Five Years Follow-up of Opsoclonus-Myoclonus-Ataxia Syndrome-Associated Neurogenic Tumors in Children.

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/m2/d for 1-2 days, 10 mg/m2/d for 3-4 days, and 5 mg/m2/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.

Brucellosis in pregnancy: results of multicenter ID-IRI study.

Brucellosis in pregnant women is reported to be associated with obstetric complications (OCs), and adequate data for human brucellosis during pregnancy are largely lacking. We performed this multicenter retrospective cross-sectional study to evaluate the epidemiology, clinical course, treatment responses, and outcomes of brucellosis among pregnant women. The study period comprised a 14-year period from January 2002 to December 2015. All consecutive pregnant women diagnosed with brucellosis in 23 participating hospitals were included. Epidemiological, clinical, laboratory, therapeutic, and outcome data along with the assessment data of the neonate were collected using a standardized questionnaire. Data of 242 patients were analyzed. The OC rate was 14.0% (34/242) in the cohort. Of the 242 women, 219 (90.5%) delivered at term, 3 (1.2%) had preterm delivery, 15 (6.2%) aborted, and 5 (2.1%) had intrauterine fetal demise. Seventeen (7.0%) of the newborns were considered as low birth weight. Spontaneous abortion (6.1%) was the commonest complication. There were no maternal or neonatal deaths and pertinent sequelae or complications were not detected in the newborns. Splenomegaly (p = 0.019), nausea and/or vomiting (p < 0.001), vaginal bleeding (p < 0.001), anemia (blood hemoglobin < 11 g/dL; p < 0.001), high level of serum aspartate aminotransferase (> 41 IU/L; p = 0.025), oligohydramnios on ultrasonography (p = 0.0002), history of taking medication other than Brucella treatment during pregnancy (p = 0.027), and Brucella bacteremia (p = 0.029) were the significant factors associated with OCs. We recommend that pregnant women with OC or with fever should be investigated for brucellosis if they live in or have traveled to an endemic area.

MicroRNA-17, MicroRNA-19b, MicroRNA-146a, MicroRNA-302d Expressions in Hepatoblastoma and Clinical Importance.

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.

Secondary Hemophagocytic Lymphohistiocytosis: Do We Really Need Chemotherapeutics for All Patients?

Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1.02, P=0.006), pediatric logistic organ dysfunction scores (hazard ratio=1.01, P=0.001) were the predictors of the survival. The hospital survival was 83% for patients with sHLH who were treated with less immunosuppressive therapy. In conclusion initiation of HLH-specific therapy for the patients with hyperferritinemia-associated sHLH should be delayed while awaiting resolution of systemic inflammation with less immunosuppressive therapy.

A Case of Idiopathic Pulmonary Hemosiderosis Presenting With Signs and Symptoms Mimicking Hemolytic Anemia.

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.

Quality of life and related factors among chronic hepatitis B-infected patients: a multi-center study, Turkey.

BACKGROUND: The aim of this study was to assess health-related quality of life (HRQOL) among chronic hepatitis B (CHB) patients in Turkey and to study related factors. METHODS: This multicenter study was carried out between January 01 and April 15, 2015 in Turkey in 57 centers. Adults were enrolled and studied in three groups. Group 1: Inactive HBsAg carriers, Group 2: CHB patients receiving antiviral therapy, Group 3: CHB patients who were neither receiving antiviral therapy nor were inactive HBsAg carriers. Study data was collected by face-to-face interviews using a standardized questionnaire, Short Form-36 (SF-36) and Hepatitis B Quality of Life (HBQOL). Values equivalent to p < 0.05 in analyses were accepted as statistically significant. RESULTS: Four thousand two hundred fifty-seven patients with CHB were included in the study. Two thousand five hundred fifty-nine (60.1 %) of the patients were males. Groups 1, 2 and 3, consisted of 1529 (35.9 %), 1721 (40.4 %) and 1007 (23.7 %) patients, respectively. The highest value of HRQOL was found in inactive HBsAg carriers. We found that total HBQOL score increased when antiviral treatment was used. However, HRQOL of CHB patients varied according to their socio-demographic properties. Regarding total HBQOL score, a higher significant level of HRQOL was determined in inactive HBV patients when matched controls with the associated factors were provided. CONCLUSIONS: The HRQOL score of CHB patients was higher than expected and it can be worsen when the disease becomes active. Use of an antiviral therapy can contribute to increasing HRQOL of patients.

International Nosocomial Infection Control Consortium (INICC) national report on device-associated infection rates in 19 cities of Turkey, data summary for 2003-2012.

BACKGROUND: Device-associated healthcare-acquired infections (DA-HAI) pose a threat to patient safety, particularly in the intensive care unit (ICU). We report the results of the International Infection Control Consortium (INICC) study conducted in Turkey from August 2003 through October 2012. METHODS: A DA-HAI surveillance study in 63 adult, paediatric ICUs and neonatal ICUs (NICUs) from 29 hospitals, in 19 cities using the methods and definitions of the U.S. NHSN and INICC methods. RESULTS: We collected prospective data from 94,498 ICU patients for 647,316 bed days. Pooled DA-HAI rates for adult and paediatric ICUs were 11.1 central line-associated bloodstream infections (CLABSIs) per 1000 central line (CL)-days, 21.4 ventilator-associated pneumonias (VAPs) per 1000 mechanical ventilator (MV)-days and 7.5 catheter-associated urinary tract infections (CAUTIs) per 1000 urinary catheter-days. Pooled DA-HAI rates for NICUs were 30 CLABSIs per 1000 CL-days, and 15.8 VAPs per 1000 MV-days. Extra length of stay (LOS) in adult and paediatric ICUs was 19.4 for CLABSI, 8.7 for VAP and 10.1 for CAUTI. Extra LOS in NICUs was 13.1 for patients with CLABSI and 16.2 for patients with VAP. Extra crude mortality was 12% for CLABSI, 19.4% for VAP and 10.5% for CAUTI in ICUs, and 15.4% for CLABSI and 10.5% for VAP in NICUs. Pooled device use (DU) ratios for adult and paediatric ICUs were 0.54 for MV, 0.65 for CL and 0.88 for UC, and 0.12 for MV, and 0.09 for CL in NICUs. The CLABSI rate was 8.5 per 1,000 CL days in the Medical Surgical ICUs included in this study, which is higher than the INICC report rate of 4.9, and more than eight times higher than the NHSN rate of 0.9. Similarly, the VAP and CAUTI rates were higher compared with U.S. NHSN (22.3 vs. 1.1 for VAP; 7.9 vs. 1.2 for CAUTI) and with the INICC report (22.3 vs. 16.5 in VAP; 7.9 vs. 5.3 in CAUTI). CONCLUSIONS: DA-HAI rates and DU ratios in our ICUs were higher than those reported in the INICC global report and in the US NHSN report.

Is very high platelet count always associated with essential thrombocythemia? An unusual presentation in a child.

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.

Long-Term Symptoms and Quality of Life in Persons with COVID-19.

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.

Treatment of intrathecal methotrexate overdose with folinic acid rescue and lumbar cerebrospinal fluid exchange: A report of two cases.

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.

Risk factors for in-hospital mortality in infective endocarditis: five years' experience at a tertiary care hospital in Turkey.

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.

Propranolol for infantile hemangiomas: a preliminary report on efficacy and safety in very low birth weight infants.

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.

Giant hydatid cyst of spleen: a case report.

Hydatidosis is endemic in many Mediterranean countries, the Middle East, South America, Australia, New Zealand and Africa. It is caused by an infection from the Echinococcus granulosus larvae which can lead to the development of cysts. The most frequently affected organ is the liver. Splenic involvement alone is very rare. In this article, a case of an isolated giant splenic hydatid cyst is reported. The patient underwent splenectomy. The diagnosis of a hydatid cyst was confirmed by histopathology. This case suggests that hydatid disease should be considered as a differential diagnosis in every patient with a cystic mass of the spleen in endemic areas.

