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Introduction Recurrent hemarthroses disturbing force/movement control are likely to impair postural balance. Aim To investigate the possible changes in static postural balance parameters in children with haemophilia (CwH) and to reveal its relationship with Haemophilia Joint Health Score (HJHS). Methods Twenty-one CwH aged 6-18 who had haemophilic arthropathy in at least one lower limb joint and 21 healthy peers were evaluated by using a force platform. Centre of pressure (CoP) signals were displayed as a map in both anteroposterior direction (APD) and mediolateral direction (MLD) by statokinesigram. Accordingly, the amplitude, velocity and standard deviation of CoP displacements along with the perimeter and ellipse area were measured. Assessments were made under eyes opened and eyes closed in bipedal stance for 60 s. Joint health and muscle strength were evaluated with HJHS and digital dynamometer, respectively. Results Velocity and amplitude of CoP displacements in MLD were increased in CwH (p˂0.05). It was also found that these parameters were moderate correlated with the clinical score of the lower limb joints (p˂0.05). In CwH, standard deviation of CoP displacements in the APD was significantly higher in eyes closed, while standard deviation in MLD was significantly higher in eyes opened (p˂0.05). Conclusion Disturbances during bipedal stance reveal the need for balance evaluation in CwH. Increased mediolateral oscillations may be an early sign of disorders of the musculoskeletal system in CwH. In addition to improving joint health, postural balance exercises that increase MLD stability have to be included in the rehabilitation programme of CwH.

The etiology of bacterial meningitis in Turkey changed after the implementation of conjugated vaccines against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib) in the Turkish National Immunization Program (NIP). Administration of Hib vaccine and PCV-7 (7-valent pneumococcal conjugate vaccine) was implemented in NIP in 2006 and 2009, respectively. In 2011, PCV-7 was replaced with PCV-13. Meningococcal vaccines have not yet been included in Turkish NIP. This prospective study comprised 27 hospitals located in seven regions of Turkey and represented 45% of the population. Children aged between 1 month and 18 years who were hospitalized with suspected meningitis were included. Cerebrospinal fluid (CSF) samples were collected, and bacterial identification was made according to the multiplex PCR assay results. During the study period, 994 children were hospitalized for suspected meningitis, and Hib (n = 3, 2.4%), S. pneumoniae (n = 33, 26.4%), and Neisseria meningitidis (n = 89, 71%) were detected in 125 samples. The most common meningococcal serogroup was MenB. Serogroup W comprised 13.9% (n = 5) and 7.5% (n = 4) of the meningococci in 2015 to 2016 and 2017 to 2018, respectively. Serogroup C was not detected. There were four deaths in the study; one was a pneumococcus case, and the others were serogroup B meningococcus cases. The epidemiology of meningococcal diseases has varied over time in Turkey. Differing from the previous surveillance periods, MenB was the most common serogroup in the 2015-to-2018 period. Meningococcal epidemiology is so dynamic that, for vaccination policies, close monitoring is crucial. IMPORTANCE Acute bacterial meningitis (ABM) is one of the most common life-threatening infections in children. The incidence and prevalence of ABM vary both geographically and temporally; therefore, surveillance systems are necessary to determine the accurate burden of ABM. The Turkish Meningitis Surveillance Group has been performing a hospital-based meningitis surveillance study since 2005 across several regions in Turkey. Meningococcus was the major ABM-causing agent during the 2015-to-2018 period, during which MenB was the dominant serogroup.

The etiology of bacterial meningitis in Turkey changed after the implementation of conjugated vaccines against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib) in the Turkish National Immunization Program (NIP). Administration of Hib vaccine and PCV-7 (7-valent pneumococcal conjugate vaccine) was implemented in NIP in 2006 and 2009, respectively. in 2011, PCV-7 was replaced with PCV-13. Meningococcal vaccines have not yet been included in Turkish NIP. This prospective study comprised 27 hospitals located in seven regions of Turkey and represented 45% of the population. Children aged between 1 month and 18 years who were hospitalized with suspected meningitis were included. Cerebrospinal fluid (CSF) samples were collected, and bacterial identification was made according to the multiplex PCR assay results. During the study period, 994 children were hospitalized for suspected meningitis, and Hib (n = 3, 2.4%), S. pneumoniae (n = 33, 26.4%), and Neisseria meningitidis (n = 89, 71%) were detected in 125 samples. The most common meningococcal serogroup was MenB. Serogroup W comprised 13.9% (n = 5) and 7.5% (n = 4) of the meningococci in 2015 to 2016 and 2017 to 2018, respectively. Serogroup C was not detected. There were four deaths in the study; one was a pneumococcus case, and the others were serogroup B meningococcus cases. The epidemiology of meningococcal diseases has varied over time in Turkey. Differing from the previous surveillance periods, MenB was the most common serogroup in the 2015-to-2018 period. Meningococcal epidemiology is so dynamic that, for vaccination policies, close monitoring is crucial. IMPORTANCE Acute bacterial meningitis (ABM) is one of the most common life-threatening infections in children. The incidence and prevalence of ABM vary both geographically and temporally; therefore, surveillance systems are necessary to determine the accurate burden of ABM. The Turkish Meningitis Surveillance Group has been performing a hospital-based meningitis surveillance study since 2005 across several regions in Turkey. Meningococcus was the major ABM-causing agent during the 2015-to-2018 period, during which MenB was the dominant serogroup., GlaxoSmithKlineGlaxoSmithKline, Funding for this study was provided by GlaxoSmithKline.

