Evaluation of Clinical and Laboratory Characteristics of Children with Pulmonary and Extrapulmonary Tuberculosis.

Background and objective: Tuberculosis (TB) is an important public health problem in both developing and developed countries. Childhood TB is also an important epidemiological indicator in terms of forming the future TB pool. The diagnosis of TB is difficult in children due to the lack of a standard clinical and radiological description. We aimed to evaluate and compare the clinical, laboratory, and radiologic findings of childhood pulmonary and extrapulmonary TB. Material and Methods: The medical records of patients hospitalized with the diagnosis of pulmonary tuberculosis (PTB) and extrapulmonary tuberculosis (EPTB) between December 2007 and December 2017 were evaluated retrospectively. Results: There were 163 patients diagnosed with TB with 94 females (57.7%) and 69 males (42.3%). Seventy-three patients (44.8%) had PTB, 71 (43.6%) patients had EPTB, and 19 patients (11.7%) had both PTB and EPTB, called as disseminated TB. Ninety-six (58.9%) patients had tuberculin skin test (TST) positivity and 64 patients (39.3%) had interferon-gamma release assay (IGRA) positivity. Acid-resistant bacteria were observed in 34 (20.9%) body fluid samples and culture positivity was observed in 33 (20.2%) samples. Comparison of PTB, EPTB, and disseminated TB revealed that low socioeconomic status, TB contact, and low body weight were more common in disseminated TB, and TST positivity was more common in PTB. Conclusion: Malnutrition, low socioeconomic status, and TB contact were important diagnostic variables in our study and all three parameters were more common in disseminated TB. Tuberculosis should be considered in patients admitted with different complaints and signs in populations with high TB incidence and low socioeconomic status.

Genotypic characterization of Pseudomonas aeruginosa isolates from Turkish children with cystic fibrosis.

Objective: To identify epidemic and other transmissible Pseudomonas aeruginosa strains, genotypic analyses are required. The aim of this study was to assess the distribution of P. aeruginosa strains within the Turkish pediatric cystic fibrosis (CF) clinic population. Methods: Eighteen patients attending the pediatric CF clinic of Cerrahpasa Medical Faculty were investigated in the study. Throat swab and/or sputum samples were taken from each patient at 3-month intervals. The isolates of patients were analyzed by pulsed-field gel electrophoresis (PFGE). The intra- and interpatient genotypic heterogeneity of isolates was examined to determine the clonal isolates of P. aeruginosa within the cohort. Results: A total of 108 clinical isolates of P. aeruginosa were obtained from 18 patients between May 2013 and May 2014. The pulsotypes of the first patient's isolates could not be obtained by PFGE. From the remaining 17 patients and 101 isolates, 55 distinct pulsotypes were detected. The number of pulsotypes observed in more than one patient (minor clonal strains, cluster strains) was 8 (14.5%), and one of them colonized three patients. However, none of them was detected in more than three patients. These pulsotypes were composed of 20 isolates. In addition, with the PFGE analysis of 81 isolates, we detected 47 (85.6%) pulsotypes, which belonged to only one patient. Over different periods of this study, only 2 (11.8%) patients were colonized with the same pulsotype. Conclusion: Our study indicates that there was considerable genomic diversity among the P. aeruginosa isolates in our clinic. The presence of shared pulsotypes supports cross-transmission between patients.

Tuberculin skin test response in patients with juvenile idiopathic arthritis on anti-TNF therapy

Background/aim: The aim of this study is to evaluate the effect of biologic drugs on the tuberculin skin test in patients with juvenile idiopathic arthritis. Materials and methods: A total of 234 biologic drug-using juvenile idiopathic arthritis patients and 45 healthy controls were enrolled in the study. The tuberculin skin test results of the patients, which had been routinely provided during follow-up, were obtained from the patient files. Tuberculin skin test values of ≥5 mm were considered to be positive. Results: The mean diameter of tuberculin skin test induration was 4.99 ± 6.84 mm (IQR: 0­10 mm) and 7.83 ± 3.47 mm (IQR: 0­16 mm) in patients and controls, respectively (P < 0.05). Tuberculin skin test positivity (≥5 mm) was found in 96 (41%) and 38 (84.4%) of patients and controls, respectively (P < 0.001). There was no induration in 125 (53.4%) patients and 3 (6.6%) healthy controls, respectively (P < 0.001). Conclusion: In the patients with juvenile idiopathic arthritis who were using biologic drugs, tuberculin skin test induration was significantly lower compared to the control group. Tuberculin skin tests alone seem inadequate for recognition of latent tuberculosis in juvenile idiopathic arthritis patients on anti-TNF therapy.

Profile of the patients who present to immunology outpatient clinics because of frequent infections.

