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Introduction: Enzyme replacement therapy is already recognized as the gold standard of care for patients with Gaucher disease. Taliglucerase alfa is one of the three alternatives recommended for treatment of Gaucher disease in children and adults., Aim: This study aims to evaluate the long-term efficacy and safety of Taliglucerase alfa in children and adolescents with Type 1 Gaucher disease., Patients and Methods: Over a six-year period, we monitored the efficacy of continuous treatment in 10 patients by assessing various parameters, including hemoglobin concentration, platelet count, liver and spleen volume, bone mineral density, glucosylsphingosine level, chitotriosidase activity, and growth parameters . Safety was evaluated by immunogenicity and adverse event monitoring., Results: The mean age of patients was 13.4 ± 3.6 years and the treatment duration was 60.24 ± 13.4 months. From baseline to end line the parameters change as follows: hemoglobin concentration improved from 12.7 (±1.3) to 14.6 (±1.5) and platelet count from 180 (±74) to 198 (±79). The spleen volume, was reduced by 46% ( p  = 0,007). The chitotriosidase activity decreased from 4,019.7 (±3,542.0) nmoles/ml/hr to 2,039.5 (±1,372.2) nmoles/ml/hr (46% reduction). Glucoylsphingosine level dropped from 119.2 (±70.4) ng/ml to 86.2 (±38.1) ng/ml, indicating a reduction of 28%. Bone mineral density Z-score, improved from -1.47 (±1.76) to -0.46 (±0.99) (69.7% reduction). Out of the 1,301 total administrations, our patients reported only 37 (2.8%) infusion-related adverse events which were mild and transitory., Conclusion: Taliglucerase alfa exhibits good efficacy and a safe profile in the treatment of children and adolescents with Type 1 Gaucher disease., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Cullufi, Tomori, Velmishi, Gjikopulli, Akshija, Tako, Dervishi, Hoxha, Tanka, Troja and Tabaku.)

Introduction: The COVID-19 pandemic has disproportionately affected patients with preexisting comorbidities, particularly dialysis patients. The aim of this study was to determine predictors of mortality in this population., Methodology: We conducted an observational, retrospective, cohort study collecting data from pre and post-vaccine from the electronic medical records of a single dialysis center at Hygeia International Hospital Tirana, Albania., Results: Of 170 dialysis patients, 52 were diagnosed with COVID-19. The prevalence of COVID-19 infection in our study was 30.5%. The mean age was 61.5 ± 12.3 years and 65.4% were men. The mortality rate in our cohort was 19.2%. Mortality rates were higher in patients with diabetic nephropathy (p < 0.04) and peripheral vascular disease (p < 0.01). Elevated C- reactive protein (CRP) (p < 0.018), high red blood cell distribution width (RDW) (p < 0.03), and low lymphocyte and eosinophil counts, were found to be risk factors for severe COVID-19 disease. ROC analysis identified lymphopenia and eosinopenia as the strongest predictors of mortality. After the vaccine administration, the mortality rate in the vaccinated population was 8%, in contrast to the 66.7% mortality rate that was found in the unvaccinated group (p < 0.001)., Conclusions: Our study revealed that risk factors for the development of severe COVID-19 infection were RDW, low lymphocyte and eosinophil counts, elevated levels of CRP. Lymphopenia and eosinopenia were determined as the most important predictors of mortality, in our cohort. Mortality was notably lower among vaccinated patients., Competing Interests: No Conflict of Interest is declared, (Copyright (c) 2023 Elvana Rista, Dorina Dervishi, Vilma Cadri, Ilir Akshija, Kristi Saliaj, Silvia Bino, Edmond Puca, Arjan Harxhi.)

Introduction: Leptospirosis is a zoonotic spirochetal disease with global importance, which continues to have a major impact on public health in developing countries. The prevalence of the disease is much higher in males. The objectives of this study were: to give some data and to share our experience with human leptospirosis in Albania; to describe the prevalence regarding to the role of gender in the prevalence of human leptospirosis; to make a gender specific analysis of the clinical manifestations in patients diagnosed and treated for leptospirosis in our service and to make a review of literature related to this hypothesis., Methodology: We reviewed the epidemiologic data, risk factors and differences in clinical presentation between males and females' patients with leptospirosis. These data are analysed from hospitalized patients. Diagnosis of leptospirosis was established based on clinical presentation, epidemiological data and subsequently confirmed serologically by Anti-Leptospira IgM antibodies through ELISA test., Results: Between 2005-2016, 233 cases of confirmed leptospirosis were analysed. Males were 208 (89.27%) and 25 of patients (10.72%) were females in ratio 9:1 p < 0.001. The highest prevalence was observed in the 45-64 age groups. Overall mortality was found to be 8.58%, 19 were males and one female p < 0.001., Conclusions: There is a much higher prevalence of leptospirosis in middle aged men. Mortality rate seems to be similar in males and females. While the difference in prevalence may be related to exposure to risk factors, further investigation is necessary to study gender-based genetic and immunological predisposition.

