How significant is the BIG score in childhood traumatic brain injury?

BACKGROUND: This study aims to evaluate the reliability of the BIG score in predicting mortality in children with traumatic brain injury (TBI) and to compare it with the literature and other scoring systems. METHODS: Patients who were followed up in the Pediatric Intensive Care Unit (PICU) for TBI between 2014 and 2019 in a tertiary reference hospital were evaluated retrospectively. RESULTS: One hundred fifty-nine patients met the inclusion criteria. The most common injury mechanisms were falling from a height (39.6%). The mortality rate was 12.6% (n = 20). The mean BIG score, ISS, and PRISM III were statistically significantly higher in the mortality group (p < 0.001). The AUC values found in the ROC analysis in the whole study group, respectively, 0.962 (CI 0.920-0.986) for the BIG score, 0.952 (CI 0.906-0.979) for the ISS, 0.957 (CI 0.913-0.983) for the GCS, and 0.981 (CI 0.946-0.996) for the PRISM III. In the patients with isolated TBI, the AUC value for the BIG score was 0.988 (0.967-1.000) and higher than the ISS and PRISM 3 [0.983 (0.956-1.000), 0.969 (0.932-1.000) respectively]). The cut-off point for the BIG score in the whole group was 19 (sensitivity 95%, specificity 88%, positive predictive value 0.58, negative predictive value 0.99). In logistic regression model, we found that BIG score is an independent variable for mortality (AOR:1.4, 95%CI 1.22-1.63). CONCLUSION: In children with traumatic brain injury, the BIG score is simple, quickly calculated, and a good predictor of mortality and disease severity. Prospective studies with more extensive series are needed on this subject.

International Nosocomial Infection Control Consortium (INICC) report of health care associated infections, data summary of 45 countries for 2015 to 2020, adult and pediatric units, device-associated module.

BACKGROUND: Reporting on the International Nosocomial Infection Control Consortium study results from 2015 to 2020, conducted in 630 intensive care units across 123 cities in 45 countries spanning Africa, Asia, Eastern Europe, Latin America, and the Middle East. METHODS: Prospective intensive care unit patient data collected via International Nosocomial Infection Control Consortium Surveillance Online System. Centers for Disease Control and Prevention/National Health Care Safety Network definitions applied for device-associated health care-associated infections (DA-HAI). RESULTS: We gathered data from 204,770 patients, 1,480,620 patient days, 936,976 central line (CL)-days, 637,850 mechanical ventilators (MV)-days, and 1,005,589 urinary catheter (UC)-days. Our results showed 4,270 CL-associated bloodstream infections, 7,635 ventilator-associated pneumonia, and 3,005 UC-associated urinary tract infections. The combined rates of DA-HAIs were 7.28%, and 10.07 DA-HAIs per 1,000 patient days. CL-associated bloodstream infections occurred at 4.55 per 1,000 CL-days, ventilator-associated pneumonias at 11.96 per 1,000 MV-days, and UC-associated urinary tract infections at 2.91 per 1,000 UC days. In terms of resistance, Pseudomonas aeruginosa showed 50.73% resistance to imipenem, 44.99% to ceftazidime, 37.95% to ciprofloxacin, and 34.05% to amikacin. Meanwhile, Klebsiella spp had resistance rates of 48.29% to imipenem, 72.03% to ceftazidime, 61.78% to ciprofloxacin, and 40.32% to amikacin. Coagulase-negative Staphylococci and Staphylococcus aureus displayed oxacillin resistance in 81.33% and 53.83% of cases, respectively. CONCLUSIONS: The high rates of DA-HAI and bacterial resistance emphasize the ongoing need for continued efforts to control them.

Decreasing central line-associated bloodstream infections rates in intensive care units in 30 low- and middle-income countries: An INICC approach.

