Clinical Deterioration Research Articles

Derivation of the Quebec Brain Injury Categories for complicated mild traumatic brain injuries.

Approximately 10% of patients with mild traumatic brain injury (TBI) present with intracranial bleeding, and only 3.5% eventually require neurosurgical intervention, which often necessitates interhospital transfer. Better guidelines and recommendations are needed to manage complicated mild TBI in the emergency department (ED). The main objective of this study was to derive a clinical decision rule, the Quebec Brain Injury Categories (QueBIC), to predict the risk of adverse outcomes for complicated mild TBI in the ED. The secondary objective was to compare the QueBIC's performance with those of other existing guidelines. The authors conducted a retrospective multicenter cohort study in 3 level I trauma centers. Consecutive patients with complicated mild TBI (Glasgow Coma Scale [GCS] score 13-15) who were aged ≥ 16 years were included. The primary outcome was a combination of neurosurgical intervention, mild TBI-related death, and clinical deterioration. Statistical analyses included set covering machine analyses. In total, 477 patients were included in the study. The mean age was 62.9 years, and 68.1% were male. The algorithm classified patients into three risk categories (low, moderate, and high risk). The high-risk group (128 patients) (subdural hemorrhage [SDH] width > 7 mm or any midline shift) presented a sensitivity of 84% (95% CI 71%-93%) and a specificity of 80% (95% CI 76%-84%) to detect neurosurgical intervention and mild TBI-related death, leaving 8 undetected cases. Patients in the moderate-risk group (169 patients) had at least 1 variable: SDH width > 4 mm, initial GCS score ≤ 14, > 1 intraparenchymal hemorrhage, or intraparenchymal hemorrhage width > 4 mm. The combined QueBIC high- and moderate-risk category had a sensitivity of 100% (95% CI 63%-100%) and a specificity of 53% (95% CI 47%-58%) to detect mild TBI-related death or neurosurgical intervention. The sensitivity and specificity values for clinical deterioration when no death or neurosurgical intervention occurred were 81% (95% CI 64%-93%) and 44% (95% CI 39%-49%), respectively. The remaining 180 patients (37.7%) did not meet any high-risk or moderate-risk criteria and were considered low risk. None had neurosurgical intervention or mild TBI-related death. Only 6 (3.3%) low-risk patients showed clinical deterioration. QueBIC is a safe and effective tool to guide the management of patients presenting to the ED with complicated mild TBI. It accurately identifies patients at low risk for specialized neurotrauma or neurosurgical care. Further validation is required before its use in EDs.

Paediatric out-of-hospital clinical deterioration: a mixed-methods scoping review protocol

IntroductionIn pre-hospital settings, identifying a deteriorating child can be challenging, especially considering that the proportion of paediatric patients with acute illnesses is lower compared with adults. This challenge is exacerbated...

Prosthetic Graft Migration in Living Donor Liver Transplantation: A Case for Conservative Management?

Abstract A 61-year-old male underwent living donor liver transplantation (LDLT) for decompensated chronic liver disease with hepatocellular carcinoma. The right lobe from the live donor was used as the graft. During implantation, the drainage of the anterior sector of the right lobe graft was through a reconstructed middle hepatic vein using a prosthetic polytetrafluoroethylene (PTFE) graft. Routine follow-up at 6 weeks showed a thrombosed PTFE graft with no clinical, radiological, or biochemical deterioration of the allograft. He was therefore managed conservatively. A routine computed tomography scan at 2 years demonstrated that the PTFE graft had eroded into the duodenum. Given the total absence of symptoms and the risk of complications associated with removing the graft, we decided against any surgical or endoscopic intervention. After a further 3 year period of follow-up, the patient continues to remain well. Enteric erosion of a prosthetic graft following LDLT can be managed without intervention, although long-term consequences are unknown.

Evaluation of Pediatric Early Warning Score (PEWS) in Unintentional Childhood Injuries Admitted to the Critical Care Unit.

