Sirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study.

Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune-mediated destruction of haematopoietic cell lines. Effective and well-tolerated treatment options for relapsed-refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7 months (range, 0-9 months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow-up and two of them had remission with low-dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed-refractory immune cytopenia.

The effect of the closed intravenous catheter system on first insertion success, indwelling time, and complications in pediatric hematology and oncology patients: A randomized controlled study.

PURPOSE: The primary purpose of this research is to evaluate the effect of two different catheter systems (closed IV catheter system: BD Nexiva™, peripheral open IV catheter: BD Insyte™ Autoguard™) on first insertion success, catheter indwelling time, and the catheter complications. METHOD: This randomized controlled study used a single-blind and parallel trial design guided by the CONSORT checklist. The "Peripheral Intravenous Catheter (PIVC) Bundle" was applied to all patients. A total of 214 catheters of 38 patients were included in the intervention (BD Nexiva™) (n = 107 catheter) and control (open IV catheter) groups (n = 107 catheter) of the study. The indwelling time and PIVC complications were followed. RESULTS: The mean age of the patients in the study group was 5.9 ± 2.2, and the mean age of the patients in the control group was 5.7 ± 1.9. The PIVC was successfully placed in 68.2% of the patients in the study group and in 65.4% of the patients in the control group at the first attempt. It was determined that the indwelling time was 4.9 ± 3.9 (max. 20.25 days) in the study group and 2.9 ± 2.8 (max. 11.25 days) days in the control group. The complication rates were found to be 86.8 for the study group and 166.9 for the control group in 1000 catheter days. In this study, no difference was found in terms of complication. CONCLUSIONS: The PIVC indwelling time is longer in patients with the closed IV catheter system. These new technology PIVCs can be used for this special patient population. GOV IDENTIFIER: NCT05769452.

Etiology and Factors Affecting Severe Complications and Mortality of Febrile Neutropenia in Children with Acute Leukemia

Objective: Febrile neutropenia (FN) is an important complication that causes high rates of morbidity and mortality in patients with malignancies. We aimed to investigate the etiology, epidemiological distribution and its change over the years, clinical courses, and outcomes of FN in children with acute leukemia. Materials and Methods: We retrospectively analyzed the demographic data, clinical characteristics, laboratory results, severe complications, and mortality rates of pediatric patients with FN between January 2010 and December 2020. Results: In 153 patients, a total of 450 FN episodes (FNEs) occurred. Eighty-four (54.9%) of these patients were male, the median age of the patients was 6.5 (range: 3-12.2) years, and 127 patients (83%) were diagnosed with acute lymphoblastic leukemia. Fever with a focus was found in approximately half of the patients, and an etiology was identified for 38.7% of the patients. The most common fever focus was bloodstream infection (n=74, 16.5%). Etiologically, a bacterial infection was identified in 22.7% (n=102), a viral infection in 13.3% (n=60), and a fungal infection in 5.8% (n=26) of the episodes. Twenty-six (23.2%) of a total of 112 bacteria were multidrug resistant (MDR) The rate of severe complications was 7.8% (n=35) and the mortality rate was 2% (n=9). In logistic regression analysis, refractory/relapsed malignancies and high C-reactive protein (CRP) at first admission were found to be the most important independent risk factors for mortality. Prolonged neutropenia after chemotherapy, diagnosis of acute myeloid leukemia, identification of fever focus or etiological agents, invasive fungal infections, polymicrobial infections, and need for intravenous immunoglobulin treatment increased the frequency of severe complications. Conclusion: We found that there was no significant change in the epidemiological distribution or frequency of resistant bacteria in our center in the last 10 years compared to previous years. Prolonged duration of fever, relapsed/refractory malignancies, presence of fever focus, and high CRP level were significant risk factors for poor clinical course and outcome.

Central nervous system thrombosis in pediatric acute lymphoblastic leukemia in Turkey: A multicenter study.

