How to follow the guidelines, when the appropriate fluid is missing?

Intravenous maintenance fluid therapy (IV-MFT) is probably the most prescribed drug in paediatric hospital care. Recently paediatric societies have produced evidence-based practice guidelines that recommend the use of balanced isotonic fluid when prescribing IV-MFT in both acute and critical paediatric care. Unfortunately, the applicability of these guidelines could be called into question when a ready-to-use glucose-containing balanced isotonic fluid is not available. The main objective of this study was to describe the availability of glucose-containing balanced isotonic fluids in European and Middle Eastern paediatric acute and critical care settings. This work is an ancillary study of the survey dedicated to IV-MFT practices in the paediatric acute and critical care settings in Europe and Middle East, a cross-sectional electronic 27-item survey, emailed in April-May 2021 to paediatric critical care physicians across 34 European and Middle East countries. The survey was developed by an expert multi-professional panel within the European Society of Peadiatric and Neonatal Intensive Care (ESPNIC). Balanced isotonic fluid with glucose 5% was available for only 32/153 (21%) responders. Balanced isotonic fluid with glucose 5% was consistently available in the UK (90%) but not available in France, Greece, The Netherlands and Turkey.    Conclusion: Ready-to-use isotonic balanced IV solutions containing glucose in sufficient amount exist but are inconsistently available throughout Europe. National and European Medication Safety Incentives should guarantee the availability of the most appropriate and safest IV-MFT solution for all children. What is Known: • Intravenous maintenance fluid therapy (IV-MFT) is probably the most prescribed drug in paediatric hospital care. • Balanced isotonic fluid is recommended when prescribing IV-MFT in both acute and critical paediatric care. What is New: • Balanced isotonic fluid with glucose 5% is available for less than 25% of the prescribers in Europe and the Middle East. Availability of balanced isotonic fluid with glucose 5% varies from one country to another but can also be inconsistent within the same country. • Clinicians who have access to a ready-to-use balanced isotonic fluid with glucose 5% are more likely to consider its use than clinicians who do not have access to such an IV solution.

Fulminant Viral Hepatitis in Two Siblings with Inherited IL-10RB Deficiency.

Fulminant viral hepatitis (FVH) caused by hepatitis A virus (HAV) is a life-threatening disease that typically strikes otherwise healthy individuals. The only known genetic etiology of FVH is inherited IL-18BP deficiency, which unleashes IL-18-dependent lymphocyte cytotoxicity and IFN-γ production. We studied two siblings who died from a combination of early-onset inflammatory bowel disease (EOIBD) and FVH due to HAV. The sibling tested was homozygous for the W100G variant of IL10RB previously described in an unrelated patient with EOIBD. We show here that the out-of-frame IL10RB variants seen in other EOIBD patients disrupt cellular responses to IL-10, IL-22, IL-26, and IFN-λs in overexpression conditions and in homozygous cells. By contrast, the impact of in-frame disease-causing variants varies between cases. When overexpressed, the W100G variant impairs cellular responses to IL-10, but not to IL-22, IL-26, or IFN-λ1, whereas cells homozygous for W100G do not respond to IL-10, IL-22, IL-26, or IFN-λ1. As IL-10 is a potent antagonist of IFN-γ in phagocytes, these findings suggest that the molecular basis of FVH in patients with IL-18BP or IL-10RB deficiency may involve excessive IFN-γ activity during HAV infections of the liver. Inherited IL-10RB deficiency, and possibly inherited IL-10 and IL-10RA deficiencies, confer a predisposition to FVH, and patients with these deficiencies should be vaccinated against HAV and other liver-tropic viruses.

JAK Inhibitor Therapy in a Child with Inherited USP18 Deficiency.

Deficiency of ubiquitin-specific peptidase 18 (USP18) is a severe type I interferonopathy. USP18 down-regulates type I interferon signaling by blocking the access of Janus-associated kinase 1 (JAK1) to the type I interferon receptor. The absence of USP18 results in unmitigated interferon-mediated inflammation and is lethal during the perinatal period. We describe a neonate who presented with hydrocephalus, necrotizing cellulitis, systemic inflammation, and respiratory failure. Exome sequencing identified a homozygous mutation at an essential splice site on USP18. The encoded protein was expressed but devoid of negative regulatory ability. Treatment with ruxolitinib was followed by a prompt and sustained recovery. (Funded by King Saud University and others.).

