Ahu Kara

Osteopetrosis and Glanzmann's thrombasthenia in a child

Autosomal recessive osteopetrosis is a rare, fatal disease characterized by accumulation of excessive bone mass due to defective bone resorption. The pathogenesis of osteopetrosis is controversial. Osteoblast-osteoclast interaction defects, incorrect differentiation of osteoclasts, abnormal contact between osteoclast and extracellular matrix, and abolished signaling are included in this process. Recently, mutations in the gene of the vacuolar proton pump have been described in some cases of recessive osteopetrosis. Glanzmanns thrombasthenia (GT) is a rare hereditary qualitative platelet disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin αIIbβ3. Several mutations on either integrin αIIb [glycoprotein (GP) IIb] or integrin β3 (GP IIIa) were reported in GT. We report on a patient with autosomal recessive osteopetrosis concurrently diagnosed with variant type Glanzmanns thrombasthenia. To our knowledge, our patient was the first case reported in the literature in which osteopetrosis and Glanzmanns thrombasthenia were diagnosed together.

Bacterial agents causing meningitis during 2013–2014 in Turkey: A multi-center hospital-based prospective surveillance study

ABSTRACT This is an observational epidemiological study to describe causes of bacterial meningitis among persons between 1 month and 18 y of age who are hospitalized with suspected bacterial meningitis in 7 Turkish regions. covering 32% of the entire population of Turkey. We present here the results from 2013 and 2014. A clinical case with meningitis was defined according to followings: any sign of meningitis including fever, vomiting, headache, and meningeal irritation in children above one year of age and fever without any documented source, impaired consciousness, prostration and seizures in those < 1 y of age. Single tube multiplex PCR assay was performed for the simultaneous identification of bacterial agents. The specific gene targets were ctrA, bex, and ply for N. meningitidis, Hib, and S. pneumoniae, respectively. PCR positive samples were recorded as laboratory-confirmed acute bacterial meningitis. A total of 665 children were hospitalized for suspected acute meningitis. The annual incidences of acute laboratory-confirmed bacterial meningitis were 0.3 cases / 100,000 population in 2013 and 0.9 cases/100,000 in 2014. Of the 94 diagnosed cases of bacterial meningitis by PCR, 85 (90.4%) were meningococcal and 9 (9.6%) were pneumococcal. Hib was not detected in any of the patients. Among meningococcal meningitis, cases of serogroup Y, A, B and W-135 were 2.4% (n = 2), 3.5% (n = 3), 32.9% (n = 28), and 42.4% (n = 36). No serogroup C was detected among meningococcal cases. Successful vaccination policies for protection from bacterial meningitis are dependent on accurate determination of the etiology of bacterial meningitis. Additionally, the epidemiology of meningococcal disease is dynamic and close monitoring of serogroup distribution is comprehensively needed to assess the benefit of adding meningococcal vaccines to the routine immunization program.

Spontaneous haemothorax: an uncommon presentation of Glanzmann thrombasthenia

Glanzmann thrombasthenia is a rare hereditary qualitative platelet disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin αIIbβ3. Common clinical manifestations include purpuric type skin bleeding, prolonged bleeding from minor cuts, epistaxis, gingival bleeding and menorrhagia. Less frequently, gastrointestinal system bleeding may occur. Haemarthrosis, haematuria, intracranial and visceral haemorrhage are very rare symptoms. This study reports a 3‐y‐old girl with Glanzmann thrombasthenia who presented with life‐threatening haemothorax. There was no history of recent trauma or drug usage and no vascular or parenchymal abnormalities to explain the development of haemothorax.

Risk of vancomycin-resistant enterococci bloodstream infection among patients colonized with vancomycin-resistant enterococci