Pediatric Early Warning Score (PEWS), also known as Brighton PEWS or Monaghan PEWS, was developed to identify children at risk for clinical deterioration in hospitals. We designed this study to describe the epidemiology of unintentional injuries in children admitted to the critical care unit in a tertiary healthcare setting, and to determine the predictive properties of PEWSin these injuries. This is a cross-sectional study. Injury-related data were based on Haddon's matrix of agent, host, and environment factors. Each child was evaluated using PEWS on admission. We noted the following outcomes: duration of stay in the intensive unit; major intervention required; and death. We analyzed data from 157 children. Most of the children were in the age group of one to five years (57.7%), followed by more than five to 12 years old (37.6%). The most common injuries were bites (35.7%), falls (24.2%), and poisoning (21.7%). These injuries occurred at home (52.7%) and in the presence of a caretaker (40.0%). On admission, 11% of children were classified as green, 40% as yellow, 36% as orange, and 13% as red by PEWS. Classification of red versus the rest (orange/yellow/green) had a high sensitivity (100%), specificity (88.3%), and negative predictive value (100%) for "death" as an outcome. Most of the injuries occurred at home and at a time when the caretaker was around. Thus, it will be useful to develop "safe home interventions" and train parents in first aid to take care of these injuries on-site. Baseline PEWS was a good predictor of "poor"as well as "positive" outcomes. It may be worthwhile to implement this score regularly in the management of childhood injuries in hospitals.

Takotsubo cardiomyopathy in a 7-month-old infant with familial hemophagocytic lymphohistiocytosis: A case report

Abstract Takotsubo cardiomyopathy constitutes an acute cardiac condition that mimics acute myocardial infarction in the absence of coronary artery disease. We present a case of a 7-month-old girl, who was admitted in the pediatric intensive care unit (PICU) at a tertiary care hospital for clinical deterioration with underlying familial hemophagocytic lymphohistiocytosis. During the PICU stay, on echocardiography, the patient had severe cardiac contractility compromise with a characteristic pattern of regional wall motion abnormalities of the left ventricle. This, in combination with elevated cardiac enzymes, led to the diagnosis of takotsubo cardiomyopathy.

Selexipag in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD): Real-world experience from EXPOSURE.

Selexipag is indicated for the treatment of pulmonary arterial hypertension (PAH), including PAH associated with connective tissue disease (CTD), and further insights into the management of selexipag-treated PAH-CTD patients in clinical settings are needed. These analyses of the ongoing, multicenter, prospective EXPOSURE (EUPAS19085) study describe characteristics, treatment patterns, tolerability, and outcomes of PAH-CTD patients initiating selexipag in Europe/Canada. All analyses were descriptive, with idiopathic PAH patients who typically display better prognosis included for context. Six hundred ninety-eight selexipag-treated patients had follow-up information; 178 (26%) had PAH-CTD.The median age was 68 years, patients were predominantly female (88%), and with WHO functional class III symptoms (63%); the median time since diagnosis was 1.7 years.There were 5% patients at low, 25% intermediate-low, 40% intermediate-high, and 30% high risk of 1-year mortality, according to the ESC/ERS 4-strata risk score. Most (80%) initiated selexipag as a triple oral therapy, and most of these (62%) remained on triple therapy 6 months post-baseline. Over a median (Q1-Q3) selexipag exposure period of 8.6 (2.5-17.2) months, 79 (44%) patients discontinued selexipag; 36 (20%) due to tolerability/adverse events. Sixty (34%) patients were hospitalized at least once; 120 hospitalizations occurred, with 49 (48%) deemed PAH-related. Survival at 1 year was 85%, and at 2 years was 71%; 29 (16%) patients died. These results describe the use of combination therapy with selexipag for patients with PAH-CTD. These findings suggest an opportunity to optimize the benefits of selexipag among patients with PAH-CTD by moving from escalating after years in response to clinical deteriorationto escalating sooner to prevent clinical deterioration.

Fulfilling last wishes: improving the compassionate discharge process

BackgroundCompassionate discharges (ComD), commonly known as rapid discharges, are urgent one-way discharges for critically ill hospitalised patients with death expected within hours or less than 7 days, to die at...

A theoretical framework for identifying sociocultural factors that influence nurses' compliance with early warning systems for acute clinical deterioration: A cross-sectional survey

BackgroundSystematic adoption of early warning systems in healthcare settings is dependent on the optimal and reliable application by the user. Psychosocial issues and hospital culture influence clinicians' patient safety behaviours. Objective(i) To examine the sociocultural factors that influence nurses' EWS compliance behaviours, using a theory driven behavioural model and (ii) to propose a conceptual model of sociocultural factors for EWS compliance behaviour. DesignA cross-sectional survey. SettingNurses employed in public hospitals across Queensland, Australia. ParticipantsUsing convenience and snowball sampling techniques eligible nurses accessed a dedicated web site and survey containing closed and open-ended questions. 291 nurses from 60 hospitals completed the survey. MethodsQuantitative data were analysed using ANOVA or t-tests to test differences in means. A series of path models based on the theory were conducted to develop a new model. Directed or theory driven content analysis informed qualitative data analysis. ResultsNurses report high levels of previous compliance behaviour and strong intentions to continue complying in the future (M=4.7; SD 0.48). Individual compliance attitudes (β 0.29, p<.05), perceived value of escalation (β 0.24, p<.05) and perceived ease or difficulty complying with documentation (β −0.31, p<.05) were statistically significant, predicting 24% of variation in compliance behaviour. Positive personal charting beliefs (β 0.14, p<.05) and subjective norms both explain higher behavioural intent indirectly through personal attitudes. High ratings of peer charting beliefs indirectly explain attitudes through subjective norms (β 0.20, p<.05). Perceptions of control over one's clinical actions (β −0.24, p<.05) and early warning system training (β −0.17, p<.05) directly contributed to fewer difficulties complying with documentation requirements. Prior difficulties when escalating care (β −0.31, p<.05) directly influenced the perceived value of escalating. ConclusionsThe developed theory-based conceptual model identified sociocultural variables that inform compliance behaviour (documenting and escalation protocols). The model highlights areas of clinical judgement, education, interprofessional trust, workplace norms and cultural factors that directly or indirectly influence nurses' intention to comply with EWS protocols. Extending our understanding of the sociocultural and system wide factors that hamper nurses' use of EWSs and professional accountability has the potential to improve the compliance behaviour of staff and subsequently enhance the safety climate attitudes of hospitals. Tweetable abstractA newly developed model reports nurse's personal attitudes, peer influence, perceived difficulties encountered documenting and escalation beliefs all predict early warning system compliance behaviour.

ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN HOSPITALIZED IN THE INTENSIVE CARE UNIT: A RETROSPECTIVE ANALYSIS

Relevance: The most common pediatric malignancy is acute lymphoblastic leukemia (ALL), with a prevalence of 4.2 per 100,000 children. Some patients diagnosed with ALL will require hospitalization in intensive care units (ICU) at some point in their treatment. The study aimed to identify significant clinical and laboratory parameters in prognosis to identify indicators of clinical deterioration in children with ALL and develop a scale for early detection of precursors of critical illness in children with ALL. Methods: This retrospective study included inpatient medical records of all patients (0-18 years old) diagnosed with ALL and hospitalized in the intensive care unit (ICU) for oncohematological patients at the Scientific Center for Pediatrics and Pediatric Surgery (Almaty, Kazakhstan). The study period was three years – 2020 to 2022. Results: The clinical and laboratory parameters of children with ALL were analyzed a day before admission to the ICU since those patients demonstrated worsening clinical status. This window of time was chosen to allow us to understand what were the precursors of early deterioration in children with ALL before hospitalization in the ICU. Study data were collected before hospitalization in the ICU during admission to the ICU, and there is data at transfer from the ICU depending on the outcome of the disease. During the study period, 188 patients with ALL were admitted to the ICU. Leukopenia and neutropenia were present in 60-80% of the patients, and elevated leukocyte count was observed in 30-40%, while normal leukocyte count was only 10%-20%. CRP and procalcitonin levels were elevated, showing the presence of inflammation. Conclusions: Based on the retrospective analysis of the data, it can be said that the presented data are harbingers of critical conditions in children with ALL. Neurological, respiratory, cardiac, renal, and other parameters are important for patient treatment and management.

Acute kidney injury in acute heart failure–when to worry and when not to worry?

ABSTRACT Acute kidney injury is common in patients with acute decompensated heart failure. It is more common in patients with acute heart failure who suffer from chronic kidney disease. Worsening renal function is often defined as a rise in serum creatinine of more than 0.3 mg/dL (26.5 µmol/L) which, by definition, is acute kidney injury (AKI) stage 1. Perhaps the term AKI is more appropriate than worsening renal function as it is used universally by nephrologists, internists and other medical practitioners. In health, the heart and the kidney support each other to maintain the body's homeostasis. In disease, the heart and the kidney can adversely affect each other's function, causing further clinical deterioration. In patients presenting with acute heart failure and fluid overload, therapy with diuretics for decongestion often causes a rise in serum creatinine and AKI. However, in the longer term the decongestion improves survival and prevents hospital admissions despite rising serum creatinine and AKI. It is important to realize that renal venous congestion due to increased right-sided heart pressures in acute heart failure is a major cause of kidney dysfunction and hence decongestion therapy improves kidney function in the longer term. This review provides a perspective on the acceptable AKI with decongestion therapy, which is associated with improved survival, as opposed to AKI due to tubular injury related to sepsis or nephrotoxic drugs, which is associated with poor survival.