Hepatitis A immunity in Yozgat, Turkey.

BACKGROUND: Since routine immunization could change the epidemiological profile of hepatitis A virus (HAV) infection in the future, it is important to determine the baseline immunity to HAV across Turkey. OBJECTIVE: The aim of this study was to determine the seroprevalence of hepatitis A among individuals 6 years of age and older in Yozgat, Turkey. DESIGN: Cross-sectional. SETTING: Community in central region of Turkey. PATIENTS AND METHODS: Questionnaires and blood specimens were collected and the presence of hepatitis A IgG antibodies against hepatitis A virus was determined quantitatively by ELISA. MAIN OUTCOME MEASURES: The rates of hepatitis A immunity by age group. SAMPLE SIZE: 1862. RESULTS: Immunity to hepatitis A was 79.1% (n=1473). The mean (SD) age was 17.1 (14.7) years in the nonimmune group and 37.8 (19.5) years in the immune group (P less than .001), and immunity increased with age. No significant difference in immunity rate was detected between gen.ders in children and adults. The seropositivity rate for subjects ages 6-19 years was lower than in subjects aged 20-96 years (52.2% versus 93.9%; P less than .001). CONCLUSION: A catch-up vaccination program is needed for persons aged 6-19 years in Yozgat. LIMITATIONS: Single region data which can not be generalized. For this reason, a multi-centered study that can reflect the whole country is recommended. CONFLICT OF INTEREST: None.

The evaluation of hTERT mRNA expression in acute leukemia children and 2 years follow-up of 40 cases.

The aim of this study is to evaluate (1) the human telomerase-specific reverse transcriptase (hTERT) mRNA expression in childhood acute leukemia, (2) the association between the hTERT mRNA expression with the patients' characteristics and the known prognostic factors and (3) the correlation of the patients' survival with the initial hTERT mRNA value at diagnosis. A total of 40 newly diagnosed patients consist of children [31 cases with acute lymphoblastic leukemia (ALL) and 9 cases with acute myeloblastic leukemia (AML)] were prospectively included into the study. The online real-time reverse-transcriptase PCR was used for the quantification of hTERT in bone marrow (BM). All cases were re-evaluated for their survival after 2 years. The highest hTERT mRNA value was observed in Pre B-cell ALL patients followed by B-cell ALL, T-cell ALL and AML. The hTERT mRNA relative ratio difference between the ALL and AML groups was significant. No significant association was found when hTERT mRNA expression was evaluated in relation with the hematological parameters (except hemoglobin level), blast percentages and the risk groups. No significant difference was determined between the rate of complete remission and relapse of cases with the hTERT mRNA values in all malignancy groups. Patients who had higher initial hTERT mRNA values showed significantly longer disease-free survival (DFS) and overall survival (OS) in ALL (P = 0.000 and 0.01, respectively). Although DFS and OS was longer in AML patients with lower initial hTERT mRNA, the difference was not significant. In conclusion, the hTERT mRNA expression values were not significantly associated with the known prognostic factors in children both with ALL and AML. hTERT mRNA value is a significant factor for childhood ALL at diagnosis in relation to the estimated survival.

Risk factors for nosocomial imipenem-resistant Acinetobacter baumannii infections.

