Evaluation of pediatric trauma score and pediatric age-adjusted shock index in pediatric patients admitted to the hospital after an earthquake.

BACKGROUND: In our earthquake-prone country, it is crucial to gather data from regional hospitals following earthquakes. This information is essential for preparing for future disasters and enhancing healthcare services for those affected by earthquakes. This study aimed to evaluate the Pediatric Trauma Score (PTS) and the Shock Index, Pediatric Age-Adjusted (SIPA), in children affected by earthquakes, to provide clinicians with insights into the severity of trauma and hemodynamic stability. METHODS: The study included patients admitted to our hospital's pediatric emergency service within the three weeks following the earthquake. We evaluated their age, sex, admission vital signs, mechanical ventilation requirements, development of crush syndrome, length of hospital stay, PTS, and SIPA. RESULTS: Our study included 176 children (89 females and 87 males) with trauma. Fifty-eight (32.95%) children had crush syndrome, and 87 (49.43%) were hospitalized. The median PTS was 10 (ranging from -3 to 12), and the median SIPA was 1.00 (ranging from 0.57 to 2.10). We observed a negative correlation between the time spent under debris and PTS (r=-0.228, p=0.002) and a positive correlation with the SIPA score (r=0.268, p<0.001). The time spent under debris (p<0.001) and SIPA score (p<0.001) were significantly higher in hospitalized children. PTS was significantly lower in hospitalized children than in others. A PTS cutoff point of 7.5, and a SIPA cutoff point of 1.05, predicted hospitalization in all children. Time spent under debris and SIPA were significantly higher in children with crush syndrome than in others (p<0.001). PTS at a cutoff point of 8.5 and SIPA at a cutoff point of 1.05 predicted crush syndrome in all children. CONCLUSION: PTS and SIPA are important practical scoring systems that can be used to predict the severity of trauma, hospitalization, crush syndrome, and the clinical course in pediatric patients admitted to the hospital due to earthquake trauma.

Evaluation of Intestinal Microbiota in Children With Sickle Cell Disease.

BACKGROUND AND AIMS: Sickle cell disease (SCD) is a chronic hemolytic anemia that may be life-threatening due to multisystemic effects. Identification of the factors which affect the pathophysiology of the disease is important in reducing mortality and morbidity. This study aimed to determine gut microbial diversity in children and adolescents with SCA compared with healthy volunteers and to evaluate the clinical impact of microbiota. MATERIALS AND METHODS: The study included 34 children and young adolescents with SCD and 41 healthy volunteer participants. The microbiome was assessed by 16S rRNA sequencing in stool samples. Laboratory parameters of all participants, such as complete blood count and C-reactive protein values and clinical characteristics of SCD patients, were determined and compared, as well as clinical conditions of the patients, such as vascular occlusive crisis and/or acute chest syndrome, frequency of transfusions, intake of penicillin, hydroxyurea, and chelation therapy were recorded. RESULTS: White blood cell count, hemoglobin, immature granulocyte and C-reactive protein levels were significantly higher in the patient group ( P <0.05). Microbiota analysis revealed 3 different clusters among subjects; controls and 2 clusters in the SCD patients (patient G1 and G2 groups). Bacteroides spp. were more prevalent, while Dialester spp. and Prevotella spp. were less prevalent in SCD compared with controls ( t =2.142, P <0.05). Patient G2 (n=9) had a higher prevalence of Bacteroides and a lower prevalence of Prevotella than patient G1 (n=25). CONCLUSION: In our study, there was a difference between SCD patients and the control group, while 2 different microbiota profiles were encountered in SCD patients. This difference between the microbiota of the patients was not found to affect the clinical picture (such as vascular occlusive crisis, acute chest syndrome).

Thromboprophylaxis in pediatric patients with earthquake-related crush syndrome: a single centre experience.

PURPOSE: Injuries increase the risk of venous thromboembolism (VTE). However, the literature on the management of anticoagulant therapy in pediatric patients with crush injury is limited. In this study, we aimed to share our experience about anticoagulant thromboprophylaxis in pediatric patients with earthquake-related crush syndrome. METHODS: This study included patients who were evaluated for VTE risk after the Turkey-Syria earthquake in 2023. Since there is no specific pediatric guideline for the prevention of VTE in trauma patients, risk assessment for VTE and decision for thromboprophylaxis was made by adapting the guideline for the prevention of perioperative VTE in adolescent patients. RESULTS: Forty-nine patients [25 males and 24 females] with earthquake-related crush syndrome had participated in the study. The median age of the patients was 13.5 (8.8-15.5) years. Seven patients (14.6%) who had no risk factors for thrombosis were considered to be at low risk and did not receive thromboprophylaxis. Thirteen patients (27.1%) with one risk factor for thrombosis were considered to be at moderate risk and 28 patients (58.3%) with two or more risk factors for thrombosis were considered to be at high risk. Moderate-risk patients (n = 8) and high-risk patients aged < 13 years (n = 11) received prophylactic enoxaparin if they could not be mobilized early, while all high-risk patients aged ≥ 13 years (n = 13) received prophylactic enoxaparin. CONCLUSION: With the decision-making algorithm for thyromboprophylaxis we used, we observed a VTE rate of 2.1% in pediatric patients with earthquake-related crush syndrome.