Background and Aim: Neutrophil gelatinase-associated lipocalin (NGAL) is found in the granules of human neutrophils, with many diverse functions. Expression of NGAL is induced under various pathophysiological conditions, for example, infection, inflammation, kidney injury, cardiovascular disease, burn injury, and intoxication, and it has an important antiapoptotic and anti-inflammatory role. Patients and Methods: A total of 37 patients with thalassemia disease beta-thalassemia major (24 male and 13 female individuals) and 37 healthy controls (17 male and 20 female) were enrolled in this study. All patients were regularly transfused, and all of them were using oral chelator, deferasirox. Results: NGAL and cystatin C levels were significantly higher in the case group than in the control group. Conclusion: In patients with beta-thalassemia major, renal damage can occur owing to regular blood transfusion. Urinary NGAL levels in these patients may be considered as a marker for early renal injury.

EgeUn###, Typhoid fever is an acute and systemic infectious disease that is specific to humans and is caused by Salmonella typhi. Patients may present with different findings ranging from simple fever to multiple organ failure. Hemophagocytosis and rhabdomyolysis are rare complications of typhoid fever. In this study, we presented a case of hemophagocytic lymphohistiocytosis and rhabdomyolysis in a 14-year-old girl who migrated from Mosul, Iraq, 1 month ago.

The aim of the present study was to present the hematologic findings of 622 cases diagnosed with acute brucellosis. Medical records of children with brucellosis, admitted to Yuzuncu Yil University Hospital over a 6-year period, were analyzed retrospectively. Three hundred ninety-seven (63.8%) patients were male and 225 (36.2%) were female. The mean age was 11 years (range, 1 to 16 y). The mean hematologic values were as follows: hemoglobin 9.4 (3.4 to 17) g/dL, hematocrite 28% (12% to 50%), leukopenia 2.8 (1.1 to 33)x10(9)/L, leukocytosis 13.7 (1.1 to 33)x10(9)/L, and platelets 79 (3 to 972)x10(9)/L. An overall 28.6% of patients were found to have anemia, alone or in combination with leukopenia or thrombocytopenia. Sixteen percent of patients had thrombocytopenia, 13.9% had leukopenia, 7.7% had pancytopenia, and 4% had both leukopenia and thrombocytopenia. Hematologic complications such as anemia, thrombocytopenia, and leukopenia are more frequently seen in acute brucellosis cases. However, acute brucellosis should also be considered in the differential diagnosis in the presence of other hematologic abnormalities such as severe thrombocytopenia, bicytopenia, and pancytopenia.

WOS: 000433166100006, PubMed ID: 29545231, Hereditary factor X (FX) deficiency is a rare bleeding disorder more prevalent in countries with high rates of consanguineous marriage. In a prospective, open-label, multicenter phase 3 study, 25 IU/kg plasma-derived factor X (pdFX) was administered as on-demand treatment or short-term prophylaxis for 6 months to 2 years. In Turkish subjects (n=6), 60.7% of bleeds were minor. A mean of 1.03 infusions were used to treat each bleed, and mean total dose per bleed was 25.38 IU/kg. Turkish subjects rated pdFX efficacy as excellent or good for all 84 assessable bleeds; investigators judged overall pdFX efficacy to be excellent or good for all subjects. Turkish subjects had 51 adverse events; 96% with known severity were mild/moderate, and 1 (infusion-site pain) was possibly pdFX-related. These results demonstrate that 25 IU/kg pdFX is safe and effective in this Turkish cohort, Bio Products Laboratory (Elstree, UK), Bio Products Laboratory (Elstree, UK) provided support for this study and funding for medical writing and editorial support in the development of this manuscript. A.F.O.: Received educational support from Pfizer. M.N.: Employee of Bio Products Laboratory. K.K.: Advisory board member for Bayer, Novo Nordisk, Pfizer, and Shire; received educational and investigational support from Bayer, Bio Products Laboratory, CSL Behring, Novo Nordisk, Octapharma, Pfizer, and Shire.

