Genomic Spectrum and Phenotypic Heterogeneity of Human IL-21 Receptor Deficiency.

Biallelic inactivating mutations in IL21R causes a combined immunodeficiency that is often complicated by cryptosporidium infections. While eight IL-21R-deficient patients have been reported previously, the natural course, immune characteristics of disease, and response to hematopoietic stem cell transplantation (HSCT) remain to be comprehensively examined. In our study, we have collected clinical histories of 13 patients with IL-21R deficiency from eight families across seven centers worldwide, including five novel patients identified by exome or NGS panel sequencing. Eight unique mutations in IL21R were identified in these patients, including two novel mutations. Median age at disease onset was 2.5 years (0.5-7 years). The main clinical manifestations were recurrent bacterial (84.6%), fungal (46.2%), and viral (38.5%) infections; cryptosporidiosis-associated cholangitis (46.2%); and asthma (23.1%). Inflammatory skin diseases (15.3%) and recurrent anaphylaxis (7.9%) constitute novel phenotypes of this combined immunodeficiency. Most patients exhibited hypogammaglobulinemia and reduced proportions of memory B cells, circulating T follicular helper cells, MAIT cells and terminally differentiated NK cells. However, IgE levels were elevated in 50% of IL-21R-deficient patients. Overall survival following HSCT (6 patients, mean follow-up 1.8 year) was 33.3%, with pre-existing organ damage constituting a negative prognostic factor. Mortality of non-transplanted patients (n = 7) was 57.1%. Our detailed analysis of the largest cohort of IL-21R-deficient patients to date provides in-depth clinical, immunological and immunophenotypic features of these patients, thereby establishing critical non-redundant functions of IL-21/IL-21R signaling in lymphocyte differentiation, humoral immunity and host defense against infection, and mechanisms of disease pathogenesis due to IL-21R deficiency. Outcome following HSCT depends on prior chronic infections and organ damage, which should thus be considered as early as possible following molecular diagnosis.

Nailfold capillaroscopy: A sensitive method for evaluating microvascular involvement in children with SARS-CoV-2 infection.

OBJECTIVES: The hyperinflammatory state and the viral invasion may result in endothelial dysfunction in SARS-CoV-2 infection. Although a method foreseeing microvascular dysfunction has not been defined yet, studies conducted in patients diagnosed with COVID-19 have demonstrated the presence of endotheliitis. With this study, we aimed to investigate the microvascular circulation in patients diagnosed with COVID-19 and multisystem inflammatory syndrome in children (MIS-C) by nailfold videocapillaroscopy (NVC). METHODS: Thirty-one patients with SARS-CoV-2 infection, 25 of whom were diagnosed with COVID-19 and 6 with MIS-C and 58 healthy peers were included in the study. NVC was performed in eight fingers with 2 images per finger and 16 images were examined for the morphology of capillaries, presence of pericapillary edema, microhemorrhage, avascular area, and neoangiogenesis. Capillary length, capillary width, apical loop, arterial and venous width, and intercapillary distance were measured from three consecutive capillaries from the ring finger of the non-dominant hand. RESULTS: COVID-19 patients showed significantly more capillary ramification (p < 0.001), capillary meandering (p = 0.04), microhemorrhage (p < 0.001), neoangiogenesis (p < 0.001), capillary tortuosity (p = 0.003). Capillary density (p = 0.002) and capillary length (p = 0.002) were significantly lower in the patient group while intercapillary distance (p = 0.01) was significantly longer compared with healthy volunteers. Morphologically, patients with MIS-C had a higher frequency of capillary ramification and neoangiogenesis compared with COVID-19 patients (p = 0.04). CONCLUSION: Abnormal capillary alterations seen in COVID-19 and MIS-C patients indicate both similar and different aspects of these two spectra of SARS-CoV-2 infection and NVC appears to be a simple and non-invasive method for evaluation of microvascular involvement.

Evaluation of respiratory syncytial virus IgG antibody dynamics in mother-infant pairs cohort.

RSV is one of the most important agents of lower respiratory infections in childhood. In this study, anti-RSV antibody levels in mother-infant pairs and factors related to antibody transfer ratio were investigated. One hundred and twenty-seven women that had term babies and their babies and 84 mother-infant pairs of them who continued the study after 6 months were enrolled. Anti-RSV IgG antibodies of the mothers and infants were positive in 46.5% and 61.5%, respectively. At the sixth month, anti-RSV antibodies were negative in all infants. Median of the anti-RSV antibody levels of the mothers and infants at birth were 12.08 IU/ml (1.21-119.27) and 13.78 IU/ml (3.99-108.6), respectively. There was a significant correlation between anti-RSV antibody levels of mothers and infants at birth (p: 0.0001, r: 0.667) and anti-RSV antibody levels of infants at birth and at 6th month (p: 0.0001, r: 0.343). Median ratio of infant and mother antibody levels was 1.22 (0.14-6.05). Median ratio that was detected in appropriate for gestational age infants was significantly higher than in small for gestational age or large for gestational age infants. In this study, the significant positive correlation between maternal antibody levels and infants' antibody levels at birth suggests that maternal vaccination strategies may be logical. We showed that antibody transfer rate was highest in appropriate for gestational age infants. It should be kept in mind that maternal vaccination strategies may be less effective in small for gestational age and large for gestational age infants.

Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single-center experience.

Deficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoprotein CD40L (CD154) gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality. However, the therapeutic decision to use HSCT proves challenging in many cases, mainly due to the lack of a phenotype-genotype correlation. We retrospectively reviewed patients with CD40L deficiency who were transplanted in Antalya and Göztepe MedicalPark Pediatric HSCT units from 2014 to 2019 and followed by Akdeniz University School of Medicine Department of Pediatric Immunology. The records of eight male cases, including one set of twins, were evaluated retrospectively. As two transplants each were performed on the twins, a total of ten transplants were evaluated. Conditioning regimens were predominantly based on myeloablative protocols, except for the twins, who received a non-myeloablative regimen for their first transplantation. Median neutrophil and platelet engraftment days were 13 (range 10-19) and 14 (range 10-42) days, respectively. In seven of ten transplants, a CMV reactivation was developed without morbidity. None of the patients developed GVHD, except for one mild case of acute GVHD. All patients survived, and the median follow-up was 852 days. Our data show that HSCT for patients with CD40 ligand deficiency is a potentially effective treatment for long-term disease control.

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood.

Tuberculosis (TB), caused by Mycobacterium tuberculosis (M.tb) and a few related mycobacteria, is a devastating disease, killing more than a million individuals per year worldwide. However, its pathogenesis remains largely elusive, as only a small proportion of infected individuals develop clinical disease either during primary infection or during reactivation from latency or secondary infection. Subacute, hematogenous, and extrapulmonary disease tends to be more frequent in infants, children, and teenagers than in adults. Life-threatening primary TB of childhood can result from known acquired or inherited immunodeficiencies, although the vast majority of cases remain unexplained. We review here the conditions conferring a predisposition to childhood clinical diseases caused by mycobacteria, including not only M.tb but also weakly virulent mycobacteria, such as BCG vaccines and environmental mycobacteria. Infections with weakly virulent mycobacteria are much rarer than TB, but the inherited and acquired immunodeficiencies underlying these infections are much better known. Their study has also provided genetic and immunological insights into childhood TB, as illustrated by the discovery of single-gene inborn errors of IFN-γ immunity underlying severe cases of TB. Novel findings are expected from ongoing and future human genetic studies of childhood TB in countries that combine a high proportion of consanguineous marriages, a high incidence of TB, and an excellent clinical care, such as Iran, Morocco, and Turkey.

Acquired Immune Deficiency Syndrome in Differential Diagnosis of Hyper-IgE-Immunoglobulinemia: Pediatric Case Report.

Acquired immune deficiency syndrome can be encountered with hypereosinophilia and hyperimmunoglobulin E (hyper-IgE) values, though these levels are rarely so high to be compared with hyperimmunoglobulin E syndrome. A 9-year-old boy presented with the complaint of fatigue, weakness, weight loss and generalized pruritic rash lasting for a year. He had frequent respiratory tract infections, wheezing episodes and urticarial skin lesions before that. On admission, he was cachectic and he had generalized lymphadenopathy, hepatosplenomegaly, oral moniliasis and pruritic rash all over his body. Laboratory evaluation revealed marked lymphopenia and hypergammaglobulinemia with extremely high IgE values (IgE: 59 300 kU/l). He was diagnosed with stage 4 human immunodeficiency virus (HIV) infection and started on antiretroviral treatment. In conclusion, HIV infection can be presented with increased IgE values.

Influenza in the pediatric population in Istanbul: a one center experience 2009-2014.

