Neurocognitive Impairment in Patients With Ataxia Telangiectasia and Their Unaffected Parents: Is It Similar?

BACKGROUND: Ataxia telangiectasia (AT) is a genetic multisystemic disorder affecting the nervous system. Data on neurocognitive functioning in AT are limited and focused on patients at various stages of disease. Because of the genetic nature of the disorder, parents of patients may also display subtle neurological problems. This study aimed to evaluate neurocognitive functioning in patients with AT and their unaffected parents. METHODS: The study included 26 patients with AT and 41 parents among which 13 patients and 18 parents were evaluated with neurocognitive tests. Clinical and radiological data were reviewed retrospectively. Data were analyzed with descriptive statistics. RESULTS: The median ages of patients and parents were 12.5 years (interquartile range [IQR] = 9.5) and 38.0 years (IQR = 12.0), respectively. Median intelligence quotients were 62.0 (IQR = 21.3) and 82.5 (IQR = 16.8), respectively, for patients and parents. Rates of intellectual disability for patients and parents were 100.0% and 83.3%, respectively. Areas of impairment in patients in decreasing order of frequency were motor skills, visual perception/memory, visual-manual coordination, spontaneous/focused and sustained attention (100.0% for each), social judgment, as well as vocabulary and arithmetic skills (75.0% for each). Areas of impairment in unaffected parents in decreasing order of frequency were visual-manual coordination (77.8%), working memory (76.5%), and visual perception and motor skills (66.7% for each). CONCLUSION: Intellectual disabilities, visual-spatial disabilities, and reduced visual-motor coordination seem to be similar in patients with AT and their parents. These results should be replicated with larger samples from multiple centers and may form putative cognitive endophenotypes for the disorder.

Emerging importance of multidrug-resistant Stenotrophomonas maltophilia infections in neonatal intensive care unit in a tertiary center in Turkey.

OBJECTIVE: Stenotrophomonas maltophilia is an emerging multi-drug resistant, opportunistic pathogen in the neonatal intensive care unit (NICU). In this study, we aimed to assess the incidence, clinical features, antibiotic susceptibility, and treatment options of S. maltophilia infection among the healthcare-associated infections (HAIs) in the neonatal unit. METHODS: In this study, the patients who were hospitalized in the NICU between January 2020 and December 2021 with S. maltophilia isolated from clinical samples were included. Demographic, clinic features, and microbiological findings of the patients were retrospectively evaluated by using the medical records. The samples (lower respiratory tract, urine, peritoneal fluid) were first examined microscopically by gram preparation and cultured. Antibiotic susceptibility tests were performed according to the recommendations of The European Committee on Antimicrobial Susceptibility Testing (EUCAST) for TMP-SMX. RESULTS: S. maltophilia was isolated in 38 clinical samples of the 20 patients who were hospitalized at the NICU between January 2020 and December 2021. A total of 40 % (n = 8) of samples from different patients were accepted as colonization. Ventilator-associated pneumonia was determined in 55 % (n = 11), and urinary tract infection in 5 % (n = 1). S. maltophilia-associated bacteremia was not detected in any of the cases. The TMP-SMX susceptibilities of the strains were as it follows: 3 (15 %) were resistant (R), 7 (28 %) were susceptible (S), and 10 (47 %) were susceptible-increased exposure (I). Three of these patients were given dual antibiotics therapy (levofloxacin plus TMP-SMX) and nine of them were given only TMP-SMX. The most common hospital-acquired infectious agents are Gram negative microorganisms (51 %), followed by coagulase negative staphylococci (CNS), Staphylococcus aureus (24 %) and S. maltophilia (24 %). CONCLUSION: Increasing TMP-SMX resistance and specific drug and dosage-related problems in the neonatal unit are important problems in treatment management.

Antibiotic associated diarrhea in outpatient pediatric antibiotic therapy.

BACKGROUND: Antibiotic-associated diarrhea is one of the most frequent side effects of antimicrobial therapy. We assessed the epidemiological data of antibiotic-associated diarrhea in pediatric patients in our region. METHODS: The prospective multi-center study included pediatric patients who were initiated an oral antibiotic course in outpatient clinics and followed in a well-established surveillance system. This follow-up system constituded inclusion of patient by the primary physician, supply of family follow-up charts to the family, passing the demographics and clinical information of patient to the Primary Investigator Centre, and a close telephone follow-up of patients for a period of eight weeks by the Primary Investigator Centre. RESULTS: A result of 758 cases were recruited in the analysis which had a frequency of 10.4% antibiotic-associated diarrhea. Among the cases treated with amoxicillin-clavulanate 10.4%, and cephalosporins 14.4% presented with antibiotic-associated diarrhea. In the analysis of antibiotic-associated diarrhea occurrence according to different geographical regions of Turkey, antibiotic-associated diarrhea episodes differed significantly (p = 0.014), particularly higher in The Eastern Anatolia and Southeastern Anatolia. Though most commonly encountered with cephalosporin use, antibiotic-associated diarrhea is not a frequent side effect. CONCLUSION: This study on pediatric antibiotic-associated diarrhea displayed epidemiological data and the differences geographically in our region.

