Pediatric Patients Research Articles

Clinical outcomes prediction in kidney transplantation by use of biomarkers from hypothermic machine perfusion.

The clinical outcomes of kidney transplantation from deceased donors have seen significant improvements with the use of machine perfusion (MP), now a standard practice in transplant centers. However, the use of perfusate biomarkers for assessing organ quality remains a subject of debate. Despite this, some centers incorporate them into their decision-making process for donor kidney acceptance. Recent studies have indicated that lactate dehydrogenase (LDH), glutathione S-transferase, interleukin-18, and neutrophil gelatinase-associated lipocalin (NGAL) could predict post-transplant outcomes. Between August 2016 and June 2017, 31 deceased-donor after brain death were included and stroke was the main cause of death. Pediatric patients, hypersensitized recipients were excluded. 43 kidneys were subjected to machine perfusion. Perfusate samples were collected just before the transplantation and stored at -80ºC. Kidney transplant recipients have an average age of 52 years, 34,9% female, with a BMI 24,6±3,7. We employed receiver operating characteristic analysis to investigate associations between these perfusate biomarkers and two key clinical outcomes: delayed graft function and primary non-function. The incidence of delayed graft function was 23.3% and primary non-function was 14%. A strong association was found between NGAL concentration and DGF (AUC=0.766, 95% CI, P=0.012), and between LDH concentration and PNF (AUC=0.84, 95% CI, P=0.027). Other perfusate biomarkers did not show significant correlations with these clinical outcomes. The concentrations of NGAL and LDH during machine perfusion could assist transplant physicians in improving the allocation of donated organs and making challenging decisions regarding organ discarding. Further, larger-scale studies are required.

The Role of Pediatric Nurses During Preventable Adverse Event Disclosure: A Scoping Review.

Preventable adverse events (PAEs) occur across the healthcare spectrum and unfortunately, errors, adverse events, and PAEs are common in pediatric care. Historically, the role of disclosure of PAEs to patients and their families occurred between the dyad of physician and patient, with physicians assuming the responsibility of disclosure. In recent years, a trend toward a multidisciplinary team-based approach has emerged in some institutions, yet the role of pediatric nurses within the team disclosing a PAE is not fully understood. Given the unique relationship between pediatric nurses and their patients and their families, it is essential to understand does the literature tell us about the role of pediatric nurses during PAE disclosure? The Arksey and O'Malley scoping review method guided this study protocol and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews framework guided reporting. The final sample included five articles for synthesis: none reported or described a specific role for pediatric nurses during PAE disclosure. There is a gap in the literature on the role of pediatric nurses during PAE disclosure. Two themes emerged from this review: the use of a team-based approach to disclosure, and the need to provide emotional support to the pediatric patient and their family. There is a need for additional investigation into the role of pediatric nurses as part of a team-based disclosure process and how pediatric nurses currently provide, or desire to provide, emotional support to the patient and their family.

Post-traumatic hyperoxia after pediatric TBI.

Hyperoxia has been suggested as a mechanism for secondary injury following adult traumatic brain injury (TBI), but its effects have not been well described in pediatric patients. Pediatric (≤18yo) TBI patients were identified in a prospective institutional registry from October 2008 to April 2022. The first, highest, and the Area Under the Curve (AUC) PaO2 in the first 24 hours were collected and calculated for each patient from arterial blood gas reports after admission to the ICU. Neurological outcome after 6 months was measured using dichotomized modified Rankin Scale (mRS) and Glasgow Outcome Scale - Extended (GOS-E). Multivariable logistic regression models were used to determine if the three measurements for hyperoxia predicted an unfavorable outcome after controlling for well-established clinical and imaging predictors of outcome. We identified 98 pediatric patients with severe accidental TBI during the study period. Hyperoxia (PaO2 > 300 mmHg) occurred in 33% of the patients. The presence of elevated PaO2 values, determined by all three evaluations of hyperoxia, was not associated with unfavorable outcome after 6 months. Utilizing multiple methods to assess exposure, hyperoxia was present in a substantial number of patients with severe TBI but was not associated with an unfavorable outcome.

Characteristics of Intracranial Injuries in Pediatric Patients Following Blunt Head Trauma.

Pediatric head trauma is a frequent reason for presentation to the emergency department. Despite this, there are few reports on specific characteristics and injury patterns in head injured children. The goal of this study was to evaluate head injury patterns in children with blunt head injury and their prevalence by age group. This is a planned secondary analysis of the NEXUS II Head CT validation study. Consecutive patients with blunt head trauma were enrolled between 2006 and 2015. Demographics and criteria from 2 clinical decision instruments (NEXUS and Canadian Head CT rules) were gathered at the time of enrollment. We abstracted and cataloged injuries for pediatric patients based on radiologist report. Frequencies of injuries and severity were analyzed by developmental age group. A total of 1018 pediatric patients were enrolled, 128 (12.6%) of whom had an injury on computed tomography scan. Median age was 11.9 (Interquartile range 4.5-15.5) for all patients and 12 (4.8-15.5) for injured patients. Of injured patients, 49 (38.3%) had a significant injury, and 27 (21.1%) received an intervention. Teenagers had the highest rate of significant injury (50%) and intervention (30%). Injuries were most frequently noted in the temporal (46.1%), frontal (45.3%), and parietal (45.3%) regions. Subarachnoid hemorrhage (29.7%) and subdural hematoma (28.9%) were the most common injuries observed.Intraparenchymal hemorrhage and cerebral edema were more prevalent in older age groups. The most common injury mechanism overall was fall from height (24.7%). Motor vehicle accidents and nonmotorized wheeled vehicle accidents were more common in older patients. Serious injuries requiring intervention were rarely encountered in pediatric patients experiencing blunt head trauma. Mechanisms of injury, type of injury, and rates of intervention varied between developmental age groups.

Synthetic suture tape for medial patellofemoral ligament reconstruction is an effective treatment for complex paediatric patellofemoral instability.

To assess the outcomes of medial patellofemoral ligament (MPFL) reconstruction using synthetic suture tape in paediatric patients with patellofemoral instability (PFI). This ambispective comparative study, conducted from 2014 to 2022, included paediatric patients who underwent MPFL reconstruction with synthetic suture tape and had a minimum follow-up of 1 year. Pre- and postoperative clinical and functional outcomes, patient satisfactionand complications were assessed. The study comprised 22 patients (29 knees), with a median age at surgery of 14.4 years (interquartile range [IQR] 10.9-16.7) and a median follow-up of 46.5 months (24.7-66). Notably, 13 knees (44.8%) had open growth plates at the time of surgery. The cohort included patients with Down syndrome (3 patients), Ehlers-Danlos (2), arthrogryposis (1), generalized joint hypermobility (8)and previous unsuccessful PFI surgeries (8). Concomitant procedures were performed on 16 knees. Postoperatively, improvements were observed in all but one patient, who reported residual pain. There were two other complications: one suture-tape rupture and one surgical wound infection. Functional scores significantly improved: Kujala, +14 points (7-29) (p < 0.001); IKDC, +10.5 points (5.2-25.3) (p < 0.001); Tegner, +2 points (0-4) (p < 0.001);Lysholm, +15 points (0-37.5) (p < 0.001). Most patients achieved excellent outcomes by Crosby-Insall criteria (21 patients, 72.4%) and reported high satisfaction (23 patients, 79.3%). MPFL reconstruction using synthetic suture tape is a viable and effective treatment for paediatric patients with PFI, particularly for those with connective tissue disorders, generalized joint hypermobilityor past surgical failures, significantly enhancing clinical and functional outcomes with an acceptable complication rate. Level IV.

Characteristics and Risk Factors for Pediatric Sepsis.