BACKGROUND Vancomycin-resistant enterococci colonization has been reported to increase the risk of developing infections, including bloodstream infections. AIM In this study, we aimed to share our experience with the vancomycin-resistant enterococci bloodstream infections following gastrointestinal vancomycin-resistant enterococci colonization in pediatric population during a period of 18 months. METHOD A retrospective cohort of children admitted to a 400-bed tertiary teaching hospital in Izmir, Turkey whose vancomycin-resistant enterococci colonization was newly detected during routine surveillances for gastrointestinal vancomycin-resistant enterococci colonization during the period of January 2009 and December 2012 were included in this study. All vancomycin-resistant enterococci isolates found within 18 months after initial detection were evaluated for evidence of infection. FINDINGS Two hundred and sixteen patients with vancomycin-resistant enterococci were included in the study. Vancomycin-resistant enterococci colonization was detected in 136 patients (62.3%) while they were hospitalized at intensive care units; while the remaining majority (33.0%) were hospitalized at hematology-oncology department. Vancomycin-resistant enterococci bacteremia was present only in three (1.55%) patients. All these patients were immunosuppressed due to human immunodeficiency virus (one patient) and intensive chemotherapy (two patients). CONCLUSION In conclusion, our study found that 1.55% of vancomycin-resistant enterococci-colonized children had developed vancomycin-resistant enterococci bloodstream infection among the pediatric intensive care unit and hematology/oncology patients; according to our findings, we suggest that immunosupression is the key point for developing vancomycin-resistant enterococci bloodstream infections.

Burn-associated bloodstream infections in pediatric burn patients: Time distribution of etiologic agents

BACKGROUND Infections are the leading cause of morbidity and mortality in patients with burns in burn units. Bloodstream infections (BSIs) in patients with burns may result from burn wound infection, use of invasive devices such as central venous catheters, and translocation of the gastrointestinal flora. OBJECTIVE In this study, we investigated the distribution and antimicrobial drug resistance of causative pathogens in children with burns and the durational changes of microorganisms in the distribution of BSIs in children. METHODS This study was conducted at the Pediatric Burn Unit (PBU) of Dr. Behçet Uz Children Research and Training Hospital during the period of November 2008-April 2015. The study subjects were all the patients admitted to the PBU, in whom microorganisms were isolated at least from one of the cultures, including blood and catheter cultures. RESULTS Gram-positive bacteria were the most common causative agents of BSI in patients with burns (66.4%), followed by gram-negative bacteria (22.1%) and fungi (11.5%). The median duration of development of BSIs caused by gram-positive bacteria from the time of burn was 5 days (ranging from 2 to 54 days of burn), which was significantly shorter than that of BSIs caused by gram-negative bacteria (12 days) and fungal pathogens (13 days). CONCLUSION The etiologic agents of BSIs in children may differ from those in adults. Gram-negative drug-resistant bacteria such as multidrug-resistant Pseudomonas aeruginosa and Acinetobacter baumannii were important agents of BSI in patients with burns, especially in the long term; however, gram-positive bacteria should also be considered while deciding the antimicrobial therapy, especially in the early periods of burn.

Varicella-Zoster Virus Infections in Pediatric Malignancy Patients: A Seven-Year Analysis

Primary varicella-zoster virus (VZV) infection is a benign self-limited disease. In this study, we review our experience in focusing on the outcome and treatment of VZV infection in pediatric malignancy patients. During the study period, a total of 41 patients with pediatric malignancy had been hospitalized with the diagnosis of VZV infection. All the patients were treated with intravenous acyclovir for a median of 7 days (ranging from 5 to 21 days). The calculated attributable delay of chemotherapy due to VZV infections was 8 days (ranging from 2 to 60 days). VZV-related complications were observed in 3 of 41 patients (7%) who suffered from acute respiratory distress syndrome, and one of them with hemophagocytic lymphohistiocytosis died due to respiratory failure despite acyclovir and broad-spectrum antimicrobial treatment plus supportive treatment. VZV infections are still important contagious diseases in pediatric cancer patients, because they cause not only significant mortality but also a delay in chemotherapy.

Antifungal consumption, indications and selection of antifungal drugs in pediatric tertiary hospitals in Turkey: Results from the first national point prevalence survey

