Taliglucerase alfa in the longterm treatment of children and adolescents with type 1 Gaucher disease: the Albanian experience.

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.

What do we know about the epidemiology and the management of human echinococcosis in Albania?

Echinococcosis is a life-threatening neglected zoonotic disease. Cystic echinococcosis (CE) due to Echinococcus (E.) granulosus usually involves livestock and dogs; alveolar echinococcosis (AE) due to E. multilocularis involves rodents and canines such as foxes and dogs. Human hosts are infected accidentally via hand to mouth and/or foodborne/waterborne pathways. Albania is deemed to be endemic for cystic echinococcosis (CE), but there is a scarcity of data to confirm this. A systematic literature search was performed in PubMed, Google Scholar, and in other medical sources. Because of the scarcity of existing information, data confirming CE cases were reviewed from the medical hospital records of Albania's largest Hospital, the Mother Teresa University Hospital (UHCMT) Tirana, and from a large private laboratory in Tirana (Pegasus laboratory). A total of eight eligible publications on 540 CE patients were found. Three hundred forty seven additional cases hospitalized in UHCMT from 2011 to 2020 were confirmed, as well as 36 laboratory cases and 10 Albanian cases notified in Germany. Taking all cases into account and considering 162 overlapping cases, 771 cases were documented from 2011 to 2020. The only case reported as AE was most likely a multi-organic CE. Surgery was the most frequent therapy approach used (84.7%). Autochthonous human CE seems to be widespread, and transmission is ongoing in Albania. CE patients in Albania undergo surgery more frequently compared with CE cases in other European countries. In order to establish a realistic estimate of prevalence and incidence of CE in Albania, mandatory notification should be reinforced. Stage-specific therapy can be used in CE to reduce therapy cost and diminish mortality by avoiding surgical overtreatment.

Predictors of mortality in hemodialysis patients with COVID-19: A single-center experience.

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.

Unveiling the incidences and trends of the neglected zoonosis cystic echinococcosis in Europe: a systematic review from the MEmE project.

The neglected zoonosis cystic echinococcosis affects mainly pastoral and rural communities in both low-income and upper-middle-income countries. In Europe, it should be regarded as an orphan and rare disease. Although human cystic echinococcosis is a notifiable parasitic infectious disease in most European countries, in practice it is largely under-reported by national health systems. To fill this gap, we extracted data on the number, incidence, and trend of human cases in Europe through a systematic review approach, using both the scientific and grey literature and accounting for the period of publication from 1997 to 2021. The highest number of possible human cases at the national level was calculated from various data sources to generate a descriptive model of human cystic echinococcosis in Europe. We identified 64 745 human cystic echinococcosis cases from 40 European countries. The mean annual incidence from 1997 to 2020 throughout Europe was 0·64 cases per 100 000 people and in EU member states was 0·50 cases per 100 000 people. Based on incidence rates and trends detected in this study, the current epicentre of cystic echinococcosis in Europe is in the southeastern European countries, whereas historical endemic European Mediterranean countries have recorded a decrease in the number of cases over the time.

Ischemic Heart Disease Correlates with Muslim Names in a Population of Ten Year First Admissions at Tirana University Hospital Center.

AIMS: Ischemic heart disease is coded under ICD-9 as the subgroup (410-414). Findings about these diagnoses, especially acute myocardial infarction, vary from study to study even when performed in the same ethno-religious group because of weaknesses in measuring in-group differences approach towards well-known risk factors, which is the intention of comparing our findings with similar studies. METHODS: This retrospective study used all hospital admissions retrieved from the electronic hospital database during 10 years period, 2005-2014 at Tirana University Hospital Center, "Mother Teresa". We examined all patients born between years 1905-1965, analyzing differences between patients discharged with diagnoses of ischemic heart disease to the totality of patients discriminating these diagnoses among groups of personal names coded as 'Muslim names' and 'other'. RESULTS: From 108,948 admissions there were extracted 11,411 patients fulfilling the criteria for ischemic heart disease. Admission ratio man to women was 2.2. No statistically significant difference between two group names regarding the age at first hospital admission, p=0.685. Cases coded as 'Muslim name', were more likely to encounter ischemic heart disease (IHD) during study time, (OR = 1.13; 95% CI, 1.08-1.17), p < 0.01, although after adjustment for age, sex and place of residence there seems little evidence of association, (OR = 1.06; 95% CI, 1.02-1.11), p=0.05. The subgroup of female 'Muslim name' population shows a higher risk to IHD, even after adjustment for age and place of residence (OR = 1.20; 95% CI, 1.12-1.29), p < 0.01. Results persisted when tests are replicated only for the diagnosis of acute myocardial infarction. CONCLUSIONS: Practicing religious groups would be expected to follow similar patterns of lifestyles. Taking this conclusion for granted generally oversimplifies religious affiliation as a dichotomous variable forgetting to take in consideration large in-group lifestyle differences mistakenly engendering a plurality of results, which make necessary the conceptions of evaluation tools to measure adherence to religious recommendations. Other studies in the same population as ours find Muslim men with a higher risk of non-fatal myocardial infarction (OR = 1.32; 95% CI, 0.95-1.82) which upon adjustment becomes (OR = 1.20; 95% CI, 0.85-1.70) but not significant, and another one finds Islamic religious affiliation a higher risk of acute coronary syndrome, (OR = 1.60; 95% CI, 0.95-1.82) than their Christian compatriots.

