Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9 ± 68.7 months (range: 2–202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3 days (1–67 days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1 = 2.3[1.48–3.43]) and etoposide usage (OR2 = 1.22 [1.14–1.89]). The patient’s 30-day survival was inferior among patients whose albumin level was 2 g/dL or less compared to those over 2 g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.

Children are at higher risk of influenza complications. The goals of this article are, estimating influenza vaccination coverage of Health Care Workers (HCWs) in tertiary children hospital, evaluating attitudes and practices of HCWs and evaluating whether HCWs vaccination uptake improved with onsite vaccination campaign. This was a before-after trial, which was carried out in a tertiary children hospital at 2017–2018 influenza season. The vaccination team visited all participants and collected information about previous vaccination uptake, attitudes and beliefs of HCWs by means of an anonymous questionnaire. Moreover, the influenza vaccine was offered onsite to all participants. A total of 572 HCWs participated in this study (response rate: 94.2%). Coverage was 10.8% in 2016–17 season and 39.9% in 2017–18 season (p < 0.0001). Multivariate regression analysis showed that being younger than 35 years (OR: 2.09), being vaccinated in previous season (OR: 47.02) and professional category of the participant (clinicians being reference group; OR: 1.73 for support staff and OR: 0.23 for nurses,) were significantly associated with vaccination uptake in 2017–18 season [95% CI]. None of the participants with former bad experience about vaccination was vaccinated in 2017–2018 season. And 90% of the participants having lack of knowledge about the vaccine were vaccinated in 2017–2018 season. After onsite vaccination campaign, influenza vaccination coverage improved significantly among HCWs. In order to achieve target vaccination coverage we should break down the prejudices with a comprehensive education program. Abbreviations: OR- Odds ratio; CI- confidence interval

Background: Glycogen synthase deficiency, also known as glycogenosis (GSD) type 0 is an inborn error of glycogen metabolism caused by mutations in theCase presentation: Herein we report three new cases of liver glycogen synthase deficiency (GSD0). The first patient presented at the 4 years of age with recurrent hypoglycemic seizures. The second patient who is the brother of the first patient presented at 15 months with asymptomatic incidental hypoglycemia. Glucose monitoring in both patients revealed daily fluctuations from fasting hypoglycemia to postprandial hyperglycemia and lactic acidemia. A third patient was consulted for ketotic hypoglycemia and postprandial hyperglycemia at the 5 years of age.Conclusions: Genetic analyses of the siblings revealed homozygosity for mutation c.736C>T on the