INTRODUCTION: The aim of the study was to evaluate the clinical characteristics of pediatric patients with influenza infection. MATERIAL AND METHODS: The patients hospitalized with confirmed influenza between October 2009 and May 2014 were enrolled in this study. RESULTS: The mean age of the patients was 66 ± 53 months (1-204 months). Fifty-four percent of patients had a chronic underlying disease. Twenty-four patients needed mechanical ventilation support and a two-month-old patient with liver disease died. Except for the 2009-2010 season, all patients who received mechanical ventilation had underlying disease. The hospital admission months were December-February in 2010-2011 and January-March in 2011-2012 as well as in 2012-2013. Convulsion was observed frequently in influenza A cases, and influenza B tended to be detected in older patients (p = 0.024). The most common symptoms in pediatric patients were fever and cough. CONCLUSION: It is obvious that to protect against circulating influenza viruses, the risk-based strategy of annual influenza immunization should target school-aged children and children with underlying conditions, especially neurological and pulmonary diseases.

Atypical presentation of human bocavirus: Severe respiratory tract infection complicated with encephalopathy.

Human bocavirus (HBOV) has been reported as a worldwide distributed respiratory pathogen. It has also been associated with encephalitis recently by detection of the virus in cerebrospinal fluid (CSF) of patients presented with encephalitis. This retrospective study aimed to present clinical features of HBOV infections in children with respiratory symptoms and describe unexplained encephalopathy in a subgroup of these patients. Results of 1,143 pediatric nasal samples from mid-December 2013 to July 2014 were reviewed for detection of HBOV. A multiplex real time polymerase chain reaction assay was used for viral detection. Medical records of the patients were retrospectively analyzed. HBOV was detected in 30 patients (2.6%). Median age was 14 months (5-80). Clinical diagnoses were upper respiratory tract infection (n = 10), bronchopneumonia (n = 9), acute bronchiolitis (n = 5), pneumonia (n = 4), acute bronchitis (n = 1), and asthma execarbation (n = 1). Hospitalization was required in 16 (53.3%) patients and 10 (62.5%) of them admitted to pediatric intensive care unit (PICU). Noninvasive mechanical ventilation modalities was applied to four patients and mechanical ventilation to four patients. Intractable seizures developed in four patients while mechanically ventilated on the 2nd-3rd days of PICU admission. No specific reason for encephalopathy was found after a thorough investigation. No mortality was observed, but two patients were discharged with neurological sequela. HBOV may lead to respiratory infections in a wide spectrum of severity. This report indicates its potential to cause severe respiratory infections requiring PICU admission and highlights possible clinical association of HBOV and encephalopathy, which developed during severe respiratory infection.

Brucella infection in a child with progressive familial intrahepatic cholestasis type 2 who had undergone liver transplantation.

Brucellosis is considered the most widespread zoonosis in the world. In endemic regions of brucellosis, childhood brucellosis includes up to one-third of all cases of human brucellosis. Brucellosis constitutes a public health problem in Turkey. A boy aged 12 yr who had PFIC2 had undergone deceased-donor liver transplantation in 2008 at the age of seven. The boy presented with fatigue, fever, and pain in the right leg and hip and was admitted to the hospital. Brucella melitensis grew in the blood culture, and the SAT was positive at a titer of 1:640. The patient was treated with oral doxycycline and rifampicin for eight wk. After treatment, the patient recovered and his blood cultures became negative. The patient's mother also had a high Brucella agglutination titer of 1:320 positive and was treated in the internal medicine department with spiramycin and doxycycline. Brucella infection should be suspected in liver transplant recipients with fever of unknown origin, especially in recipients who live in an endemic area.

Comparison of tuberculin skin test and QuantiFERON®-TB Gold In-Tube for the diagnosis of childhood tuberculosis.