BACKGROUND: In patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary to the primary disease and due to treatment-related factors. In this multicenter study, we aimed to research the frequency of central nervous system (CNS) thrombosis occurring during treatment, hereditary and acquired risk factors, clinical and laboratory features of patients with thrombosis, treatment approaches, and thrombosis-related mortality and morbidity rates in pediatric ALL patients. PROCEDURE: Pediatric patients who developed CNS thrombosis during ALL treatment from 2010 to 2021 were analyzed retrospectively in 25 different Pediatric Hematology Oncology centers in Türkiye. The demographic characteristics of the patients, symptoms associated with thrombosis, the stage of the leukemia treatment during thrombosis, the anticoagulant therapy applied for thrombosis, and the final status of the patients recorded through electronic medical records were determined. RESULTS: Data from 70 patients with CNS thrombosis during treatment, out of 3968 pediatric patients with ALL, were reviewed. The incidence of CNS thrombosis was 1.8% (venous: 1.5 %; arterial: 0.03%). Among patients with CNS thrombosis, 47 had the event in the first 2 months. Low molecular weight heparin (LMWH) was the most commonly used treatment with a median of 6 months (min-max: 3-28 months). No treatment-related complications occurred. Chronic thrombosis findings occurred in four patients (6%). In five (7%) patients who developed cerebral vein thrombosis, neurological sequelae (epilepsy and neurological deficit) remained. One patient died related to thrombosis, and the mortality rate was 1.4%. CONCLUSION: Cerebral venous thrombosis and, less frequently, cerebral arterial thrombosis may develop in patients with ALL. The incidence of CNS thrombosis is higher during induction therapy than during other courses of treatment. Therefore, patients receiving induction therapy should be monitored carefully for clinical findings suggestive of CNS thrombosis.

Evaluation of vincristine-induced peripheral neuropathy in children with cancer: Turkish validity and reliability study.

BACKGROUND: The evaluation of peripheral neuropathy in children receiving Vincristine treatment is challenging. This study examined the Turkish validity and reliability of the Total Neuropathy Score-Pediatric Vincristine (TNS-PV) measurement tool, which can measure Vincristine-induced peripheral neuropathy symptoms in children with cancer. METHODS: A total of 53 children aged 5-17 years who received Vincristine treatment in two pediatric hematology-oncology centers participated in the study. Data was collected using the Total Neuropathy Score-Pediatric Vincristine (TNS-PV), the Common Terminology Criteria for Adverse Events (CTCAE), the Wong-Baker FACES Pain Scale, and the Adolescent Pediatric Pain Tool (APPT). The correlation between the TNS-PV total score and other scales and the inter-rater reliability coefficient was evaluated. FINDINGS: Of the children, 81.1% were diagnosed with ALL and 13.2% with Ewing Sarcoma. Cronbach's alpha values of form A and B of the TNS-PV scale were 0.628 and 0.639, respectively. As the cumulative Vincristine dose increased, the children's scores on TNS-PV were higher. A moderate and significant positive correlation was found between the TNS-PV form A total score and the worst subjective symptoms a, b (A), strength, tendon reflexes, and autonomic / constipation (r = 0.441, r = 0.545, r = 0.472, r = 0.536, p < 0.01). DISCUSSION: The TNS-PV form B total score was found to have a moderate level, significant correlation with CTCAE sensory neuropathy score and Wong-Baker FACES Pain Scale, and a high level, significant positive correlation with CTCAE motor neuropathy score. APPLICATION TO PRACTICE: The TNS-PV is valid and reliable for measuring Vincristine-induced peripheral neuropathy in practice in Turkish children 5 years and older.

Timing of Central Venous Line Insertion During Induction in Children With Acute Lymphoblastic Leukemia.

PURPOSE: Central venous lines (CVL) in children with acute lymphoblastic leukemia (ALL) provide comfortable administration of intensive chemotherapy and blood sampling. The optimal time for the insertion of CVL in patients with ALL during induction therapy is controversial. This study aimed to investigate the frequency of CVL-related complications in children with ALL concerning the time of CVL insertion. PATIENTS AND METHODS: We reviewed the records of 52 pediatric ALL patients with CVL. CVL placement before or on treatment day 15 was defined as "early insertion", and after treatment day 15 was defined as "late insertion". Demographics, preoperative blood counts, type of central line, time of CVL placement, CVL-related complications, and blood counts during complications were all noted. All the data were collected from those with the first catheter use. RESULTS: CVL was placed ≤15 days in 26 patients (50%) and after 15 days in 26 patients (50%). Regarding the infection rates, no statistical difference was found between early and late CVL-inserted groups ( P =n.s.). Five patients developed thrombosis, and risk was found to be similar between early and late CVL-inserted groups ( P =n.s.). Catheter-related mechanical complications were recorded in 7 patients (3 in early and 4 in late CVL-inserted group, ( P =n.s.). CONCLUSION: The present study showed no relation between the timing of CVL placement during induction therapy and the occurrence of infection and thrombosis. Our results suggest that CVL can be placed safely at the time of diagnosis or early induction treatment to provide a comfortable administration of chemotherapy and decrease painful blood samplings.

Examination of the psychometric properties of pediatric-modified total neuropathy score in Turkish children with cancer.

BACKGROUND: Evaluation of chemotherapy-induced peripheral neuropathy has gained importance in symptom management of pediatric patients with cancer. This study aimed to perform the Turkish validity and reliability study of the Pediatric-Modified Total Neuropathy Score (Ped-mTNS). METHODS: A methodological, descriptive, and cross-sectional design was used in the study. Forty children aged between 5 and 18 and were treated for cancer and 40 age- and gender-matched healthy children (control group) were included in the study. The mean scores of the items on the Ped-mTNS were compared, and item-total score correlations were evaluated. Cronbach's alpha coefficient of the Ped-mTNS was calculated for internal consistency. FINDINGS: Cronbach's alpha value of the scale was found as 0.709. The item-total correlations of the scale items ranged from 0.260 to 0.658. The mean score of cancer patients on the Ped-mTNS was found as 4.4 ± 3.8. DISCUSSION: Ped-mTNS scores of children with cancer indicated more deficits than those of the control group. In the evaluation of children in the patient and control groups, a difference was found in terms of light touch sensation, which is one of the sensory symptoms in the items of the Ped-mTNS, and pin sensibility and strength, which are among the clinical symptoms. APPLICATION TO PRACTICE: The Ped-mTNS was determined to be a valid and reliable measurement tool for children with cancer aged between 5 and 18 in the Turkish population.

Evaluation of voriconazole related adverse events in pediatric patients with hematological malignancies.

BACKGROUND: Despite therapeutic drug monitoring and pharmacogenetic-guided dose selection are recommended for pediatric patients, safety of voriconazole is mostly monitored by clinical assessment. Having comprehensive knowledge of safety profile and distinguishing incidental events from the reactions that are truly related to voriconazole use are crucial for safer and uninterrupted treatment. OBJECTIVES: This study aimed to address adverse reactions during the first month of voriconazole use by systematically evaluating retrospective records of all adverse events. Patients/Methods: It is a single-center, retrospective analysis of patients who received voriconazole from 1 September 2010 to 1 September 2020. Severity of abnormal findings in medical records were systematically graded. Causality between voriconazole and the events was evaluated by Liverpool Causality Assessment Tool (LCAT), Naranjo Algorithm and World Health Organization Causality Assessment System. The events with possible or probable causal relation to voriconazole are classified as adverse reaction. RESULTS: Records of 45 patients included in the study. The overall frequency of adverse reactions was 51.1%. Hepatobiliary laboratory adverse reactions identified in 48.9% of the patients and led to treatment discontinuation in 20.0%. Amylase and lipase elevation (2.2%), ventricular extra systoles (2.2%), hallucination and nightmares (2.2%) were other adverse reactions. CONCLUSIONS: Hepatobiliary abnormalities were the most common adverse reactions and the most common cause of treatment discontinuation. For safer treatment in critically ill patients, the dose should be personalized. To clearly identify the accurate frequency and the causality of all adverse reactions, prospective studies with much larger sample size are needed.

Roles of Vitamin-K-dependent Factors Protein S and GAS6 With TAM Receptors and HMGB1 in Pediatric COVID-19 Disease.

OBJECTIVE: This study was designed to evaluate serum high-mobility group box 1 (HMGB1), protein S (PS), growth arrest-specific gene 6 (GAS6), and TAM receptor (TYRO3, AXL, and MERTK) levels in children with COVID-19 disease. METHODS: A prospective case-control study was conducted in our pediatric emergency department and 57 patients with SARS-CoV-2 polymerase chain reaction (PCR) positivity, 6 patients with multisystem inflammatory syndrome in children (MIS-C), and 17 healthy children were included. Demographic data, clinical findings, laboratory and radiologic data, the need for hospitalization, and prognosis were recorded. Serum HMGB1, PS, GAS6, and TAM receptor levels were studied by enzyme-linked immunosorbent assay method. RESULTS: While SARS-CoV-2 PCR-positive patients and healthy controls were similar in terms of gender and age, GAS6 and MERTK levels were significantly lower in SARS-CoV-2 PCR-positive patients compared with healthy controls. Among SARS-CoV-2 PCR-positive patients, no difference was found in terms of serum markers in those with and without gastrointestinal or respiratory system symptoms. However, in patients with respiratory distress at admission, PS and TYRO3 levels were significantly lower. AXL levels were lower in patients diagnosed with MIS-C compared with healthy controls. Activated partial thromboplastin time was negatively correlated with HMGB1, PS, GAS6, and AXL levels. CONCLUSION: Our results suggest that such measurements may be informative and warranted in children with COVID-19 who show evidence of coagulopathy and respiratory distress. Further studies are needed to clarify the roles of these markers in diagnosis, to predict clinical severity, and to evaluate their roles in treatment approaches for COVID-19 disease.

A novel Mecom gene mutation associated with amegakaryocytic thrombocytopenia in a premature infant.

BACKGROUND: Hereditary bone marrow failure syndromes are a category of biologically different syndromes that can cause cytopenia in at least one hematopoietic cell lineage. CASE: We present a 29-week-old male infant who had a low Apgar Score, advanced delivery room resuscitation, widespread petechial rash, and ecchymoses at birth, without any dysmorphic features. Initial laboratory tests revealed bicytopenia (platelet count 7x10 3 /uL, hemoglobin of 3.9 g/dL, neutrophil 2.0x103 /uL) with findings of disseminated intravasculer coagulation (DIC). Imaging studies demonstrated accompanying left-sided congenital pulmonary airway malformation. On the second postnatal week pancytopenia occurred and the bone marrow findings were consistent with congenital amegakaryocytic thrombocytopenia. Further evaluations for differential diagnosis of pancitopenia were performed and the results of congenital viral infections, metabolic and immunologic tests were negative. While supportive treatments were in progress, haploidentical bone marrow transplantation (BMT) was performed from the father at 84th day due to unavailability of HLA-matched relative or nonrelative donor. Whole exome sequencing revealed a novel heterozygous frameshift variation (c.1242dupT [p. Thr538fs]) in exon 8 of the MECOM gene and validated by Sanger sequencing. No variation was detected in the parents genetic analysis. CONCLUSIONS: In this report, we present a patient with congenital bone marrow failure successfully treated with haploidentic BMT and describe a novel, de novo pathogenic variant in MECOM gene.

Evaluation of paediatric immune thrombocytopenia patients with clinical and laboratory findings: emphasizing the role of monocytosis.

We aimed to investigate the relationship between demographics, clinical features, laboratory findings including monocytosis and clinical course in children with immune thrombocytopenia (ITP). Data of 100 ITP patients were analysed. Complete blood count findings of the patients at certain time points were evaluated to classify the disease as acute, persistent and chronic. An effect of sex on chronicity was not observed ( P  = 0.166). Of the patients enrolled in the study, 38% ( n  = 38) had chronic course. The mean age of patients with the chronic course was 7 ±â€Š4.1 years, which was significantly higher than the other groups ( P  = 0.007). Sixty-five percent ( n  = 13) of the patients presenting with mucosal bleeding and 27.4% ( n  = 20) of the patients presenting with skin bleeding became chronic ( P  = 0.008). MPV was found to be significantly high in chronic ITP patients ( P  = 0.049). Monocytosis was noted in 80% of the patients at diagnosis. Intravenous immunoglobulin was used in 84% of the patients with acute ITP; 33% of them developed chronic ITP. The age at diagnosis, presence of mucosal bleeding and increased MPV on admission were high-risk factors for the development of the chronic course. Monocytosis was detected in 80% of the patients on admission, and it may play a role in the pathogenesis of ITP.

Castleman Disease: A Multicenter Case Series from Turkey

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.

Assessment of Minimal Residual Disease in Childhood Acute Lymphoblastic Leukemia: A Multicenter Study From Turkey.

Assestment of minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) is of utmost importance both for risk classification and tailoring of the therapy. The data of pediatric ALL patients that received treatment with Berlin-Frankfurt-Münster (BFM) protocols were retrospectively collected from 5 university hospitals in Turkey. Of the 1388 patients enrolled in the study 390 were treated according to MRD-based protocols. MRD assestment was with real time quantitative polymerase chain reaction (qPCR) in 283 patients and with multiparametric flow cytometry (MFC)-MRD in 107 patients. MRD monitoring had upstaged a total of 8 patients (2%) from intermediate risk group to high-risk group. Univariate analysis revealed age 10 years or above, prednisone poor response, PCR-MRD ≥10-3 on day 33 and on day 78 as poor prognostic factors affecting event-free survival (EFS). Detection of >10% blasts on day 15 with MFC (MFC-high-risk group) was not shown to affect EFS and/or overall survival (log-rank P=0.339). Multiple logistic regression analysis revealed PCR-MRD ≥10-3 on day 78 as the only poor prognostic factor affecting EFS (odds ratio: 8.03; 95% confidence interval: 2.5-25; P=0.000). It is very important to establish the infrastructure and ensure necessary standardization for both MRD methods for optimal management of children with ALL.

Which Device Is Favorable for Intubation Attempts of Pediatric Residents on Four Different Pediatric Airway Simulations?

OBJECTIVES: Endotracheal intubation is an essential skill for the pediatric airway management. Although direct laryngoscopy (DL) is the standard method, several videolaryngoscopes (VLs) have been recently enhanced as an alternative especially for difficult intubations. We aimed to compare McGrath MAC (McG) and Storz C-MAC PM (ST) VLs with DL in terms of intubation success rate, time to intubation (TTI), and duration of obtaining glottis view of intubation attempts performed by pediatric residents on different pediatric airway manikins. METHODS: The pediatric residents with no experience in videolaryngoscopy were included. After a brief demonstration, intubation attempts with 3 randomly handled different devices with 6 different blades were performed on a child manikin, an infant manikin, a Pierre-Robin sequence infant manikin, and a child manikin with cervical immobilization sequentially. RESULTS: Fifty pediatric residents were enrolled. The attempts of DL on child manikin and attempts of all devices on child with cervical immobilization simulation were completely successful. For the attempts on both infant manikin and Pierre-Robin sequence infant manikin, the success rate of McG was significantly lower than ST and DL (P = 0.011 and P = 0.001). In the child manikin, McG and ST had prolonged TTI compared with DL (P = 0.016 and P = 0.001). For the child with cervical immobilization simulation, TTI of DL was significantly shorter than McG and ST (P = 0.011 and P = 0.001). Time to intubation of McG was significantly longer than DL and ST for the attempts on both 2 infant manikins. The rate of Cormack-Lehane grade I glottis view was similar for the attempts on both 2 child airway simulations. For infant manikin and Pierre-Robin sequence infant simulation, the rate of Cormack-Lehane grade I of ST was higher than attempts of DL and McG. CONCLUSIONS: The attempts of pediatric residents on infant normal airway and Pierre-Robin sequence infant airway simulations resulted with enhanced glottis view by the utilization of Storz C-MAC PM, but both 2 VLs did not provide the improvement of intubation success rate and TTI compared with DL on these 4 different pediatric manikins. Further clinical studies of different VLs in different clinical courses are required for a reliable utilization in children.

Evaluation of Possible Effects of the COVID-19 Pandemic on Febrile Neutropenia Episodes in Children With Acute Leukemia.

With the rapid spread of coronavirus disease 2019 (COVID-19) around the globe, concerns about the management of patients with malignancy have risen significantly. This study aimed to investigate the possible impact of the COVID-19 pandemic and prevention policies on the incidence and etiology of febrile neutropenia (FN) episodes in children with acute leukemia. Children who had acute leukemia and were diagnosed as FN in a tertiary center from March 2018 to March 2021 were included in the study. FN episodes were grouped as prepandemic and postpandemic based on the date that pandemic was declared. Relevant data were collected retrospectively. We evaluated 113 FN episodes (75.2% were prepandemic) of 46 patients, a median of 4.7 (2.6 to 12.6) years of age. The number of FN episodes per patient did not differ between prepandemic and postpandemic periods ( P =0.476). There was no significant difference among the 2 groups regarding the microbiologic causes, focus of fever, and clinical outcomes in FN episodes. Two of the patients were diagnosed as COVID-19 and recovered without any complications. In conclusion, we showed that the incidence and etiology of FN episodes were similar before and during the COVID-19 pandemic in children with acute leukemia.

Hepatitis-Associated Aplastic Anemia: Etiology, Clinical Characteristics and Outcome.