The burden of viral infections in pediatric intensive care unit between endemic and pandemic coronavirus infections: A tertiary care center experience.

OBJECTIVES: To measure the prevalence of viral infections, length of stay (LOS), and outcome in children admitted to the pediatric intensive care unit (PICU) during the period preceding the COVID-19 pandemic in a MERS-CoV endemic country. METHODS: A retrospective chart review of children 0-14 years old admitted to PICU with a viral infection. RESULTS: Of 1736 patients, 164 patients (9.45%) had a positive viral infection. The annual prevalence trended downward over a three-year period, from 11.7% to 7.3%. The median PICU LOS was 11.6 days. Viral infections were responsible for 1904.4 (21.94%) PICU patient-days. Mechanical ventilation was used in 91.5% of patients, including noninvasive and invasive modes. Comorbidities were significantly associated with intubation (P-value = 0.025). Patients infected with multiple viruses had median pediatric index of mortality 2 (PIM 2) scores of 4, as compared to 1 for patients with single virus infections (p < 0.001), and a median PICU LOS of 12 days, compared to 4 in the single-virus group (p < 0.001). Overall, mortality associated with viral infections in PICU was 7 (4.3%). Patients with viral infections having multiple organ failure were significantly more likely to die in the PICU (p = 0.001). CONCLUSION: Viral infections are responsible for one-fifth of PICU patient-days, with a high demand for mechanical ventilation. Patients with multiple viral infections had longer LOS, and higher PIM 2 scores. The downward trend in the yearly rate of PICU admissions for viral infections between the end of the MERS-CoV outbreak and the start of the COVID-19 pandemic may suggest viral interference that warrants further investigations.

Intravenous maintenance fluid therapy practice in the pediatric acute and critical care settings: a European and Middle Eastern survey.

The ideal fluid for intravenous maintenance fluid therapy (IV-MFT) in acutely and critically ill children is controversial, and evidence-based clinical practice guidelines are lacking and current prescribing practices remain unknown. We aimed to describe the current practices in prescribing IV-MFT in the context of acute and critically ill children with regard to the amount, tonicity, composition, use of balanced fluids, and prescribing strategies in various clinical contexts. A cross-sectional electronic 27-item survey was emailed in April-May 2021 to pediatric critical care physicians across European and Middle East countries. The survey instrument was developed by an expert multi-professional panel within the European Society of Pediatric and Neonatal Intensive Care (ESPNIC). A total of 154 respondents from 35 European and Middle East countries participated (response rate 64%). Respondents were physicians in charge of acute or critically ill children. All respondents indicated they routinely use a predefined formula to prescribe the amount of IV-MFT and considered fluid balance monitoring very important in the management of acute and critically ill children. The use of balanced solution was preferred if there were altered serum sodium and chloride levels or metabolic acidosis. Just under half (42%, 65/153) of respondents believed balanced solutions should always be used. Respondents considered the use of isotonic IV solutions as important for acute and critically ill children. In terms of the indication and the composition of IV-MFT prescribed, responses were heterogeneous among centers. Almost 70% (107/154) respondents believed there was a gap between current practice and what they considered ideal IV-MFT due to a lack of guidelines and inadequate training of healthcare professionals.   Conclusions: Our study showed considerable variability in clinical prescribing practice of IV-MFT in acute pediatric settings across Europe and the Middle East. There is an urgent need to develop evidence-based guidelines for IV-MFT prescription in acute and critically ill children. What is Known: • The administration of maintenance intravenous fluid therapy is a standard of care for a lot of hospitalized children • Maintenance intravenous fluid therapy prescriptions are often based on Holliday and Segar's historical guidelines even if this practice has been associated with several complications. What is New: • This study provided information on the prescribing practice regarding fluid restriction, fluid tonicity, and balance. • This study showed considerable variability in clinical prescribing practice of intravenous maintenance fluid therapy across Europe and the Middle East.

Initial Fluid Balance Associated Outcomes in Children With Severe Sepsis and Septic Shock.