AIM: We aimed to determine the rate of primary immune deficiency (PID) among children presenting to our immunology outpatient clinic with a history of frequent infections and with warning signs of primary immune deficiency. MATERIAL AND METHODS: The files of 232 children aged between 1 and 18 years with warning signs of primary immune deficiency who were referred to our pediatric immunology outpatient clinic with a complaint of frequent infections were selected and evaluated retrospectively. RESULTS: Thirty-six percent of the subjects were female (n=84) and 64% were male (n=148). PID was found in 72.4% (n=164). The most common diagnosis was selective IgA deficiency (26.3%, n=61). The most common diseases other than primary immune deficiency included reactive airway disease and/or atopy (34.4%, n=22), adenoid vegetation (12.3%, n=8), chronic disease (6.3%, n=4) and periodic fever, aphtous stomatitis and adenopathy (4.6%, n=3). The majortiy of the subjects (90.5%, n=210) presented with a complaint of recurrent upper respiratory tract infection. PID was found in all subjects who had bronchiectasis. The rates of the diagnoses of variable immune deficiency and Bruton agammaglubulinemia (XLA) were found to be significantly higher in the subjects who had lower respiratory tract infection, who were hospitalized because of infection and who had a history of severe infection compared to the subjects who did not have these properties (p<0.05 and p<0.01, respectively). Growth and developmental failure was found with a significantly higher rate in the patients who had a diagnosis of severe combined immune deficiency or hyper IgM compared to the other subjects (p<0.01). No difference was found in the rates of PID between the age groups, but the diagnosis of XLA increased as the age of presentation increased and this was considered an indicator which showed that patients with XLA were being diagnosed in a late period. CONCLUSIONS: It was found that the rate of diagnosis was considerably high (72.4%), when the subjects who had frequent infections were selected by the warning signs of PID.

Primary diffuse leptomeningeal gliomatosis mimicking tuberculous meningitis.

Primary diffuse leptomeningeal gliomatosis is a disease with an aggressive course that can result in death. To date, 82 cases have been reported. Here, the case of a 3-year-old male patient presenting with strabismus, headache, and restlessness is reported. Physical examination revealed paralysis of the left abducens nerve, neck stiffness, and bilateral papilledema. Tuberculous meningitis was tentatively diagnosed, and antituberculosis treatment was initiated when cranial imaging revealed contrast enhancement around the basal cistern. Craniocervical magnetic resonance imaging (MRI) was performed when there was no response to treatment, and it revealed diffuse leptomeningeal contrast enhancement around the basilar cistern, in the supratentorial and infratentorial compartments, and in the spinal region. Primary diffuse leptomeningeal gliomatosis was diagnosed by a meningeal biopsy.

A case of oculoglandular tularemia resistant to medical treatment.

Tularemia is a zoonotic disease of the northern hemisphere. Oculoglandular tularemia is the rarest form, comprising 1.4-4.2% of all cases. We present a patient with oculoglandular tularemia that did not respond to gentamicin to demonstrate the need for prolonged antimicrobial treatment and surgical intervention.

Toxicological evaluation of two children diagnosed as Munchausen syndrome by proxy.

Munchausen syndrome by proxy is a kind of child abuse in which affected children are often hospitalized for long periods and endure repetitive, painful and expensive diagnostic attempts. We present herein two toxicologically confirmed cases of Munchausen syndrome by proxy. Case 1 is a 16-month-old male who had fever, peripheral cyanosis, tremor, and reported cardiac arrest. Symptoms recurred in the hospital when the mother administered fluids. Toxicology detected 3.5 ng/ml mercury (Hg) in the fluid and 9.4 microg Hg/g creatinine in the urine. Case 2 is a 14-year-old female who had irregular blood findings and multiple hospitalizations. Serum analysis detected warfarin. Both mothers were transferred to psychiatric care. Munchausen syndrome by proxy should be suspected when clinical/laboratory findings are negative, illness descriptions are inconsistent, and frequent hospitalization yields no diagnosis. Psychiatric evaluation and toxicological analysis are recommended.

Primary intracranial abscess localized in the basal ganglia: a case report.

Brain abscesses are serious infections that cause neurological problems and generally require antibiotic treatment and surgical drainage. Hematogenous brain abscesses are generally located in the region fed by the middle cerebral artery, but are occasionally found in deep tissues, such as the basal ganglia and thalamus.

Is it safe to use anti-TNF-α agents for tuberculosis in children suffering with chronic rheumatic disease?