BACKGROUND: Central line (CL)-associated bloodstream infections (CLABSIs) occurring in the intensive care unit (ICU) are common and associated with a high burden. METHODS: We implemented a multidimensional approach, incorporating an 11-element bundle, education, surveillance of CLABSI rates and clinical outcomes, monitoring compliance with bundle components, feedback of CLABSI rates and clinical outcomes, and performance feedback in 316 ICUs across 30 low- and middle-income countries. Our dependent variables were CLABSI per 1,000-CL-days and in-ICU all-cause mortality rates. These variables were measured at baseline and during the intervention, specifically during the second month, third month, 4 to 16 months, and 17 to 29 months. Comparisons were conducted using a two-sample t test. To explore the exposure-outcome relationship, we used a generalized linear mixed model with a Poisson distribution to model the number of CLABSIs. RESULTS: During 1,837,750 patient-days, 283,087 patients, used 1,218,882 CL-days. CLABSI per 1,000 CL-days rates decreased from 15.34 at the baseline period to 7.97 in the 2nd month (relative risk (RR) = 0.52; 95% confidence interval [CI] = 0.48-0.56; P < .001), 5.34 in the 3rd month (RR = 0.35; 95% CI = 0.32-0.38; P < .001), and 2.23 in the 17 to 29 months (RR = 0.15; 95% CI = 0.13-0.17; P < .001). In-ICU all-cause mortality rate decreased from 16.17% at baseline to 13.68% (RR = 0.84; P = .0013) at 17 to 29 months. CONCLUSIONS: The implemented approach was effective, and a similar intervention could be applied in other ICUs of low- and middle-income countries to reduce CLABSI and in-ICU all-cause mortality rates.

Factors Affecting the Quality of Life of Parents Caring for Pediatric Patients with a Tracheostomy.

Objectives This study aimed to evaluate factors affecting the quality of life (QOL) of parents of children who underwent placement of a tracheostomy while in the pediatric intensive care unit (PICU) through postdischarge use of a standardized questionnaire, Functional Status Scale (FSS) for patients, and WHOQoL-BREF (a QOL scale) for parents. Methods The parents were initially contacted by telephone, postdischarge, during which the standardized questionnaire was completed. The functional status of the patients was evaluated using the FSS, and the QOL of parents was determined through use of the WHOQoL-BREF scale. Results From 2011 to 2021, tracheostomy was performed in 119 PICU patients. Overall, 93 patients were excluded due to death in 66 (56%), decannulation in 24 (20%) and, 3 (2%) were not available for follow-up. The parents of 26 (22%) patients were available for follow-up and for which the standardized questionnaire FSS and WHOQoL-BREF QOL scales were completed. The mean FSS score of the patients was elevated at 17.84. In comparison, reduced mean scores were observed for parental physical health of 20.61, psychological health of 20.57, social health of 11.15, and environmental health of 29.00. As a result, a moderate ( r < 0.80), yet significant ( p ≤ 0.004) negative correlation was found between the FSS scores of patients and the physical, social relationships, environmental, and psychological health QOL scores of parents. Conclusion This study is unique in that, to our knowledge, it is the first to compare parental QOL with the FSS of pediatric patients who have undergone a tracheostomy while hospitalized in the PICU. Our findings indicate that the parental QOL was reduced in four areas and correlates with an elevation in FSS score (indicating a greater functional disorder) of pediatric patients who had previously undergone a tracheostomy while hospitalized in the PICU.

Role of Albumin-corrected Anion Gap and Lactate Clearance in Predicting Mortality in Pediatric Intensive Care Patients