Sepsis is considered a major cause of health loss in children and had high mortality and morbidity. Currently, there is no reliable model for predicting the prognosis of pediatric patients with sepsis. This study aimed to analyze the clinical characteristics of sepsis in children and assess the risk factors associated with poor prognosis in pediatric sepsis patients to identify timely interventions and improve their outcomes. This study analyzed the clinical indicators and laboratory results of septic patients hospitalized in the Pediatric Intensive Care Unit of Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, China, from January 1, 2019, to December 31, 2021. Risk factors for sepsis were identified by logistic regression analyses. A total of 355 children with sepsis were enrolled, with 333 children (93.8%) in the good prognosis group, and 22 children (6.2%) in the poor prognosis group. Among them, there were 255 patients (71.8%) in the sepsis group, and 100 patients (28.2%) in the severe sepsis group. The length of hospital stay in the poor prognosis group was longer than that in the good prognosis group (P<0.01). The levels of interleukin 1β (IL-1β) in the poor prognosis group were higher than those in the good prognosis group (P>0.05), and the platelet (PLT), albumin (ALB), and hemoglobin (Hb) levels were lower in the poor prognosis group (P<0.01). The IL-8 levels in the severe sepsis group were higher than those in the sepsis group (P<0.05). Multiple logistic regression analysis suggested that lower Hb levels, ALB levels, peak PLT counts, and higher IL-1β levels were independent risk factors for poor prognosis in children with sepsis. Lower Hb, ALB, and PLT counts and elevated IL-1β are independent risk factors for poor prognosis in children with sepsis.

2023 catastrophic Turkey earthquake: clinical outcomes of pediatric patients rescued under the rubble

ABSTRACT Objectives Children are the most vulnerable population affected by the earthquake. We aimed to examine the characteristics, morbidity, mortality, and the factors affecting these pediatric earthquake victims to guide the follow-up of pediatric patients who were rescued under the rubble in possible future disasters. Methods This is a retrospective observational study. The files of pediatric earthquake victims aged 0-18 years who were admitted to Adana City Training and Research Hospital between 6 February-30 April 2023 were analyzed. 318 patients were included in the study. Results Of all patients 48.7% (n = 155) were female and 51.3% (n = 163) were male. The mean age of the patients was 114.8 months. 65.4% (n = 208) of the patients were discharged from our hospital, eighteen patients (5.7%) died. Information on the hour when the patients were rescued under the rubble could be reached in 117 (36.8%) patients. The mean rescue time of these patients was 50.5 hours. 62 (53%) victims were rescued in the first 24 hours after the earthquake. There were 111 patients (34.9%) with fractures. There were 118 patients (37.1%) who underwent fasciotomy surgery. The total number of patients with amputation was 48 (15.1%). There were 36 patients (11.3%) with one limb amputated, 12 patients (%3.7) with more than one limb amputation. Internal organ injury was present in 30.5% (n = 97) of the patients. Of these patients, 85.6% (n = 83) had single organ trauma and 14.4% (n = 14) had multiple organ trauma. There were 58 patients (18.2%) with acute renal failure in follow-up. The mean rescue time for patients with amputation was 83.65 ± 62.9 hours, and for patients without amputation was 36.44 ± 50.6 hours. This difference was statistically significant (p = 0.001). No statistically significant difference was found when the ages of the patients were compared to fasciotomy, amputation, and fracture status. Conclusion While evaluating earthquake-induced trauma, pediatric patients should be approached carefully.

Surgical management of a thoraco-lumbar extradural cyst in a pediatric patient with Klippel-Trenaunay Syndrome: A case report

Surgical management of a thoraco-lumbar extradural cyst in a pediatric patient with Klippel-Trenaunay Syndrome: A case report

Use of rotational angiography with 3D reconstruction as a guide for stent aortoplasty in a pediatric patient with coarctation of the aorta

Use of rotational angiography with 3D reconstruction as a guide for stent aortoplasty in a pediatric patient with coarctation of the aorta

Loss of heterozygosity for chromosomes 16q in Wilms tumors predicts outcomes: A meta-analysis

BACKGROUND The research findings suggest that the prognosis of children with Wilms tumor (WT) is affected by various factors. Some scholars have indicated that loss of heterozygosity (LOH) on chromosome 16q is associated with a poor prognosis in patients with WT. AIM To further elucidate this relationship, we conducted a meta-analysis. METHODS This meta-analysis was registered in INPLASY (INPLASY2023100060). We systematically searched databases including Embase, PubMed, Web of Science, Cochrane, and Google Scholar up to May 31, 2020, for randomized trials reporting any intrapartum fetal surveillance approach. The meta-analysis was performed within a frequentist framework, and the quality and network inconsistency of trials were assessed. Odds ratios and 95%CIs were calculated to report the relationship between event-free survival and 16q LOH in patients with WT. RESULTS Eleven cohort studies were included in this meta-analysis to estimate the relationship between event-free survival and 16q LOH in patients with WT (I 2 = 25%, P &lt; 0.001). As expected, 16q LOH can serve as an effective predictor of event-free survival in patients with WT (risk ratio = 1.95, 95%CI: 1.52–2.49, P &lt; 0.001). CONCLUSION In pediatric patients with WT, there exists a partial correlation between 16q LOH and an unfavorable treatment prognosis. Clinical detection of 16q chromosome LOH warrants increased attention to the patient’s prognosis.

