Exploring congenital sucrase-isomaltase deficiency in autism spectrum disorder patients with irritable bowel syndrome symptoms: A prospective SI gene sequencing study.

Congenital sucrase-isomaltase deficiency (CSID) is an inherited metabolic disorder causing chronic gastrointestinal symptoms and malnutrition when untreated. Most CSID patients are likely to remain under- or misdiagnosed. This study aimed to investigate prevalence of CSID among patients with autism spectrum disorder (ASD) presenting with irritable bowel syndrome (IBS) symptoms via prospective SI gene sequencing.

Use of ivabradine in children with junctional ectopic tachycardia after pediatric cardiac surgery; two-centre experience.

Introduction: Although amiodarone is traditionally used in the treatment of postoperative junctional ectopic tachycardia (JET), the search for new treatments is ongoing. We present our experience with ivabradine at two medical centers.

Materials And Methods: Between January 2022 and January 2023, patients who developed JET after pediatric cardiac surgery were prospectively followed up and documented.

Management of patients developing axillary pseudoaneursym after ductal stenting: Report of three cases.

Pseudoaneurysms develop as a result of disruption of the arterial wall due to trauma or iatrogenic reasons such as catheterization, and it is important due to the high risk of bleeding and rupture. Until recently, the main treatment of pseudoaneurysms was surgical repair. However, in recent years, minimally invasive methods such as ultrasound-guided compression and percutaneous thrombin injection have been used more frequently.

Factors Associated with the Development of Anti-drug Antibodies to TNFi and the Consequences for Axial Spondyloarthritis: A Two-year Follow-up Study.

Objective: To evaluate the development of anti-drug antibodies (ADAb) against tumor necrosis factor inhibitors (TNFi) therapy during a 2-year period and search the factors linked to patients with axial spondyloarthritis (axSpA).

Methods: Biologic-naive patients with axSpA were included in this observational study. Serum drug levels and ADAb were measured at weeks 12, 24, 52, and 104 of treatment by enzyme-linked immunosorbent assay (ELISA).

Comparative Value of CRP and FCP for Endoscopic and Histologic Remissions in Ulcerative Colitis.

We have previously shown that CRP < 2.9 mg/L is a better predictor of endoscopic remission (ER) than CRP < 5 mg/L in ulcerative colitis (UC). Here, we prospectively evaluate CRP and FCP cut-offs and compare them in predicting ER and histological remission (HR) in UC.

Behçet's syndrome: one year in review 2024.

The aim of this review is to provide a critical summary of studies published during 2023 that contribute to our understanding of Behçet's syndrome. An increase in the incidence of BS was reported in Northern Spain after 2014.Studies on patient perspectives showed the impact of Behçet's syndrome on quality of life, daily activities, education, work, and relationships.

Assessment of the frequency and risk factors of gastrointestinal bleeding after cardiopulmonary bypass in paediatric cases.

Introduction And Aim: Gastrointestinal bleeding is a potential complication in paediatric patients undergoing cardiopulmonary bypass, as it develops secondary to low gastrointestinal perfusion. This study aimed to examine the incidence of gastrointestinal bleeding and identify its risk factors in these patients.

Method: This retrospective study was undertaken to examine the demographic features, clinical findings, and operative data of paediatric patients under years old who had undergone congenital heart surgery with cardiopulmonary bypass between November 1, 2021, and November 1, 2023.

Impact of high-flow nasal oxygen therapy on postoperative atelectasis and reintubation rate after paediatric cardiac surgery.

Introduction: Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients.

Case report: Dysphagia aortica.

Dysphagia, characterized by abnormal swallowing, presents as oropharyngeal or esophageal dysphagia. Dysphagia aortica, a rare manifestation, results from external aortic compression, leading to swallowing difficulties. Limited literature exists on this condition.

Myocardial infarction and narrowed peripheral arterial vessels secondary to generalised arterial calcification syndrome in a two-month-old girl.

Generalised arterial calcification of infancy, an autosomal recessive disorder characterised by abnormal calcification of medium and large-sized arteries, represents a rare cause of dilated cardiomyopathy. We present the case of a two-month-old girl diagnosed posthumously with dilated cardiomyopathy. Studies suggest that early initiation of treatment can improve prognosis in generalised arterial calcification of infancy, so clinicians should be alert to the condition, especially in patients displaying generalised narrowing of medium and large-sized arteries.