Background: Identifying mortality risk in critically ill children is central to diagnostic and treatment practices. For this purpose, scoring systems, such as the Pediatric Index of Mortality 3 (PIM 3), have been proposed; however, the role of biochemical markers, such as albumin-corrected anion gap (cAG) and lactate clearance (LC), in predicting mortality in pediatric intensive care unit (PICU) patients is yet to be explored. Aims: To evaluate the predictive value of the cAG and LC for mortality in pediatric patients admitted to a PICU. Study Design: Retrospective single-center cohort study. Methods: Clinical and laboratory data from the time of PICU admission were collected, and patients were classified into based on their 0- and 6-hour of admission lactate levels into an LC(+) group (patients with normal or decreasing lactate levels) or an LC(−) group (increasing lactate levels). LC and cAG levels were compared using the Mann-Whitney U test and Student's t-test, respectively. Additionally, multiple logistic regression analysis was performed to evaluate the effect of LC and cAG on mortality. Results: We included 825 patients in the study; the mortality rate was 8.6%. The absence of LC [adjusted odds ratio (AOR) =4.735; 95% confidence interval (CI): 2.163-10.367; p < 0.001], cAG (AOR =1.064; 95% CI: 1.010-1.122; p = 0.019) and PIM 3 (AOR = 1.871; 95% CI: 1.553-2.254; p < 0.001) were independent risk factors for mortality. Using the receiver operating characteristic curve analysis of PIM 3 as a predictor of mortality, area under the curve values of 0.832 (95% CI: 0.805-0.857; p < 0.001) for the original score and 0.858 for a revised PIM 3 score (based on the ß coefficients obtained for cAG and LC; 95% CI 0.832-0.881; p < 0.001) were obtained, which was significantly different (p = 0.027). Conclusion: A cAG value > 18 at the time of PICU admission high lactate levels which do not decrease within 6 hours of hospitalization are associated with an increased risk of mortality. The revised PIM 3 score, which includes cAG and LC, is a better predictor of mortality than the classical PIM 3 score.

Evaluation of nutritional status in pediatric intensive care unit patients: the results of a multicenter, prospective study in Turkey.

Introduction: Malnutrition is defined as a pathological condition arising from deficient or imbalanced intake of nutritional elements. Factors such as increasing metabolic demands during the disease course in the hospitalized patients and inadequate calorie intake increase the risk of malnutrition. The aim of the present study is to evaluate nutritional status of patients admitted to pediatric intensive care units (PICU) in Turkey, examine the effect of nutrition on the treatment process and draw attention to the need for regulating nutritional support of patients while continuing existing therapies. Material and Method: In this prospective multicenter study, the data was collected over a period of one month from PICUs participating in the PICU Nutrition Study Group in Turkey. Anthropometric data of the patients, calorie intake, 90-day mortality, need for mechanical ventilation, length of hospital stay and length of stay in intensive care unit were recorded and the relationship between these parameters was examined. Results: Of the 614 patients included in the study, malnutrition was detected in 45.4% of the patients. Enteral feeding was initiated in 40.6% (n = 249) of the patients at day one upon admission to the intensive care unit. In the first 48 h, 86.82% (n = 533) of the patients achieved the target calorie intake, and 81.65% (n = 307) of the 376 patients remaining in the intensive care unit achieved the target calorie intake at the end of one week. The risk of mortality decreased with increasing upper mid-arm circumference and triceps skin fold thickness Z-score (OR = 0.871/0.894; p = 0.027/0.024). The risk of mortality was 2.723 times higher in patients who did not achieve the target calorie intake at first 48 h (p = 0.006) and the risk was 3.829 times higher in patients who did not achieve the target calorie intake at the end of one week (p = 0.001). The risk of mortality decreased with increasing triceps skin fold thickness Z-score (OR = 0.894; p = 0.024). Conclusion: Timely and appropriate nutritional support in critically ill patients favorably affects the clinical course. The results of the present study suggest that mortality rate is higher in patients who fail to achieve the target calorie intake at first 48 h and day seven of admission to the intensive care unit. The risk of mortality decreases with increasing triceps skin fold thickness Z-score.

Acute Fulminant Hepatic Failure Caused by Sevoflurane Linked to Influenza A.

Sevoflurane is a volatile anesthetic agent that does not tend to cause clinically significant hepatotoxicity, but there are some reported hepatotoxicity cases in the literature. In the case presented here, adenotonsillectomy was performed during influenza infection, and sevoflurane was administered, after which acute fulminant hepatitis developed. At hour 24 of hospitalization after fulminant hepatic failure, liver transplant was performed in a 3.5-year-old patient without any known diseases. In such cases, etiology investigations should be planned, life support therapy should be administered, and information should be given to the patient to avoid exposure to sevoflurane in the future.

Posttraumatic epilepsy in critically ill children with traumatic brain injury.