BACKGROUND: Tuberculosis (TB) is an important worldwide ongoing health issue. To be able to control TB, one should not only cure active TB but also identify childhood TB patients who have the possibility to develop active disease in the future. The aim of this study was to compare a century-old tuberculin skin test (TST) and QuantiFERON-TB Gold In-Tube (QFT-GIT) test, developed as an alternative to TST and which has been claimed to be superior to TST in several ways, in the diagnosis of childhood TB. METHODS: Fifty-three children with TB between 5 months and 17.5 years of age and 92 healthy children from the same age group with no risk factors for TB were recruited into the study. All children underwent TST and QFT-GIT test and their demographic, clinic and laboratory data were recorded. Data were analyzed using SPSS 14. RESULTS: A total of 53 patients were diagnosed with TB. Mean patient age was 8.5 ± 4.3 years (range, 5 months-17.5 years). A total of 41.7% of the patients were female. Sixteen of 53 patients had confirmation on culture. QFT-GIT test was positive in 16 and TST was positive in 15 among 16 children with culture-confirmed TB. The sensitivity of TST and QFT-GIT were 93.8% and 100.0%, and the specificity of TST and QFT-GIT were 100.0% and 97.8%, respectively. With regard to the 53 TB children including those without bacteriological confirmation, QFT-GIT was positive in 33 children, and TST was positive in 44 children. The sensitivity of TST and QFT-GIT was then 83.0% and 62.3%, and the specificity, 100.0% and 97.8%, respectively. CONCLUSION: Although positive QFT-GIT test is very significant for TB, negative results will not exclude TB infection. TST and QFT-GIT used together may provide more efficient results.

Factors Affecting the Implementation and Acceptance of the Cocoon Strategy in the NICU in a Tertiary Center in Türkiye.

Pertussis is an important cause of mortality and morbidity in infancy. It is recommended that close contacts of the baby be vaccinated with Tdap, and this practice is called the cocoon strategy. This study aimed to investigate the applicability of the cocoon strategy and to determine the factors affecting the process. Mothers of babies who were hospitalized in the neonatal intensive care unit were included in the study. In the first stage, a face-to-face questionnaire was given to the mothers to measure their level of knowledge about whooping cough and its vaccine. In the second stage, written and verbal information about the cocoon strategy was given, and then vaccination intentions for Tdap were learned. In the third stage, all mothers were contacted 3 weeks after and asked whether they had received a Tdap vaccination and why. Of these mothers, 68% could not answer any questions about pertussis disease and vaccines correctly. After the information, 35% (n = 78) of the mothers stated that they were considering getting vaccinated, while only 2% (n = 5) of the mothers were able to get the Tdap vaccine. The most important reasons for not getting vaccinated were a lack of time (24%) and the cost of vaccination (23%). It is predicted that Tdap vaccination rates may increase if the cost of vaccine, availability of vaccine, and the access of mothers to the vaccine application are facilitated.

Clinical and epidemiological characteristics of pandemic influenza A/(H1N1) in hospitalized pediatric patients at a university hospital, Istanbul, Turkey.

BACKGROUND: The aim of this study was to describe the clinical and epidemiological characteristics of pandemic influenza in hospitalized children. METHODS: A total of 114 patients with suspected H1N1 virus infection were hospitalized, and nasal swabs were sent to National Influenza Reference Laboratory for confirmation of pandemic influenza A (H1N1) virus infection by rRT-PCR assay. RESULTS: Forty-six female and 68 male patients were included in the study. Age of the patients ranged from 40 days to 16 years. Clinical and/or radiological pneumonia were detected in 96% of all. Sixteen patients required mechanical ventilation due to hypoxemia. Previously healthy children required mechanical ventilation and oxygen therapy more than patients with chronic diseases. Elevated levels of CRP and LDH in patients with respiratory distress and patients who required mechanical ventilation were statistically significant. CONCLUSION: Our study showed that progress of pandemic influenza infection in previously healthy children is as severe as their counterparts with chronic underlying diseases.

Progressive Multifocal Leukoencephalopathy in Children with Primary and Secondary Immune Deficiency.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system that is caused by John Cunningham virus (JCV). It occurs almost exclusively in immunosuppressed individuals, for example, patients with AIDS and hematological and lymphoreticular malignancies. In this article, we present a review of the literature and 2 case reports with PML. The first report examines a 15-year-old male (who presented with dedicator of cytokinesis 8 deficiency) who was diagnosed as having PML based on characteristic magnetic resonance imaging (MRI) lesions and a positive PCR for JCV in cerebrospinal fluid. He was transferred for bone marrow transplantation after stabilization with therapy of maraviroc and cidofovir. The second report examines a 6-year-old male who presented with encephalitis and was also diagnosed with AIDS. He was diagnosed with PML and started treatment with cidofovir. His clinical status and MRI findings deteriorated rapidly. In immunosuppressive patients who developed encephalopathy, JCV ought to be considered.

LRBA deficiency: a rare cause of type 1 diabetes, colitis, and severe immunodeficiency.

The biological role of the lipopolysaccharide-responsive beige-like anchor (LRBA) protein associated with the immune system is not to date well known. However, it is thought to regulate the CTLA4 protein, an inhibitory immunoreceptor. Chronic diarrhea, autoimmune disorders, organomegaly, frequent recurrent infections, hypogammaglobulinemia, chronic lung manifestations, and growth retardation are some features of LRBA deficiency. This rare disease is observed as a result of homozygous mutations in the LRBA gene. An 11.3-year-old male patient presented because of short stature and high blood glucose level. He had a previous history of lymphoproliferative disease, chronic diarrhea, and recurrent infections. His parents were first-degree consanguineous relatives. A diagnosis of type 1 diabetes mellitus (T1DM) was added to the preexisting diagnoses of immunodeficiency, recurrent infection, enteropathy, chronic diarrhea, lymphadenopathy, hepatomegaly, and short stature. Genetic analysis revealed a homozygous mutation in the LRBA gene, c.5047C>T (p.R1683*) (p.Arg1683*). Abatacept treatment was started: the patient's hospital admission frequency decreased, and glucose regulation improved. At follow-up, growth hormone (GH) deficiency was diagnosed, although it was not treated because the underlying disease was not under control. Nevertheless, the patient's height improved with abatacept treatment. LRBA deficiency should be considered in the presence of consanguineous marriage, diabetes, immunodeficiency, and additional autoimmune symptoms. LRBA phenotypes are variable even when the same variants in the LRBA gene are present. Genetic diagnosis is important to determine optimal treatment options. In addition to chronic malnutrition and immunosuppressive therapy, GH deficiency may be one of the causes of short stature in these patients.

Multicenter prospective surveillance study of viral agents causing meningoencephalitis.

The frequency of bacterial factors causing central nervous system infections has decreased as a result of the development of our national immunization program. In this study, it is aimed to obtain the data of our local surveillance by defining the viral etiology in cases diagnosed with meningoencephalitis for 1 year. Previously healhty 186 children, who applied with findings suggesting viral meningoencephalitis to 8 different tertiary health centers between August 2018 and August 2019, in Istanbul, were included. The cerebrospinal fluid (CSF) sample was evaluated by polymerase chain reaction. The M:F ratio was 1.24 in the patient group, whose age ranged from 1 to 216 months (mean 40.2 ± 48.7). Viral factor was detected in 26.8%. Enterovirus was the most common agent (24%) and followed by Adenovirus (22%) and HHV type 6 (22%). In the rest of the samples revealed HHV type 7 (10%), EBV (6%), CMV (6%), HSV type 1 (6%), Parvovirus (4%) and VZV (2%). The most common symptoms were fever (79%) and convulsions (45.7%). Antibiotherapy and antiviral therapy was started 48.6% and 4% respectively. Mortality and sequela rate resulted 0.53% and 3.7%, respectively. This highlights the importance of monitoring trends in encephalitis in Turkey with aview to improving pathogen diagnosis for encephalitis and rapidly identifying novel emerging encephalitis-causing pathogens that demand public health action especially in national immunisation programme.

SARS-CoV-2 seropositivity among pediatric health care personnel after the first peak of the pandemic: nationwide surveillance in Turkey.

BACKGROUND: Understanding SARS-CoV-2 seroprevalence among health care personnel is important to explore risk factors for transmission, develop elimination strategies and form a view on the necessity and frequency of surveillance in the future. METHODS: We enrolled 4927 health care personnel working in pediatric units at 32 hospitals from 7 different regions of Turkey in a study to determine SARS Co-V-2 seroprevalence after the first peak of the COVID-19 pandemic. A point of care serologic lateral flow rapid test kit for immunoglobulin (Ig)M/IgG was used. Seroprevalence and its association with demographic characteristics and possible risk factors were analyzed. RESULTS: SARS-CoV-2 seropositivity prevalence in health care personnel tested was 6.1%. Seropositivity was more common among those who did not universally wear protective masks (10.6% vs 6.1%). Having a COVID-19-positive co-worker increased the likelihood of infection. The least and the most experienced personnel were more likely to be infected. Most of the seropositive health care personnel (68.0%) did not suspect that they had previously had COVID-19. CONCLUSIONS: Health surveillance for health care personnel involving routine point-of-care nucleic acid testing and monitoring personal protective equipment adherence are suggested as important strategies to protect health care personnel from COVID-19 and reduce nosocomial SARS-CoV-2 transmission.

Evaluation of cases of pediatric extrapulmonary tuberculosis: a single center experience.

AIM: Extrapulmonary tuberculosis is observed more frequently and leads to complications with a higher rate in children compared with adults because the risk of lymphohematogen spread is higher. In this study, the clinical, laboratory, and radiologic findings and treatment outcomes were evaluated in pediatric patients who were followed up in our clinic with a diagnosis of extrapulmonary tuberculosis. MATERIAL AND METHODS: Seventy patients aged 0-18 years who were followed up with a diagnosis of extrapulmonary tuberculosis between 2008 and 2017 in the Division of Pediatric Infectious Diseases in our hospital were examined retrospectively. RESULTS: The median age of the patients was 8,8 (range, 0,4-17) years and 47.1% were female (n=33). Twenty-seven patients (38.6%) were aged 0-4 years, 15 (21.4%) were aged 5-9 years, and 28 patients (40%) were aged 10-18 years. Forty-four patients (62.9%) were diagnosed as having extrapulmonary tuberculosis and 26 (37.1%) had pulmonary + extrapulmonary tuberculosis. The most common form of extrapulmonary tuberculosis was extrathoracic lymphadenopathy, which was found in 22 patients (31.4%). The other patients were diagnosed as having musculoskeletal system tuberculosis (n=10, 14.3%), gastrointestinal system tuberculosis (n=9, 12.9%), miliary tuberculosis (n=8, 11.4%), intrathoracic lymphadenopathy (n=7, 10%), renal tuberculosis (n=6, 8.6%), central nervous system tuberculosis (n=5, 7.1%), and pleural tuberculosis (n=3, 4.3%). Among a total of 58 patients in whom tuberculin skin test and interferon gamma release tests were studied together, tuberculin skin test positivity (n=37, 63.8%) was found with a higher rate compared with interferon gamma release test positivity (n=32, 55.2%), but the difference was not statistically significant (p=0.35). The median treatment period was 12 (range, 6-24) months. Among the patients whose treatments were terminated, improvement was observed in 52 patients (74.2%) and the development of sequela was observed in six patients (8.5%). Two patients who were diagnosed as having central nervous system tuberculosis (2.8%) died. CONCLUSION: Clinical, laboratory, and radiologic data should be evaluated together when making a diagnosis of extrapulmonary tuberculosis in children. Interferon gamma release tests alone are not superior to tuberculin skin test, but should be considered to be used in combination in the diagnosis.

AMAÇ: Çocuklarda, lenfohematojen yayilim riskinin yüksek olmasi nedeniyle akciger disi tüberküloz eriskinlere göre daha sik gözlenmekte ve daha fazla komplikasyona yol açmaktadir. Bu çalismada, klinigimizde akciger disi tüberküloz tanisi ile izledigimiz çocuk olgularin klinik, laboratuvar ve radyolojik bulgulari ile tedavi sonuçlari degerlendirilmistir. GEREÇ VE YÖNTEMLER: Hastanemiz Çocuk Enfeksiyon Hastaliklari Bilim Dali'nda 2008­2017 yillari arasinda akciger disi tüberküloz ile izlenen 0­18 yas arasi 70 olgu geriye dönük olarak incelendi. BULGULAR: Yas ortancasi 8,8 (0,4­17) yil olan olgularin %47,1'i kiz (n=33) idi. Sifir­4 yas arasi 27 olgu (%38,6), 5­9 yas arasi 15 olgu (%21,4), 10­18 yas arasi 28 olgu (%40) vardi. Kirk dört olgu (%62,9) akciger disi tüberküloz, 26 olgu (%37,1) akciger+akciger disi tüberküloz tanisi almisti. Yirmi iki olguda (%31,4) en sik toraks disi lenfadenopati saptandi. Diger olgular siklik sirasina göre; kas-iskelet sistemi (n=10, %14,3), gastrointestinal sistem (n=9, %12,9), miliyer (n=8, %11,4), toraks içi lenfadenopati (n=7, %10), renal (n=6, %8,6), merkezi sinir sistemi- (n=5, %7.1) ve plevra tüberkülozu (n=3,%4.3) tanilari almisti. Tüberkülin deri testi ve interferon gamma salinim testi birlikte çalisilan toplam 58 olguda, tüberkülin deri testi pozitifligi (n=37, %63,8) interferon gamma salinim testi pozitifligine göre (n=32, %55,2) daha fazlaydi, ancak istatiksel olarak anlamli bulunmadi (p=0,35). Olgularin ortanca tedavi süresi 12 (6­24) ay idi. Tedavisi sonlandirilan olgularin 52'sinde (%74,2) iyilesme, altisinda (%8,5) sekel gelisimi izlendi. Merkezi sinir sistemi tüberkülozu tanili iki olgu (%2,8) yasamini kaybetti. ÇIKARIMLAR: Çocuklarda akciger disi tüberküloz tanisi konulurken klinik, laboratuvar, radyolojik veriler birlikte degerlendirilmelidir. Interferon gamma salinim testleri tek basina tüberkülin deri testine üstün olmayip, tanida birlikte kullanilmasi düsünülmelidir.