INTRODUCTION: Net fluid balance and its role in sepsis-related mortality is not clear; studies suggest that aggressive fluid resuscitation can help in treatment, whereas others consider it is associated with poor outcomes. This study aimed to clarify the possible association of initial 24 hours' fluid balance with poor outcomes in pediatric patients with sepsis. METHODS: Retrospective data analysis included pediatric patients admitted with suspected or proven sepsis or septic shock to pediatric intensive care unit (PICU) of a tertiary care teaching hospital in Saudi Arabia. RESULTS: The study included 47 patients; 13 (28%) died, and mortality rate was significant in children with neurologic failure (P < 0.02), mechanical ventilation within 24 hours of admission (P < 0.03), leukopenia (P < 0.02), abnormal international normalized ratio (P < 0.02), initial blood lactate levels higher than 5 mmol/L (P < 0.02), or positive fluid balance at 24 hours of admission to the PICU (P < 0.001). CONCLUSION: Among children with sepsis and/or septic shock, there is significant association between mortality and initial high blood lactate levels and positive fluid balance at 24 hours from admission to the PICU.

Pediatric resident's perception of night float system compared to 24 hours system, a prospective study.

BACKGROUND: The study aims to evaluate the perceptions of pediatric residents under the night float (NF) on-call system and its impact on well-being, education, and patient safety compared with the traditional 24-h on-call system. METHODS: The study is prospective in nature and conducted on two pediatric resident training centers who apply the NF on-call system as a pilot project. Senior residents (PGY-3 and PGY-4) enrolled in the two training centers were invited to participate before and 6 months after the implementation of the change in the on-call system. A self-administered online questionnaire was distributed. Responses were rated using a five-point Likert-type scale (1 = strongly disagree; 5 = strongly agree). The items covered three main domains, namely, residents' well-being, ability to deliver healthcare, and medical education experience. Pre- and post-intervention scores were presented as means and compared by t-test for paired samples. RESULTS: A total of 42 residents participated in the survey (female = 24; 57.1%). All participants were senior residents; 25 (59.6%) were third-year residents (PGY-3), whereas 17 (40.4%) were fourth-year residents (PGY-4). The participants reported that many aspects of the three domains were improved with the introduction of the NF system. The system was perceived to exert less adverse health effect on the residents (mean: 2.37 ± 1.01) compared with the 24-h on-call system (mean: 4.19 ± 0.60; P < 0.001). In addition, the NF system was perceived to lead to less exposure to personal harm and result in less negative impact on quality of care, better work efficiency, reduced potential for medical errors, more successful teaching, and less disruptions to other rotations compared with the 24 h on-call system (P < 0.001). CONCLUSION: The perception of senior residents toward the 24-h on-call system pertains to negative impacts on well-being, education, and patient safety compared with on-call systems with restrictive duty hours, such as the NF system, which is perceived to be less harmful, to exert positive impacts on the quality of delivered healthcare services, and more useful from pedagogic aspect.

Remote interviews for medical residency selection during the initial COVID-19 crisis: a national survey.

BACKGROUND: Medical training programs candidate's interview is an integral part of the residency matching process. During the coronavirus disease 2019 (COVID-19) pandemic, conducting these interviews was challenging due to infection prevention restrains (social distancing, namely) and travel restrictions. E-interviews were implemented by the Saudi Commission for Healthcare Specialties (SCFHS) since the matching cycle of March 2020 to hold the interviews in a safer virtual environment while maintaining the same matching quality and standards. AIM: This study was conducted to assess the medical training residency program applicants' satisfaction, stress, and other perspectives for the (SCFHS) March 2020 Matching-cycle conducted through an urgently implemented E-interviews process. METHOD: A cross-sectional, nationwide survey (Additional file 1) was sent to 4153 residency-nominated applicants to the (SCFHS) March 2020 cycle. RESULTS: Among the 510 candidates who responded, 62.2% applied for medical specialties, 20.2% applied for surgical specialties, and 17.6% applied for critical care and emergency specialties. Most respondents (61.2%) never had previous experience with web-based video conferences. Most respondents (80.2%) used the Zoom application to conduct the current E-interviews, whereas only 15.9% used the FaceTime application. 63.3% of the respondents preferred E-interviews over in-person interviews, and 60.6% rated their experience as very good or excellent. 75.7% of the respondents agreed that all their residency program queries were adequately addressed during the E-interviews. At the same time, 52.2% of them agreed that E-interviews allowed them to represent themselves accurately. 28.2% felt no stress at all with their E-interviews experience, while 41.2% felt little stressed and only 8.2% felt highly stressed. The factors that were independently and inversely associated with applicants' level of stress with E-interviews experience were their ability to represent themselves during the interviews (p = 0.001), cost-savings (p < 0.001), their overall rating of the E-interviews quality (p = 0.007) and the speed of the internet connection (p < 0.006). CONCLUSION: Videoconferencing was implemented on an urgent basis during the COVID-19 pandemic in the medical residency application process in Saudi Arabia. It was perceived as an adequate and promising tool to replace in-person interviews in the future. Applicants' satisfaction was mainly driven by good organization, cost-saving, and their ability to present themselves. Future studies to enhance this experience are warranted.