Background: Parenchymal changes after stereotactic body radiation therapy (SBRT) make differential diagnosis between treatment outcomes and disease recurrence often difficult. The purpose of our study was to identify the radiographic features detectable at computed tomography (CT) scan [high-risk features (HRFs)] that allow enough specificity and sensitivity for early detection of recurrence., Methods: We retrospectively evaluated patients who underwent SBRT for inoperable early stage non-small cell lung cancer (NSCLC). The median delivered dose performed was 50 Gy in 5 fractions prescribed to 80% isodose. All patients underwent chest CT scan before SBRT and at 3, 6, 12, 18, 24 months after, and then annually. Each CT scan was evaluated and benign and HRFs were recorded. 18 F-fluorodeoxyglucose-CT was not used routinely., Results: Forty-five patients were included (34 males, 11 females; median age: 77 years; stage IA: 77.8%, stage IB: 22.2%; median follow-up: 21.7 months). Two year and actuarial local control was 77%. HRFs were identified in 20 patients. The most significant predictor of relapse was an enlarging opacity at 12 months (P<0.001) with 84.6% sensitivity and 71.8% specificity. The presence of ≥2 HRFs demonstrated a high sensibility (92.3%) and specificity (71.9%) (P<0.0001)., Conclusions: Detection of HRFs is predictive of relapse with a sensibility that increases with the number of HRFs observed. This observation may allow to better define the diagnostic follow algorithm up suggesting to performing further exams only in patients with >2 HRFs., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Aim: To assess "predictors" of esophageal varices (EV) and variceal bleeding using non-invasive markers in Albanian patients diagnosed with liver cirrhosis., Methods: One hundred thirty-nine newly diagnosed cirrhotic patients without variceal bleeding were included in this analysis. Model for end-stage liver disease (MELD), aspartate aminotransferase (AST) to alanine aminotransferase (ALT) ratio (AST/ALT), AST to platelet ratio index (APRI), platelet count to spleen diameter (PC/SD), fibrosis-4-index (FIB-4), fibrosis index (FI) and King's Score were measured for all participants. All patients underwent endoscopic assessment within two days of hospitalization. The major end point was the first esophageal variceal bleeding (EVB) event. The diagnostic performance of "predictors" for the presence of EV and EVB were assessed by sensitivity and specificity values obtained from the receiver operating characteristics procedure., Results: FIB-4 was the only strong and significant "predictor" of esophageal varices (multivariable-adjusted OR = 1.57 for one unit increment; 95%CI: 1.15-2.14). Furthermore, a cut-off value of 3.23 for FIB-4 was a significant predictor of esophageal varices, with a sensitivity of 72%, a specificity of 58% and a proportion of area under the curve (AUC) of 66% ( P = 0.01). During the follow-up (median: 31.5 mo; interquartile range: 11-59 mo), 34 patients (24%) experienced a first EVB. FIB-4 was a poor predictor of EVB (the AUC was only 51%) for a cut-off value of 5.02. Furthermore, the AUC of AST/ALT, APRI, PC/SD, FI, MELD and King's Score ranged from 45% to 55%. None of the non-invasive markers turned out to be a useful predictor of EVB., Conclusion: Despite the low diagnostic accuracy, FIB-4 appears the most efficient non-invasive liver fibrosis marker which can be used as an initial screening tool for cirrhotic patients., Competing Interests: Conflict-of-interest statement: None of the authors have declared any conflict of interest.

There is no or little evidence on postoperative pain assessment and treatment in Albanian hospitals. This study is based on our every day work and aims to highlight our experience. We conducted a descriptive drug utilization study which implied data collection over 6 months. Evidence of the enrolled patients was kept by maintaining records and the completed structured questionnaires. Postoperative pain was assessed through a five-category verbal rating scale (VRS). Metamizole was the most prescribed and administered analgesic drug as single therapy and in combination therapy, and acetaminophen was the least prescribed drug. The compliance between the prescribed dosages and those administered was higher in patients treated with a single analgesic compared to multiple therapies. A few patients reported adverse events (4.2 percent). There is much variability in postoperative pain management methods used by medical staff within the Tirana University Hospital. In Albania to date there is no standard protocol for postoperative pain treatment. This study shows that there are no essential differences in patient outcomes in terms of efficacy of analgesic treatment. This leads to the conclusion that a postoperative protocol/guideline for pain management should be prepared, based on our local study findings and also on international experience. Moreover, the guidelines should consider use of balanced analgesia.