Genotype-Phenotype Relationship among 785 Unrelated White Women with Inherited Congenital Factor VII Deficiency: A Three-Center Database Study.

Background: Congenital factor VII (FVII) deficiency, a rare bleeding disorder resulting from mutations in the F7 gene with autosomal recessive inheritance, exhibits clinical heterogeneity that lacks a strong correlation with FVII:C levels. The objective of this study was to discern genetic defects and assess their associations with the clinical phenotype in a substantial cohort comprising 785 white women exhibiting FVII:C levels below the age-dependent cut-off percentage.

Patients And Methods: Individuals with verified inherited factor VII deficiency underwent i) genotyping using the Sanger method and multiplex ligation-dependent probe amplification (MLPA) to identify F7 mutations, including common polymorphic variants.

Outcome and quality of life after intracranial meningioma surgery in cats.

Objectives: The present study aimed to evaluate the postoperative quality of life (QOL) after surgery for the treatment of intracranial meningioma in cats.

Methods: The study included 14 cases that underwent craniotomy from May 2009 to March 2021. Owners were contacted via telephone after a median time of 967 (range 227-4209) days after surgery and surveyed with a specially designed questionnaire that consisted of three domains, subdivided into different items.

Pharyngeal dermoid cyst causing partial upper airway obstruction in a cat.

Case Summary: A 2-year-old cat was presented with nasopharyngeal stridor and stertor. Radiographs of the upper neck region showed a mass lesion in the nasopharynx. A nasopharyngeal polyp was suspected, but an attempt at endoscopic removal failed, owing to fragmentation of the mass and excessive haemorrhage.

Comparison of Extracapsular Stabilization Techniques Using an Ultrasonically Implanted Absorbable Bone Anchor (Weldix) after Cranial Cruciate Ligament Rupture in Cats-An In Vitro Study.

Background: This study evaluated joint stability after surgical repair of cranial cruciate ligament (CrCL)-deficient stifle joints in cats using a novel absorbable polylactide bone anchor in an ex vivo model.

Methods: Thirty-six hindlimbs from cats with intact (G group) and transected CrCLs were treated with fabellotibial suture alone (G group), suture combined with an absorbable polylactide bone anchor (G group), or suture combined with a nonabsorbable bone anchor (G group), positioned in a limb press with predefined joint angles (stifle joint: 120 ± 5°; hock joint: 120 ± 5°) and loaded with 10%, 20%, and 30% of body mass (BM). Predefined points were measured on lateral radiographs and with a coordinate measurement machine.

Interleukin-11 (IL-11) receptor cleavage by the rhomboid protease RHBDL2 induces IL-11 trans-signaling.

Interleukin-11 (IL-11) is a pleiotropic cytokine with both pro- and anti-inflammatory properties. It activates its target cells via binding to the membrane-bound IL-11 receptor (IL-11R), which then recruits a homodimer of the ubiquitously expressed, signal-transducing receptor gp130. Besides this classic signaling pathway, IL-11 can also bind to soluble forms of the IL-11R (sIL-11R), and IL-11/sIL-11R complexes activate cells via the induction of gp130 homodimerization (trans-signaling).

Impact of atrial fibrillation burden on cognitive function after left atrial ablation - Results of the MACPAF study.

Atrial fibrillation (AF) is associated with cognitive decline and dementia irrespective of AF-related ischemic stroke. We investigated whether AF burden after ablation in patients with symptomatic paroxysmal AF has an impact on cognitive function. After enrolment to the prospective MACPAF study, study patients received an insertable loop recorder (ILR) and underwent serial neurological/cognitive assessment.

Loop Recorder Detected High Rate of Atrial Fibrillation Recurrence after a Single Balloon- or Basket-Based Ablation of Paroxysmal Atrial Fibrillation: Results of the MACPAF Study.

Purpose: Pulmonary vein isolation (PVI) is an established approach to treat symptomatic non-permanent atrial fibrillation (AF). Detecting AF recurrence after PVI is important, if discontinuation of oral anticoagulation after ablation is considered.

Methods: Patients with symptomatic paroxysmal AF were enrolled in the prospective randomized mesh ablator vs.

Neuropsychological effects of MRI-detected brain lesions after left atrial catheter ablation for atrial fibrillation: long-term results of the MACPAF study.

Background: MRI-detected brain lesions are common after left atrial catheter ablation for symptomatic atrial fibrillation. The clinical relevance of these acute ischemic lesions is not fully understood, but ablation-related cerebral injury could contribute to cognitive dysfunction.

Methods And Results: In the prospective Mesh Ablator versus Cryoballoon Pulmonary Vein Ablation of Symptomatic Paroxysmal Atrial Fibrillation (MACPAF) study, serial 3-T brain MRIs and neuropsychological assessment were performed to analyze the rate of ablation-related brain lesions and their effect on cognitive function.

3 Tesla MRI-detected brain lesions after pulmonary vein isolation for atrial fibrillation: results of the MACPAF study.

Background: Left atrial catheter ablation (LACA) is an established therapeutic approach to abolish symptomatic atrial fibrillation (AF).

Objective: Based on the prospective MACPAF study (clinicaltrials.gov NCT01061931) we report the rate of ischemic brain lesions postablation and their impact on cognitive function.

Mesh ablator vs. cryoballoon pulmonary vein ablation of symptomatic paroxysmal atrial fibrillation: results of the MACPAF study.