Isolated Fallopian Tube Torsion in Children With Hydrosalpinx: Is Conservative Management an Option?

Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX). A retrospective review was conducted. Pediatric patients with IFTT operated in the same center were included. Seventeen girls (aged: 11-16 years) were managed for acute abdominal pain between 2008 and 2018, with intraoperative diagnosis of IFTT. All patients underwent laparoscopic exploration, with laparoscopically fallopian tube detorsion in all patients. Based on the association of IFTT with HSX after fallopian tube detorsion, patients were divided into 2 groups: group 1 (IFTT without HSX; 12 girls) and group 2 (IFTT with HSX; 5 girls). During the same surgery, complementary surgical procedures were done. In group 1: salpingectomies (4), partial salpingectomies (2) and cystectomies (6) were done. In group 2: salpingectomy (1), salpingotomy (1), and cyst ablation (1). The treatment was called conservative when the tube was preserved.Follow-up was uneventful in group 1. In group 2, for all patients with initial fallopian tube preservation, further surgical procedures were necessary (1-4 surgeries/patient), and, finally, another 3 patients required salpingectomy. Conservative treatment with tube preservation of IFTT without HSX appeared to be beneficial compared to those with HSX, with no recurrence of torsion or symptoms during the follow-up. However, the same conservative treatment was not sufficiently effective for IFTT with HSX and required further procedures due to recurrence of torsion. IV.

Displaced proximal humeral fractures in skeletally immature patients: functional outcomes of surgical treatment.

To report the functional results of our experience and to describe intraoperative findings and complications due to the techniques used in our service. From January 2018 to December 2022, 27 Pediatric patients aged from 8 to 16years underwent surgery to treat proximal humerus fractures. Their demographic characteristics were evaluated, as well as their clinical characteristics on admission, type of reduction (closed/open), presence of interposition in open reductions, type of implant, complications, and functional range of movement according to two shoulder functional scores. Mean age was 11.2years (8-15), there was a predominance of males, who accounted for 70% of the cases, and sports accidents were the cause of injury in 44% of the cases. Anatomical location was balanced between patients, including 55% of physeal fractures and 45% of metaphyseal ones. Overall, 81.5% of patients required open reduction to achieve axis correction, and that 55.5% of fractures were fixed with Kirschner wires, and 44, 4% with elastic titanium nails. Mean QuickDASH score was 0.58 (0-1.7), and Constant score was 9 (3-24). There were no major complications, but 27% of cases subjected to open reduction presented a hypertrophic scar. Surgical treatment of severely displaced proximal humerus fractures is successful, both with closed and open reduction; type of implant does not play a key role and should be selected based on the characteristics of each case. Surgical training should prevail at the time of decision-making. When these recommendations are followed, results can be excellent and sequelae are infrequent.

Diagnosis of pneumonia in paediatric age group by comparing two modalities chest x-ray versus ultrasonography at tertiary care hospital

: Up to 20 million cases of pneumonia in children ages 0 to 5 years require hospitalization each year, from total 150 million cases of pneumonia. The most common cause of death in children worldwide is pneumonia. Due to the general non-specificity of the signs and symptoms, clinical history and physical examination cannot reliably distinguish between the causes of respiratory cases. As a result, accurate diagnosis frequently requires medical imaging, but two-thirds of the world's population lack access to diagnostic imaging, which lead to increasing the consequences of respiratory illnesses. : To know the characteristic of pneumonia in x-ray and ultrasonography. To compare X-ray chest and USG for early diagnosis. : A Prospective observational study was conducted among 100 paediatric patients diagnosed with pneumonia and admitted in paediatric ward, at New Civil Hospital, Surat. : Out of total, 52.7% and 47.3% patients were Males and Females, respectively. 87.2% cases were fully immunized, while remaining were partially immunized. Sensitivity and specificity of chest X-ray is 83.4 % and 86.8 % respectively. Sensitivity and specificity of USG thorax is 96.8 % and 90.5 % respectively. Young children are at least four times more susceptible to radiation damage than adults.: In comparison to chest radiography, lung ultrasound has the potential advantages of being more sensitive and specific, radiation-free, subject to less regulatory constraints, relatively less expensive so it can be used in diagnosis in patients with pneumonia.

