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.

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

Objectives: Lumbar puncture (LP) is fundamental for diagnosis and treatment; however, some parents do not provide consent for their children to undergo the procedure, which can make diagnosis and determination of the optimal treatment difficult. The present study aimed to describe the level of knowledge and attitudes toward LP of parents whose children were scheduled to undergo the procedure. Methods: A prospective cross-sectional descriptive study of a convenience sample of parents of 84 children aged 2 months to 17 years scheduled for LP at a single academic children's hospital between 2015 and 2017. Parents were administered a written survey and interviewed by a physician other than the person who did the LP. Data on parental level of knowledge and attitudes regarding LP, in addition to reasons for refusal, were collected. The parents of 84 patients scheduled for LP due to various indications were administered a face-to-face survey interview. The survey was used to collect parental demographic data, as well as opinions and knowledge about LP and postinterventional complications. Results: The mean age of the 84 patients (57% male and 43% female) was 6.4 ± 5.17 years. Lumbar puncture was planned for the presumptive diagnosis of neurological disease in 45.25% of the patients, central nervous system infection in 45.25%, and acute encephalopathy in 9.5%. Among the parents, 65% (n = 55) had no knowledge or attitude about LP prior to the survey interview. The most common parental concern related to LP was paralysis (25%), followed by infertility (2%), mental retardation (1%), and disease progression (1%). Only 4.7% of the parents did not provide consent for their child to undergo LP. Conclusions: We found that most parents had little knowledge about LP, and the most common parental concern was paralysis. Despite this, in our study, only 5% of parents did not consent to LP.

Amaç:Human Papilloma Virüs (HPV) serviks kanserinin en sık nedenidir. Serviks kanserini ve HPV ilişkili diğer hastalıkları önlemede aşılama birincil olarak etkili yöntemdir. Bu çalışmanın amacı ebeveynlerin HPV aşısı hakkındaki bilgi düzeyleri ve yaklaşımlarını değerlendirmektir. Gereç ve Yöntemler:Tanımlayıcı olan bu çalışma Aralık 2018-Ocak 2019 tarihlerinde 9-18 yaş arası kız çocuğuna sahip 203 ebeveyn üzerinde yapılmıştır. Ebeveynlerin demografik verileri, HPV hakkındaki bilgileri ve HPV aşılamasına ilişkin tutumlarını içeren anketler değerlendirildi. Bulgular:203 ebeveynin (% 80 anne ve% 20 baba) yaş ortalaması 39,1 ± 6,1 idi. Ebeveynlerin% 70'i HPV aşısını duymamıştı ve% 67'si rahim ağzı kanserinin önlenebilir bir hastalık olduğunu bilmiyordu. Ebeveynlerin% 62'si bilgilendirme sonrasında kızlarına HPV aşısını yaptırmayı kabul etti. Aşının fiyatı ve doz sayısı hakkında bilgilendirme sonrasında, ebeveynlerin% 24'ü 'Devlet tarafından ücretsiz karşılansa dahi yaptırmam' şeklinde cevap verdi. En önemli nedenleri aşı hakkında yeterli bilgiye sahip olmamaları idi (% 69).Sonuç:Çoğu ebeveyn HPV aşısını bilmemektedir ve bilgilendirme sonrası çocuklarına aşı yaptırmayı kabul etmektedir. Bununla birlikte bazı ebeveynler, ücretsiz karşılanmış olsa dahi çocuklarına aşı yaptırmayı düşünmemektedir. Ailelerin bu konuda daha fazla aydınlatılması için sağlık çalışanlarına büyük görev düşmektedir.