PURPOSE: The aim of this study was to determine the clinical, laboratory, and radiological factors related with posttraumatic epilepsy (PTE). METHODS: The study is a multicenter descriptive cross-sectional cohort study. Children who followed up for TBI in the pediatric intensive care unit between 2014 and 2021 were included. Demographic data and clinical and radiological parameters were recorded from electronic case forms. All patients who were in the 6-month posttraumatic period were evaluated by a neurologist for PTE. RESULTS: Four hundred seventy-seven patients were included. The median age at the time of trauma was 66 (IQR 27-122) months, and 298 (62.5%) were male. Two hundred eighty (58.7%) patients had multiple traumas. The mortality rate was 11.7%. The mean duration of hospitalization, pediatric intensive care unit hospitalization and mechanical ventilation, Rotterdam score, PRISM III score, and GCS at admission were higher in patients with epilepsy (p < 0.05). The rate of epilepsy was higher in patients with severe TBI, cerebral edema on tomography and clinical findings of increased intracranial pressure, blood transfusion in the intensive care unit, multiple intracranial hemorrhages, and intubated patients (p < 0.05). In logistic regression analysis, the presence of intracranial hemorrhage in more than one compartment of the brain (OR 6.13, 95%CI 3.05-12.33) and the presence of seizures (OR 9.75, 95%CI 4.80-19.83) were independently significant in terms of the development of epilepsy (p < 0.001). CONCLUSIONS: In this multicenter cross-sectional study, intracranial hemorrhages in more than one compartment and clinical seizures during intensive care unit admission were found to be independent risk factors for PTE development in pediatric intensive care unit patients with TBI.

Serum Zonulin Levels in Pediatric Migraine.

BACKGROUND: Migraine is a complex neurogenic inflammatory disorder. There are strong neuronal, endocrine, and immunologic connections between the brain and gastrointestinal system. Damage to the intestinal barrier is thought to cause systemic immune dysregulation. Zonulin is a protein produced by the small intestine epithelium in humans that regulates intestinal permeability through intracellular tight junctions and is a potential marker for inflammation. Zonulin increases in positive correlation with permeability. In our study, we aimed to research the correlation between serum zonulin levels in the period between attacks in pediatric patients with migraine. METHODS: The study included 30 patients with migraine and 24 healthy controls, matched in terms of sex and age. Demographic and clinical characteristics were recorded. Serum zonulin levels were studied with the enzyme-linked immunosorbent assay method. RESULTS: Patients had a mean of 5.6 ± 3.5 attacks per month. The mean serum zonulin was 5.68 ± 1.21 ng/mL in the migraine group and 5.72 ± 2.1 ng/mL in the control group with no significant difference found (P = 0.084). In the migraine group, no correlations were identified between serum zonulin levels and age, body mass index, pain frequency, pain duration, onset time, visual analog scale score, and presence of gastrointestinal systems apart from nausea-vomiting. CONCLUSIONS: More than 50 proteins were identified to affect the intestinal permeability apart from zonulin. There is a need for prospective studies encompassing the time of attack, but our study is important as it is the first study about zonulin levels in pediatric migraine.

The role of height and weight growth in spontaneous resolution of vesicoureteral reflux.

BACKGROUND: The aim of this study was to investigate the role of height and weight growth in the resolution of primary vesicoureteral reflux (VUR). METHODS: We retrospectively evaluated the data of 74 males and 135 females who were diagnosed with primary VUR. According to the vesicoureteral reflux resolution, patients were divided into two groups. Patients with complete or partial resolution of VUR were included in Group 1 and patients with no resolution of VUR were included in Group 2. Patients were evaluated for weight Z-score, height Z-score and body mass index at the diagnosis and in the follow-up. In addition, age, sex, grade of reflux and laterality were recorded. RESULTS: There were no significant differences between the two groups, according to height Z-score, weight Z-score and body mass index at the diagnosis, in the follow-up and also annual changes of these parameters. In addition, the same parameters did not significantly differ in Groups 1 and 2, between the initial and final evaluations. However, when we evaluate the patients older than 12 months old, weight Z-scores were significantly higher at the final evaluation than at the diagnosis, in Group 1. This significant difference was not detected in Group 2 at the same age. CONCLUSIONS: Although we could not detect the hectic pace of height growth as being important in the prediction of long-term outcomes of VUR, weight growth should be considered to predict the resolution of VUR.

