COVID-19 and Vaccination Status in Lysosomal Storage Diseases: A Single-Center Experience.

OBJECTIVE: Coronavirus disease 2019 (COVID-19) causes significant morbidity and mortality in individuals with chronic disease. There is not enough information about the course of coronavirus disease in lysosomal storage diseases. This study aimed to evaluate coronavirus disease vaccination status and the impact of coronavirus disease on lysosomal storage disease. MATERIALS AND METHODS: The study included 87 lysosomal storage disease patients. The patients' diagnoses were Gaucher, mucopolysaccharidosis I, II, IVA, VI, VII, Fabry, and Pompe. A questionnaire assessing severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) exposure, coronavirus disease symptoms, and vaccine status was administered in person or by phone calls. RESULTS: The number of coronavirus disease positive patients was 8 (9.1%). Only 2 patients were treated in the intensive care unit. Other coronavirus disease patients had mild symptoms and stayed in-home quarantine. Patients over 12 years of age could receive a COVID-19 vaccine. 63.5% of those aged ≥12 years were vaccinated. CONCLUSION: Lysosomal storage disease patients did not have an increased risk of COVID-19 compared to the healthy population, despite the chronic inflammatory disease. Vaccination of lysosomal storage disease patients will be protective against severe coronavirus disease.

Antifungal consumption, indications and selection of antifungal drugs in paediatric tertiary hospitals in Turkey: Results from the first national point prevalence survey.

OBJECTIVES: The aim of this point prevalence survey was to evaluate the consumption, indications and strategies of antifungal therapy in the paediatric population in Turkey. METHODS: A point prevalence study was performed at 25 hospitals. In addition to general data on paediatric units of the institutes, the generic name and indication of antifungal drugs, the presence of fungal isolation and susceptibility patterns, and the presence of galactomannan test and high-resolution computed tomography (HRCT) results were reviewed. RESULTS: A total of 3338 hospitalised patients were evaluated. The number of antifungal drugs prescribed was 314 in 301 patients (9.0%). Antifungal drugs were mostly prescribed in paediatric haematology and oncology (PHO) units (35.2%), followed by neonatal ICUs (NICUs) (19.6%), paediatric services (18.3%), paediatric ICUs (PICUs) (14.6%) and haematopoietic stem cell transplantation (HSCT) units (7.3%). Antifungals were used for prophylaxis in 147 patients (48.8%) and for treatment in 154 patients (50.0%). The antifungal treatment strategy in 154 patients was empirical in 77 (50.0%), diagnostic-driven in 29 (18.8%) and targeted in 48 (31.2%). At the point of decision-making for diagnostic-driven antifungal therapy in 29 patients, HRCT had not been performed in 1 patient (3.4%) and galactomannan test results were not available in 12 patients (41.4%). Thirteen patients (8.4%) were receiving eight different antifungal combination therapies. CONCLUSION: The majority of antifungal drugs for treatment and prophylaxis were prescribed in PHO and HSCT units (42.5%), followed by ICUs. Thus, antifungal stewardship programmes should mainly focus on these patients within the availability of diagnostic tests of each hospital.

Change in Pathogens Causing Late-onset Sepsis in Neonatal Intensive Care Unit in Izmir, Turkey.

OBJECTIVE: Neonatal sepsis is a common cause of morbidity and mortality among newborns in the developing world. We have investigated the causative agents and their antimicrobial susceptibility of late-onset sepsis (>72 h post-delivery), and determined the possible association between various risk factors and the mortality due to neonatal sepsis in 2008. To view the changes in years, we compared them with the data which we gained in 2004. METHODS: Medical records of all neonates with late-onset sepsis were reviewed for demographic characteristics (birth weight, gestational age, gender, type of delivery, and mortality rate), positive cultures and risk factors of mortality. FINDINGS: One hundred and forty-seven and 227 neonates had been diagnosed as late-onset sepsis in 2004 and 2008, respectively. Coagulase-negative staphylococcus was the most frequent microorganisms. Gram-negative bacilli, particularly Pseudomonas aeruginosa showed a significant increase in years. The mortality rate was 11.5% and 19% in 2004 and 2008, respectively. Birth weight, gestational age, and infection with Klebsiella spp. isolates were found to have significant association with sepsis mortality in our neonatal intensive care unit (NICU). CONCLUSION: The present study emphasizes the importance of periodic surveys of sepsis encountered in particular neonatal setting to recognize the trend. Increased Gram-negative bacilli rate was possibly related to the widespread use of antibiotics in our NICU.