Syndromic Retinitis Pigmentosa : A Narrative Review

Retinitis pigmentosa (RP) encompasses inherited retinal dystrophies, appearing either as an isolated eye condition or as part of a broader systemic syndrome, known as syndromic RP. In these cases, RP includes systemic symptoms impacting other organs, complicating diagnosis and management. This review highlights key systemic syndromes linked with RP, such as Usher, Bardet–Biedl, and Alström syndromes, focusing on genetic mutations, inheritance, and clinical symptoms. These insights support clinicians in recognizing syndromic RP early. Ocular signs like nystagmus and congenital cataracts may indicate systemic disease, prompting genetic testing. Conversely, systemic symptoms may necessitate eye exams, even if vision symptoms are absent. Understanding the systemic aspects of these syndromes emphasizes the need for multidisciplinary collaboration among ophthalmologists, pediatricians, and other specialists to optimize patient care. The review also addresses emerging genetic therapies aimed at both visual and systemic symptoms, though more extensive studies are required to confirm their effectiveness. Overall, by detailing the genetic and clinical profiles of syndromic RP, this review seeks to aid healthcare professionals in diagnosing and managing these complex conditions more effectively, enhancing patient outcomes through timely, specialized intervention

AF-FLOW Global Registry Confirms Validity of Electrographic Flow Mapping as a Phenotyping Tool for Atrial Fibrillation

Electrographic flow (EGF) mapping allows for the visualization of global atrial wavefront propagations. One mechanism of initiation and maintenance of atrial fibrillation (AF) is stimulation from EGF-identified focal sources that serve as driver sites of fibrillatory conduction. Electrographic flow consistency (EGFC) further quantifies the concordance of observed wavefront patterns, indicating that a healthier substrate shows more organized wavefront propagation and higher EGFC. Freedom from AF (FFAF) recurrence has accordingly been shown to be higher in patients with ablated vs. unablated sources and with high vs. low EGFC.(1) Measure FFAF across EGF-derived phenotypes in patients enrolled in the AF-FLOW Global Registry; (2) determine if a relationship exists between EGFC and percentage of healthy voltage as measured from bipolar voltage maps.The AF-FLOW Global Registry is a multicenter, prospective study of 25 all-comer AF patients who underwent concomitant high-density bipolar voltage mapping with a 16-electrode grid mapping catheter and EGF mapping with a 64-pole basket catheter. The EGF algorithm detects extra-pulmonary vein sources as origins of excitation from a singularity of divergent flow vectors and was used to localize RF ablation targets. Overall, EGFC per atrium was also computed as the average of the modulus of individual EGF vectors, where the vector length represents the consistency of flow patterns. Patients were then assigned phenotypes on the basis of source presence or absence and EGFC, and rates of FFAF at 1-year were compared across the four resulting phenotypes. Atrial EGFC was also compared to the percentage of healthy tissue determined by bipolar voltage mapping.Patients with paroxysmal AF had higher FFAF than persistent AF (PeAF) and long-standing PeAF patients; patients receiving de novo ablation had higher FFAF than those receiving redo ablation. Patient phenotyping revealed that those with high EGFC had higher FFAF than those with low EGFC (p = 0.015). Atrial EGFC was also correlated to the percent of high voltage tissue across all patients (r = 0.651, p < 0.0001).EGF mapping provides insights into the mechanistic nature of AF and the atrial health of the underlying substrate. Therefore, further studies are needed to develop phenotype-specific treatments for the disease.ClinicalTrials.gov identifier: NCT05481359

Opioid Utilisation in Hungary: National and Regional Analysis in Ambulatory and Hospital Care Sector

Background/Objectives: Opioid consumption analysis in Hungary, particularly through ambulatory and hospital sales data, including regional information, is lacking. This study examines opioid use in both sectors, explores regional variations, and identifies influencing factors. Methods: A cross-sectional analysis was conducted using sales data from ambulatory and hospital care, quantifying opioid consumption in defined daily doses (DDD) per 1000 inhabitants (DID) and per day, or DDD per 100 patient days (DHPD) at national and regional levels. Correlations between opioid utilisation and regional variables were assessed using Spearman’s rank test. Results: Total opioid use has risen from 4.73 DID in 2012 to 6.75 DID in 2021, with weak and oral opioids being the most used. Ambulatory care experienced significant increases in weak (61.48%) and oral opioid use (60.01%). Hospital care experienced a decline in DID and stagnation in DHPD. Tramadol combinations grew notably in ambulatory care, with tramadol-paracetamol rising from 0.37 DID to 2.17 DID (484.61% increase) and tramadol-dexketoprofen from 0.12 DID to 0.91 DID (650.27% increase). Interregional differences showed a maximum to minimum ratio of 1.79 in ambulatory and 3.03 in hospital care in 2021. Positive correlations were found between opioid use and geriatric population percentage (r = 0.475; p = 0.035) and, also, unemployment rate (r = 0.546; p = 0.014). A moderate negative correlation was observed between the number of general practitioners (r = −0.458; p = 0.043) and ambulatory care opioid use. Conclusions: Opioid use is increasing in Hungarian ambulatory care while remaining steady in the hospital sector. Regional variations are possibly linked to demographic and economic factors in ambulatory care