[Long COVID in Children: A Pediatric Center Experience]. / COVID-19 Nedeniyle Hastanede Yatan Çocuk Hastalarda Uzun Dönem Etkilerin ve Uzamis COVID Sikliginin Degerlendirilmesi.

Although COVID-19 (Coronavirus disease-2019) is observed to be milder in children, it has been observed that the symptoms continue for a long time in many people after the acute period of the disease, especially the multisystemic inflammatory syndrome (MISC) that developed after COVID-19 with the progression of the pandemic. Although it was first defined by different names such as long COVID and post COVID in adults, it has been observed in studies that similar complaints such as cough, fatigue and difficulty in concentrating continue for a long time in children, just as in adults. In our study, we aimed to evaluate the status of long COVID in childhood. Our study included patients aged from one month to 18 years with moderate and severe symptoms who were hospitalized and discharged for SARS-CoV-2 infection in Istanbul University Faculty of Medicine between November 1, 2020 and November 1, 2021. A questionnaire form was created to learn about the complaints of the patients and their ongoing complaints. The patients/parents were called by phone and their complaints were recorded in the questionnaire. The patients were classified according to the definitions in the guidelines published by NICE, RCGP and SIGN. In total, 116 patients were included; 57.8% (n= 64) male, 42.5% (n= 49) female; 53.4% (n= 62) 0-9, 46.6% (n= 54) 10-18 years old. Comorbid conditions were found in 64 (55.2%) patients. The mean follow-up period was 5.90 ± 3.61 [min-max (1-12)] months; longest symptom durations: decrease in effort loss/fatigue 19.25 ± 74.56 (0-365) days, concentration difficulties 11.12 ± 49.75 (0-365) days, fatigue 9.61 ± 34.96 (0-365) days and cough were 8.34 ± 35.37 (0-365) days. The percentage of the patients who met the definition of subacute/ongoing symptomatic COVID-19 was 37.9% (n= 44). The most common symptoms were the decrease of effort capacity/fatigue 12.1% (n= 14) and the concentration difficulties 10.3% (n= 12) in subacute symptomatic patients. The percentage of patients matching the definition of chronic/post COVID-19 was 11.2% (n= 13). In the first year of the disease, ongoing complaints such as fatigue and concentration difficulties were observed in eight patients. The rate of concentration difficulties in the 10-18 age group was statistically significantly higher than the 0-9 age group (p= 0.037). In terms of other symptoms, no significant difference was found according to age, gender and concomitant disease status. Out of these, one patient was diagnosed with Type 2 diabetes mellitus during the acute illness, and two patients were diagnosed with allergic rhinitis after COVID-19. A statistically significant difference was found in the rates of concentration disorders according to age groups with subacute/ongoing symptoms. Although only the hospitalized patients were included, fatigue and difficulty in concentration were among the most common ongoing symptoms in our study, similar to the literature, and they were seen to be more common in older children. It is important both for early diagnosis and awareness to follow up children with COVID-19 in terms of symptoms, not only in terms of prolonged symptoms but also in terms of new diseases.

The effectiveness of topical 1% lidocaine with systemic oral analgesics for ear pain with acute otitis media.

BACKGROUND: Acute otitis media (AOM) is one of the most common childhood infections. Ear pain, the main symptom of AOM, results in parents frequently seeking medical assistance for their children. The aim of this study was to compare the effectiveness of topical 1% lidocaine ear drops administered with oral analgesics with that of oral analgesics alone. METHODS: This multicenter randomized, open-labeled study was conducted at 15 centers with 184 pediatric AOM patients with bilateral ear pain (aged 1-5 years) between May 1, 2016, and June 31, 2018. All patients received oral paracetamol or ibuprofen and topical 1% lidocaine, which was administered to each ear according to the randomization list. The ear pain score was evaluated within 48 h using the Face, Legs, Activity, Cry, and Consolability (FLACC) scale, and the patients were followed up for 10 days. RESULTS: The median age was 31.8 months (min-max, 12-84.2 months). Of those patients enrolled, 22.3% received paracetamol, and 24.5% received paracetamol with lidocaine ear drops; 23.4% received ibuprofen, and 29.9% received ibuprofen with lidocaine ear drops. Lower pain scores were significantly measured at baseline and 10th minutes by a reduction 25% (RR 13.64, 95% CI 4.47-41.63, p = 0.001, RR 0.14, 95% CI 0.06-0.35, p = 0.001) and 50% (RR 4.76, 95% CI 1.63-13.87, p = 0.004, RR 0.14, 95% CI 0.05-0.4, p = 0.001) in the paracetamol and lidocaine versus paracetamol groups and the ibuprofen and lidocaine versus ibuprofen groups, respectively. No serious side effects were evident during follow-up. CONCLUSION: This randomized study suggests that topical 1% lidocaine ear drops with paracetamol or ibuprofen seems to provide effective and rapid relief for children presenting with ear pain attributed to AOM.