Bone densitometry measurements in children with neurofibromatosis Type 1 using quantitative computed tomography.

Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease characterised by multisystemic involvement, including bone tissue. Deformities and reduced bone mass are the main bone manifestations in NF1. Quantitative computed tomography (QCT) provides true volumetric bone mineral density (BMD) measurement. This study aimed to evaluate bone metabolism parameters and BMD in children with NF1 using QCT. Methods: The data of 52 paediatric NF1 patients (23 female, 29 male) was evaluated retrospectively. We investigated anthropometric measurements, biochemical parameters like total calcium, phosphate, magnesium, alkaline phosphatase, 25-hydroxyvitamin D (25OHD), parathyroid hormone, calcitonin, urinary calcium/creatinine ratio, and QCT parameters like lumbar trabecular and cortical BMD, trabecular area and cortical thickness. Comparisons of gender and puberty status were performed. Results: 25% of patients had skeletal deformities and 42.3% had 25OHD inadequacy (<20 ng/mL). The frequency of 25OHD inadequacy was significantly higher in pubertal/postpubertal patients than prepubertal patients (61.9% vs. 29.0%, P = 0.019). Trabecular BMD Z-score was <-2.0 in 11.5% of patients; all with low BMD were at the pubertal/postpubertal stage. There was a significant negative correlation between age and trabecular Z-score (r = -0.41, P = 0.003). Mean cortical BMD was statistically similar between the genders and puberty groups. Puberty status, anthropometric Z-scores, and biochemical and QCT parameters were statistically similar between the genders (P > 0.05). Conclusion: Paediatric NF1 patients may present with low BMD and 25OHD inadequacy, especially at puberty. QCT may be a useful tool to evaluate trabecular and cortical bone separately in NF1 patients.

El uso excesivo de pantallas está asociado con labilidad emocional en niños preescolares / Excessive screen time is associated with emotional lability in preschool children

Introducción. En estudios anteriores, el uso excesivo o la exposición temprana a pantallas se asoció con atención deficiente, falta de control de la conducta, retraso del lenguaje y déficit en la función ejecutiva. El objetivo de este estudio fue investigar la relación entre el tiempo de uso de pantallas y la regulación emocional, que afecta las relaciones sociales de los niños de 2 a 5 años.Población y métodos. Estudio descriptivo transversal en un hospital universitario del 1.º de enero al 1.º de marzo de 2018. Se incluyó a madres de niños sanos de 2 a 5 años con un uso de pantallas inferior a 1 hora o superior a 4 horas. A quienes aceptaron participar se les administró una encuesta estructurada y la Emotion Regulation Checklist para padres.Resultados. De los 240 niños participantes, 98 (el 40,8 %) tenían un uso de pantallas ≥ 4 horas. Ser cuidado por la madre, tener 12 meses o más durante la primera exposición y no estar acompañado por los padres al usarlas se asociaron con ≥ 4 horas de uso de pantallas (p = 0,002; p = 0,002; p = 0,012, respectivamente). La proporción de participantes con una puntuación alta de labilidad/negatividad (L/N) fue significativamente mayor entre los niños con ≥ 4 horas de uso de pantallas y que no estaban acompañados por sus padres al usarlas (p = 0,004; p = 0,033, respectivamente).Conclusiones. Este estudio determinó que un uso excesivo de pantallas se asocia con labilidad emocional durante esta etapa temprana de la infancia.

