Prophylactic endoscopic vacuum therapy for stricture prevention following wide-field endoscopic submucosal dissection of superficial esophageal cancer.

Background And Aims: Esophageal stricture (ES) is a severe adverse event following wide-field endoscopic submucosal dissection (ESD) of superficial esophageal carcinoma. This study evaluated the efficacy and safety of combining endoscopic vacuum therapy (EVT) and budesonide orodispersible tablet (BOT) in preventing post-ESD strictures.

Methods: This prospective case series included patients with superficial esophageal squamous cell carcinoma and adenocarcinoma who had wide-field ESD (≥75% circumference, resection length ≥50 mm).

P2RX7 gene variants associate with altered inflammasome assembly and reduced pyroptosis in chronic nonbacterial osteomyelitis (CNO).

Chronic nonbacterial osteomyelitis (CNO), an autoinflammatory bone disease primarily affecting children, can cause pain, hyperostosis and fractures, affecting quality-of-life and psychomotor development. This study investigated CNO-associated variants in P2RX7, encoding for the ATP-dependent trans-membrane K channel P2X7, and their effects on NLRP3 inflammasome assembly. Whole exome sequencing in two related transgenerational CNO patients, and target sequencing of P2RX7 in a large CNO cohort (N = 190) were conducted.

Prophylactic clipping using the over-the-scope clip (OTSC) system after complex ESD and EMR of large colon polyps.

Background: Delayed bleeding is the most frequent complication after endoscopic submucosal dissection (ESD) and endoscopic mucosal resection (EMR) of large colon polyps. Today, prophylactic clipping with through-the-scope clips (TTSCs) is commonly used to reduce the risk of bleeding. However, the over-the-scope clip (OTSC) system might be superior to TTSCs in achieving hemostasis.

Real-world evidence on efmoroctocog alfa in patients with haemophilia A: A systematic literature review of treatment experience in Europe.

Introduction: The real-world effectiveness of the efmoroctocog alfa (recombinant FVIII Fc fusion protein, a rFVIIIFc) has been investigated in numerous studies, however, currently, there exists no comprehensive collection of the existing real-world evidence (RWE) on the performance of prophylactic use of rFVIIIFc.

Aim: The aims of this systematic literature study were to identify, review, evaluate and collate the RWE of prophylactic rFVIIIFc for patients with haemophilia A reported in Europe.

Methods: We searched Medline and Embase from 2014 to February 2022 to identify publications reporting the effectiveness of rFVIIIFc in patients with haemophilia A.

Design of a Real-World Observational Study in Previously Untreated and Minimally Treated Hemophilia A Patients: Protect-NOW.

 The efficacy, safety, and immunogenicity of each of Octapharma's factor VIII (FVIII) products, Nuwiq, octanate, and wilate, have been investigated in previously untreated patients (PUPs) with severe hemophilia A in prospective clinical trials. The aim of the Protect-NOW study is to evaluate the effectiveness, safety, and utilization patterns of Nuwiq, octanate, and wilate in PUPs and minimally treated patients (MTPs; <5 exposure days [EDs] to FVIII concentrates or other blood products containing FVIII) with severe hemophilia A in a real-world setting. Real-world data provide valuable information that complement data obtained from interventional clinical trials.

Efficacy and safety of damoctocog alfa pegol prophylaxis in patients ⩾40 years with severe haemophilia A and comorbidities: analysis from the PROTECT VIII study.

Background: Advances in treatment have enabled patients with haemophilia A to live longer and therefore may be subjected to comorbidities associated with ageing, in addition to disease-associated morbidities. There have been few reports to date on efficacy and safety of treatment specifically in patients with severe haemophilia A and comorbidities.

Objective: To explore the efficacy and safety of damoctocog alfa pegol prophylaxis in patients with severe haemophilia A aged ⩾40 years with comorbidities of interest.

Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis.

Background And Objectives: Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (anti-MDAR ncephalitis ne-Year Functional tatus) score was introduced as a tool to predict disease progression in NMDARE.

Gestational diabetes mellitus and COVID-19: results from the COVID-19-Related Obstetric and Neonatal Outcome Study (CRONOS).

Background: Gestational diabetes mellitus is one of the most frequent pregnancy complications with a global prevalence of 13.4% in 2021. Pregnant women with COVID-19 and gestational diabetes mellitus are 3.