The clinical utility of rapid exome sequencing in a consanguineous population.

BACKGROUND: The clinical utility of exome sequencing is now well documented. Rapid exome sequencing (RES) is more resource-intensive than regular exome sequencing and is typically employed in specialized clinical settings wherein urgent molecular diagnosis is thought to influence acute management. Studies on the clinical utility of RES have been largely limited to outbred populations. METHODS: Here, we describe our experience with rapid exome sequencing (RES) in a highly consanguineous population. Clinical settings included intensive care units, prenatal cases approaching the legal cutoff for termination, and urgent transplant decisions. RESULTS: A positive molecular finding (a pathogenic or likely pathogenic variant that explains the phenotype) was observed in 80 of 189 cases (42%), while 15 (8%) and 94 (50%) received ambiguous (variant of uncertain significance (VUS)) and negative results, respectively. The consanguineous nature of the study population gave us an opportunity to observe highly unusual and severe phenotypic expressions of previously reported genes. Clinical utility was observed in nearly all (79/80) cases with positive molecular findings and included management decisions, prognostication, and reproductive counseling. Reproductive counseling is a particularly important utility in this population where the overwhelming majority (86%) of identified variants are autosomal recessive, which are more actionable in this regard than the de novo variants typically reported by RES elsewhere. Indeed, our cost-effectiveness analysis shows compelling cost savings in the study population. CONCLUSIONS: This work expands the diversity of environments in which RES has a demonstrable clinical utility.

Outcomes of Cardiopulmonary Resuscitation in the Pediatric Intensive Care of a Tertiary Center.

Understanding the factors affecting survival and modifying the preventable factors may improve patient outcomes following cardiopulmonary resuscitation (CPR). The aim of this study was to assess the prevalence and outcomes of cardiac arrest and CPR events in a tertiary pediatric intensive care unit (PICU). Outcomes of interest were the return of spontaneous circulation (ROSC) lasting more than 20 minutes, survival for 24 hours post-CPR, and survival to hospital discharge. We analyzed data from the PICU CPR registry from January 1, 2011 to January 1, 2018. All patients who underwent at least 2 minutes of CPR in the PICU were included. CPR was administered in 65 PICU instances, with a prevalence of 1.85%. The mean patient age was 32.7 months. ROSC occurred in 38 (58.5%) patients, 30 (46.2%) achieved 24-hour survival, and 21 (32.3%) survived to hospital discharge. Younger age ( p < 0.018), respiratory cause ( p < 0.001), bradycardia ( p < 0.018), and short duration of CPR ( p < 0.001) were associated with better outcomes, while sodium bicarbonate, norepinephrine, and vasopressin were associated with worse outcome ( p < 0.009). The off-hour CPR had no impact on the outcome. The patients' cumulative predicted survival declined by an average of 8.7% for an additional 1 minute duration of CPR ( p = 0.001). The study concludes that the duration of CPR, therefore, remains one of the crucial factors determining CPR outcomes and needs to be considered in parallel with the guideline emphasis on CPR quality. The lower survival rate post-ROSC needs careful consideration during parental counseling. Better anticipation and prevention of CPR remain ongoing challenges.

Enhancing Expert Panel Discussions in Pediatric Palliative Care: Innovative Scenario Development and Summarization With ChatGPT-4.

This study presents a novel approach to enhance expert panel discussions in a medical conference through the use of ChatGPT-4 (Generative Pre-trained Transformer version 4), a recently launched powerful artificial intelligence (AI) language model. We report on ChatGPT-4's ability to optimize and summarize the medical conference panel recommendations of the first Pan-Arab Pediatric Palliative Critical Care Hybrid Conference, held in Riyadh, Saudi Arabia. ChatGPT-4 was incorporated into the discussions in two sequential phases: first, scenarios were optimized by the AI model to stimulate in-depth conversations; second, the model identified, summarized, and contrasted key themes from the panel and audience discussions. The results suggest that ChatGPT-4 effectively facilitated complex do-not-resuscitate (DNR) conflict resolution by summarizing key themes such as effective communication, collaboration, patient and family-centered care, trust, and ethical considerations. The inclusion of ChatGPT-4 in pediatric palliative care panel discussions demonstrated potential benefits for enhancing critical thinking among medical professionals. Further research is warranted to validate and broaden these insights across various settings and cultures.