Multisystem inflammatory syndrome in children associated with COVID-19 in 101 cases from Turkey (Turk-MISC study).

AIM: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. METHODS: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. RESULTS: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6-9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112-228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30-84) and procalcitonin levels (86/89, median 5 µg/L; IQR 0.58-20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. CONCLUSION: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.

Correlation of laboratory parameters and Chest CT findings in young adults with COVID-19 and comparison of imaging findings with children.

PURPOSE: We aimed to compare COVID-19 imaging findings of young adults (19-35 years of age) with those of children (0-18 years) and to correlate imaging findings of young adults with their laboratory tests. MATERIALS AND METHODS: This retrospective study included Real Time-Polymerase Chain Reaction (RT-PCR) confirmed 130 young adults (mean age: 28.39 ± 4.77; 65 male, 65 female) and 36 children (mean age: 12.41 ± 4.51; 17 male, 19 female), between March and June 2020. COVID-19 related imaging findings on chest CT were examined in young adults and compared with children by the Mann-Whitney U, and Chi-square or Fisher's exact test. Laboratory examinations of young adults were assessed in terms of correlation with radiological findings by the Spearman's correlation analysis. RESULTS: Bilateral multiple distributions (p = 0.014), subpleural involvement, and pleural thickening (p = 0.004), GGOs with internal consolidations were more frequent in adults (p = 0.009). Infiltrations were significantly larger than 20 mm in young adults (p = 0.011). The rates of feeding vessel sign, vascular enlargement, and halo sign were significantly higher in young adults (p < 0.003). Highly significant positive correlations were found between radiological and biochemical parameters. CONCLUSION: Distribution, size, and pattern of COVID-19 related imaging findings differed in children and young adults. Radiological findings were correlated with biochemical parameters but not with blood count results of young adults.

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.

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.

Plasma D-dimer : a promising indicator of COVID-19 infection severity or only an acute phase reactant.

OBJECTIVE: Serum D-dimer levels, as well as other biomarkers related to coagulation, are significantly elevated during severe community acquired pneumonia. The aim of this study is to investigate the utility of plasma D-dimer levels determining the severity of inflammation and prognosis in pediatric patients with COVID-19 infection. METHODS: We retrospectively chart reviewed medical records of pediatric patients (< 18 years of age) admitted to Istanbul Fcaulty of Medicine, Department of Pediatrics Infectious Disease Service between March 11, and June 30, 2020. We collected demographic, clinical, biochemical and radiographic data. RESULTS: A hundred and seventy-one pediatric patients (1 - 216 months of age) admitted to pediatric infecitous disease service included in this study. Patients were classified into 4 categories; 1) COVID-19 infection confirmed by PCR, 2) Suspected COVID-19 infection due to close exposure history and radiographic findings, 3) Lower respiratory tract infection other than COVID-19 confirmed with multiplex respiratory viral panel, and 4) Systemic infections other than lower respiratory tract infection. Lymphopenia was observed significantly higher in patients with COVID-19 infection compared to patients with other respiratory viral infections (p=0.06). In patients with radiographic findings concerning for COVID-19 infection, elevated serum D-dimer levels were detected significantly higher than lymphopenia (p=0.07). CONCLUSIONS: Elevated serum D-dimer levels at baseline are associated with inflammation especially in patients with COVID-19 infection with radipgraphic findings. Monitoring serum D-dimer levels may be used for early identification of severe cases in children.

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.

Imaging Features of Pediatric COVID-19 on Chest Radiography and Chest CT: A Retrospective, Single-Center Study.