OBJECTIVES: To identify the risk factors for nosocomial imipenem-resistant Acinetobacter baumannii (IRAB) infections. METHODS: A prospective case-control study, set in an 1100-bed referral and tertiary-care hospital, of all patients who had nosocomial A. baumannii infections between January 1 and December 31, 2004. Only the first isolation of A. baumannii was considered. RESULTS: IRAB was isolated from 66 (53.7%) patients and imipenem-sensitive Acinetobacter baumannii (ISAB) was isolated from 57 (46.3%) patients during the study period. The mean duration of hospital stay until A. baumannii isolation was 20.8+/-13.6 days in IRAB infections, whereas it was 15.4+/-9.4 days in ISAB infections. Of the patients, 65.2% with IRAB infections and 40.4% with ISAB infections were followed at the intensive care unit (ICU). Previous carbapenem use was present in 43.9% of the patients with IRAB and 12.3% of the patients with ISAB infection. In univariate analysis female sex, longer duration of hospital stay until infection, ICU stay, emergent surgical operation, total parenteral nutrition, having a central venous catheter, endotracheal tube, urinary catheter or nasogastric tube, previous antibiotic use, and previous administration of carbapenems were significant risk factors for IRAB infections (p<0.05). In multivariate analysis, longer duration of hospital stay until A. baumannii isolation (odds ratio (OR) 1.043; 95% confidence interval (CI) 1.003-1.084; p=0.032), previous antibiotic use (OR 5.051; 95% CI 1.004-25.396; p=0.049), and ICU stay (OR 3.100; 95% CI 1.398-6.873; p=0.005) were independently associated with imipenem resistance. CONCLUSIONS: Our results suggest that the nosocomial occurrence of IRAB is strongly related to an ICU stay and duration of hospital stay, and that IRAB occurrence may be favored by the selection pressure of previously used antibiotics.

Clinical and laboratory features of Crimean-Congo hemorrhagic fever: predictors of fatality.

OBJECTIVE: To determine the predictors of fatality among patients with Crimean-Congo hemorrhagic fever (CCHF) based on epidemiological, clinical, and laboratory findings. METHODS: Among the patients with possible CCHF who were referred to Ankara Numune Education and Research Hospital (ANERH) from the surrounding hospitals between 2003 and 2006, those with IgM antibodies and/or reverse transcriptase-polymerase chain reaction (RT-PCR) results positive for CCHF virus in their blood, and who had received only supportive treatment, were included in the study. RESULTS: Sixty-nine patients with CCHF were admitted to ANERH from various cities of the northeastern part of the central region and southern parts of the Black Sea region of Turkey. Eleven (15.9%) patients died. Age, gender, days from the appearance of symptoms to admission, and initial complaints except bleeding were similar between fatal and non-fatal cases (p>0.05). Among the clinical findings, ecchymosis (p=0.007), hematemesis (p=0.030), melena (p<0.001), somnolence (p<0.001), and gingival bleeding (p=0.044) were more common among fatal cases. The mean platelet count was 47.569 x 10(9)/l in non-fatal cases and 12.636 x 10(9)/l in fatal cases (p=0.003). Among the fatal cases, the mean prothrombin time (PT; 18.4s vs. 13.4s; p<0.001) and the mean activated partial thromboplastin time (aPTT; 69.4s vs. 42.7s; p=0.001) were longer, and the mean alanine aminotransferase (ALT; 1688 vs. 293; p<0.001), mean aspartate aminotransferase (AST; 3028 vs. 634; p<0.001), mean lactate dehydrogenase (LDH; 4245 vs. 1141; p<0.001), mean creatine phosphokinase (CPK; 3016 vs. 851; p=0.004) levels and the mean international normalized ratio (INR; 1.38 vs. 1.1; p<0.001) were higher. In a Cox proportional hazards model, thrombocytopenia of < or = 20 x 10(9)/l (hazard rate (HR) 9.67; 95% confidence interval (CI) 1.16-80.68; p=0.036), a prolonged aPTT > or = 60s (HR 11.62; 95% CI 2.40-56.27; p=0.002), existence of melena (HR 6.39; 95% CI 1.64-24.93; p=0.008), and somnolence (HR 6.30; 95% CI 1.80-22.09; p=0.004) were independently associated with mortality. CONCLUSIONS: Thrombocytopenia of < or = 20 x 10(9)/l, a prolonged aPTT > or = 60s, the existence of melena, and somnolence were independent predictors of fatality.