Therapeutic plasma exchange in critically ill children: A single center experience.

Therapeutic plasma exchange (TPE) is used in a wide spectrum of diseases in critically ill pediatric patients. We aim to review the indications, complications, safety, and outcomes of critically ill children who received TPE. All of the TPE procedures performed in a pediatric intensive care unit providing tertiary care during 19 years (January 2013-January 2023) were evaluated retrospectively. A total of 154 patients underwent 486 TPE sessions. Median age was 6 years (2-12.5) and 35 children had a body weight of <10 kg (22.7%). Number of organ failure was 4 (2-6). Liver diseases were the most common indication for TPE (31.2%) followed by sepsis with multiorgan dysfunction syndrome (27.3%). Overall survival rate was 72.7%. The highest mortality was observed in hemophagocytic lymphohistiocytosis group. Non-survivors had significantly higher number of organ failure (p < 0.001), higher PRISM score (p < 0.001), and higher PELOD score on admission (p < 0.001). Adverse events were observed in 68 (13.9%) sessions. Hypotension (7.8%) and hypocalcemia (5.1%) were the most frequent adverse events. TPE is safe for critically ill pediatric patients with experienced staff. Survival rate may vary depending on the underlying disease. Survival decreases with the increase in the number of failed organs.

Fecal proteolytic profiling of pediatric inflammatory bowel disease: A pilot study.

Colonoscopy is the gold standard for diagnosing inflammatory bowel disease (IBD). However, this invasive procedure has a high burden for pediatric patients. Previous research has shown elevated fecal amino acid concentrations in children with IBD versus controls. We hypothesized that this finding could result from increased proteolytic activity. Therefore, the aim of this study was to investigate whether fecal protease-based profiling was able to discriminate between IBD and controls. Protease activity was measured in fecal samples from patients with IBD (Crohn's disease (CD) n = 19; ulcerative colitis (UC) n = 19) and non-IBD controls (n = 19) using a fluorescence resonance energy transfer (FRET)-peptide library. Receiver operating characteristic (ROC) curve analysis was used to determine the diagnostic value of each FRET-peptide substrate. Screening the FRET-peptide library revealed an increased total proteolytic activity (TPA), as well as degradation of specific FRET-peptides specifically in fecal samples from IBD patients. Based on level of significance (p < .001) and ROC curve analysis (AUC > 0.85), the fluorogenic substrates W-W, A-A, a-a, F-h, and H-y showed diagnostic potential for CD. The substrates W-W, a-a, T-t, G-v, and H-y showed diagnostic potential for UC based on significance (p < .001) and ROC analysis (AUC > 0.90). None of the FRET-peptide substrates used was able to differentiate between protease activity in fecal samples from CD versus UC. This study showed an increased fecal proteolytic activity in children with newly diagnosed, treatment-naïve, IBD. This could lead to the development of novel, noninvasive biomarkers for screening and diagnostic purposes.

Dupilumab Safety and Efficacy up to 1 Year in Children Aged 6 Months to 5 Years with Atopic Dermatitis: Results from a Phase 3 Open-Label Extension Study.