Determinants of Quality of Life after Pediatric Traumatic Brain Injury.

Introduction Pediatric traumatic brain injury (TBI) is a significant cause of death and long-term disability. There is a paucity of data on quality of life in survivors of pediatric TBI. The aim of this study is to determine the factors affecting the quality of life after TBI in children. Methods Consecutively admitted 104 of 156 patients to the pediatric intensive care unit (PICU) with TBI between 1 month and 18 years were included in the study. Demographics were obtained from electronic records. Injury severity and mortality scores were calculated. The Pediatric Quality of Life Inventory (PedsQL) scale and Glasgow Outcome Scale (GOS) score were evaluated by interview with patient or the caregiving parents. The Rotterdam computed tomography (CT) score was calculated from the radiology images taken within the first 24 hours after admission to the emergency service. Results Severe TBI, multiple trauma, intracranial hemorrhage from multiple sites, convulsions, high intracranial pressure, emergency operation on admission, and hypotension on admission were associated with low PedsQL values according to results of univariate analysis ( p < 0.05). There was a negative correlation between PedsQL and GOS, mechanical ventilation duration, PICU length of stay (LOS), and hospital LOS. In the linear regression model made by considering the univariate analysis results, it was shown that Rotterdam CT score and PICU LOS are independent variables that determine low PedsQL score. PedsQL scores were lower in children ≥ 8 years of age and in those evaluated within the first year after discharge ( p = 0.003). Conclusion In pediatric TBI, Rotterdam CT score and PICU LOS were found as independent variables determining PedsQL score after discharge.

Intestinal Mucormycosis in a Child With Maple Syrup Urine Disease After Orthotopic Liver Transplant.

Mucormycosis can result in serious morbidity and mortality, especially in transplant recipients. In this case report, we present a 3-year-old female patient with maple syrup urine disease who developed mucormycosis infection after deceased donor split liver transplant. Progressive segmental necrosis of the small intestines and new ischemic areas were observed after repeated abdominal surgeries. Microscopic examination of biopsy material revealed mucormycosis. Early recognition is crucial for treatment, and patients with clinical suspicion can be treated empirically with antifungal medicine. However, diagnostic tests with accurate and fast results are needed and more effective therapeutic methods should be developed for better outcomes.

Clinical characteristics of firearm-related injuries in children in Turkey.

BACKGROUND: A significant number of children are injured by or die from firearm-related incidents every year, although there is a lack of global data on the number of children admitted to pediatric emergency departments (PEDs) and pediatric intensive care units (PICU) with firearm injuries. This study is the most comprehensive analysis of firearm injuries sustained by children in Turkey to date. METHODS: This multicenter, retrospective, cohort study was conducted between 2010 and 2020 with the contributions of the PEDs, PICUs, intensive care units, and surgery departments of university hospitals and research hospitals. RESULTS: A total of 508 children were admitted to hospital with firearm-related injuries in the research period, although the medical records of only 489 could be obtained. Of the total admissions to hospitals, 55.0% were identified as unintentional, 8.2% as homicide, 4.5% as self-harm, and 32.3% as undetermined. The Glasgow Coma Scale (GCS) and ventilation support were found to be the most significant predictors of mortality, while head/neck injury, length of stay (LOS) in the hospital and surgical interventions were found to be the most significant predictors of disability. The overall mortality of firearm-related injuries was 6.3%, and the mortality for children admitted to the PICU was 19.8%. The probability of disability was calculated as 96.0% for children hospitalized with firearm injuries for longer than 75 days. CONCLUSIONS: Head/neck injury, LOS in the hospital, and surgical interventions were found to be the most significant parameters for the prediction of disability. Hospitalization exceeding 6 days was found to be related to disability.

Combined immunoglobulin and plasmapheresis treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES).