Hepatitis-associated aplastic anemia (HAA) is a form of acquired aplastic anemia (AA) in which bone marrow failure develops after an acute attack of hepatitis. Bone marrow failure leading to AA is generally severe in cases of HAA and fatal if left untreated. This retrospective multicenter study investigated clinical and laboratory characteristics, possible causes, treatment, and outcome of HAA in children. Twenty patients from 8 centers were included in the study. Aspartate aminotransferase and alanine aminotransferase were <3 to 5×upper limit of normal (ULN) in 2 patients, <5 to 10×ULN in 2 patients, and >10×ULN in 16 patients. Acute liver failure developed in 5 (29%) patients. Pancytopenia was simultaneously present in 6 of 20 (30%) patients. Eleven of the 20 patients (55%) were alive, in remission and transfusion free. Those who were alive either had undergone hematopoietic stem cell transplantation and/or immunosuppressive treatment, except 1 patient who had received no treatment. Patients with the diagnosis of acute hepatitis should be evaluated and followed up carefully for presence of cytopenia, so that definitive treatment of AA can be initiated in a timely and appropriate manner when needed.

Invasive Fungal Infections in Children with Leukemia: Clinical Features and Prognosis

Objective: The incidence of invasive fungal infections (IFIs) has increased due to intensive chemotherapy in childhood leukemia. The aim of this study was to evaluate the incidence, risk factors, causative pathogens, and impact on survival of IFIs among pediatric leukemia patients. Materials and Methods: The hospital records of 307 children with acute lymphoblastic leukemia (ALL, n=238), acute myeloid leukemia (AML, n=51), and relapsed leukemia (n=18) between January 2010 and December 2015 were retrospectively evaluated. Results: A total of 1213 febrile neutropenia episodes were recorded and 127 (10.4%) of them were related to an IFI. Of 307 children, 121 (39.4%) developed IFIs. The mean age was significantly older in the IFI group compared to children without IFIs (p<0.001). IFIs were defined as possible, probable, and proven in 73.2%, 11.9%, and 14.9% of the attacks, respectively. Invasive aspergillosis (81.9%) was the most frequent infection, followed by invasive candidiasis (13.4%) and rare fungal diseases (4.8%). The majority of IFI attacks in both ALL and AML occurred during the induction phase. In total, the death rate was 24% and the IFI-related mortality rate was 18%. The mortality rate among children with IFIs was found to be significantly higher than that of children without IFIs (p<0.001). Overall and event-free survival rates at 5 years were also found to be significantly lower in the IFI group (p<0.001). Relapse (odds ratio: 8.49) was the most effective risk factor for mortality, followed by developing an IFI episode (odds ratio: 3.2) and AML (odds ratio: 2.33) according to multivariate regression analysis. Conclusion: Our data showed that IFIs were more common in older children. Although proven and probable IFI episodes were more frequently diagnosed in cases of relapse and AML, children with ALL and AML had similar frequencies of experiencing at least one episode Conclusion: Our data showed that IFIs were more common in older children. Although proven and probable IFI episodes were more frequently diagnosed in cases of relapse and AML, children with ALL and AML had similar frequencies of experiencing at least one episode

Pediatric trauma: Blood product transfusion characteristics in a pediatric emergency department, a single center experience.

AIM: To investigate clinical and laboratory data, management and outcomes of pediatric trauma patients who initially received blood product transfusions. METHODS: Between January 2011-January 2021, traumatic children who underwent blood product transfusions within 24 h of arrival at the emergency department were included. Demographics, clinical and laboratory data, Injury Severity Score (ISS), volume of transfused blood products and crystalloid boluses in 24 h were recorded. Massive transfusion (MT) was defined as transfusion of ≥40 mL/kg of all blood products in 24 h. RESULTS: Among 32 cases, 8 (25.0 %) patients met the MT threshold criterion. Length of pediatric intensive care unit (PICU) stay and mechanical ventilation (MV) were longer for patients who received MT although there was no difference for age, ISS, volume of crystalloid boluses, length of hospital stay, and 30-day mortality between those who received MT or not. Volume of crystalloid boluses was higher in patients who died than those who survived but the volume of blood products was similar for two groups. An APTT value of >37.5 s was identified as a predictor of 30-day mortality (OR = 48.000, 95 % CI: 3.704-621.998, p: 0.003). CONCLUSION: Children who received MT had longer durations of MV and PICU stay than those who did not receive, but there was no significance for ISS, volume of crystalloid boluses, hospital stay, or mortality between two groups. Volume of crystalloid boluses was higher in patients who died than those who survived. An APTT value of >37.5 s can be used to predict 30-day mortality.