Pediatric patients with moderate-to-severe atopic dermatitis (AD) often experience a high disease burden and have a high risk of persistent disease. Standard-of-care immunosuppressive systemic treatments have been used off-label for AD in pediatric patients despite concerns for suboptimal safety with continuous use and risk of relapse upon discontinuation. The biologic agent dupilumab is the first systemic treatment approved for moderate-to-severe AD in children as young as 6 months. Long-term safety and efficacy data in this patient population are needed to inform continuous AD management. The purpose of this work was to determine the long-term safety and efficacy of dupilumab treatment up to 1 year in an open-label extension (OLE) study [LIBERTY AD PED-OLE (NCT02612454)] in children aged 6 months to 5 years with moderate-to-severe AD who previously participated in the 16-week, double-blind, phase 3 LIBERTY AD PRESCHOOL trial (NCT03346434 part B; parent study) and were subsequently enrolled in PED-OLE. In PED-OLE, patients received dupilumab every 4 weeks according to a weight-tiered regimen (body weight ≥ 5 kg to < 15 kg: 200 mg; ≥ 15 kg to < 30 kg: 300 mg). Data for 142 patients were analyzed, 60 of whom had completed the 52-week visit at time of database lock. Mean age at baseline was 4.1 y [SD, 1.13; range, 1.0-5.9 years]. A majority (78.2%) of patients reported ≥ 1 treatment-emergent adverse event (TEAE), most of which were mild or moderate and transient. The most frequently reported TEAEs were nasopharyngitis (19.7%), cough (15.5%), and pyrexia (14.1%). One TEAE led to treatment discontinuation (severe urticaria, which resolved in 1 day). By week 52, 36.2% of patients had achieved an Investigator's Global Assessment score of 0/1 (clear/almost clear skin), and 96.6%, 79.3%, and 58.6% had at least 50%, 75%, or 90% improvement, respectively, in Eczema Area and Severity Index scores. Consistent with results seen in adults, adolescents, and older children (aged 6-11 years), treatment with dupilumab for up to 1 year in children aged 6 months to 5 years with inadequately controlled moderate-to-severe AD demonstrated an acceptable long-term safety profile and sustained efficacy. These results support the long-term continuous use of dupilumab in this patient population. ClinicalTrials.gov Identifiers: NCT02612454 and NCT03346434 (part B).

Comparative Analysis of Pediatric Patients with Different Involvements of Brucellosis.

Brucellosis is a zoonotic disease that mimics many other diseases, making diagnosis difficult in pediatric patients. If the clinical signs of the disease are not well known and there is a delay in diagnosis and treatment, complicated brucellosis involving different body sites can develop. This study aimed to analyze the demographics, clinical information, laboratory test results, and imaging findings of children with brucellosis. Particular attention was paid to the involvement of organs and potential complications. Pediatric patients diagnosed with brucellosis at the Sanlıurfa Training and Research Hospital in Turkey were retrospectively evaluated. In total, 101 patients diagnosed with brucellosis were included in this study. Bone-joint involvement was observed in 21 (20.7%) patients. High Brucella Coombs gel test (CT) titers (≥1/640) and increased erythrocyte sedimentation rate (ESR) were significant in the bone-joint brucellosis group (P = 0.022 and P = 0.0205, respectively). Fourteen (13.9%) patients had at least one organomegaly in which Brucella CT titers and C-reactive protein (CRP) levels were substantially higher than those in patients without organomegaly (P = 0.02 and P = 0.0049, respectively). Anemia, leukopenia, and thrombocytopenia were observed in 36 (35.6%), 10 (9.9%), and 4 (3.9%) patients, respectively. At least one elevation in liver function test (LFT) results was observed in 32 (31.7%) patients. High acute-phase reactants, such as ESR, CRP levels, and Brucella CT titers, may help identify complicated brucellosis. This study highlights that pediatric brucellosis can lead to cytopenia and elevated LFT results and should therefore be considered as a differential diagnosis.

Children with Congenital Heart Diseases Exhibit Altered Deep Gray Matter Structures.