Febrile infection-related epilepsy syndrome (FIRES) is a rarely observed and destructive syndrome progressing with resistant seizures or refractory status epilepticus. In this report we present in a treatment procedure with plasmapheresis of a pediatric patient with FIRES and currently unknown etiology in order to contribute to the literature.

Neuroimaging Findings in Pediatric Patients with Thalassemia Major.

BACKGROUND: Cranial magnetic resonance imaging (MRI) studies about iron accumulation in children with thalassemia major are quite limited. AIM: This study aimed to detect neurological findings with cranial MRIs in the pediatric patients with thalassemia major who did not develop any neurological complications. MATERIALS AND METHODS: Pediatric patients with thalassemia major who followed in the Pediatric Hematology Unit between 1 July 2017 and 1 January 2019 were included in the study. The patients underwent cranial MRI scans. RESULTS: A total of 30 patients were included. The median age was 15 (range from 4-18) years old. We found that 7 patients had a splenectomy and 19 of the remaining 23 patients had splenomegaly. In addition, 13 of the patients had hepatomegaly, 10 had skeletal deformities, and 17 had growth retardation. The mean ferritin level was 3772.3 ± 2524.8. We detected various pathologies on cranial MRI images of 10 (33.3%) patients. In 3 of these patients, millimeter-sized ischemia-compatible lesions were found in the cerebral white matter, which did not fit any arterial area, and 5 patients had hyperintense lesions in the basal ganglia. CONCLUSION: Our study is valuable since 1/3 of our pediatric patients with thalassemia major were detected with intracranial pathology.

The skills of defibrillation practice and certified life-support training in the healthcare providers in Turkey.

AIM OF THE STUDY: Successful cardiopulmonary resuscitation and early defibrillation are critical in survival after in- or out-of-hospital cardiopulmonary arrest. The scope of this multi-centre study is to (a) assess skills of paediatric healthcare providers (HCPs) concerning two domains: (1) recognising rhythm abnormalities and (2) the use of defibrillator devices, and (b) to evaluate the impact of certified basic-life-support (BLS) and advanced-life-support (ALS) training to offer solutions for quality of improvement in several paediatric emergency cares and intensive care settings of Turkey. METHODS: This cross-sectional and multi-centre survey study included several paediatric emergency care and intensive care settings from different regions of Turkey. RESULTS: A total of 716 HCPs participated in the study (physicians: 69.4%, healthcare staff: 30.6%). The median age was 29 (27-33) years. Certified BLS-ALS training was received in 61% (n = 303/497) of the physicians and 45.2% (n = 99/219) of the non-physician healthcare staff (P < .001). The length of professional experience had favourable outcome towards an increased self-confidence in the physicians (P < .01, P < .001). Both physicians and non-physician healthcare staff improved their theoretical knowledge in the practice of synchronised cardioversion defibrillation (P < .001, P < .001). Non-certified healthcare providers were less likely to manage the initial doses of synchronised cardioversion and defibrillation: the correct responses remained at 32.5% and 9.2% for synchronised cardioversion and 44.8% and 16.7% for defibrillation in the physicians and healthcare staff, respectively. The indications for defibrillation were correctly answered in the physicians who had acquired a certificate of BLS-ALS training (P = .047, P = .003). CONCLUSIONS: The professional experience is significant in the correct use of a defibrillator and related procedures. Given the importance of early defibrillation in survival, the importance and proper use of defibrillators should be emphasised in Certified BLS-ALS programmes. Certified BLS-ALS programmes increase the level of knowledge and self-confidence towards synchronised cardioversion-defibrillation procedures.

Associations Between Transcranial Doppler Flow Velocities, Laboratory Parameters, and Clinical Features in Turkish Children With Sickle Cell Disease.

OBJECTIVE: Stroke is one of the major complications of sickle cell disease (SCD). Stroke features either occlusion of, or stenosis at, the origin of one of the large intracerebral arteries, the internal carotid artery (ICA), and/or the middle cerebral artery (MCA). PURPOSE: We sought correlations between cerebral blood flow velocities and the laboratory and clinical findings of patients with SCD. MATERIALS AND METHODS: Fifty-three pediatric SCD patients (39 with HbSS, 14 with HbSß0) were analyzed. The mean patient age was 12.9±3.9 years. The control group contained 24 healthy individuals. The time-averaged maximum mean velocity (TAMMV) and resistive index (RI) of the MCA, the TAMMVs of the ICA and vertebral artery (VA), and the diameter of the VA were estimated through transcranial Doppler ultrasonography using a 2.5 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. We evaluated the relationships between the TAMMVs, laboratory parameters, and clinical findings. RESULTS: The mean±SD MCA TAMMV was 161.2±35.4 cm/s in patients with HbSS and 185.8±62.9 cm/s in patients with HbSß0. The mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 168.5±43.9 cm/s, 0.63±0.06, 116.8±25.5 cm/s, 69.2±18.5 cm/s, and 3.5±0.61 mm for all SCD patients, respectively. In the control group, the mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 103.8±28.8 cm/s, 0.53±0.04, 96.4±27.8 cm/s, 40.3±12.1 cm/s, and 3.4±0.6 mm, respectively. Although the differences were not significant, TAMMVs were higher in HbSß0 patients taking hydroxyurea; those with hemoglobin levels <8 g/dL, ferritin levels >1000 ng/dL, mean platelet volume >12 fL, or red cell distribution width >18%; or required chelation, or were below the third percentiles of weight and height. The TAMMV was significantly higher only in SCD patients who complained of headache. CONCLUSIONS: High ferritin and low hemoglobin levels, a high mean platelet volume, a high red cell distribution width, low weight (below the third percentile), and a short height (below the third percentile) may be associated with elevated cerebral blood flow velocities and an increased stroke risk in children with SCD. Children with such features should be closely followed-up through transcranial Doppler ultrasonography examination.

Evaluation of GLUT1, IGF-2, VEGF, FGF 1, and angiopoietin 2 in infantile hemangioma.

INTRODUCTION: Infantile hemangioma (IH) is a common vascular tumor in children. It is reported that IHs are associated with immunochemical markers such as vascular endothelial growth factor (VEGF)-A, glucose transporter isoform 1 (GLUT1), and insulin-like growth factor-2 (IGF-2). MATERIAL AND METHODS: This cross-sectional study focused on pediatric patients with IH. A total of 46 patients (mean age 14.2±21.9 months) with IH and 45 healthy controls (mean age 21.8±15.08 months) were enrolled. Demographic data, clinical findings, and laboratory parameters were recorded. Blood samples were collected. Serum GLUT1, IGF-2, VEGF-A, fibroblast growth factor 1 (FGF1), and angiopoietin 2 levels were assessed by enzyme-linked immunosorbent assay. RESULTS: Serum GLUT1, IGF-2, and VEGF-A levels were significantly higher in patients with IH than in healthy controls (8.80±4.07pg/mL vs. 5.66±4.34pg/mL, 281.10±84.12pg/mL vs. 234.19±75.38pg/mL, 1196.99±389.34pg/mL vs. 996.99±349.16pg/mL, respectively, p=0.026, p=0.030, and p=0.036). Serum GLUT1, IGF-2, and VEGF-A levels in patients with complicated hemangioma were significantly higher than in healthy controls (9.69±3.94pg/mL vs. 5.66±4.34pg/mL, 289.94±83.18pg/mL vs. 234.19±75.38pg/mL, 1276.22±388.24pg/mL vs. 996.99±349.16pg/mL, respectively, p=0.017, p=0.022, and p=0.011). Serum GLUT1, IGF-2, and VEGF-A levels in patients with hemangioma receiving propranolol treatment were significantly higher than in healthy controls. Serum FGF1 levels were higher in patients with IH, complicated hemangioma, and hemangioma receiving propranolol treatment than in healthy controls but the difference was not statistically significantly. CONCLUSION: Serum GLUT1, IGF-2, and VEGF-A levels were positively correlated with disease severity in patients with hemangioma, for example, in complicated hemangioma and hemangioma requiring propranolol treatment. However, further research on larger and different age subgroups is warranted to assess these markers.

