemaker; Kearns-Sayre Syndrome; Propofol; Idiographic Approach; Case Report; Oman

A Newly Detected Left Ventricular Mass Following A Complex Intracardiac Repair

Appearance of unexpected masses in the chambers of the heart during cardiac surgery can be intriguing. We report the case of a mass in the left ventricle that appeared at the time of separation from cardiopulmonary bypass in a child after a complex intracardiac repair. The child presented for surgery to a tertiary care hospital in Muscat, Oman, in 2022. Prior to the surgical repair the mass was not appreciated by echocardiography. An intraventricular baffle was used to divert left ventricular blood flow towards the outflow tract, after which an intraventricular “mass” was observed. Intraoperative transoesophageal echocardiography identified the mass as a portion of the interventricular septum that was located between the inlet and outlet ventricular septal defects. Keywords: Echocardiography; Heart Septal Defects; Heart Ventricles; Diagnostic Imaging

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience

Sinus venosus atrial septal defects present a wide variety of anatomical features and are frequently associated with partial anomalous pulmonary venous drainage of one or more right pulmonary veins. Surgical correction used to be the standard treatment. In recent times, transcatheter correction of superior sinus venosus atrial septal defects has come into vogue. The transcatheter closure of these defects with covered stents at a tertiary care centre in Oman between 2018 and 2023 is reported. Keywords: Heart Septal Defects, Atrial; Vena Cava, Superior; Endovascular Procedures; Stents; Cardiac Catheterization; Echocardiography, Transesophageal; Oman