Resistant gram-negative infections in a pediatric intensive care unit: a retrospective study in a tertiary care center.

AIM: Healthcare-associated infections cause increased morbidity and mortality in intensive care units. In this study, it was aimed to compare infections with multi-drug resistance and extended drug resistance, while evaluating the characteristics of resistant Gram-negative infections in the pediatric intensive care unit in our university hospital. MATERIAL AND METHODS: In this study, pediatric patients who were found to have Gram-negative infections during hsopitalization in the pediatric intensive care unit in our faculty between January 2011 and December 2015, were evaluated retrospectively. RESULTS: One thousand thirty patients were internalized in our unit in the study period. The incidence for healthcare-associated infection was found as 17.2% and the incidence density was found as 32.7 per 1000 patient days. The incidence for healthcare-related infection per 1000 device days and the rate for device use were calculated as 66.9 and 0.59, respectively. One hundred thirty Gram-negative infection episodes were found in 79 patients whose median age was 22 (1-205) months. The most common infections included ventilator-related pneumonia (n=78, 60%) and bloodstream infections (n=38, 29.2%). The most common causative agents included Pseudomonas aeruginosa (n=50, 38.5%), Kleibsiella pneumonia (n=32, 24.6%) and Acinetobacter baumannii (n=28, 21.5%). Among A. baumannii isolates, the rates for resistance against piperacillin-tazobactam and meropenem were found as 96.4% and 89.3%, respectively. Empirical use of carbapenems, aminoglycosides, and fluoroquinolones, the presence of total parenteral nutrition and history of Gram-negative bacterial infections prior to pediatric intensive care unit admission were significantly more common among extended-drug Gram-negative bacterial infections. The late mortality rate was found to be higher in presence of extended drug resistance. History of Gram-negative infection was found to be an independent risk factor in terms of extended drug resistance. CONCLUSION: Healthcare-associated infections are an important health problem and it is important for infection control committees of hospitals to determine and apply strategies according to hospital colonization in prevention.

AMAÇ: Saglik bakimi iliskili enfeksiyonlar yogun bakim birimlerinde yüksek hastalik ve ölüme neden olmaktadir. Bu çalismada, üniversite hastanemiz çocuk yogun bakim birimindeki dirençli gram negatif enfeksiyonlarin özellikleri degerlendirilirken; çok ilaca dirençli ve genisletilmis ilaç direnci olan enfeksiyonlarin karsilastirilmasi amaçlanmistir. GEREÇ VE YÖNTEMLER: Bu çalismada Ocak 2011­Aralik 2015 yillari arasinda, fakültemiz çocuk yogun bakim biriminde yatisi sirasinda gram negatif enfeksiyon saptanan çocuk hastalar geriye dönük olarak degerlendirildi. BULGULAR: Çalisma döneminde birimimize 1 030 hasta yatirildi; saglik bakimi iliskili enfeksiyon sikligi %17,2, siklik yogunlugu 1 000 hasta günü basina 32,7 idi. 1 000 cihaz günü basina saglik bakimi iliskili enfeksiyon sikligi ve cihaz kullanim orani sirasiyla 66,9 ve 0,59 olarak hesaplandi. Yas ortancasi 22 (1­205) ay olan 79 hastada, 130 gram negatif enfeksiyon atagi saptandi. En sik saptanan enfeksiyonlar, ventilatör iliskili pnömoni (n=78, %60) ve kan akim enfeksiyonu (n=38, %29,2) idi. En sik etkenler Pseudomonas aeruginosa (n=50, %38,5), Kleibsiella pneumonia (n=32, %24,6) ve Acinetobacter baumannii (n=28, %21,5) idi. A. baumannii izolatlari arasinda piperasilin-tazobaktam ve meropenem direnci sirasi ile %96,4 ve %89,3 saptandi. Ampirik karbapenem, aminoglikozid ve florokinolon kullanimi, total parenteral nütrisyon varligi, yogun bakim öncesi geçirilmis gram negatif enfeksiyon öyküsü anlamli olarak daha yüksekti. Geç dönem ölüm hizi genisletilmis ilaç direnci varliginda daha yüksek saptandi. Geçirilmis gram negatif enfeksiyon öyküsü, genisletilmis ilaç direnci açisindan bagimsiz risk etmeni olarak bulundu. ÇIKARIMLAR: Saglik bakimi iliskili enfeksiyonlar önemli bir saglik sorunudur ve önlemede mümkünse her hastanenin kendi enfeksiyon kontrol kurulunun hastane kolonizasyonuna göre stratejiler belirleyip uygulamasi önemlidir.