Assessment of PELOD-2 and PIM-3 scores of children coming from the war in Syria.

OBJECTIVES: Since the civil war in Syria began, millions of Syrians have left the country and been forced to migrate to other countries. Turkey is the country with the most refugees hosting 3.6 million refugees. This study aimed to compare the PIM-3 score, PELOD-2 score, PELOD-2 predicted death rate (PDR), mortality rates, demographic data, and outcomes of patients admitted to pediatric intensive care units between refugee children living in Turkey, pediatric patients brought directly from the border by the emergency services, and the general Turkish population. METHODS: This was a retrospective study performed between February 2018 and February 2019 at Hatay State Hospital, very close to the Syrian border. The study included 158 patients. Patients were divided into three groups: Turkish citizens, those living in Turkey as refugees, and those brought from the border. RESULTS: Of the patients, 57 were Turkish citizens, 33 were refugees, and 68 were brought from the border. For patients, the mean PIM-3 score was 25.62±27.70, the PELOD-2 score was 8.03±4.72, and PELOD2-PDR was 16.07±23.45. The median scores for PIM-3, PELOD-2, and PELOD2-PDR of patients brought from the Syrian border were higher compared with Turkish citizens and refugees. There was no significant difference between refugees and Turkish citizens. Of the patients, 27 died, with the distribution being 15% Turkish citizens, 26% refugees, and 59% brought from the border. The mortality of patients transported from the border was statistically significant (P=0.03). CONCLUSION: We consider that the source of the difference between patients brought from the border and those living in Turkey may be associated with the continuing war beyond our borders and children experiencing insufficient care conditions. In conclusion, it is not just weapons that cause death in war, and children unfortunately suffer because of this situation.

Acidemia propiónica: una causa sumamente rara de linfohistiocitosis hemofagocítica en un lactante / Propionic acidemia: an extremely rare cause of hemophagocytic lymphohistiocytosis in an infant

La linfohistiocitosis hemofagocítica (LHH) puede ser primaria (hereditaria) o secundaria a infecciones, tumores malignos, trastornos reumatológicos, síndromes de inmunodeficiencia y metabolopatías. Se informaron casos de intolerancia a la proteína lisinúrica, deficiencia de múltiples sulfatasas, galactosemia, enfermedad de Gaucher, síndrome de Pearson y galactosialidosis. No se sabe cómo se desencadena la LHH en las metabolopatías. Se diagnosticó LHH en un lactante de 2 meses con letargo, palidez, alimentación deficiente, hepatoesplenomegalia, fiebre y pancitopenia, y se instauró el protocolo HLH-2004. Se realizaron, en conjunto, análisis para detectar mutaciones genéticas y pruebas metabólicas; los resultados fueron negativos para las mutaciones genéticas de LHH primaria, pero se detectaron hiperamoniemia y concentración elevada de metilcitrato. Se diagnosticó acidemia propiónica. Aquí informamos sobre un caso de LHH secundaria a acidemia propiónica. Es posible la realización simultánea de pruebas de detección de trastornos metabólicos y de mutaciones genéticas para el diagnóstico temprano en los lactantes con LHH

Hemophagocytic lymphohystiocytosis (HLH) may be primary (inherited/familial) or secondary to infections, malignancies, rheumatologic disorders, immune deficiency syndromes and metabolic diseases. Cases including lysinuric protein intolerance, multiple sulfatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis have previously been reported. It is unclear how the metabolites trigger HLH in metabolic diseases. A 2-month-old infant with lethargy, pallor, poor feeding, hepatosplenomegaly, fever and pancytopenia, was diagnosed with HLH and the HLH-2004 treatment protocol was initiated. Analysis for primary HLH gene mutations and metabolic screening tests were performed together; primary HLH gene mutations were negative, but hyperammonemia and elevated methyl citrate were detected. Propionic acidemia was diagnosed with tandem mass spectrometry in neonatal dried blood spot. We report this case of HLH secondary to propionic acidemia. Both metabolic disorder screening tests and gene mutation analysis may be performed simultaneously especially for early diagnosis in infants presenting with HLH.