The role of infliximab in treating refractory cardiac sarcoidosis. Case series and systematic review of literature.

Cardiac sarcoidosis is associated with significant morbidity and mortality. Immunosuppressive treatment focuses on suppressing myocardial inflammation, which can lead to major adverse events especially when progressing to fibrosis. Conventional management usually includes steroids and steroid sparing agents such as methotrexate and azathioprine. Tumour necrosis factor alpha inhibitors are often reserved for those with a worsening clinical status and/or evidence of persistent inflammatory activity despite conventional therapy. Refractory cardiac sarcoidosis (CS) can be defined as the persistence or progression of active disease, evidenced either by lack of clinical response or persistence or progression of imaging abnormalities, despite being on conventional therapy. In the United Kingdom, tumour necrosis factor alpha inhibitors are currently not licensed for cardiac sarcoidosis as there are no randomised controlled trials to assess the efficacy of infliximab in this patient cohort. In this study, we present the outcomes of six patients treated with infliximab for refractory cardiac sarcoidosis at Royal Brompton Hospital and performed a systematic review of the existing literature on use of infliximab in cardiac sarcoidosis. We searched the Cochrane Library, OVID Medline, OVID Embase, Web of Science and Pubmed to identify 7 full-text studies assessing the role of infliximab in the management of cardiac sarcoidosis. Infliximab was found to play a vital role in stabilising refractory cardiac sarcoidosis by stemming clinical deterioration, arrythmia burden and even reducing steroids requirements. Further prospective trial data is necessary to validate these findings.

The Impact of Location and Asset Type on the Success of Advanced Airway Management in a Critical Care Transport Environment

ObjectiveAdvanced airway management (AAM) is a critical component of prehospital critical care. Airway management in flight can be more challenging because of spatial, ergonomic, and environmental factors. This study examines the frequency of in-flight intubation (IFI), first-pass success (FPS) rates, and definitive airway sans hypoxia/hypotension on first attempt (DASH-1A) across different locations of airway management. MethodsWe conducted a retrospective database analysis of all patients transported between January 2016 and July 2021 who received AAM from a single air medical service. Patient records were reviewed for location of intubation, patient characteristics, and FPS and DASH-1A rates. The primary outcome was the frequency of IFI. The secondary outcomes included FPS and DASH-1A rates by location and type of transport asset. ResultsDuring the study period, 473 patients required AAM. Three percent (15/473) of patients were intubated in an in-flight setting, 28% (130/473) were intubated on scene, and 70% (328/473) were intubated in a health care facility. The primary reason for IFI was unanticipated cardiac arrest or clinical deterioration. The overall FPS rate was 69% (328/473), and the DASH-1A rate was 49% (194/399). Based on the location of AAM, the FPS and DASH-1A rates were the lowest for on-scene intubations (56% [74/130] and 27% [20/74], respectively). Most of the on-scene AAM took place with rotor wing flight crews. ConclusionAirway management occurs infrequently in an in-flight setting and is necessary because of patient deterioration or cardiac arrest. Based on our results, we identified opportunities for targeted AAM quality improvement and clinical governance.

Association between Neuroimaging Scores and Clinical Status in Pediatric Patients Diagnosed with Metachromatic Leukodystrophy

Purpose: Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by arylsulfatase A deficiency, which leads to progressive demyelination in both the central and peripheral nervous systems, resulting in significant gross motor deterioration. This study aimed to analyze data concerning neuroimaging and clinical phenotypes of MLD patients, categorized by disease subtype.Methods: Patients diagnosed with MLD based on arylsulfatase A enzymatic activity, demyelination observed in brain magnetic resonance images, and/or pathogenic mutations were included in this study. The medical charts of 10 patients with confirmed MLD were retrospectively reviewed. We used a simplified magnetic resonance imaging (MRI) scoring system and clinical status, including survival. We analyzed the correlations between the scores of specific neuroimaging lesions and clinical status in two groups, categorized as late-infantile and juvenile types based on the age at symptom onset.Results: We detected a positive relationship between clinical function deterioration and MRI score (rho=0.59, P=0.002) in patients with MLD. This correlation was stronger in the late-infantile type (rho=0.700, P=0.003) than in the juvenile type (rho=0.513, P=0.029). A strong relationship was also noted in patients with high signal intensities in the pons and basal ganglia, and cerebellar atrophy, but not in those with lesions in the midbrain. MLD with a high MRI score was associated with poor clinical function. Conclusion: The identified correlations between modified MRI scores and clinical function scales may help predict the prognosis of patients with MLD, thereby aiding in the identification of treatment options and enhancing the quality of life for these patients.