Evaluation of Short-Term Neurological Outcomes in Children with Brain Abscesses.

AIM: To evaluate the neurological outcomes of children diagnosed with brain abscesses in the early post-treatment period. < b > MATERIAL and METHODS: This study was a retrospective analysis of pediatric brain abscess patients between January 2000 and December 2015, during a 16-years period. Patients were divided into two groups according to their outcome at the end of the treatment. The patients with "good outcome" were the ones without any neurological sequelae [Glasgow Outcome Scale (GOS) score 5]. "Unfavorable outcome" was defined as having any kind of neurological deficit (GOS score 1-4). RESULTS: A total number of 31 patients (22 male, 71%) with the median age at diagnosis of 84 months (range, 1-202 months) were enrolled in this study. The most common presenting symptom was fever being encountered in 71% of the patients (n=22), followed by focal neurological deficit (FND)(n=17, 54.8%), vomiting (n=14, 45.2%), headache (n=13, 41.9%), seizure (n=13, 41.9%), change in mental status (n=12, 38.7%) and visual disturbance (n=2, 6.5%). Twenty-four patients (77.4%) had predisposing factors. The most common pathogens were gram-positive cocci (n=9, 29%). Seventeen patients (54.8%) had unfavorable outcome; 2 patients (6.4%) died. All patients were treated with parenteral antibiotherapy with median duration of 73 days (range, 28-540 days). Surgical procedures were performed in 83.9% (n=26) of patients [isolated aspiration (n=19, 61.3%), only resection (n=5, 16.1%), aspiration and resection (n=2, 6.5%)]. CONCLUSION: Glasgow coma scale score below 12 and the presence of FND on admission were found to be independent risk factors for unfavourable neurological outcome in children with brain abscesses.

Clinical differences of influenza subspecies among hospitalized children.

AIM: Clinical findings, mortality, and morbidity rates differ among influenza subspecies. Awareness of these differences will lead physicians to choose the proper diagnostic and therapeutic strategies and to foresee possible complications. The aim of this study was to evaluate the clinical differences of influenza subspecies among hospitalized children. MATERIAL AND METHODS: Hospitalized children with proven influenza infection by polymerase chain reaction on nasopharyngeal swab specimens in our clinic, between December 2013 and March 2016, were enrolled. These children were divided into 3 groups as Influenza A/H1N1 (n=42), Influenza A/H3N2 (n=23), and Influenza B (n=35). RESULTS: The median age of the children was 51.5 months (range, 3-204 months). The most common presenting symptoms were fever (n=83), cough (n=58), and difficulty in breathing (n=25). The most common non-respiratory findings were lymphadenopathy (n=18) and gastrointestinal system involvement (n=17). Sixty-two percent of the patients (n=62) had chronic diseases. H1N1 and H3N2 were significantly more common among patients with chronic neurologic disorders and renal failure, respectively. Leukopenia (n=32) and thrombocytopenia (n=22) were the most common pathologic laboratory findings. Neutropenia, elevated CRP levels, and antibiotic use were significantly more common among patients with H1N1 infection. Seven patients were transferred to the intensive care unit with diagnoses of acute respiratory distress syndrome (n=4), encephalitis (n=2), and bronchiolitis (n=1). Two patients with chronic diseases and H1N1 infection died secondary to acute respiratory distress syndrome. CONCLUSIONS: Influenza A/H1N1 infection represented more severe clinical disease.