Introduction. Previous studies have found that excessive screen time or early screen exposure is associated with poor attention, lack of behavioral control, delayed language and deficit in executive functions. The aim of this study was to investigate the relationship between screen time and emotion regulation skills, which is one of the important life components affecting the social relations of children aged 2 to 5 years.Population and methods.This cross-sectional descriptive study was carried out in a university hospital between January 1, 2018 and March 1, 2018. Mothers of healthy children aged 2-5 years with a daily screen time of less than 1 hour or over 4 hours were included in the study. A structured survey and the Emotion Regulation Checklist for parents were applied to the mothers who agreed to participate.Results. Of 240 children participating in the study, 98 (40.8 %) had ≥4 hours of screen time. Caring by mother, age at first screen exposure 12 months and older, not co-viewing with parents were found to be associated with ≥ 4 hours of screen time (p = 0.002, p = 0.002, p = 0.012, respectively). The ratio of participants with high-lability/negativity (L/N) score was significantly higher in children with screen time of ≥ 4 hours and not co-viewing with parents (p = 0.004, p = 0.033, respectively).Conclusions. This study investigating the relationship between the emotion regulation skill and screen time revealed that excessive screen time is associated with emotional lability in this early childhood period.

Bone marrow involvement in pediatric malignancies: a comparison study of Positron emission tomographycomputed tomography and bone marrow biopsy.

BACKGROUND AND OBJECTIVES: The comparison of Positron emission tomography- computed tomography (PETCT) and bone marrow biopsy (BMB) modalities in detecting bone marrow disease is an up to date research topic. In this study, we aimed to compare the results of PET-CT and BMB procedures in detecting bone marrow involvement in pediatric malignancies. METHOD: At the time of diagnosis, PET-CT imaging and BMB performed patients` data were evaluated, retrospectively. Malign diagnoses were Hodgkin's lymphoma in 23 (30.7%), non-Hodgkin's lymphoma in 20 (26.7%), neuroblastoma in 11 (14.7%), Ewing sarcoma in 10 (13.7%), Langerhans cell histiocytosis in 6 (8%), and rhabdomyosarcoma in 5 (6.6%) patients. RESULTS: Bone marrow involvement was detected in 39 (52%) of 75 patients. Bone marrow involvement was identified by both PET-CT and BMB in 18 (46.1%) patients, by only PET-CT in 12 (30.7%) patients, by only BMB in 9 (23%) patients. The sensitivity of PET-CT was 66%, specificity was 75%, positive predictive value was 60%, and negative predictive value was 80%. Sensitivity, spesificity, positive and negative predictive values of PETCT were different in before mentioned malignancy groups. CONCLUSION: PET-CT may not have high sensitivity and specificity to identify bone marrow involvement for each type of cancer. The approach of using bone marrow biopsy and PET-CT as complementary modalities seems reliable.

Effective High-dose Interferon-α Therapy in a 13-Year-Old Girl With Erdheim-Chester Disease.

Erdheim-Chester disease (ECD) is a proliferative disorder of non-Langerhans histiocytes with a higher incidence in the fifth to seventh decades and rarer occurrence in the pediatric population. Although ECD typically involves bone, it can also affect the central nervous system, cardiovascular system, retro-orbital space, retroperitoneal space, and kidneys, lungs, and skin. A 13-year-old Syrian girl who presented with multisystemic involvement was diagnosed with ECD. The B-Raf proto-oncogene V600E mutation was not detected in ECD lesions. Response to the high-dose interferon-α therapy was excellent in this pediatric patient. In this article, pediatric ECD case reports are also reviewed.

A novel mutation of the transcobalamin II gene in an infant presenting with hemophagocytic lymphohistiocytosis.

Transcobalamin II (TC II) deficiency is a rare disorder of cobalamin (CBL, vitamin B12) metabolism that occurs due to mutations in transcobalamin gene (TCN2). Hemophagocytic lymphohistiocytosis (HLH) in contrast is a syndrome characterized by uncontrolled immune response with hyperinflammation. A 2-month-old male baby was admitted with complaints of fever, cough, diarrhea, and respiratory distress. The parents were first cousins. The baby exhibited five of the eight diagnostic criteria for HLH-2004 and was diagnosed as HLH. A second bone marrow aspiration demonstrated megaloblastic changes in the erythroid series. The patient's vitamin B12 level was normal; however, hyperhomocysteinemia was present. A genetic deficiency of TC II was suspected. The patient and his parents were tested for TCN2 mutation. He had a homozygote mutation that was not included in Human 'Gene Mutation Database Cardiff'. The patient was treated with intramuscular vitamin B12, which was followed by improvement in both clinical and laboratory findings. He was 12 months old at the time of this report, with normal physical and neuromotor development. In this case presenting with the clinical and laboratory findings of HLH, TC II deficiency was diagnosed. A new mutation was found that was not reported before. Potential causative mechanisms of HLH induced by defects of cobalamin synthesis merit further investigation.