Clinical and epidemiological analysis of intestinal infections in a children's infectious diseases hospital, experience in using the PEWS scale

Purpose of the work: to conduct a clinical-epidemiological analysis of intestinal infections in a pediatric infectious diseases hospital and the effectiveness of the PEWS scale.Materials and methods: a retrospective study was conducted at the Moscow City Clinical Hospital «Communarka» and City Clinical Hospital No. 1 in the pediatric infectious disease departments from June 1, 2023, to August 31, 2023. Laboratory, clinical, and anamnestic data were obtained from the medical information system «EMIAS» and the laboratory information system (LIS) «ALISA». The study analyzed 838 medical records of children with acute respiratory infections.Results: when conducting a statistical analysis of indicators in two groups (group 1 — PEWS ≥ 3 points, recorded at least once during the entire period of hospitalization, group 2 — PEWS from 1 to 2 points, recorded at least once during hospitalization period) no significant differences were observed in terms of etiology, age, day of illness, blood test results, and length of hospital stay. All patients had a moderate form of acute respiratory infection, however, patients with PEWS ≥ 3 points required increased attention from medical staff and had a higher risk of deterioration without additional medical intervention, with the most significant risk in predicting deterioration being related to changes in the cardiovascular system. All children were discharged home in satisfactory condition with PEWS = 0.Conclusion: In our conducted research, for the first time in the Russian Federation, the experience of using the PEWS scale in pediatric practice for patients with gastrointestinal infections was applied with the aim of early detection of the risk of clinical deterioration. It can be assumed that the most significant risk in predicting deterioration in the condition of a child with acute gastrointestinal infection, as well as with acute respiratory infection, is predominantly due to disturbances in the cardiovascular system.

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment.

Macrophage activation syndrome (MAS) is potentially fatal; so, early diagnosis and timely treatment are essential. However, detecting MAS is sometimes challenging because its principal features can be observed in other pediatric diseases that cause severe inflammation. Cytokine storm due to immune dysregulation represents the clinical and laboratory features of MAS that are included in the diagnostic criteria. Most cases of MAS occur as an underlying condition worsens and progresses. Therefore, a patient with autoimmune or autoinflammatory disease who shows unexplained clinical deterioration despite appropriate management should be considered at high risk for MAS (i.e., occult MAS). The basic principles of treatment are control of triggering factors, supportive care, and relief of hyperinflammation. Systemic steroids and cyclosporine A are frequently used as a first-line treatment. For the treatment of refractory MAS, cytokine-specific biologic agents such as anakinra have recently become preferred over traditional immunosuppressive agents such as etoposide. MAS might be underrecognized in pediatric patients with infectious and inflammatory diseases due to its diverse clinical presentations. Clinical suspicion of MAS is of the utmost importance for early recognition of the disease.

Discontinuation of Palliative Brain Radiotherapy in Patients with Brain Metastases: A Case-Control Study.

Background: Discontinuation of radiotherapy is rarely discussed in the scientific literature. The goal of this study was, therefore, to estimate the frequency of and reasons for treatment discontinuations in patients receiving radiotherapy for brain metastases from solid tumors and to identify factors predicting said discontinuations. Methods: All patients treated for brain metastases from solid tumors between 2010 and 2020 at our institution were retrospectively reviewed. In addition to collecting relevant patient characteristics, the Recursive Partitioning Analysis (RPA) and disease-specific Graded Prognostic Assessment (GPA) groups for each patient were calculated to assess the performance of these scores in predicting treatment discontinuations. Results: Out of 468 patients who underwent cranial radiotherapy, 35 treatments (7.5%) were discontinued. The most frequent reason was clinical deterioration, which was documented in 26 (74.3%) of discontinued treatments. Patients whose radiotherapy was discontinued had, on average, more leptomeningeal disease (20.0% vs. 12.6%), worse ECOG performance status (mean ECOG performance status 1.86 vs. 1.39), and more uncontrolled extracranial metastases (85.3% vs. 70.8%). The frequencies of treatment discontinuation increased with worse prognosis and differed significantly across RPA groups (p = 0.037) but not across GPA groups (p = 0.612). Conclusions: Treatment discontinuation occurred in 7.5% of cases, mostly due to clinical deterioration. Poor performance status, as well as more advanced disease and, in turn, poor prognosis, were associated with higher discontinuation rates.