Varicella remains a dangerous viral pathogen for pediatric cancer patients. The incidence of primary varicella infection in Eastern of Turkiye is still high because of low vaccination rate. Medical records of 27 pediatric patients with hematological malignancies that developed varicella/herpes zoster were retrospectively reviewed. All were treated at a single medical center in Eastern Turkiye between 1997 and 2006. Varicella (n = 22) or herpes zoster (n = 5) were diagnosed in 27 cases, of whom 14 had acute lymphoblastic leukemia (ALL), five had acute myeloblastic leukemia (AML), and eight had non-Hodgkin lymphoma (NHL). The mean age of the children was 5.7 ± 3.2 years. None of these children had evidence of disseminated disease. The mean total leukocyte count was 2600 ± 1500/mm3 in ALL group, 2300 ± 1800/mm3 in AML group, and 3100 ± 1700/mm3 in NHL group. While intravenous acyclovir was administered to 21 patients, remaining six patients were received oral acyclovir. Fever was reduced in mean 5.6 ± 2.1, 5.2 ± 1.7 and 4.4 ± 1.8 days in ALL, AML, and NHL groups, respectively. None of the 27 children worsened or developed features of visceral dissemination. Outcome of the patients were not different in terms of oral and intravenous acyclovir use. These results suggest that in our institution varicella is not associated with a high incidence of mortality in cancer patients. Early administration of acyclovir can prevent the visceral dissemination of varicella-zoster virus. Prednisolone usage during the incubation period of varicella did not result in increased severe varicella infection.

Introduction: The goal of this study was to evaluate whether any characteristics that are evident at presentation for urgent medical attention could be used to differentiate cases of H5N1 in the absence of viral testing. Methodology: Information about exposure to poultry, clinical signs and symptoms, treatments, and outcomes was abstracted from existing data in the global avian influenza registry (www.avianfluregistry.org) using standardized data collection tools for documented and possible cases of H5N1 infection who presented for medical attention between 2005-2011 during known H5N1 outbreaks in Azerbaijan, Indonesia, Pakistan and Turkey. Results: Demography, exposure to poultry, and presenting symptoms were compared, with only the common symptoms of fever and headache presenting significantly more frequently in confirmed H5N1 cases than in possible cases. Reported exposure to infected humans was also more common in confirmed cases. In contrast, unexplained respiratory illness, sore throat, excess sputum production, and rhinorrhea were more frequent in possible cases. Overall, oseltamivir treatment showed a survival benefit, with the greatest benefit shown in H5N1 cases who were treated within two days of symptom onset (51% reduction in case fatality). Conclusion: Since prompt treatment with antivirals conferred a strong survival benefit for H5N1 cases, presumptive antiviral treatment should be considered for all possible cases presenting during an outbreak of H5N1 as a potentially life-saving measure.

Brucellosis can affect any organ system and result in possible complications. We present an adolescent male who had brucellar aortitis and meningitis simultaneously. Brucellar aortic involvement is a rare complication of brucellosis and can occur without concomitant endocarditis. Here, the patient was managed with prolonged antibiotic therapy without any surgery.

Background. Oseltamivir is widely used as treatment for influenza virus A subtype H5N1 (hereafter, “H5N1”) infection but, like any intervention, is not always effective. Methods. We used Avian Influenza Registry data from 10 countries to examine the risk of death in 215 patients with confirmed H5N1 infection who were treated with oseltamivir, according to viral clade, age, respiratory failure, and adjunctive treatment with corticosteroids or antibiotics. Results. The median age of infected individuals was 18 years, and 50% were male. The highest fatality rate occurred in a country with clade 2.1 virus circulation, and the lowest occurred in countries with clade 2.2 virus circulation (P< .001). In univariate analyses, age of ≤5 years and treatment ≤2 days after symptom onset were protective against fatality. When accounting for all risk factors, early initiation of oseltamivir was found to be particularly effective in individuals without respiratory failure (odds ratio, 0.17; P= .04). Patients who had advanced respiratory failure requiring ventilatory support at the time of oseltamivir initiation were more likely to die from the episode of H5N1 infection than patients who did not (P< .001). Adjunctive therapy did not improve the likelihood of surviving the episode. Conclusions. Oseltamivir is especially effective for treating H5N1 infection when given early and before onset of respiratory failure. The effect of viral clade on fatality and treatment response deserves further investigation.

Epidemiological studies indicate that in chronic HD patients, bacterial and viral infection rank second place in mortality and morbidity, behind cardiovascular disease. Chronic hepatitis C virus (HCV) is very prevalent in some Hemodialysis (HD) centers. We have investigated lymphocyte subtype count and immunoglobulin levels in hemodialyzed patient with HCV. We studied 55 patients with end stage renal disease (ESRD) on chronic HD and 21 healty subjects. Patients group included 34 female and 21 male with mean age 46.5±16.1(18-77) years. Serum concentrations of IgG, IgM, IgA, CD4, CD8, CD19, CD16-56 lymphocytes were measured. Kt/V values were calculated according to DOQI guideline. Lymphocyte and lymphocyte subgroups values of HD patients were significant lower than healty persons. IgG, IgM and ALT levels of HCV positive HD patients compared with significant higher than HCV negative HD patients. CD4/CD8 ratios within groups were no differences. Lymphocyte subtype count of between HCV positive and HCV negative HD patients was no differences. IgG and IgM levels of HCV positive HD patients were higher than HCV negative HD patients. High Ig levels were also associated with ALT levels

Background. Avian influenza continues to pose a threat to humans and maintains the potential for greater transmissibility. Understanding the clinical presentation and prognosis in children will help guide effective diagnosis and treatment. Methods. A global patient registry was created to enable systematic collection of clinical, exposure, treatment, and outcomes data on confirmed cases of H5N1. Bivariate and multivariate statistical tools were used to describe clinical presentation and evaluate factors prognostic of survival. Results. Data were available from 13 countries on 193 children

Objective: To identify the well-known common translocations and FLT3 mutations in childhood acute myelogenousleukemia (AML) patients in Turkey. Material and Methods: The study included 50 newly diagnosed patients in which t(15;17), t(8;21), and inv(16)chromosomal translocations were identified using real-time PCR and FLT3 gene mutations were identified via direct PCR amplification PCR-RE analysis. Results: In all, t(15;17) chromosomal aberrations were observed in 4 patients (8.0%), t(8;21) chromosomal aberrationswere observed in 12 patients (24.0%), inv(16) chromosomal aberrations were observed in 3 patients (6.0%), and FLT3-ITD mutations were observed in 2 patients (4.0%); FLT3-D835 point mutation heterozygosity was observed in only 1patient (2.0%) patient. Conclusion: Despite of the known literature, a patient with FLT3-ITD and FLT3-D835 double mutation shows a bettersurvival and this might be due to the complementation effect of the t(15;17) translocation. The reportedmutation ratein this article (4%) of FLT3 gene seems to be one of the first results for Turkish population.

Long used in established industrialized nations to treat patients with haemophilia and inhibitors, factor eight inhibitor bypassing activity (FEIBA) has, in recent years, been introduced into more geographically diverse settings. Data are needed on how successfully FEIBA therapy has been implemented in new regions. To determine the efficacy and safety of FEIBA for the treatment of acute bleeding and surgical haemostasis in a newly industrialized country. A multicentre registry of haemophilia A patients with inhibitors receiving FEIBA treatment was established in Turkey. With a standardized case report form, data were collected retrospectively on: patient demographics; characteristics of acute bleeding episodes and surgical interventions; FEIBA regimen; and treatment outcomes. Thirty-seven patients received a total of 112 FEIBA treatment courses, 90 for acute bleeding and 22 for surgical haemostasis. The median FEIBA dose per infusion for acute bleeding was 50 IU kg(-1), and for surgery was 100 IU kg(-1). For both acute joint and muscle/soft tissue bleeding and in surgery, haemostasis was attained in a median of two FEIBA infusions. FEIBA was judged effective in 92% of treatment courses for acute bleeding, with a 95% confidence interval (CI) of 85-97%. Rates of haemostatic efficacy did not differ significantly between anatomical sites of acute bleeding. The haemostatic efficacy rate of FEIBA in surgery was 86% (CI, 65-97%). No thromboembolic complications or other adverse events occurred during any treatment course. FEIBA has been successfully integrated into clinical practice in Turkey, with rates of haemostatic efficacy comparable to those reported in countries with a longer history of FEIBA usage.

Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitted to Yuzuncu Yil University Hospital between 2004 and 2010, were analyzed retrospectively. Over this time period, 187 patients with brucellosis were diagnosed. Twenty-five (13.3%%) of 187 patients had pancytopenia during admission to hospital. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titers were 1:320 in 1 patient and 1:1280 in 24 patients. Blood culture was positive for Brucella melitensis in 3 patients (12%%). Fever was the most common manifestation, followed by malaise, anorexia, sweating, and weight loss. Fever and splenomegaly were the common signs in most patients. In addition, arthritis was observed in 5 patients, and epistaxis, headache, and abdominal pain were observed in 3 patients. The common bone marrow aspiration findings consisted of increased megakaryocytes and hyperplasia of erythroid series, with a shift to the left of the granulocytic series. Histiocytic hyperplasia was observed in the bone marrow smear of 2 patients. Mild hemophagocytosis was observed in the bone marrow of 3 patients. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 6 weeks after antibiotic treatment of brucellosis. In conclusion, the authors would like to emphasize that brucellosis should be considered in the differential diagnosis of children with pancytopenia.

Neutropenic enterocolitis is a potentially life-threatening complication of malignancies and also the other neutropenic conditions. It characterized by typically clinical course of fever, diarrhoea and abdominal pain that may be localized to the right lower quadrant with radiological evidence of ileocecal inflamation. We present a case of an 9-year-old girl with neutropenic enterocolitis who managed successfully by medical treatment and show radiological findings consistent with enterocolitis.