Children with congenital heart diseases (CHDs) have an increased risk of developing neurologic deficits, even in the absence of apparent brain pathology. The aim of this work was to compare quantitative macro- and microstructural properties of subcortical gray matter structures of pediatric CHD patients with normal appearing brain magnetic resonance imaging to healthy controls. We retrospectively reviewed children with coarctation of the aorta (COA) and hypoplastic left heart syndrome (HLHS) admitted to our hospital. We identified 24pediatric CHD patients (17COA, 7HLHS) with normal-appearing brain MRI. Using an atlas-based approach, the volume and apparent diffusion coefficient (ADC) were determined for the thalamus, caudate, putamen, pallidum, hippocampus, amygdala, nucleus accumbens, cerebral white matter, cerebral cortex, and brainstem. Multivariate statistics were used to compare the extracted values to reference values from 100 typically developing children without any known cardiac or neurological diseases. Multivariate analysis of covariance using the regional ADC and volume values as dependent variables and age and sex as co-variates revealed asignificant difference between pediatric CHD patients and healthy controls (p < 0.001). Post-hoc comparisons demonstrated significantly reduced brain volumes in most subcortical brain regions investigated and elevated ADC values in the thalamus for children with CHD. No significant differences were found comparing children with COA and HLHS. Despite normal appearing brain MRI, children with CHD exhibit wide-spread macro-structural and regional micro-structural differences of subcortical brain structures compared to healthy controls, which could negatively impact neurodevelopment, leading to neurological deficits in childhood and beyond.

Evaluation of T-wave memory after accessory pathway ablation in pediatric patients with Wolff-Parkinson-White syndrome.

T-wave memory (TWM) is a rare cause of T-wave inversion (TWI). Alterations in ventricular activation due to abnormal depolarization may cause repolarization abnormalities on the ECG, even if myocardial conduction returns to normal. These repolarization changes are defined as TWM. In our study, we aimed to determine the frequency of TWM development and the predictors affecting it in the pediatric population who underwent accessory pathway (AP) ablation due to Wolff-Parkinson-White (WPW) syndrome. The data of patients with manifest AP who underwent electrophysiological studies and ablation between 2015 and 2021 were retrospectively analyzed. The study included 180 patients who were under 21 years of age and had at least one year of follow-up after ablation. Patients with structural heart disease, intermittent WPWs, recurrent ablation, other arrhythmia substrates, and those with less than one-year follow-up were excluded from the study. The ECG data of the patients before the procedure, in the first 24h after the procedure, three months, and in the first year were recorded. The standard ablation technique was used in all patients. Postprocedure TWM was observed in 116 (64.4%) patients. Ninety-three patients (51.7%) had a right-sided AP, and 87 patients (48.3%) had a left-sided AP. The presence of posteroseptal AP was found to be significantly higher in the group that developed TWM. Of these patients, 107 (93.1%) patients showed improvement at the end of the first year. Preprocedural absolute QRS-T angle, postprocedural PR interval, and right posteroseptal pathway location were identified as predictors of TWM. The development of TWM is particularly associated with the right-sided pathway location, especially the right posteroseptal pathway location. The predictors of TWM are the preprocedural QRS-T angle, the postprocedural PR interval, and the presence of the right posteroseptal AP.

Establishment, characterization, and genetic profiling of patient-derived osteosarcoma cells from a patient with retinoblastoma

Osteosarcoma is the most common malignant bone cancer in pediatric patients. Patients who respond poorly to chemotherapy experience worse clinical outcomes with a high mortality rate. The major challenge is the lack of effective drugs for these patients. To introduce new drugs for clinical approval, preclinical studies based on in vitro models must demonstrate the potency of the tested drugs, enabling the drugs to enter phase 1 clinical trials. Patient-derived cell culture is a promising testing platform for in vitro studies, as they more accurately recapitulate cancer states and genetic profiles compared to cell lines. In the present study, we established patient-derived osteosarcoma cells (PDC) from a patient who had previously been diagnosed with retinoblastoma. We identified a new variant of a germline mutation in the RB1 gene in the tissue of the patient. The biological effects of this PDC were studied to observe whether the cryopreserved PDC retained a feature of fresh PDC. The cryopreserved PDC preserved the key biological effects, including cell growth, invasive capability, migration, and mineralization, that define the conserved phenotypes compared to fresh PDC. From whole genome sequencing analysis of osteosarcoma tissue and patient-derived cells, we found that cryopreserved PDC was a minor population in the origin tissue and was selectively grown under the culture conditions. The cryopreserved PDC has a high resistance to conventional chemotherapy. This study demonstrated that the established cryopreserved PDC has the aggressive characteristics of osteosarcoma, in particular the chemoresistance phenotype that might be used for further investigation in the chemoresistant mechanism of osteosarcoma. In conclusion, the approach we applied for primary cell culture might be a promising method to generate in vitro models for functional testing of osteosarcoma.