Efficacy and safety of infliximab in patients with autoimmune hepatitis.

Background And Aims: A limited number of drugs are used as standard or alternative therapies in autoimmune hepatitis (AIH). No specific recommendations are available for patients failing to respond to these therapies. We analyzed the efficacy and safety of infliximab in patients with AIH.

Mimickers of nervous system involvement among patients with Behçet's syndrome.

Objectives: We aimed to identify conditions mimicking nervous system involvement among patients with Behçet's syndrome (BS) and to determine clinical, laboratory and imaging findings that may help in the differential diagnosis.

Methods: We screened the charts of 500 consecutive BS patients to identify those who were referred to neurology at any time during their follow-up. The final diagnoses, presenting signs and symptoms, laboratory and imaging results were retrieved from patient charts.

Development of Posterior Uveitis in Behçet Syndrome Patients with Vitreous Cells at Baseline.

Purpose: The prognostic importance of vitreous cells (VC) in patients with Behçet syndrome (BS) is unknown. We aimed to determine the frequency of developing posterior uveitis (PU) and any additional risk factors associated with the development of PU in BS patients with VC at diagnosis.

Methods: The charts of 572 consecutive BS patients who were registered between 2010 and 2012 were reviewed.

Tocilizumab may not be a good option for vascular involvement due to Behçet's syndrome.

Objectives: Tocilizumab has been increasingly reported as an alternative therapeutic agent in the management of Behçet's syndrome (BS) and it has been mostly tried in BS patients with neurological and eye involvement. As therapeutic responses to each drug may vary across different types of BS involvement, we aimed to report seven patients with large vessel involvement treated with tocilizumab.

Methods: We enrolled seven BS patients with vascular involvement who were given tocilizumab at the Behçet's Disease Research Centre in Istanbul University-Cerrahpaşa between 2000 and 2022.

Potential antiplatelet agents with grape seed - backbone polyphenols: computational studies.

The study focused on grape seed-derived polyphenols for their antiplatelet, anti-inflammatory, and fibrinolytic properties through molecular docking and dynamics simulations. Compounds were evaluated for their effects on P2Y12, PTP1B, thromboxane A2, and other targets. Compounds 1 and 6 showed strong inhibitory potential on P2Y12.

A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System.

Introduction/objective: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort.

Methods: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment.

Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

Validation of the Behçet's Syndrome Overall Damage Index (BODI) for Retrospective Studies and a Proposal for Modification.

Objective: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data.

Ascites does not accompany pleural effusion developing under dasatinib therapy in patients with CML-CP.

Objectives: Pleural effusion (PE) is the most frequent pulmonary complication of dasatinib, a tyrosine kinase inhibitor (TKI). Concurrent pericardial effusions have been reported in about one-third of the cases. In this study, we aimed to investigate ascites generation in chronic-phase chronic myeloid leukemia (CML-CP) patients developing PE under dasatinib.

Screening for pulmonary arterial hypertension in patients with systemic sclerosis in the era of new pulmonary arterial hypertension definitions.

Objectives: This study compares the performance of three composite pulmonary arterial hypertension (PAH) screening tools in a real-life SSc cohort, according to both the previous 2015 ESC/ERS guideline and the recent 2022 ESC/ERS guideline haemodynamic criteria.

Methods: Consecutive SSc patients without a previous diagnosis of pulmonary hypertension (PH) were screened for PAH using the European Society of Cardiology/European Respiratory Society (ESC/ERS), DETECT, and Australian Scleroderma Interest Group (ASIG) algorithms. Right heart catheterisation (RHC) referral performances for PAH were compared according to the 2022 ESC/ERS PAH criteria.

Behçet's syndrome.

Behçet's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Behçet's syndrome is a multifactorial disease and infectious, genetic, epigenetic, and immunological factors contribute to its pathogenesis. Its heterogeneous spectrum of clinical features include mucocutaneous, articular, ocular, vascular, neurological, and gastrointestinal manifestations that can present with a relapsing and remitting course.

Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis.

The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment.