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Aim: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare autoimmune disorder. Approximately half of the cases are associated with neuroblastoma in children. This study's aim is to review management of our cases with OMAS-associated neuroblastoma for treatment approach as well as long-term follow-up., Methods: Age at onset of symptoms and tumor diagnosis, tumor location, histopathology, stage, chemotherapy, OMAS protocol, surgery, and follow-up period were evaluated retrospectively in six patients between 2007 and 2022., Results: Mean age of onset of OMAS findings was 13.5 months and mean age at tumor diagnosis was 15.1 months. Tumor was located at thorax in three patients and surrenal in others. Four patients underwent primary surgery. Histopathological diagnosis was ganglioneuroblastoma in three, neuroblastoma in two, and undifferentiated neuroblastoma in one. One patient was considered as stage 1 and rest of them as stage 2. Chemotherapy was provided in five cases. The OMAS protocol was applied to five patients. Our protocol is intravenous immunoglobulin (IVIG) 1 g/kg/d for 2 consecutive days once a month and dexamethasone for 5 days (20 mg/m 2 /d for 1-2 days, 10 mg/m 2 /d for 3-4 days, and 5 mg/m 2 /d for the fifth day) once a month, alternatively by 2-week intervals. Patients were followed up for a mean of 8.1 years. Neuropsychiatric sequelae were detected in two patients., Conclusion: In tumor-related cases, alternating use of corticosteroid and IVIG for suppression of autoimmunity as the OMAS protocol, total excision of the tumor as soon as possible, and chemotherapeutics in selected patients seem to be related to resolution of acute problems, long-term sequelae, and severity., Competing Interests: None declared., (Thieme. All rights reserved.)

Myeloproliferative neoplasms are rare in childhood. They are categorized as Philadelphia chromosome-positive and Philadelphia chromosome-negative. Chronic myeloid leukemia (CML) is the most common myeloproliferative disease in which the Philadelphia chromosome is detected as a result of BCR-ABL rearrangements. In others, the most common genetic abnormality is JAK2V617F mutation. The coexistence of these 2 abnormalities in CML is unexpected, and rare cases have recently been reported in adults. We present a child who had a very high platelet count in which we found this coexistence. The clinical presentation, laboratory findings, management, and prognosis of this coexistence is challenging in such a rare condition., (© The Author(s) 2023. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: After three years since the first cases of COVID-19, many people suffer from post-COVID symptoms, reducing their quality of life. In this study, we aimed to evaluate the symptoms and prevalence of long COVID and its effect on the quality of life and also the effect of SARS-CoV-2 vaccine on the quality of life., Materials and Methods: Patients with COVID-19 between April 01, 2020, and December 31, 2021, were evaluated with a survey at least three and at most 12 months (mean 7.43±3.3 months) after diagnosis. Patients answered the questions face-to-face or via telephone interview. The survey included questionnaires on demographic features, current complaints, and complaints that persisted or developed after recovery, Short Form 36 Health Survey (SF-36), and European Quality of Life 5 Dimensions 3 Level Version (EQ-5D-3L) Scale., Results: The study was conducted with 521 participants, 81% complaining of post-COVID symptoms. The most common symptoms were fatigue and shortness of breath. Long COVID was found to significantly reduce the quality of life in both sub-dimensions of the scale. Compared to SF-36 Turkish population norms, all participants showed a significant decrease in quality of life subscales. Vaccination against severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) was found to improve quality of life and reduce long-term COVID-19 symptoms., Conclusion: COVID-19 has significantly affected quality of life. Post-illness follow-up of people with COVID-19 is important to provide patients with medical, physical, and psychosocial rehabilitation support. SARS-COV-2 vaccine reduces the negative effects of COVID-19 on quality of life. For this reason, the community should be made aware of vaccination, and COVID-19 vaccination rates should be increased in the community., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Infectious Diseases and Clinical Microbiology.)

Hepatoblastoma (HB) is the most common liver malignancy in children. The prognosis changes according to the histologic subtypes of HB. In the present study, we aimed to characterize the expression level of selected microRNAs (miRNAs) in HB as well as in histologic subtypes, and to consider the association with the prognosis. A total of 22 HB tumor samples, subtyped as fetal (n=16) and embryonal (n=6), and 10 nontumorous surrounding liver samples were evaluated in this study. Expressions of miR-17, miR-146a, miR-302d, and miR-19b were analyzed in 22 HB tumor samples and 10 nontumorous surrounding liver samples by quantitative real-time polymerase chain reaction. Lower miRNA-17 expression levels were obtained in tumor samples in comparison with nontumorous surrounding liver samples (P=0.028). Lower miRNA-17 expression was significant for predicting prognosis in HB patients (area under receiver-operator characteristic curve=0.875, P=0.044). A higher-level of miR-19b was found in embryonal samples (P=0.008). Overall and event-free survival was not found to correlate with miRNA expression levels (P>0.05). This research finds miRNA-17 and miRNA-19b expression levels can provide important data on diagnosis and prognosis in HB showing different clinical behaviors.

Hepatitis A is a worldwide vaccine-preventable infection. The aim of our study was to determine the serological status of hepatitis A virus (HAV) among first-year nursing students in Turkey. A sample of 423 students was used and immunoglobulin G antibodies against HAV were determined quantitatively by enzyme-linked immunosorbent assay on each. Overall, 84.6% had no immunity to HAV, making them at risk for HAV, and so susceptible to nosocomial transmission. Nursing students who work in high-risk wards must be vaccinated against hepatitis A.

Purpose: The aim of this study was to investigate differences in retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thicknesses in neurofibromatosis 1 (NF1) cases, with and without optic pathway gliomas (OPGs)., Materials and Methods: In total, 33 eyes of 33 subjects were evaluated in this prospective observational case series. Twenty-one patients with a diagnosis of NF1 were enrolled. Patients with NF1 and OPGs were included in Group 1 (n = 9), and patients with NF1 without OPGs were included in Group 2 (n = 12). The control group (Group 3) was comprised of 12 age- and sex-matched subjects with no history of ophthalmic or systemic diseases. All of the subjects underwent complete ophthalmic examinations, including best-corrected visual acuity (BCVA), slit lamp microscopy, and indirect ophthalmoscopy. Additionally, optical coherence tomography (OCT) measurements were obtained., Results: There were no statistically significant between-group differences in age and sex (p = 0.227 and 0.986, respectively). The average RNFL thickness in Group 1 (NF1 patients with OPGs) was significantly lower than in Groups 2 and 3 (86.6 ± 22.5, 107.4 ± 6.65, and 108.4 ± 5.05 µm, respectively; p = 0.001). Furthermore, the average GCC thickness in Group 1 was significantly lower than in Groups 2 and 3 (78.6 ± 16.3, 94.8 ± 3.55, and 94.9 ± 3.82 µm, respectively; p < 0.001)., Conclusions: Both RNFL and GCC thicknesses were significantly lower in NF1 patients with OPGs. The use of OCT to quantify damage to the visual pathway may enable earlier detection of OPGs in NF1 patients.

Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Hemangiomas, like pure arteriovenous malformations, can cause hyperdynamic heart failure, and diffuse nodular-type hemangiomas can present with hypothyroidism. Respiratory problems and hepatic failure can be associated with diffuse nodular-type liver hemangiomas. There is a spectrum of approaches to management, varying from "watchful waiting" to liver transplant. In the age of propranolol, there has been a prominent change in the infantile hepatic hemangioma treatment algorithm. Our suggestion is early treatment with 3 mg/kg/day propranolol plus 1.0 to1.5 mg/kg/day prednisolone in all patients. This protocol is the most effective strategy for type 3 infantile hepatic hemangioma. Approximately one-third of patients with abdominal compartment syndrome in the era before propranolol treatment required liver transplant; this new treatment obviates transplant for many of these patients.

Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia. While he was being investigated for clinical and laboratory signs mimicking hemolytic anemia, he developed cough and dyspnea. He had infiltrates on chest x-ray and scattered patchy infiltrates in both lungs on high-resolution computed tomography. Hemosiderin laden macrophages were identified in fasting gastric juice and bronchoalveolar lavage fluid. The patient was diagnosed with idiopathic pulmonary hemosiderosis and started corticosteroid therapy.

Background/aim: To assess the immune status against tetanus in relation to self-declared vaccination status among an adult population in Yozgat, Turkey., Methods: Questionnaires and blood specimens were collected from 267 individuals over 18 years of age in Yozgat, Turkey. Antitetanus antibodies were determined quantitatively by enzyme-linked immunosorbent assay., Results: Among the 267 subjects (mean age 43.4 ± 15.6; male:female, 133:134) 168 (62.9%) had protective level of tetanus antibody titers. There was a characteristic distribution of the tetanus antibody titers in different age groups as it dropped with increasing age. Protective immunity was obtained in 74.1% of urban and 47.9% of rural participants. The lowest immunity was observed among farmers (53.3%) and housewives (55.6%) when compared to other occupations. Protective antibody titers were detected in 25% of the individuals who had not finished any school, while the rate was 83.7% in those who had graduated from university. While the protection rate in women who had been vaccinated during pregnancy was 78.3%, this rate was 39.2% in nonvaccinated women., Conclusion: Our findings confirm that only 62.9% of the population is immunized against tetanus, with a lack of coverage in particular for the elderly and in rural areas. It is necessary to follow the recommendations for 10-year boosters for tetanus.

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system, that predominantly affects the spinal cord and the optic nerve. Its key features include transverse myelitis, commonly associated with extensive inflammation spanning three or more consecutive vertebral segments. Longitudinal extensive spinal cord lesions can also occur in systemic autoimmune diseases, infections, vascular and metabolic disorders, subsequent to irradiation, intramedullary tumors and paraneoplastic myelopathies. We present a case study of an 8-year-old girl seropositive for antibodies against the aquaporin 4 who displayed longitudinal extensive spinal cord lesions, that was initially misdiagnosed as an intramedullary tumor.

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.