Aims: Catheter ablation of the pulmonary veins (PVs) is a promising therapeutic approach for symptomatic atrial fibrillation (AF). The prospective randomized single-centre study 'Mesh Ablator versus Cryoballoon Pulmonary Vein Ablation of Symptomatic Paroxysmal Atrial Fibrillation' (MACPAF; clinicaltrials.gov NCT01061931) compared the efficacy and safety of two balloon-based PV ablation systems.

Comparison of skin adhesive and absorbable intracutaneous suture for the implantation of cardiac rhythm devices.

Aims: Wound healing is a major determent in the post-surgical course of patients (pts) after pacemaker (PM) and implantable cardioverter defibrillator (ICD) implantation. Insufficient closure may lead to serious complications with pocket infections leading to the device's explantation as the worst case scenario. In addition to the different types of suture and suture clips, a novel topical skin adhesive containing 2-octyl-cyanoacrylate is commercially available.

Safety and reliability of the insertable Reveal XT recorder in patients undergoing 3 Tesla brain magnetic resonance imaging.

Background: Up to now there is little evidence about the safety and reliability of insertable cardiac monitors (ICMs) in patients undergoing magnetic resonance imaging (MRI).

Objective: The purpose of this prospective single-center study (MACPAF; clinicaltrials.govNCT01061931), which we are currently performing, was to evaluate these issues for the ICM Reveal XT at a 3 Tesla MRI scanner in patients undergoing serial brain MRI.

Stroke risk associated with balloon based catheter ablation for atrial fibrillation: Rationale and design of the MACPAF Study.

Background: Catheter ablation of the pulmonary veins has become accepted as a standard therapeutic approach for symptomatic paroxysmal atrial fibrillation (AF). However, there is some evidence for an ablation associated (silent) stroke risk, lowering the hope to limit the stroke risk by restoration of rhythm over rate control in AF. The purpose of the prospective randomized single-center study "Mesh Ablator versus Cryoballoon Pulmonary Vein Ablation of Symptomatic Paroxysmal Atrial Fibrillation" (MACPAF) is to compare the efficacy and safety of two balloon based pulmonary vein ablation systems in patients with symptomatic paroxysmal AF.

Limitations of the past and latest evolutions of home monitoring: arrhythmia electrograms transmitted automatically improve the efficacy of remote monitoring.

Background: Telemetric surveillance systems are part of a well-accepted and evolving field in the care of cardiac patients. Especially in patients with implanted cardioverter-defibrillators (ICD), they are well established and their usefulness and reliability have been shown in several clinical trials. Currently, three generations of Home Monitoring™ (HM, Biotronik GmbH & Co.

Potential role of home monitoring to reduce inappropriate shocks in implantable cardioverter-defibrillator patients due to lead failure.

Aims: Lead dysfunctions in implantable cardioverter-defibrillator (ICD) patients can lead to inappropriate shocks or even complete loss of function of the device. Home monitoring (HM) systems are capable of daily data transmissions regarding the device and the lead integrity as well as information concerning anti-arrhythmic therapies. We therefore analysed the data from the Biotronik HM system whether it enables physicians to react quickly on serious ICD malfunctions and to avoid inappropriate shocks.

Detection of patients with coronary artery disease using cardiac magnetic field mapping at rest.

We studied the use of cardiac magnetic field mapping to detect patients with CAD without subjecting them to stress. Fifty-nine healthy control subjects and 101 patients with CAD without previous MI were included. The optimal positions for detecting CAD were located in the left superior parasternal and in the inferior midsternal area.

Circadian rhythm of fasting and postprandial portal blood flow in cirrhosis.

Objective: To examine the relationship of both the unstimulated and the postprandial portal blood flow (PVF) to the time of day and to determine its intra-individual reproducibility over time in patients with liver cirrhosis.

Material And Methods: In 24 cirrhotic patients, 27 PVF measurements were performed during 24 h on day 0 and day 7 using Doppler ultrasound. Three standard liquid meals were given orally.

Ligation versus propranolol for the primary prophylaxis of variceal bleeding in cirrhosis.

In this randomized controlled multicenter trial, we compared endoscopic variceal banding ligation (VBL) with propranolol (PPL) for primary prophylaxis of variceal bleeding. One hundred fifty-two cirrhotic patients with 2 or more esophageal varices (diameter >5 mm) without prior bleeding were randomized to VBL (n = 75) or PPL (n = 77). The groups were well matched with respect to baseline characteristics (age 56 +/- 10 years, alcoholic etiology 51%, Child-Pugh score 7.

Prognostic impact of renal impairment and sodium imbalance in patients undergoing transjugular intrahepatic portosystemic shunting for the prevention of variceal rebleeding.

Background/aim: Kidney function and portal pressure have prognostic relevance in nonshunted patients with cirrhosis. Since insertion of a transjugular intrahepatic portosystemic shunt (TIPS) reduces portal pressure and may improve the renal function, the aim of the present study was to investigate the prognostic role of renal impairment and portal hemodynamics in patients with compensated cirrhosis electively shunted due to recurrent variceal hemorrhages.

Methods: Data of 101 consecutive and prospectively followed patients with compensated cirrhosis (bilirubin <5 mg/dl) undergoing elective TIPS due to recurrent variceal bleeding (45 died, and 8 were transplanted during the follow-up period) were evaluated in a multivariate Cox model.