Vanishing white matter disease: imaging, clinical and molecular correlation in Brazilian families.

To characterize Vanishing White Matter Disease (VWM) cases from a Brazilian University Tertiary hospital, focusing on brain magnetic resonance image (MRI) aspects, clinical and molecular data. Medical records and brain MRI of 13 genetically confirmed VWM patients were reviewed. Epidemiological data such as age at symptom onset, gender and main symptoms were analyzed, along with genetic mutations and MRI characteristics, such as the distribution of white matter lesions and atrophy. The majority of patients were female, with the age of symptom onset ranging from 1year and 6months to 40years. All mutations were identified in the EIF2B5 gene, the most prevalent being c.338G > A (p.Arg113His), and a novel mutation related to the disease was discovered, c.1051G > A (p.Gly351Ser). Trauma or infection were significant triggers. The most frequent symptoms were ataxia and limb spasticity. All MRI scans displayed deep white matter involvement, cystic degeneration, with U-fibers relatively spared and a predilection for the frontoparietal region. Lesions in the corpus callosum and posterior fossa were present in all patients. Follow-up exams revealed the evolution of white matter lesions and cerebral atrophy, which correlated with clinical deterioration. VWM affects various age groups, with a significant clinical and genetic variability. A novel mutation associated with the disease is highlighted. MRI reveals a typical pattern of white matter involvement, characterized by diffuse lesions in the periventricular and deep regions, with subsequent extension to the subcortical areas, accompanied by cystic degeneration, and plays a crucial role in diagnosis and follow-up.

PALC-02. PALLIATIVE RADIOTHERAPY FOR HAEMOSTASIS IN CHILDREN WITH CEREBRAL METASTATIC HAEMORRHAGE

Abstract BACKGROUND Among children, bleeding from brain metastases is rare. Bleeding could be caused by: cancer, treatments and chemotherapy/other drugs such as Bevacizumab, NSAIDs and anticoagulants. We report a case of brain RT after metastases bleeding. METHODS A 14-years-old boy with a negative previous medical history presented to the Emergency Department with seizures and loss of consciousness. Full-body CT scan revealed brain and renal haemorrhages and a right testicular lesion. Serum βhCG was 223,773 mIU/mL. Neurosurgical bleeding control and right orchiectomy were performed: the histopathology revealed a metastatic mixed non-seminomatous germ cell neoplasia of the testis. First-line chemotherapy (4 cycles of Cisplatin/Etoposide/Ifosfamide) and second-line treatments (Paclitaxel/Ifosfamide/Cisplatin and Gemcitabine/Paclitaxel/Oxaliplatin) were performed. Suddenly, he presented with left-sided hyposthenia and partial left hemibody seizure. CT scan revealed 2 haemorrhagic brain lesions, one in each hemisphere. Surgery was not feasible. We started anti-oedema and anti-epileptic therapy, and whole brain RT (WBRT, 36 Gy/12 fx, BEDα/β= 10 Gy= 46.8 Gy). After 5 days from the start, RT had a 7-day stop because of acute oedema and clinical deterioration. Fourth-line chemotherapy was administered with CarboPEB (1st of further 4 cycles), and then WBRT was restarted. At the end of WBRT a stereotactic RT boost to both the haemorrhagic lesions was delivered (20 Gy/10 fx, BEDα/β= 10 Gy= 24.0 Gy). As of today, eight months after the end of therapy, the patient is free from disease and βhCG is negative. CONCLUSION Palliative RT in children is a non-conventional indication, but potentially effective at providing rapid haemostasis, especially in selected cases (surgery not feasible/contraindicated, adult-type histologies, …) and it is ensuring a good tumor control and low-grade acute toxicities. Hypofractionation is suggested to balance patient comfort and treatment effectiveness, especially when life expectancy is short. The optimal RT schedule, dose and fractionations need to be further investigated.