Congenital leukemia is a rare disease. The majority of cases of this disease are acute myelogenous leukemia (AML). Congenital acute lymphoblastic leukemia (ALL) is rare and most often is of B cell lineage. Rarely, some cases have been designated biphenotypic or mixed phenotype acute leukemia (MPAL). Herein, we report a preterm newborn referred to us as a result of the appearance of blue-violaceous dermal nodules on her body at birth. She was a twin and the product of an in vitro fertilization (IVF) pregnancy. Physical examination showed jaundice, hepatosplenomegaly, and peripheral facial nerve palsy in addition to dermal nodules. Bone marrow aspiration showed 40% blasts of lymphoid lineage; skin biopsy and its immunohistochemistry revealed myeloblastic infiltration of the dermis. Cytogenetic analysis (46,XX), fluorescence in situ hybridization (FISH) analysis, and cranial magnetic resonance were normal. The patient was diagnosed with congenital MPAL, and an association between IVF and congenital leukemia was suggested., (Copyright © 2013. Published by Elsevier B.V.)

Background: We report an outbreak of surgical site infections due to genetically related strains of Streptococcus pyogenes in a cardiovascular surgery department., Methods: The practices that were possibly related to the outbreak were investigated through direct observation and interviews with staff by an infection control team. Surveillance sampling from patients, health-care workers, and environment were done for the investigation of the source. Pulsed-field gel electrophoresis was used to investigate a clonal relationship among the S. pyogenes isolates., Results: Four patients operated on in the cardiovascular surgery department developed surgical site infection due to S. pyogenes. Molecular characterization of S. pyogenes done by pulsed-field gel electrophoresis revealed the same strain., Conclusions: Although a definite source for the outbreak could not be identified, probably lack of adherence to hand hygiene practices during surgical dressings, contamination, and cross contamination led to this outbreak.

Aim: This study was planned with the aim of retrospectively reviewing the clinical and laboratory findings and therapies of our patients diagnosed with tuberous sclerosis and redefining the patients according to the diagnostic criteria revised by the 2012 International Tuberous Sclerosis Complex Consensus Group and comparing them with the literature., Materials and Method: Twenty patients diagnosed with tuberous sclerosis complex in the Pediatric Neurology Clinic were examined retrospectively in terms of clinical findings and therapies. The diagnoses were compared again according to 1998 and 2012 criteria., Results: It was observed that the complaint at presentation was seizure in 17 of 20 patients and hypopigmented spots on the skin in 3 of 20 patients. On the initial physical examination, findings related with the disease were found in the skin in 17 of the patients, in the eye in 5, in the kidneys in 7 and in the brain with imaging in 17. No cardiac involvement was observed in the patients. Infantile spasm was observed in 7 of the patients who presented because of seizure (n=17), partial seizure was observed in 7 and multiple seizure types were observed in 3. It was found that sirolimus treatment was given to 9 of 20 patients because of different reasons, 7 of these 9 patients had epileptic seizures and sirolimus treatment had no effect on epileptic seizures. According to 2012 diagnostic criteria, no marked change occured in the diagnoses of our patients., Conclusions: It was observed that the signs and symptoms of our patients were compatible with the literature. Molecular genetic examination was planned for the patients who were being followed up because of probable tuberous sclerosis complex. It was observed that sirolimus treatment had no marked effect on the seizure frequency of our patients.

We evaluated the effect of early use of ribavirin on outcomes of patients at a secondary care hospital. The study included patients who were hospitalized between 2005 and 2010 at the Infectious Diseases Department of Kastamonu Dr. Münif İslamoğlu Hospital in the Kastamonu Province of Turkey. In total, 342 confirmed cases of Crimean-Congo hemorrhagic fever (CCHF) were included in the study. The overall case fatality rate was 2.9%. In multivariate analysis, the patients that were admitted to the hospital within 2 days after onset of symptoms (odds ratio [OR]=5, confidence interval [CI] 0.31-0.86) and received oral ribavirin (OR=0.12, CI 0.05-0.26) were less likely to become more severe cases and less likely to be transferred to the tertiary care centers. Having vaginal bleeding was a significant parameter for transfer.

Brucella endocarditis (BE) is a rare but life-threatening complication of human brucellosis. The aim of this study was to investigate the course of BE along with the therapeutic interrelations. A total of 53 patients with BE hospitalised in 19 health institutions between 2006 and 2011 were included in the Gulhane study. Diagnosis of brucellosis was established by either isolation of Brucella sp. or the presence of antibodies, and the definition of endocarditis was made according to Duke's criteria. There were four treatment groups: ceftriaxone combined with oral antibiotics (Group 1); aminoglycosides combined with oral antibiotics (Group 2); oral antibiotic combinations (Group 3); and aminoglycoside plus ceftriaxone combined with an oral antibiotic (Group 4). Involvement rates of the aortic, mitral and tricuspid valves were 49.1%, 43.4% and 5.7%, respectively. Thirty-two patients (60.4%) had an underlying cardiac valvular problem, including previous prosthetic valve replacement (n=18). Medical treatment was provided to 32 patients (60.4%), whilst concordant medical and surgical approaches were provided to 21 patients (39.6%). Mortality in Group 1 was 15% (3/20), whilst in Group 2 it was 5.3% (1/19). In Group 3, 25.0% (3/12) of the cases died, whereas none of the cases in Group 4 died. In conclusion, mortality increased 47-fold with pericardial effusion and 25-fold due to congestive heart failure that developed after BE. Although mortality was lower in the aminoglycoside-containing arm (Groups 2 and 4), statistical analysis could not be performed owing to the small number of patients., (Copyright © 2012 Elsevier B.V. and the International Society of Chemotherapy. All rights reserved.)

Trimethoprim-sulfamethoxazole (TMP-SMZ) is recommended as the treatment of choice for Stenotrophomonas maltophilia infections. However, when the administration of TMP-SMZ is not possible, alternative treatment options for S. maltophilia infections has not been clearly established. We compare the efficacy of tigecycline treatment with TMP-SMZ in nosocomial S. maltophilia infections during a 3-year period. For the treatment of S. maltophilia infection, 26 (57.8%) patients received TMP-SMZ and 19 (42.2%) patients received tigecycline. Culture positivity rate was 95.7% in TMP-SMZ group and 70.6% in tigecycline group at the seventh day (P = 0.028), whereas 26.3% versus 18.8% at the fourteenth day (P = 0.700). Clinical improvement was observed 69.2% in TMP-SMZ group and 68.4% in tigecycline group at the fourteenth day (P = 0.954). Mortality rates at the thirtieth day were respectively, 30.8 and 21.1% in TMP-SMZ and tigecycline groups (P = 0.517). There were no significant differences in mortality and clinical response rates between TMP-SMZ and tigecycline treatment. Tigecycline can be considered as an alternative option beyond TMP-SMZ in treatment of S. maltophilia infections.

Splenic abscess is a very rare complication of non-typhoidal Salmonella infections. We report a case of splenic abscess caused by Salmonella enteritidis. The patient is a 63-year-old woman with diabetes mellitus and underwent splenectomy. This case suggests that the patients with comorbities are at increased risk for invasive infections in non-typhoidal Salmonella infections.

Background: Mediastinitis due to Acinetobacter baumannii is rare., Methods: We report a case of mediastinitis caused by pan-resistant A. baumannii in a patient with multiple comorbidities who underwent cardiopulmonary bypass and we also present a literature review., Results: Successful treatment consisted of surgical debridement plus drainage, and tigecycline administration for three weeks., Conclusion: Surgical source control along with effective antibiotics is essential in the treatment of mediastinitis. Tigecycline has the potential to be an option for pan-resistant A. baumannii mediastinitis.

We report two male cases (4- and 5-years-old) of intrathecal methotrexate overdose. The two boys with acute lymphoblastic leukemia were to receive intrathecal injection of methotrexate. Instead of the prescribed 12 mg, they both received a dose of 120 mg. The initial cerebrospinal fluid samples showed methotrexate concentration of 2.24x10-2M in case 1 and 1.32x10-2M in case 2. The cases were successfully treated with cerebrospinal fluid (CSF) exchange and intravenous folinic acid rescue. The favorable outcome in our cases suggests that CSF exchange is safe and that folinic acid rescue may be adequate to prevent sequelae in patients subjected to intrathecal MTX overdoses up to 120 mg. We propose CSF exchange and intravenous folinic acid as the mainstay of treatment. In addition to the staff's failure to check the drug label carefully, the marked resemblance of the two dose preparations of MTX may have been contributory.

Objectives: Crimean-Congo hemorrhagic fever (CCHF) is a lethal hemorrhagic disease. There is currently no specific antiviral therapy for CCHF approved for use in humans. In this study we aimed to investigate the effect of oral ribavirin treatment on the viral load and disease progression in CCHF., Methods: The study population was composed of patients who had a definitive diagnosis of CCHF by means of clinical presentation plus detection of viral RNA by reverse transcriptase polymerase chain reaction (RT-PCR). Ten patients who received oral ribavirin for 10 days and 40 control patients who received supportive treatment only were included in the study. Ribavirin treatment consisted of oral ribavirin 4 g/day for 4 days and then 2.4 g/day for 6 days. Viral load and hematological and biochemical laboratory parameters, which were measured daily, were analyzed., Results: Mean age (37.4 vs. 45.5, p=0.285), gender (male 50% vs. 62.5%, p=0.470), days from the appearance of symptoms to admission (4.3 vs.4.4 days, p=0.922), and initial complaints were similar between the ribavirin group and the control group. Upon hospital admission, mean viral load was 8.2×10⁸ copies/ml in the ribavirin group and 8.3×10⁸ copies/ml in the control group (p=0.994). During follow-up, no statistically significant differences were found between the groups with regard to the decrease in viral load, the reduction in alanine aminotransferase and aspartate aminotransferase levels, and the increase in platelet count. The case-fatality rate was 20% (2/10 patients) in the ribavirin group and 15% (6/40 patients) in the control group (p=0.509)., Conclusion: In this study, oral ribavirin treatment in CCHF patients did not affect viral load or disease progression., (Copyright © 2010 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Despite the relatively recent introduction of propranolol in the treatment of infantile hemangiomas, there can be little doubt of its efficacy. With regard to safety issues, there are no prior data for very low weight infants. In this study, we used propranolol in preterm and very low weight infants. We used clinical criteria to assess the response to the therapy. We noted all side effects expected from beta-adrenergic blocking drugs, and followed the patients' weight gain during propranolol treatment. Objective, clinical evidence of hemangioma regression was seen after two months in all patients. None of the patients required treatment discontinuation due to adverse side effects. During the propranolol treatment, weight gain was normal in all patients. To the best of our knowledge, this is the first report on the use of propranolol in preterm and very low weight infants, and also the first report from Turkey on the use of propranolol in infantile hemangiomas.

