Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

Background

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

Disrupted local innervation results in less VIP expression in CF mice tissues

Vasoactive Intestinal Peptide (VIP) is the major physiological agonist of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) chloride channel activity. VIP functions as a neuromodulator and neurotransmitter secreted by neurons innervating all exocrine glands. VIP is also a potent vasodilator and bronchodilator that regulates exocrine gland secretions, contributing to local innate defense by stimulating the movement of water and chloride transport across intestinal and tracheobronchial epithelia. Previous human studies have shown that the rich intrinsic neuronal networks for VIP secretion around exocrine glands could be lost in tissues from patients with cystic fibrosis. Our research has since confirmed, in vitro and in vivo, the need for chronic VIP exposure to maintain functional CFTR chloride channels at the cell surface of airways and intestinal epithelium, as well as normal exocrine tissues morphology [1]. The goal of the present study was to examine changes in VIP in the lung, duodenum and sweat glands of 8- and 17-weeks old F508del/F508del mice and to investigate VIPergic innervation in the small intestine of CF mice, before important signs of the disease development. Our data show that a low amount of VIP is found in CF tissues prior to tissue damage. Moreover, we found a specific reduction in VIPergic and cholinergic innervation of the small intestine. The general innervation of the primary and secondary myenteric plexus was lost in CF tissues, with the presence of enlarged ganglionic cells in the tertiary layer. We propose that low amount of VIP in CF tissues is due to a reduction in VIPergic and cholinergic innervation and represents an early defect that constitutes an aggravating factor for CF disease progression.     

Evisceration with autogenous scleral graft and bioceramic implantation within the modified scleral shell: 133 cases over 17 years

Introduction: To present long-term follow-up data on evisceration performed with autogenous scleral grafting and ceramic implantation in a modified scleral shell.

Methods: This was a retrospective analysis of all consecutive eviscerations performed in the Department of Ophthalmology, Montpellier University Hospital, France, between February 1998 and October 2015. For all patients, the technique used was a conventional anterior evisceration after total keratectomy, disinsertion of the medial rectus muscle, sectioning of the optic nerve and excision of sclera centered on the papilla. The scleral graft was then sutured just behind the sutured keratectomy, and the bioceramic implant was inserted by posterior way in the scleral shell. Demographic characteristics, implant size and type, cosmetic results from pictures of all patients and complications were recorded. This study was performed with Ethics Review Committee Approval, and in compliance with the Declaration of Helsinki.

Results: In total, 133 patients (36.6% women) were identified during the study period. The mean (SD) implant size was 17.32 (1.84) mm. The median follow-up after evisceration was 57.43 (24.7, 68.3) months. Two cases of implant exposure (1.5%) were recorded. For 24 patients (17.9%), additional surgeries were performed for ptosis (2.2%), conjunctival cyst (1.5%), or post-evisceration socket syndrome (6.7%). Cosmetics results were excellent for 50.1% of cases, good for 33.3% and fair for 16.6%; using a grading scale based on the superior sulcus deformity.

Conclusion: Evisceration with autogenous scleral grafting and ceramic implantation can result in a high volume of restoration, good cosmetic results, and low risk of exposure of the implant.