LMIC-25. EXPANDING THE PEDIATRIC NEURO-ONCOLOGY TELECONFERENCE EXPERIENCES, A LUXURY, OR A NECESSITY?

Abstract INTRODUCTION Videoteleconference in neurooncology is feasible and sustainable. The well-established 20 years’ Sickkids/KHCC teleconferencing is an example. Since 2018, several regional centers joined these meetings to discuss their patients’ management plans. We aimed to evaluate the impact of this experience. METHODS We retrospectively reviewed the 56 meetings’ minutes (9/2018-12/2023) and compared the pre-conference suggested plans with the post-conference recommendations. We recorded if the recommendations were implemented and the impact perceived. RESULTS In total, 251 patients were discussed: KHCC (137) and non-KHCC (114). Four of the regional participating oncologists had dedicated pediatric neuro-oncology training. Patients were selected for discussion due to challenges in their management (i.e. with relapsed tumors, rare diagnoses, unexpected tumor behavior). Main diagnoses were: Low-grade-glioma (28%), High-grade-glioma (21%), and Medulloblastoma (17%). Of the 227 patients where the local team suggested a care plan, the teleconference recommendations concurred with the proposed plan in 50% of the cases, agreed on but provided an alternative plan option in 18% and disagreed on in 32% of the cases. The difference in recommendations mostly affected the proposed treatment modality (15%). In 64% of the discordant plans and 50% of the alternative plans, the treating team applied the recommendations. Challenges to apply the recommendations were mainly linked to patients’ factors (53%, plan refusal, traveling abroad, clinical deterioration), local multidisciplinary team consensus on a different plan (29%) or logistic difficulties (18%, e.g. drug access). CONCLUSIONS Improvement in patients’ care was likely achieved with the continuous effort of the local teams to implement the recommendations. Participating oncologists valued the shared educational experience learned from each other’s case discussions, especially those related to molecular-testing and implications on treatment. Joining such regional teleconferences is of particular importance to small-volume centers or those lacking a pediatric neurooncologist. Regional and international collaborations are a necessity when it comes to improving the care of children with CNS tumors.

METB-13. GERMLINE GENETIC TESTING IN CHILDREN WITH CNS TUMORS; FIRST-EVER EXPERIENCE FROM JORDAN

Abstract BACKGROUND It is expected that 10-15% of children with CNS tumors have an underlying cancer predisposition syndrome (CPS). This has important implications on treatment, cancer screening and family counseling. There is no published data on this genetic risk from Jordan. METHODS We retrospectively reviewed the medical charts of the Jordanian children &amp;lt;18 years old at the time of diagnosis with CNS tumors, and were treated at King Hussein Cancer Center/Jordan between January 2021 and December 2023. We reviewed their clinical characteristics and tumor diagnoses, then applied the updated Jongmans’ criteria and MIPOGG criteria and linked that with their germline genetic testing result if performed. RESULTS We identified 198 children (53% males); median age at diagnosis was 7years (range, 0-18 years). Consanguinity was found in 30% of families, and 35% had first/second degree relatives with cancer. Most common diagnoses were LGG (41%), HGG (22%) and medulloblastoma (14%). Most tumors were non-metastatic (90%) at initial diagnosis. Fifty-five percent of patients fulfilled the updated Jongmans’ criteria and 36% the MIPOGG criteria, however germline genetic testing was only performed for 34 patients (17%). Pathogenic germline variants were found in 15 patients (44%); 6 LGG (NF1 (3), TSC2, PTPN11, MUTYH), 4 medulloblastoma (SDHB, TP53, BRCA2, CHEK2), 4 HGG (MSH6 (3), TP53), and one had pathogenic NF2 gene. The presence of CPS affected the management received: children with NF1 were treated without tumor biopsy, the child with TSC2 received everolimus, and 3 children with MSH6 received checkpoint inhibitors instead of temozolomide. The underlying CPS also predicted prognosis e.g. rapid clinical deterioration in the TP53-mutated-SHH-medulloblastoma. Partial screening was provided to children with NF1, TSC, NF2, TP53 and BMMRD mutations. CONCLUSIONS High proportion of our patients fulfilled the criteria for referral to genetic clinic, but less than half were referred. Increasing awareness of these criteria is important. Identification of CPS impacted treatment and prognosis, yet families’ perceptions and attitudes toward these CPS need to be captured.