Background and Aim of the Study: Despite recent improvements in diagnostic and therapeutic interventions, infective endocarditis (IE) is still associated with high in-hospital mortality rates. The study aim was to determine the clinical, laboratory and echocardiographic features of IE, and to evaluate the risk factors for in-hospital mortality., Methods: A retrospective cohort study design was employed, with a main outcome measure of in-hospital mortality. A total of 107 patients (79 males, 28 females; mean age 45 +/- 16 years) admitted with the modified Duke criteria for definitive IE were included in the study during a five-year period between January 2004 and December 2008., Results: Among the patients, the mitral valve alone was involved in 45% of cases, the aortic valve in 36%, tricuspid valve in 11%, and multiple valves in 8%. Forty-seven patients (44%) had prosthetic valves. Blood cultures were positive in 71 patients (66%). The most common isolated microorganisms were staphylococci, streptococci and Brucella melitensis. The in-hospital mortality rate was 27%. Leading causes of death were multi-organ failure and heart failure. In univariate analysis, factors associated with death were a longer duration of symptoms before hospitalization, previous history of IE, white blood cell count > or = 10,000/mm3, serum creatinine level > or = 2 mg/dl, vegetation size >15 mm, involvement of multiple valves, existence of severe regurgitation, cardiac abscess, and neurologic complications. Multivariate analysis showed that risk factors for mortality were multivalvular involvement (hazard ratio (HR) 4.7; 95% confidence interval (CI) 1.3-17.6; p = 0.021), vegetation size >15 mm (HR 5.5; 95% CI 2.1-14.6; p = 0.001), serum creatinine > or = 2 mg/dl (HR 4.1; 95% CI 1.8-9.4; p = 0.001), and previous history of IE (HR 3.5; 95% CI 1.2-11; p = 0.026)., Conclusion: Multivalvular involvement, vegetation length >15 mm, serum creatinine level > or = 2 mg/dl on admission, and a previous history of IE, were independent predictors for in-hospital mortality in IE.

The impact of appropriate empirical antimicrobial therapy for Acinetobacter baumannii bacteraemia on patient outcomes has not been clearly established. We assessed predictors of 30-day mortality and the effect of inappropriate empirical antimicrobial treatment on mortality among patients with A. baumannii bacteraemia between July 2005 and June 2008. Initial empirical antimicrobial therapy was considered to be appropriate if the initial antibiotics that were administered within 48 h after the acquisition of a blood culture sample included at least one antibiotic that was active in vitro and when the dosage and route of administration were in accordance with current medical standards. Overall, 103 patients with nosocomial A. baumannii bacteraemia were included in the study. Appropriate empirical therapy was administered to 41.7% of patients within 48 h. The overall mortality rate was 54.4%, with rates of 39.5% and 65% for patients who received appropriate and inappropriate antimicrobial therapy within 48 h, respectively. Thus, a 25.5% reduction in the overall crude mortality rate was associated with adequate early empirical antimicrobial therapy. Multivariate analysis using a Cox regression model showed that significant independent risk factors for mortality were delayed appropriate treatment [hazard ratio (HR)=2.4, 95% confidence interval (CI) 1.3-4.2; P=0.004], development of septic shock (HR=2.6, 95% CI 1.4-4.8; P=0.004), age>65 years (HR=2.1, 95% CI 1.2-3.7; P=0.007) and mechanical ventilation (HR=3.3, 95% CI 1.5-7.4; P=0.003). It is concluded that a delay in receiving appropriate antimicrobial therapy had an adverse influence on clinical outcome in patients with A. baumannii bacteraemia.

Objectives: This study examined the association between lymphocyte subgroups and mortality in patients with Crimean-Congo hemorrhagic fever (CCHF) in Turkey., Methods: During the spring and summer of 2007, peripheral blood was collected from hospitalized patients with suspected CCHF. Lymphocyte subgroups were characterized by fluorescence-activated cell sorting. CCHF cases were confirmed by detecting viral RNA by PCR and/or IgM antibodies by ELISA. Lymphocyte subgroups were compared between fatal and non-fatal cases. The correlation between lymphocyte subgroups and viral loads was also investigated., Results: Seventy-seven confirmed cases of CCHF were included in this study (five cases were fatal (6.5 %)). No differences in lymphocyte subgroups were found between fatal and non-fatal cases, except for significantly higher CD3+CD8+ T cells in the fatal cases (p=0.017). A positive correlation between viral load and CD3+CD8+ T cells was also detected (p=0.044). There was no correlation between other lymphocyte subgroups and viral load., Conclusions: Higher levels of CD3+CD8+ T lymphocytes were detected in fatal compared to non-fatal CCHF cases. Despite this cytotoxic immune activation, a fatal outcome could not be prevented. We hypothesize that high viral load and other factors may influence this outcome, although more studies are required to explain the pathogenesis of CCHF.

Apoptosis-related proteins and proliferation activity and their relationship with Epstein-Barr Virus (EBV) are contemporary issues in pediatric non-Hodgkin's lymphoma (pNHL). In this study prognostic or pathogenetic role of EBV latent infection, proliferating activity, and apoptosis-regulating proteins in pNHL were explored. EBV-EBER, lmp-1, ki-67, bcl-2, survivin, bax, fas, c-myc, p53 and apoptotic index by TUNEL method were explored in 70 pNHL cases and evaluated statistically. Of the 70 cases evaluated, 24 were female and 46 were male. Seven cases were stage I/II and 63 cases were stage III/IV. The mean age was 7.16 + or - 3.72(1-15). EBV was positive in (25.7%) cases. Overall survival was 82%, while event free survival was 75%. Bax was expressed in 40% of the cases, while the expression of bcl-2,was 50%, survivin 42.9%, p53 8.6%, fas 18.6% and c-myc in 45.7%. Mean apoptotic index was 131.29 + or - 96.69 per 5,000 cells. Mean proliferation index was 55.97% (12-92%). Fas positivity was high in EBV positive cases (p = 0.0001). EBV positivity was not related with prognosis. Apoptotic index was found to be an independent prognostic factor (p = 0.017). Our results suggest that apoptosis-regulating proteins have a role in the pathogenesis of pNHL. EBV was correlated with apoptotic index and fas, bcl-2. No correlation was observed with proliferation index and studied factors. High apoptotic index was related with good prognosis.

Brucellosis is a systemic infection with multiple presentations. Despite its oral transmission and gastrointestinal pathogenesis, systemic symptoms are usually more prominent than gastrointestinal complaints. We report a patient with enteric fever caused by Brucella melitensis.

Cryptosporidium spp. is known to cause heavy diarrhea especially in immunosuppressed patients. In this study, eighty nine leukemia and lymphoma patients between the ages of 1 to 14 were studied for the prevalence of Cryptosporidiosis using both ELISA (Cryptosporidium Rida Screen, R-Biopharm, Germany) and the Kinyoun acid-fast staining method. These patients were sent to us by the Hematology-Oncology department where they were diagnosed with leukemia and lymphoma. Cryptosporidium spp. were detected in 11 patients (12.35%) with ELISA and in 7 patients (7.86%) with the Kinyoun acid fast stain. No cryptosporidiosis was detected in the control groups of 60 patients with neoplasia but without diarrhea. The distribution of Cryptosporidium among positive samples were 7 (14.8%) in patients who were diagnosed with ALL, 3 (10%) in patients who were diagnosed with KML, and 1 (8.3%) in patients who were diagnosed with solid tumors. Sixty-five patients (73.03%) had a fever, 43 patients (48.31%) were vomiting and 58 patients (65.16%) had stomach pain. Except for two, all the patients responded positively to paromomycin treatment. Those two patients responded positively to azitromycine treatment. We suggest that when considering cryptosporidiosis in children with cancer, the use of a more sensitive and specific method such as ELISA- in addition to the acid fast stain should be considered.

Crimean-Congo hemorrhagic fever (CCHF) is a potentially fatal disease affecting multiple organ systems. To determine the association between viral load and severity of CCHF infection, quantitative measurement of CCHF virus was performed using 1-step reverse-transcriptase polymerase chain reaction for 36 patients with CCHF infection. Viral loads ranged from 1.1x10(3) copies/mL to > or = 9.9x10(9) copies/mL. Nine (25%) of 36 patients died. In 8 of the 9 patients with fatal outcomes, viral loads were detected that were > or = 1x10(9) copies/mL, whereas in 25 of the 26 patients with nonfatal outcomes, viral loads were detected that were < 1x10(9) copies/mL (P<.001). A viral load > or = 1x10(9) RNA copies/mL can be considered to predict a fatal outcome with a positive predictive value of 80%, with 88.9% sensitivity and 92.6% specificity. We suggest that viral load is a measure of the severity of CCHF.

Apoptosis-related genes and proteins and proliferation activity and their relationship with Epstein-Barr virus (EBV) is a contemporary issue. In this study, prognostic or pathogenetic roles of EBV latent infection, proliferating activity, and apoptosis-regulating proteins in pediatric Hodgkin lymphomas were explored. EBV-EBER, lmp-1, ki-67, bcl-2, survivin, Bax, fas, c-myc, and p53, and apoptotic index were analyzed in 63 pediatric Hodgkin lymphoma cases. The results were evaluated by chi-square, Mann Whitney U test, Pearson correlation analysis, and Kaplan Meier survival analysis. Thirty-two cases were stage I or II, whereas 31 cases were stage III or IV. The mean age was 8.4 +/- 63.54 years. EBV was positive in 52 (82.5%) cases. Overall survival was 94% and event-free survival 83.6%. Bax expression was observed 74.6%, bcl-2 47.6%, survivin 43%, p53 33.3%, fas 54%, and c-myc 25.4%. The mean apoptotic index was 18.22%. The mean proliferation index was 57.83%. The proliferation index was positively related with EBV but not with prognosis. None of the parameters were related with prognosis. EBV was negatively related with the apoptotic index. There were no relationships between bax, bcl-2, survivin, p53, fas, and c-myc with EBV. These results suggest that EBV might play a role in Hodgkin lymphoma pathogenesis by inducing proliferative activity and inhibiting apoptosis. Apoptosis-related proteins were not correlated with EBV. None of the parameters was found to predict prognosis.

Objective: To identify risk factors for recurrent catheter related bloodstream infections (CR-BSIs). The study was undertaken at the University of Utah Hospital and involved patients who had a CR-BSI followed by catheter removal and reinsertion between January 1998 and February 2002., Design: A retrospective chart review for the cohort study of catheters initially infected, which were then followed to study risk factors for a subsequent infection. Both central line and peripherally inserted central line catheters were included in the study. A recurrent CR-BSI was defined as positive blood cultures after three negative cultures, coupled with positive catheter tip culture or no other evident new source of infection., Results: Twenty-five (34%) of 73 patients had a recurrent CR-BSI. The first CR-BSI occurred a mean of 20.4 days after catheter insertion whereas recurrence developed a mean of 12.1 days after reinsertion (p = 0.392). Coagulase-negative staphylococci (60%) were the most common cause of recurrent infection. The recurrence was more common among the patients who were given blood product transfusion (hazard ratio (HR) 2.3; confidence interval (CI) 1.02-5.67, p = 0.049). In 20 (27%) patients, catheters were changed over a guidewire. The guidewire catheter exchange was not found to be associated with an increased risk of recurrent infection (p = 0.582)., Conclusion: Catheter replacement to a new site, instead of rewiring, was not shown to decrease the risk for recurrent infection. The transfusion of blood products was associated with an increased risk for recurrent infection.

May-Hegglin anomaly is a hereditary thrombocytopenia associated with giant platelets and large basophilic, cytoplasmic inclusion bodies (resembling Döhle bodies) in the granulocytes. Patients may experience easy bruising, recurrent epistaxis, gingival bleeding, menorrhagia and sometimes excessive bleeding associated with surgical procedures. Failure to appropriately diagnose May-Hegglin anomaly could result in inappropriate treatment. In states of chronic thrombocytopenia associated with large platelets, including chronic idiopathic thrombocytopenic purpura, May-Hegglin anomaly should be considered in the differential diagnosis. In this case report, we present a five-year-old girl previously followed as idiopathic thrombocytopenic purpura without bleeding symptoms and a 14-year-old boy who were diagnosed with May-Hegglin anomaly.

Background: Epididymoorchitis is the most frequent genitourinary complication of brucellosis., Methods: This prospective study was conducted between February 2001 and January 2004, prospectively. Male patients diagnosed with brucellosis were included in this study and evaluated for testicular involvement., Results: Epididymoorchitis was detected in 17 out of 134 (12.7%) male patients with brucellosis. Mean age of the patients was 36.9+/-7.1 years. Twelve patients (70.6%) had acute, four patients (23.5%) had subacute, and one patient (5.9%) had chronic brucellosis. The most common symptoms were scrotal pain (94%) and swelling (82%). Eleven patients had unilateral epididymoorchitis, four had unilateral orchitis and two had unilateral epididymitis. A testicular abscess was detected in one patient. Sperm analysis was performed on 14 patients. Five patients had aspermia and eight had oligospermia. Combined antibiotic therapy was started and continued for 6-8 weeks. Orchiectomy was required for two patients and granulomatous orchitis was detected in the resected specimens. Relapse occurred in only one patient. Three patients had permanent oligospermia and one patient had permanent aspermia after the antibiotic therapy. Younger age, high C-reactive protein level and blood culture positivity were statistically significant differences between the patients with and without epididymoorchitis., Conclusions: Brucellosis should be considered in the diagnosis of scrotal diseases in endemic areas. A conservative approach is usually adequate for managing brucellar epididymoorchitis. However, infertility problems may develop in these patients. Well-designed further investigations are needed to explain the relationship between brucellar epididymoorchitis and infertility in man.

Calcinosis cutis, an uncommon disorder characterized by hydroxyapatite crystals of calcium phosphate deposited in the skin, has been described infrequently in childhood. Cutaneous calcification may be divided into four major categories: dystrophic, metastatic, idiopathic, and iatrogenic. Here, we report an example of iatrogenic type with a 4-year-old boy who diagnosed with Burkitt's lymphoma, and developed calcinosis cutis secondary to a tumour lysis syndrome with induction chemotherapy.

Human telomerase reverse transcriptase (hTERT) is the catalytic component of telomerase enzyme and has been shown to be associated with telomerase activity (TA). Although many studies in adult leukemia have established the importance of TA, very few have been reported in the children. In this study hTERT levels in childhood leukemia was evaluated and compared with the prognostic factors described before. The LightCycler instrument was used (online real-time PCR) for the quantification of hTERT in peripheral blood and bone marrow in 23 cases with acute lymphoblastic leukemia (ALL) and in 8 cases with acute myeloblastic leukemia (AML). Ten cases with normal peripheral blood (PB) and bone marrow (BM) were selected as control group. Cytogenetic analyses were available in 21 patients with leukemia. In all cases with acute leukemia and in control group, peripheral blood (PB) hTERT levels correlated significantly with bone marrow (BM) hTERT levels. Before treatment, patients with ALL had significantly higher hTERT levels than that of AML patients and control cases. Among patients with ALL, higher hTERT levels were observed in patients with pre-B leukemia, followed by B cell and T cell leukemia patients. Initially increased hTERT levels decreased to the nearly normal levels during remission in cases with ALL. No correlation was observed between the initial hTERT levels and the known prognostic factors except cytogenetic findings. Higher hTERT levels were detected in patients having karyotypic abnormalities which indicate poor prognosis. hTERT levels are significantly high in childhood ALL with the highest level of pre-B cell leukemia before treatment. Those high levels of hTERT decrease to almost normal levels in remission. hTERT levels might be useful in monitoring of leukemia in children.

Possible clinical relevance of Nm23 expression, angiogenesis and proliferative activity were evaluated as prognostic parameters in childhood embryonal rhabdomyosarcoma (RMS). Specimens of 25 RMS cases were studied for Nm23 antigen immunohistochemically. Vascular surface density (VSD) and number of vessels per stroma (NVES) calculated by stereologic methods on labeling sections with CD34 antibody. For evaluation of proliferative activity of tumors, mitotic figures and Ki67 positive cells were investigated. All findings were searched statistically. Five patients were stage 1 (20%), two were stage 2 (8%), 15 were stage 3 (60%) and three were stage 4 (12%). The mean event free survival (EFS) was 20.8 and the mean overall survival (OS) was 25.9 months. Sixteen patients (64%) were alive and without disease. The percentage of Nm23 positivity was 52%. Log rank analysis showed Nm23 as a predictor for survival (p=0.0313). In Pearson correlation analysis, there was statistical significance between OS and presence of Nm23 expression (p=0.044). VSD was also positively related with EFS (p=0.040). Despite the present parameters in use, there is a need for new prognostic markers, especially to predict the outcome of patients. These findings suggested that Nm23 expression and VSD might be useful for follow-up in RMS.

Stool specimens taken from 50 children with malignancy and from 92 healthy children were investigated for intestinal parasites, using the modified formol ethyl acetate concentration method, and native-lugol, trichrome and Kinyoun acid-fast stain methods. Thirty-eight (76.0%) of the 50 patients had lymphoma or leukemia and were considered immunosuppressed. Several different parasites were found in 21 (42.0%) of the 50 patients with malignancy and in 16 (47.3%) of the 38 patients with immune deficiency compared to in only 16 (17.3%) of the 92 healthy children. The incidence of parasites in patients with malignancy or immunosuppression was significantly higher than in the healthy control group (p<0.01, p<0.01).

The study was designed to evaluate rational antibiotic use in relation to diagnosis and bacteriological findings. All hospitalized patients who received antibiotics were evaluated by a cross-sectional study. Of the 713 patients hospitalized, 281 (39.4%) patients received 377 antibiotics. Among 30 different antibiotics the most frequently requested were first generation cephalosporins (19.9%), ampicillin-sulbactam (19.1%) and aminoglycosides (11.7%). Antibiotic use was appropriate in 64.2% of antibiotic requests. In analysis of appropriate use, a request after an infectious diseases consultation was a frequent reason (OR=14, P<0.001, CI=0.02-0.24). Antibiotics requested in conjunction with susceptibility results were found to be more appropriate than those ordered empirically (OR=4.5, P=0.017, CI=0.06-0.76). Inappropriate antibiotic use was significantly higher among unrestricted antibiotics than restricted ones (P<0.001). Irrational antibiotic use was high for unrestricted antibiotics. Additional interventions such as postgraduate training programmes and elaboration of local guidelines could be beneficial.

Thrombocytopenia is one of the rare hematologic complication of brucellosis. Herein a case of brucellosis with severe thrombocytopenia was reported. The patient was admitted to hematology service with epistaxis, ecchymoses, fever, thrombocytopenia (0.6 x 103/μL) and anemia. His initial diagnosis was idiopathic thrombocytopenic purpura. Corticosteroid treatment was started and erythrocyte and platelet suspensions were transfused. Despite this treatment clinical symptoms and laboratory disorders were not improved. No significant pathology was detected in the examination of bone marrow aspiration. On the seventh day, Brucella abortus was yielded from his blood cultures. Steroid was stopped and rifampicin plus doxycycline started. His clinical symptoms were disappeared and laboratory findings improved (thrombocyte count: 205 x 103/μL) at the second week of the antibiotic treatment. This case was interesting showing us that thrombocyte count might decrease to very low levels in brucellosis. So that, especially in the endemic areas, brucellosis should be kept in mind for the etiology of fever and thrombocytopenia.

Objective: To study natural killer cells' spontaneous cytotoxic capacity against tumor cells and their prognostic significance in classical Hodgkin's disease., Study Design: Thirty-eight pediatric mixed cellularity and nodular sclerosing Hodgkin's disease patients were included in the study. Immunohistochemical staining was performed for natural killer cells in the background using the monoclonal antibody CD57 in serial sections of B5-formalin-fixed, paraffin-embedded tissue blocks. CD57-positive cells were counted with an immersion objective among 5,000 cells on representative areas of the tumors. The degree of natural killer cells was classified as low (<150 cells) or high (> or = 150 cells). Multivariate regression analysis was performed to determine the differences between patients with and without relapse., Result: The mean of CD57-positive cell numberfor all the cases was 173.42 +/- 117.34 (range, 20-500). CD57-positive cells were high in 21 cases and low in 17. The mean of CD57-positive cell numbers was 191.85 +/- 115.33 in the disease-free group and 84.44 +/- 57.90 in the relapsing group. Log rank analysis showed statistical significance between event-free survival and number of CD57-positive cells (P = .0207)., Conclusion: In multivariate analysis, CD57 expression proved to be a prognostic factor independent from otherfactors. As a result, CD57 expression by background natural killer cells may be used as a prognostic parameter in mixed cellularity and nodular sclerosing Hodgkin's disease.