A Novel AICDA Splice-Site Mutation in Two Siblings with HIGM2 Permits Somatic Hypermutation but Abrogates Mutational Targeting.

Hyper-IgM syndrome type 2 (HIGM2) is a B cell intrinsic primary immunodeficiency caused by mutations in AICDA encoding activation-induced cytidine deaminase (AID) which impair immunoglobulin class switch recombination (CSR) and somatic hypermutation (SHM). Whereas autosomal-recessive AID-deficiency (AR-AID) affects both CSR and SHM, the autosomal-dominant form (AD-AID) due to C-terminal heterozygous variants completely abolishes CSR but only partially affects SHM. AR-AID patients display enhanced germinal center (GC) reactions and autoimmune manifestations, which are not present in AD-AID, suggesting that SHM but not CSR regulates GC reactions and peripheral B cell tolerance.

Addition of shock wave therapy to nail dynamization increases the chance of long-bone non-union healing.

Background: Long-bone non-unions after intramedullary nailing can be treated by nail dynamization or focused high-energy extracorporal shock wave therapy (fESWT). The objective of this study was to assess the effect of the combination therapy of nail dynamization and fESWT on long-bone non-unions.

Materials And Methods: 49 patients with long-bone non-unions (femur and tibia) after nailing were treated with nail dynamization (group D, n = 15), fESWT (group S, n = 17) or nail dynamization in addition to fESWT (group DS, n = 17).

Assessing Global Kidney Nutrition Care.

Background And Objectives: Nutrition intervention is an essential component of kidney disease management. This study aimed to understand current global availability and capacity of kidney nutrition care services, interdisciplinary communication, and availability of oral nutrition supplements.

Design, Setting, Participants, & Measurements: The International Society of Renal Nutrition and Metabolism (ISRNM), working in partnership with the International Society of Nephrology (ISN) Global Kidney Health Atlas Committee, developed this Global Kidney Nutrition Care Atlas.

Lifting the veil of secrecy: maternal and neonatal outcome of oocyte donation pregnancies in Germany.

Background: In Germany, performing fertility procedures involving oocyte donation is illegal, as stated by the Embryo Protection Law. Nonetheless, in our clinical routine we attend to a steadily rising number of pregnant women, who have sought oocyte donation abroad. Due to the legal circumstances many women opt to keep the origin of their pregnancy a secret.

Right ventricular function and vasoactive peptides for early prediction of bronchopulmonary dysplasia.

Background: To assess the prognostic value of early echocardiographic indices of right ventricular function and vasoactive peptides for prediction of bronchopulmonary dysplasia (BPD) or death in very preterm infants.

Methods: Prospective study involving 294 very preterm infants (median [IQR] gestational age 28.4 [26.

Telemetric capsule-based upper gastrointestinal tract - blood detection - first multicentric experience.

Introduction: Risk stratification in upper gastrointestinal bleeding (UGIB) currently relies on clinical parameters and risk scores. HemoPill acute (Ovesco Endoscopy, Tuebingen, Germany) is a pill-shaped, orally administered sensor capsule for real-time blood detection. The aim of this study was to evaluate the system in clinical routine.

Practical Guidance of the GTH Haemophilia Board on the Use of Emicizumab in Patients with Haemophilia A.

Emicizumab has been approved for bleeding prophylaxis in patients with haemophilia A (PWHAs) with or without inhibitors. Because of substantial differences between factor VIII (FVIII) and Emicizumab, the 'Ständige Kommission Hämophilie' of the German, Austrian, Swiss Society for Thrombosis and Haemostasis Research (GTH) established a practical guidance for the use of Emicizumab in PWHAs. A systematic literature research was conducted in PubMed.

Biomechanical comparison of augmented versus non-augmented sacroiliac screws in a novel hemi-pelvis test model.

Operative treatment of sacral insufficiency fractures is frequently being complicated by osteopenic bone properties. Cement augmentation of implanted sacroiliac screws may lead to superior construct stability and prevent mechanical complications. A novel hemi-pelvis test model with dissected symphysis was developed.

Mature teratoma of the temporal bone in 3.5-month-old baby girl.

Mature teratoma is a benign germ cell tumor rarely located in the temporal bone. We are reporting a case of a mature teratoma of the temporal bone in a healthy borne 3.5-month-old baby girl with a 2-day suggestive history of otitis media and polypoidal mass expulsing from the external auditory canal of the left ear.