Anthrax is a zoonosis produced by Bacillus anthracis. The aim of this study was to evaluate the clinical findings, therapy, and outcome in children with cutaneous anthrax (CA). Data on age, gender, occupation, clinical symptoms and findings, location and type of lesions, clinical history, laboratory findings, treatment, and outcome were recorded from patients’ medical records, retrospectively. The study included 65 patients between 1 month and 18 years old (9.0 ± 4.0 years), 37 patients (56.9%) were male and 28 (43.1%) were female. Most of the patients (89.1%) were admitted in summer and autumn (p

Celiac disease (CD) is a disease of the small intestine caused by an immune response to ingested gluten. Idiopathic thrombocytopenic purpura (ITP) is a common acquired bleeding disorder of childhood. It may follow a viral infection or immunization and is caused by an inappropriate response of the immune system. Autoimmune thyroiditis (AT) is a disease that occurs due to autoimmune mechanisms. Celiac disease associated with autoimmune thyroid disease is well known, but the association of CD, autoimmune thyroid disease, and ITP has been reported very rarely in the literature. In the current report, we have presented a case with CD, AT, and acute ITP, because this association is rarely seen, and to emphasize that CD and AT should be kept in mind in patients with ITP.

L-asparaginase is effective in the treatment of malignant diseases, but it has been associated with hypersensitivity reactions in 5% to 45% of the patients.To determine whether a desensitization protocol in children with native Escherichia coli L-asparaginase hypersensitivity allows subsequent safe administration of native E. coli L-asparaginase.A desensitization protocol was used in 9 children with leukemia (n=8) or Langerhans cell histiocytosis (n=1) and previous severe (n=4) or mild/moderate (n=5) L-asparaginase hypersensitivity. Dexamethasone (2 mg/kg intravenously) and pheniramine hydrogen maleate (1 mg/kg intravenously) were administered 1 hour before each of the following L-asparaginase administrations. Subsequently, 0.1%, 1%, 5%, 10%, and the remaining 84% of the total dose of L-asparaginase (10,000 IU/m2) were each prepared in 240 mL 0.9% saline and infused over 4 hours (20 h in total).After desensitization, none of the patients had recurrence of hypersensitivity reactions during the subsequent 2 to 15 doses (median: 6 doses/patient; 68 doses in total) of native E. coli L-asparaginase.This desensitization protocol was safe and allows continued administration of native E. coli L-asparaginase administration.

SUMMARYIn 2006 an outbreak of avian influenza A(H5N1) in Turkey caused 12 human infections, including four deaths. We conducted a serological survey to determine the extent of subclinical infection caused by the outbreak. Single serum samples were collected from five individuals with avian influenza whose nasopharyngeal swabs tested positive for H5 RNA by polymerase chain reaction, 28 family contacts of the cases, 95 poultry cullers, 75 individuals known to have had contact with diseased chickens and 81 individuals living in the region with no known contact with infected chickens and/or patients. Paired serum samples were collected from 97 healthcare workers. All sera were tested for the presence of neutralizing antibodies by enzyme-linked immunoassay, haemagglutination inhibition and microneutralization assays. Only one serum sample, from a parent of an avian influenza patient, tested positive for H5N1 by microneutralization assay. This survey shows that there was minimal subclinical H5N1 infection among contacts of human cases and infected poultry in Turkey in 2006. Further, the low rate of subclinical infection following contact with diseased poultry gave further support to the reported low infectivity of the virus.

A few cases with bilateral renal enlargement in acute lymphoblastic leukemia were reported in literature. In this article, we reported an unusual case of a child with precursor B-ALL presenting with massively enlarged bilateral unobstructed kidneys and acute renal failure. Renal involvement of ALL should be taken into consideration in case with massively enlarged bilateral kidneys in radiological examination. Based on radiological, clinical and laboratory findings including bone marrow aspirate examination, the patient could be diagnosed as ALL without renal biopsy.

Acute ITP is a benign, self-limiting disease. Chronic ITP is diagnosed when thrombocytopenia persists beyond 12 months. The main objective of the present study was to examine whether absolute lymphocyte counts at diagnosis has predictive value with chronic ITP. A total of 601 patients diagnosed as ITP between 1995 and 2014 were retrospectively evaluated. CBCs with differential counts were performed at presentation for 601 patients. Absolute lymphocyte counts at presentation were independently predictive of disease duration. The male to female ratio was almost 1:1 and 25.9 % (156/601) of the patients had chronic ITP. We determined that age >6.75 year, platelet counts >6.950/mm(3) and absolute lymphocyte counts