OBJECTIVES The aim of this point prevalence survey was to evaluate the consumption, indications and strategies of antifungal therapy in the paediatric population in Turkey. METHODS A point prevalence study was performed at 25 hospitals. In addition to general data on paediatric units of the institutes, the generic name and indication of antifungal drugs, the presence of fungal isolation and susceptibility patterns, and the presence of galactomannan test and high-resolution computed tomography (HRCT) results were reviewed. RESULTS A total of 3338 hospitalised patients were evaluated. The number of antifungal drugs prescribed was 314 in 301 patients (9.0%). Antifungal drugs were mostly prescribed in paediatric haematology and oncology (PHO) units (35.2%), followed by neonatal ICUs (NICUs) (19.6%), paediatric services (18.3%), paediatric ICUs (PICUs) (14.6%) and haematopoietic stem cell transplantation (HSCT) units (7.3%). Antifungals were used for prophylaxis in 147 patients (48.8%) and for treatment in 154 patients (50.0%). The antifungal treatment strategy in 154 patients was empirical in 77 (50.0%), diagnostic-driven in 29 (18.8%) and targeted in 48 (31.2%). At the point of decision-making for diagnostic-driven antifungal therapy in 29 patients, HRCT had not been performed in 1 patient (3.4%) and galactomannan test results were not available in 12 patients (41.4%). Thirteen patients (8.4%) were receiving eight different antifungal combination therapies. CONCLUSION The majority of antifungal drugs for treatment and prophylaxis were prescribed in PHO and HSCT units (42.5%), followed by ICUs. Thus, antifungal stewardship programmes should mainly focus on these patients within the availability of diagnostic tests of each hospital.

Central line bundle for prevention of central line–associated bloodstream infection for totally implantable venous access devices (ports) in pediatric cancer patients:

Objective: The clinical impact of central line bundle programs for central line–associated bloodstream infections has been well demonstrated in intensive care units. However, the experience of central line bundle programs in totally implantable venous access devices (ports) in pediatric-hematology patients was limited. Methods: A retrospective study was designed to compare and evaluate the clinical impact of implementing a central line bundle for a 2-year 5-month period, including 10 months of prebundle period, 11 months of central line bundle (that includes needleless split-septum devices), and finally 8 months of central line bundle period in which single-use prefilled flushing devices were added to the previous central line bundle. Results: During the prebundle period, the rate of 14.5 central line–associated bloodstream infections per 1000 CL-days had decreased to 5.49 CLABSIs per 1000 CL-days in the first bundle period. The incidence rate ratio with these two groups was 0.379, indicating a relative risk reduction of 62% (p = 0.005). By the addition of single-use prefilled flushing devices to the first bundle program, the central line–associated bloodstream infection rate decreased to 2.63 per 1000 CL-days. Port removal rate due to central line–associated bloodstream infections was 0.46 per 1000 catheter days in the bundle period, which was significantly lower than in the prebundle period in which port removal rate was 4.5 per 1000 catheter days (p < 0.001). Conclusion: Central line bundle programs were found to be effective in decreasing central line–associated bloodstream infection rates, improving patients’ quality of life by preventing ports removal due in pediatric cancer patients.

Hospital cost analysis of children with preseptal cellulitis

OBJECTIVE Hospitalization of the children with preseptal cellulitis creates a burden on healthcare costs. This study aimed to analyze the hospital costs for preseptal cellulitis and determine the factors contributing. METHODS Children, between 1 and 18 years old, who were admitted to hospital for preseptal cellulitis from May 2013 to December 2016 were included in the study. Patients were divided into groups by age (under or equal to five years and older than five years) and by the presence of sinusitis. Demographics, length of stay and total and categorical hospital costs were evaluated retrospectively. RESULTS The study included 54 patients with a mean age of 5 years. Thirty one of the patients were under five years of age. The most common symptoms were swelling (94.4%) and redness (83.3%) around eye. Among the predisposing factors, sinusitis was the most common one (37%). The average length of stay was 4.5 days. Total hospital cost of all patients was

The Frequency of Infective Endocarditis in Candida Bloodstream Infections: a Retrospective Study in a Child Hospital

11,841. Antibiotic costs (37%) and inpatient floor costs (36%) were the greatest expenditures. Between age groups, length of stay was longer, and inpatient floor and antibiotic costs were significantly higher in the group of >5 years (p = 0.007, p = 0.004 and p = 0.001, respectively). In the group with sinusitis, length of stay was longer, and all hospital costs were significantly higher compared to the group without sinusitis (p < 0.001). There was a strong, positive correlation between length of stay and hospital costs (r = 0.854, n = 53, p < 0.001). Sinusitis was a significant factor (p < 0.001) for longer length of stay, but age was not (p = 0.841). CONCLUSION Sinusitis was found to be an important factor contributing to longer length of stay and higher hospital costs for preseptal cellulitis. Oral or ambulatory intravenous antimicrobial treatment strategies might decrease the hospital expenditure in these patients; however care should be taken in the presence of sinusitis.