To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients diagnosed with juvenile idiopathic arthritis (JIA) and other chronic rheumatologic diseases treated with anti-TNF-α treatment (etanercept, infliximab, adalimumab) in Turkey, 144 patients were evaluated retrospectively for the development of tuberculosis. Patients were evaluated every 6 months for tuberculosis using history, physical examination, tuberculin skin test (TST), chest radiographs, and, when required, examination of sputum/early morning gastric aspirates for acid-fast bacilli and chest tomography. A tuberculin skin test over 10 mm induration was interpreted as positive. Patients were diagnosed with JIA (n = 132), enthesitis-related arthritis (ERA; n = 14), juvenile psoriatic arthritis (JPsA; n = 4), chronic idiopathic uveitis (n = 4), and chronic arthritis related to FMF (n = 8). Mean age was 12.25 ± 3.96 years (4.08-19.41 years), mean duration of illness was 5.86 ± 3.77 years (0.66-15 years), and the mean duration of anti-TNF-α treatment was 2.41 ± 1.47 years (0.6-7 years). Anti-TNF-α agents prescribed were etanercept (n = 133), infliximab (n = 30), and adalimumab (n = 6). When unresponsive to one anti-TNF-α therapy, patients were switched to another. There was no history of contact with individuals having tuberculosis. During follow-up, seven patients (4.8%) with positive TST were given INH prophylaxis. One oligoarticular JIA patient (0.69%) diagnosed with secondary uveitis who had been followed for 5 years and had been using infliximab for 2 years, developed a positive Quantiferon-TB test while on INH prophylaxis. He was started on an anti-tuberculosis drug regimen. In conclusion, anti-TNF-α treatment in children with chronic inflammatory disease is safe. Follow-up every 6 months of children on anti-TNF-α treatment with respect to tuberculosis by the pediatric infectious disease department is important to prevent possible complications.

Relation between serum Insulin-like growth factor-I and insulin-like growth factor-binding protein-3 levels, clinical status and growth parameters in prepubertal cystic fibrosis patients.

BACKGROUND: This study aims to determine the relation between anabolic hormones, Insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 (IGFBP-3), growth parameters, and clinical status in prepubertal cystic fibrosis (CF) patients. This prospective study comprises age/sex-matched control subjects and was set in a tertiary care teaching hospital. METHODS: Serum concentrations of IGF-I and IGFBP-3 were measured in 37 CF and 23 healthy subjects, whose mean ages were 5.02 +/- 3.06 and 5.27 +/- 2.82, respectively. The results were analyzed in relation to body mass index standard deviation scores (BMISD), height standard deviation scores (HSD), growth velocity standard deviation scores (GVSD), and clinical status assessed by Shwachman scores and pulmonary function parameters. RESULTS: Serum IGFBP-3 of CF patients showed significantly lower concentrations than healthy subjects (2457 vs. 3249 ng/mL) (P < 0.05), whereas IGF-I levels did not (123.35 vs. 149.8 ng/mL). There was significant positive correlation between IGF-I and IGFBP-3 with HSD (r = 0.62; r = 0.79) and BMISD (r = 0.39; r = 0.50). The pulmonary function tests in 14 CF subjects were not statistically worse than in nine healthy cases. The mean HSD (-0.67, SD 1.06) and BMISD (-0.28, SD 0.71) of CF patients were not significantly lower than those of healthy subjects (-0.02, SD 0.86 and 0.03, SD 0.49), respectively. CONCLUSION: Decreased serum IGF-I and IGFBP-3 levels may reflect growth retardation in CF. IGFBP-3 seems like a more sensitive parameter than IGF-I for growth monitoring in this study. Growth parameters of Turkish prepubertal CF patients are not markedly below national standards. Different genetic backgrounds of relevant populations certainly play an important role for the variable clinical course.

HHV-8-associated Kaposi sarcoma in a child with IFNgammaR1 deficiency.

OBJECTIVES: Mediterranean classic Kaposi sarcoma (KS) of childhood is rare and unexplained. Our objective is to describe the case of a child with complete IFNgammaR1 deficiency and severe mycobacterial disease in whom Kaposi sarcoma (KS) developed. RESULTS: Disseminated mycobacterial infection began at the age of 5 months, and at 11 years of age the child had disseminated KS lesions. The histologic appearance of these lesions was typical, with endothelial and spindle cell proliferation. Human herpesvirus-8 (HHV-8)-associated antigens were detected in situ by immunohistochemistry. HHV-8 DNA of K1 molecular subtype A was amplified from tissue lesions, and HHV-8-specific antibodies were detected in the patient's serum. The child died at 12 years of age of disseminated mycobacterial disease and KS. CONCLUSIONS: This is the first identification of a well-defined primary immunodeficiency in a child with KS. Inherited disorders of IFN-gamma-mediated immunity and severe mycobacterial disease may predispose HHV-8-infected children to KS.