Congenital factor X (FX) deficiency is a rare bleeding disorder inherited as an autosomal recessive trait with an incidence of 1 : 500 000-1 000 000. A total or partial deficiency of FX causes an impairment of clot formation, leading to a haemorrhagic disease, which manifests with bleeding symptoms of different severity, also unprovoked.We analysed the clinical manifestations, laboratory phenotype and genotype in 12 patients from Turkey affected with severe FX deficiency.Prothrombin time (PT), activated partial thromboplastin time (APTT), FX activity (FX:C) and FX antigen level (FX:Ag) were measured, and mutation analysis was performed for all patients.The most frequent bleeding episodes in patients were epistaxis and easy bruising (11/12, 91%), followed by haemarthroses (10/12, 83%). FX:C was1% in 11 patients, and 4% in one. FX:Ag was reduced in all patients, consistent with type II deficiency. Direct sequencing of the factor X gene (F10) identified two different mutations: the novel 33 bp in-frame deletion p.Thr176_Gln186, c.526_558del, which seems to be associated with milder bleeding symptoms and the c.785GA, p.Gly262Asp missense mutation (previously reported as Gly222Asp), which is associated with severe bleeding symptoms.The p.Gly262Asp missense mutation was identified in 11 of the 12 patients in this study. Previously published cases on the same p.Gly262Asp mutation were Iranian patients originating from the border between Turkey and Iran suggesting that this mutation may be candidate as a good tool for mutational screening analysis in this area.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from abnormal proliferation of histiocytes in tissues and organs. The incidence of HLH is 1:50,000-300,000. Cutaneous eruptions have been reported in 6-65% of the cases. It's important to differentiate the eruptions from other systemic diseases. We present an infant with prominent skin manifestations of HLH. On the 11th day of life, she was admitted to our hospital with complaint of a generalized rash that had started the previous day. The eruptions consisted of irregularly shaped maculopapular erythematous rash and purpura. Bone marrow aspiration on the 25th day of life revealed hemophagocytosis with increased macrophages and histiocytes, consistent with HLH. Treatment was started with dexamethasone followed by induction chemotherapy with etoposide. All skin manifestations resolved in a few days. Although the clinical features are nonspecific, HLH should be kept in mind as an accompanying disease in neonates presenting with skin eruptions.

The purposes of this study were to determine the frequency of vitamin K deficiency and to assess its effects on bleeding in patients with acute and intractable diarrhea. A total of 90 children with diarrhea and 30 healthy children (group Q were included in this study. Patients were divided into 2 groups, according to the duration of diarrhea. Complete blood count; prothrombin time (PT); activated prothrombin time (APTT); Factors ii, VII, IX, and X; and protein C levels were studied in all patients. A total of 3 mg of vitamin K was administrated to patients with prolonged PT and/or APTT. Coagulation parameters were restudied 8 to 12 h after vitamin K was administered. Mean age, sex, weight, and breastfeeding percentage, as well as history of fever and vitamin K administration at birth, were similar in the 2 groups. The duration of antibiotic administration in group B (intractable diarrhea) was significantly longer than that in group A (acute diarrhea). Gastrointestinal (GI) bleeding was observed in 3 (4.9%) infants in group A and 6 (20.7%) in group B (P

Autoimmune thrombocytopenic purpura (ITP) is a disease that presents with skin and mucous membrane bleeding due to thrombocytopenia. In the literature, there are a few studies about the effect of high-dose steroid therapy on coagulation tests in different diseases, but their results are still controversial. In this study, coagulation parameters were investigated that might have a role in hemostatic compensation in childhood acute ITP before and after high-dose methylprednisolone (HDMP) treatment. The study includes 21 children age 1.5 to 14 years with acute ITP and 21 healthy age-matched control subjects. All patients with acute ITP received HDMP for 7 days. Before and after HDMP treatment (0 and 8 days) prothrombin time, partial thromboplastin time, fibrinogen, Protein C, Protein S, antithrombin III, and the levels of factor II (FII), FV, FVII, FVIII, FIX, FX, FXI, and FXII were studied in all subjects. The results were compared with those of the control group. Pre-treatment Protein C and Protein S levels in the patient group were significantly lower than those in the control groups (p

Stevens-Johnson syndrome (SJS) is defined as a severe erythema-multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa and hemorrhagic crusting on the lips; it is associated with fever, headache, and arthralgia. The disease is a part of a continuum of immunologically mediated mucocutaneous diseases at various grades of severity. SJS is often induced by drugs, but the pathophysiologic mechanism is completely unknown. Patients and their first degree-relatives may have genetic defects in their metabolic pathways that lead to the accumulation of toxic metabolites. However, viral infections are known triggers of this skin disorder. Varicella zoster virus has been only very rarely reported as an etiological agent in SJS, despite its high incidence as a pathogen in childhood. In this study, we report a case of primary varicella infection associated with SJS because of its rare presentation. To the best of our knowledge, our case is the youngest child in the literature.

Eight-year-old girl was admitted to our clinic with the complaint of constant epiphora in the right eye. It was reported that this complaint began after the start of chemotherapy with a diagnosis of Acute Lymphoblastic Leukemia (ALL) about 5 years ago. In addition, eyelid ptosis associated with esotropia also occurred during that period. We found that nasolacrimal duct obstruction did not improve with medical treatment but ptosis and esotropia improved with pyridoxine and pyridostigmine treatment during that period. Examination of the eye on admission revealed nasolacrimal duct obstruction on the right side. Other ocular findings were normal. Nasolacrimal system obstruction, ptosis and esotropia combination has not been reported previously in patients using systemic drugs or receiving chemotherapy due to ALL.