Two cases of imported hemorrhagic fever with renal syndrome and systematic review of literature.

Many factors are involved in the epidemiology of hemorrhagic fever with renal syndrome (HFRS). Imported cases, as well as those by emigrants, have been reported in literature worldwide. Our goal is to document two cases of HFRS, imported by two immigrants from two countries, and to make a review of the imported HFRS literature data. We performed a systematic literature review (PRISMA guidelines) of imported cases of HFRS and herein describe our two clinical cases. We found 20 published papers, with 16 of them in English and 4 in other languages. Twenty-three patients with travel- or immigration-associated HFRS, including our two cases, were identified. We included only papers that were in English. The average age of the patients was 35.9 ±â€¯15.13 years, and the ratio of male to female was 8:1. Imported disease from Europe to Europe occurred in seven cases, America to Europe occurred in four cases, Europe to America occurred in two cases, America to America occurred in two cases, Asia to Asia in one case, Asia to Europe in one case, and Europe to Asia in one case. The results of the two cited cases are based on the clinical-laboratory, anamnestic, and serologic data for both the patients who tested positive for HFRS. Our systematic analysis shows that international travelers are important sources of infectious diseases. HFRS related to travel and immigration is a rare event. Principal risk factors for travelers and immigrants are camping outside recommended areas or under unsuitable conditions. In recent years, various publications have shown that international travelers and immigrants have expanded the spectrum of imported infectious diseases. The literature data show that the actual reported numbers of imported case of HFRS are limited.

The role of gender in the prevalence of human leptospirosis in Albania.

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.

Predictors of esophageal varices and first variceal bleeding in liver cirrhosis patients.

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.

Hemorrhagic fever with renal syndrome in Albania. Focus on predictors of acute kidney injury in HFRS.

BACKGROUND: Hemorrhagic fever with renal syndrome (HFRS) is a rodent borne zoonosis, caused by the members of the family Bunyaviridae, genus Hantavirus. The main clinical features of the infection by this virus family are fever, thrombocytopenia and acute kidney injury. OBJECTIVE: The aim of our study was to identify, for the first time, characteristic features of HFRS in the Albanian population. STUDY DESIGN: The study comprised 33 consecutive patients admitted with suspected HFRS from April 2011-April 2016 at one center. Clinical diagnosis was confirmed by ELISA and real-time PCR. Statistical analysis was performed to identify prognostic markers and indicators of disease severity. RESULTS: The virus strain causing HFRS was Dobrava type in all 33 cases. The disease outbreaks occurred during the period June-July. Mean hospital stay was 15.7±6.9days. 29 (88%) of the patients were male. The mean age was 39.7±14.1. 16 (48.5%) patients were from Northeast Albania. 8 (24.2%) patients required dialysis. The strongest correlation was the inverse relationship of nadir platelet count with urea and creatinine, p<0.0001, p<0.0079 respectively. Creatinine and hyponatremia were inversely correlated p=0.0007, whereas hyponatremia and nadir platelet count had the highest sensitivity and specificity for development of severe AKI, 92.6%, 100%; 88.9%, 83.3% respectively. Mortality rate was 9.09%. CONCLUSION: HFRS is a severe viral disease in Albania caused by Dobrava strain. It is associated with high mortality, 9.09% in our cohort. In our study, thrombocytopenia, urinary volume, hyponatremia were indicators of more severe disease.

Radiological differential diagnosis between fibrosis and recurrence after stereotactic body radiation therapy (SBRT) in early stage non-small cell lung cancer (NSCLC).

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. 18F-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.