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The diagnosis of mild bleeding disorders is not easy as most of the "healthy" individuals also report bleeding symptoms. In order to get a precise bleeding history, Pediatric Bleeding Questionnaire (PBQ) has been developed. In our study, Turkish children diagnosed with Von Willebrand disease (VWD), platelet function defect (PFD), and healthy children without any symptoms (control group 1) and healthy children with symptoms but found hemostatically normal (control group 2) were analyzed with PBQ. The cut off level for "positive bleeding score" was found to be ≥2 (area under the curve [AUC]: 0.785, 95% confidence interval [CI]: 0.718-0.852). The sensitivity, specificity, positive predictive value, and negative predictive value of PBQ to define VWD versus control group 1 was 100%, 97.4%, 96.4%, and 100%; VWD versus control group 2 was 100%, 53.1%, 64.3%, and 100%; PFD versus control group 1 was 93.3%, 53.1%, 73.7%, and 85%; and PFD versus control group 2 was 93.3%, 53.1%, 73.7%, and 85%, respectively., (© The Author(s) 2014.)

Objectives: Hemophagocytic lymphohistiocytosis is a syndrome of pathologic immune activation that shares similar clinical and laboratory phenotypes with severe sepsis. Recent studies led to better recognition of hemophagocytic lymphohistiocytosis by clinicians, but no consensus exists on the criteria for high-risk patients., Design: We retrospectively reviewed the medical records of patients diagnosed with hemophagocytic lymphohistiocytosis to analyze the risk factors associated with poor outcome., Setting: Pediatric intensive care and hematology units of three tertiary hospitals in Turkey., Participants: Fifty-two children with hemophagocytic lymphohistiocytosis., Interventions: None., Measurement and Main Results: There were a total of 52 children meeting the diagnostic criteria of Histiocytic Society. Of them, 28 (54%) had a primary hemophagocytic lymphohistiocytosis. Mutation studies were performed in 18 of 28 patients (65%). Fourteen of them had PRF1, STX11, STXBP2, and UNC13D mutations, and four had Rab27a and LYST mutations. The remaining 24 patients (46%) were defined as having secondary hemophagocytic lymphohistiocytosis. Twenty-one of them had infection-associated hemophagocytic lymphohistiocytosis, and three had lysinuric protein intolerance. The mortality rate was significantly higher in primary hemophagocytic lymphohistiocytosis (64%) than in secondary hemophagocytic lymphohistiocytosis (16%) (p < 0.05). There were no significant differences for survival rate between hemophagocytic lymphohistiocytosis 94 (44%) and hemophagocytic lymphohistiocytosis 2004 (64%) protocols (p > 0.05). Age below 2 years, hyperferritinemia, thrombocytopenia, high disseminated intravascular coagulation score at diagnosis, and no clinical response at 2 weeks of treatment were independent prognostic factors for poor prognosis., Conclusions: Our data suggest that disseminated intravascular coagulation score greater than or equal to 5 can be used in the definition of high-risk patients. Early recognition of poor risk factors has important prognostic and therapeutic implications.

Background: In this study, our aim was to evaluate the fatal occupational accidents that occurred in Zonguldak coal mines between the years 2005-2008., Material and Methods: We retrospectively evaluated 42 fatal coal mine occupational accidents in Zonguldak (Turkey) between the years 2005-2008. The forensic records of autopsies referred to the chief prosecutors' office during 2005-2008 were reviewed retrospectively. There were 42 cases. The cases were evaluated with respect to age, mechanism of injury, body region of wound, cause of death, and legality of the mine., Results: Ages of the deceased ranged between 17 and 52 (median: 32.9 yrs). Deaths were mostly in the 21-30 (35.8%), and 31-40 (30.9%) age groups. Only 1 case was younger than 18 years of age. Review of occupational fatalities has revealed that fatal accidents occurred mostly (76.2%) in the private, and fewer (23.8%) in the public mining enterprises. Crime scene investigation findings have demonstrated that of all occupational deaths (total n=42), 27 (64.3%) were due to subsidence, followed by methane gas poisoning (n=6, 14.2%), tram crash (n=3, 7.1%), log falls (n=2, 4.8%), electrocution (n=2, 4.8%), and methane explosion (n=2, 4.8%)., Conclusions: Despite laws and regulations concerning mining and because of problems in their implementation, Turkey leads the world in work-related accidents, occupational injuries, and deaths. Evaluation of autopsy findings of deaths in fatal occupational accidents occurring in coal mines is quite important in planning to decrease rates of occupational fatalities.

Megaloblastic anaemia due to vitamin B12 deficiency is rare in childhood. However, as most cases are due to maternal insufficiency, it is mainly seen in breastfed infants especially when the mother's socioeconomic status is low and the nutrition is not adequate. We present case of two Syrian refugee infants with severe vitamin B12 deficiency with pancytopenia, hepatosplenomegaly and leukoerythroblastosis.

Little is known about the likelihood of curing children with high-dose chemotherapy regimens for treatment of childhood acute lymphoblastic leukemia (ALL) in Turkey. The authors here report their 13 years' experience with original ALL-BFM (Berlin-Franfurt-Münster) 95 protocol in a cohort of 140 Turkish children with ALL. Complete remission rate was 97.7% with a relapse rate of 12.9% and death rate 17.9% during a median follow-up of 69 months. The event-free survival (EFS), disease-free survival (DFS), and overall survival (OS) in these patients at 12 years were 75.0%, 87.1%, and 80.6%, respectively. These results show that ALL-BFM 95 protocol is equally applicable in the experienced centers, even in developing countries without substantial treatment-related toxicity. High rate of infection deaths are to be reduced with correct policies.