Herpes zoster (HZ) is often associated with painful erythematous vesicular eruptions of the skin or mucous membranes. Approximately 10% to 30% of the population will suffer from HZ during their lifetime. HZ is infrequent in healthy children. However, diminished cellular immunity seems to increase risk of reactivation because incidence increases with age and in immunocompromised states. We report a 7-year-old girl with acute myeloblastic leukemia HZ infection on the right palmar, elbow and forearm region (C7, C8 and T1 dermatomes). We want to indicate unusual localization of HZ on the acute myeloblastic leukemia child patient.

The cranial computed tomography (CT),findings of 48 children with purulent meningitis were examined, prospectively, to determine the importance of cranial CT findings on the prognosis of childhood meningitis, in a developing country. The age of children ranged from 2 months to 13 years. Of 48 patients, 29 (60.5%) survived without sequelae, 13 (27%) survived with sequelae, and six (12.5%) died. Cranial CT was normal in 21 (43%) patients of 48 children with meningitis at admission. Abnormal CT findings were detected in 10, H, and 6 children in the groups of survived without sequelae, survived with sequelae, and deaths, respectively, at admission (p < .05) We found that CT scan results were correlated with neurological signs (p < .05). At least one or more cranial CTs were was re-taken in children in whom the first CT revealed abnormal findings; we did not find a statistically significant difference for the follow-up CT findings between the groups (p > .05). Hydrocephalus and subdural effusion were the commonest abnormal CT findings. In conclusion, our findings showed that cranial CT may safely be used to detect intracranial complications of meningitis in childhood and the ratio of sequelae and death were more common in children with abnormal cranial CT than those of normal cranial CT findings. Additionally, there was a positive correlation between CT scan results and neurological signs.

Besides large intra-joint bleedings that are frequently observed in Hemophilia A, bleeding may also rarely occur in spinal joints. Additional to traumatic or spontaneous hematomas, cord suppression may be occured due to various reasons such as epidural tumor and infections, myelosclerosis and bone tissue suppression that occurs to the enlargement in the bone marrow because of hemolytic anemia and thalassemia. In the present study a 6 months old male who developed paraparesis as a result of spinal compression of a hematoma that occurred after lumbar puncture and then diagnosed with Hemophilia A presented on account of the present case, our aim is to emphasize that perispinal hematoma may lead to paraparesis and that paraparesis may develop due to neurological retention that is rarely seen in children with Hemophilia A.

Initial presentation of Non-Hodgkin Lymphoma (NHL) as primary pleural lymphoma is extremely rare. We report a case with NHL, who was initially presented with pleural effusion and pleural thickening. Our patients at first received intensive treatment of broad spectrum antibiotics for 10 days with no change in the clinical status, and then were diagnosed with T-lymphoblastic lymphoma with the examination of pleural fluid by using cytologic and flow cytometric methods, and treated with LMT89 group B treatment protocol. Our case points out the necessity for considering the NHL within the differential diagnosis of any pediatric patient presenting with sterile pleural effusion and pleural thickening who does not respond to antimicrobial therapy.

Individual case reports and series have subsequently been published describing immune thrombocytopenia after vaccination [1,2]. Herein we report a case with acute immune thrombocytopenic purpura that were developed after DTaP-IPV-Hib combined vaccine (diphtheria/ tetanus/acellular pertussis, inactivated poliomyelitis, Haemophilus influenzae type b) and Pneumococcal Conjugate Vaccines (PCV).

WOS: 000348316800043, PubMed: 25483487, Successful vaccination policies for protection from bacterial meningitis are dependent on determination of the etiology of bacterial meningitis. Cerebrospinal fluid (CSF) samples were obtained prospectively from children from 1 month to, Novartis Vaccines and Diagnostics; GlaxoSmithKlineGlaxoSmithKline, The study was supported by Novartis Vaccines and Diagnostics (for 5 years) and by GlaxoSmithKline (for 2 years). The authors declare that they have no other conflicts of interest.

Ethylenediaminetetraacetic acid (EDTA)-dependent pseudothrombocytopenia (PTCP) is the phenomenon of a spurious low platelet count due to antiplatelet antibodies that cause platelet clumping in blood anticoagulated with EDTA. The aggregation of platelets in EDTA-dependent PTCP is usually prevented by other anticoagulants, such as sodium citrate or heparin. EDTA-dependent PTCP has never been associated with hemorrhagic diathesis or platelet dysfunction. In this article, a 10-year-old boy with EDTA- and heparin-dependent PTCP is presented because of rare presentation. We report that EDTA and heparin can induce platelet clumping, and thus spuriously low platelet counts. However, aggregation of platelets was not detected in blood samples with sodium citrate, and platelet count was normal.

The treatment of hemophilia A patients with inhibitor could be very expensive. Ankaferd blood stopper (ABS) is a unique folkloric medicinal plant extract, which has historically been used in Turkish traditional medicine as a hemostatic agent. In this article, a 16-year-old boy was presented with uncontrolled bleeding, despite the treatment of factor VIII, rVIIa, factor VIII inhibitor bypass activity (FEIBA), cyclophosphamide, and prednisolone at circumcision site that resolved with ABS in minutes. Our patient with hemophilia A and inhibitor is the first clinical pediatric case.