RATIONALE AND OBJECTIVES: This study aims to reveal the imaging features of Coronavirus Disease 2019 (COVID-19) in children. MATERIALS AND METHODS: Sixty-nine chest radiographs and 37 chest CT examinations of 74 children (36 male; median (interquartile range) age:11 (6.25-15) years, 38 female; median (interquartile range) age: 12 (5.75-16) years) with positive real-time reverse transcription-polymerase chain reaction results between March 10 and May 31, 2020, were evaluated in this retrospective study. Differences in 0-<6, 6-<12, and 12-18 years of age groups were assessed with the Fisher's exact test or Kruskal-Wallis tests. RESULTS: Right-sided (3/69, 4.3%) or bilateral (3/69, 4.3%) ground-glass opacities without significant difference in age groups were depicted as radiographic findings related to COVID-19 in children. Opacities were either single (7/37, 18.9%) or bilateral (7/37, 18.9%) around the distal third of the bronchovascular bundle on CT. There was no significant difference in the median size of the largest opacities, total numbers of opacities and involved lobes, and the distance of the closest opacity to the pleura among age groups (p > 0.05). The rate of ground-glass opacities with or without consolidation (17/37, 45.94%) was higher than consolidation alone (6/37, 16.2%). Feeding vessel sign (16/37, 43.2%), halo sign (9/37, 24.3%), pleural thickening (6/37, 16.2%), interlobular interstitial thickening (5/37, 13.5%), and lymphadenopathy (3/37, 8.1%) were other imaging findings. CONCLUSION: Unilateral or bilateral distributed ground-glass opacities often associated with feeding vessel sign, halo sign, and pleural thickening on chest CT without significant differences between age groups were findings of COVID-19 in children.

An adolescent girl with obstructive uropathy requiring nephro-ureterectomy was subsequently diagnosed with renal tuberculosis: case report.

A 15-year-old girl was followed up for 2 years in a district hospital for management of vesicoureteral reflux and, subsequently, hydronephrosis of both kidneys and required bilateral ureteroneocystostomy. Despite surgery, there was continuous progression of the left hydronephrosis. Referral to a tertiary hospital because of continued sterile pyuria prompted investigation for tuberculosis (TB): she was diagnosed with bilateral pulmonary TB and urine culture confirmed Mycobacterium tuberculosis. Despite tuberculous chemotherapy and dexamethasone, she required a left nephrectomy. Histology demonstrated necrotising granulomatous pyelonephritis. She remains well with normal function of the right kidney. Despite the rarity, chronic urinary tract disorders should always prompt investigation for tuberculosis.

Parents of ataxia-telangiectasia patients display a distinct cellular immune phenotype mimicking ATM-mutated patients.

BACKGROUND: Heterozygous relatives of ataxia-telangiectasia (AT) patients are at an increased risk for certain AT-related manifestations. We also show that there is an increase of infection frequency in parents of AT patients. Thus, we hypothesized that the parents might exhibit immune alterations similar to their affected children. METHODS: Lymphocyte phenotyping to enumerate T- and B-cell subsets was performed. Functional analyses included in vitro quantified γ-H2AX, poly (ADP-ribose) polymerase (PARP) and caspase-9 proteins. Chromosomal instability was determined by comet assay. RESULTS: We analyzed 20 AT patients (14F/6M), 31 parents (16F/15M), and 35 age-matched healthy controls. The AT patients' parents exhibited low frequency of naive CD4+ T- (n = 14, 45%) and recent thymic emigrants (n = 11, 35%) in comparison with the age-matched healthy donors. Interestingly, parents with low naive T cells also demonstrated high rate of recurrent infections (9/14, 64%). In comparison with age-matched controls, parents who had recurrent infections and low naive T cells showed significantly higher baseline γ-H2AX levels and H2 O2 -induced DNA damage as well as increased cleaved caspase-9 and PARP proteins. CONCLUSION: Parents of AT patients could present with recurrent infections and display cellular defects that mimic AT patients. The observed immunological changes could be associated with increased DNA double-strand breaks.

Investigation of human bocavirus in pediatric patients with respiratory tract infection.

INTRODUCTION: Human bocavirus (HBoV) is a linear single-stranded DNA virus belonging to the Parvoviridae family. This study aimed to investigate the incidence of HBoV and co-infections in pediatric patients with symptoms of viral respiratory tract infection. METHODOLOGY: This study included 2,310 patients between the ages of 0-18 in whom HBoV and other respiratory tract viral pathogens were analyzed in nasopharyngeal swab specimens. RESULTS: In the pediatric age group, HBoV was found in 4.5% (105/2310) of the patients and higher in children between the ages of 1 and 5. Mixed infection was detected in 43.8% (46/105) of HBoV positive patients (p = 0.10). Mono and mixed infection rates were higher in outpatients than in inpatients (p < 0.05). Respiratory syncytial virus was significantly higher than the other respiratory viral pathogens (p < 0.001). CONCLUSIONS: This study is important as it is one of the rare studies performed on the incidence of HBoV in the Marmara region. In pediatric age group, the incidence of HBoV was found 4.5%. The incidence rate of HBoV in this study was similar to those in studies around the world, but close to low rates. The incidence of HBoV was found higher especially among children between the ages of 1-5 in this study. In addition to the incidence of HBoV, accompanying co-infections in the pediatric age group were also investigated in this study. Since concurrence of RSV, HRV and hMPV with HBoV was the most common it must be considered that there may be more than one agents in patients with symptoms of respiratory tract infection.

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.

Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat.

BACKGROUND: Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. METHODS: Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. RESULTS: The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). CONCLUSION: In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.

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.