We present two patients with brucellosis concomitant with acute leukemia. Co-existence of acute leukemia with brucellosis which may have similar symptoms, have not been reported earlier. The first case presented with generalized arthralgia, fever, paleness, and pancytopenia. The second patient had mild leucopenia and thrombocytopenia. She presented with fever. We carried out the chemotherapy for both ALL and brucellosis simultaneously. While the first patient's fever disappeared within 3 days, the second patient's fever had continued on subfebril level for five days and then disappeared. We achieved the remission in both patients and no reactivation was observed during the follow-up period.

Pica is defined as developmentally inappropriate consumption of nonnutritive substances for at least 1 month. There are a few studies on serum trace element levels of patients with pica. The literature contains contracting data on the levels of oxidative stress and antioxidant levels in patients with iron-deficiency anemia (IDA). The effect of pica on oxidative stress and antioxidant capacity has not been investigated yet. The present study evaluated the effects of pica and IDA on oxidative stress and antioxidant capacity as well as on the levels of trace elements including serum zinc and selenium in 47 children with IDA plus pica, 22 children with IDA only and 21 nonanemic children as controls. The results demonstrated significantly lower levels of serum selenium and zinc in pica and IDA groups compared to the control group. Total oxidant levels were highest in the pica group and consistently, the lowest total antioxidant capacity was observed again in the pica group. Comparison of pica and IDA groups yielded significantly lower levels of total antioxidant levels and significantly higher oxidative stress index in the pica group. Consequently, it is thought that the detrimental effects of pica within the organism were mediated by adverse impacts on antioxidant capacity and oxidative stress. These effects should be kept in mind while managing patients with pica.

WOS: 000320857300004, PubMed ID: 23637041, Streptococcus pneumoniae is the most common etiological cause of complicated pneumonia, including empyema. In this study, we investigated the serotypes of S. pneumoniae that cause empyema in children. One hundred fifty-six children who were diagnosed with pneumonia complicated with empyema in 13 hospitals in seven geographic regions of Turkey between 2010 and 2012 were included in this study. Pleural fluid samples were collected by thoracentesis and tested for 14 serotypes/serogroups using a Bio-Plex multiplex antigen detection assay. The serotypes of S. pneumoniae were specified in 33 of 156 samples. The mean age +/- the standard deviation of the 33 patients was 6.17 +/- 3.54 years (range, 0.6 to 15 years). All of the children were unvaccinated according to the vaccination reports. Eighteen of the children were male, and 15 were female. The serotypes of the non-7-valent pneumococcal conjugated vaccine (non-PCV-7), serotype 1, serotype 5, and serotype 3, were detected in eight (14.5%), seven (12.7%), and five (9.1%) of the samples, respectively. Serotypes 1 and 5 were codetected in two samples. The remaining non-PCV- 7 serotypes were 8 (n = 3), 18 (n = 1), 19A (n = 1), and 7F/A (n = 1). PCV-7 serotypes 6B, 9V, 14, 19F, and 23F were detected in nine (16.3%) of the samples. The potential serotype coverages of PCV-7, PCV-10, and PCV-13 were 16.3%, 45.4%, and 60%, respectively. Pediatric parapneumonic empyema continues to be an important health problem despite the introduction of conjugated pneumococcal vaccines. Active surveillance studies are needed to monitor the change in S. pneumoniae serotypes that cause empyema in order to have a better selection of pneumococcal vaccines., GlaxoSmithKlineGlaxoSmithKline, The study was supported by GlaxoSmithKline.

Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present with congestive heart failure and asymptomatic abdominal mass were before 6 months of age. We described a patient with projectile vomiting, which is quite an unusual presentation. Following surgical resection, neither recurrence nor symptoms were seen in 2 years of follow-up.

Brucellosis is a multisystem disease with a broad spectrum of clinical manifestations; hematological abnormalities ranging from fulminant as of disseminated intravascular coagulation (DIC) to anaemia, leucopoenia, thrombocytopenia, and clotting disorders. In this report, we present DIC in a case of brucellosis because of rare presentation. A 3-year-old boy was admitted with the complaints of continuous fever, vomiting, abdominal pain, and bruise on leg. He looked pale and his physical examination revealed purpuric skin lesions on both legs. A mild hepatosplenomegaly was noted on palpation. Laboratory tests showed hematocrit 21%, hemoglobin 7 g/dL, platelet count 20,000/mm3, prothrombin time 19 seconds, activated partial thromboplastin time 48 seconds, plasma fibrinogen level 20 mg/dL, andd-dimer 8 µg/mL. Serum antibrucella titration agglutination test was found to be 1 of 1280 positive. Blood cultures performed on specimens obtained at the time of admission yielded Brucella melitensis. The clinical and laboratory findings were consistent with DIC.