Presentations and outcomes of familial hemophagocytic lymphohistiocytosis in the pediatric intensive care units (PICUs).

Objectives: We aimed to describe Familial Hemophagocytic Lymphohistiocytosis (F-HLH) patients' clinical features, intensive care courses, and outcomes. Methods: Multi-center retrospective cohort study of pediatric patients diagnosed with F-HLH from 2015 to 2020 in five tertiary centers in Saudi Arabia. Patients were classified as F-HLH based on their genetic confirmation of known mutation or on their clinical criteria, which include a constellation of abnormalities, early disease onset, recurrent HLH in the absence of other causes, or a family history of HLH. Results: Fifty-eight patients (28 male, 30 female), with a mean age of 21.0 ± 33.9 months, were included. The most common principal diagnosis was hematological or immune dysfunction (39.7%), followed by cardiovascular dysfunction in 13 (22.4%) patients. Fever was the most common clinical presentation in 27.6%, followed by convulsions (13.8%) and bleeding (13.8%). There were 20 patients (34.5%) who had splenomegaly, and more than 70% of patients had hyperferritinemia >500 mg/dl, hypertriglyceridemia >150 mg/dl and hemophagocytosis in bone marrow biopsy. Compared to deceased patients 18 (31%), survivors had significantly lower PT (p = 041), bilirubin level of <34.2 mmol/L (p = 0.042), higher serum triglyceride level (p = 0.036), and lesser bleeding within the initial 6 h of admission (p = 0.004). Risk factors for mortality included requirements of higher levels of hemodynamic (61.1% vs. 17.5%, p = 0.001) and respiratory (88.9% vs. 37.5%, p < 0.001) support, and positive fungal cultures (p = 0.046). Conclusions: Familial HLH still represents a challenge in the pediatric critical care setting. Earlier diagnosis and prompt initiation of appropriate treatment could improve F-HLH survival.

Propranolol-induced hyperkalemia in infantile hemangioma patients: How serious is it?

Background: Infantile hemangioma is the most frequent benign vascular tumor of infancy, which has a specific clinical history of early growth, followed by spontaneous remission. Since the fortuitous discovery of propranolol's efficacy in 2008, the management of infantile hemangioma has been quickly developing. Methods: This study is a retrospective cohort study. Electronic search in the patient's registry of King Khalid University Hospital, Riyadh, Saudi Arabia, was performed using the keywords hemangioma, haemangioma, infantile hemangioma, and vascular tumors. The search revealed a total of 101 subjects for which 56 were included and 45 were excluded. Results: A total of 56 patients with infantile hemangioma were evaluated in this study. The majority were females. The F: M ratio is 3.4:1. The highest percentage of the patients was delivered by the elective cesarian section, that is, 23 (41.1%), followed by spontaneous vaginal delivery, that is, 19 (33.9%). Full-term patients were 27 (48%), whereas the pre-term patients were 21 (37%). The total number of patients who developed hyperkalemia while on propranolol was 12 (31%). There was no statistically significant difference (P > 0.05) between patients who developed hyperkalemia and patients who did not develop hyperkalemia in terms of gender, gestational age, mode of delivery, size and location of hemangioma, or concomitant topical timolol use. Conclusion: Hyperkalemia is benign and transient, although solid conclusive opinions cannot be drawn because of the small sample size and the retrospective nature of the study.

Favipiravir Effectiveness and Safety in Hospitalized Moderate-Severe COVID-19 Patients: Observational Prospective Multicenter Investigation in Saudi Arabia.

Objectives: There are limited data on the efficacy and safety of favipiravir antiviral in coronavirus disease 2019 (COVID-19), particularly in the more progressed disease phase. This study aims to evaluate the favipiravir effect on reducing the length of hospital stay and in-hospital mortality among moderate and severe hospitalized COVID-19 patients. Methods: A prospective, multicenter observational study was conducted that included moderate and severe hospitalized adult COVID-19 patients in four major regions (Riyadh (Riyadh), Eastern (Dammam), Al-Qassem (Buraydah), and Macca (Jeddah) of Saudi Arabia. For the primary outcome of all-cause mortality, a Cox proportional hazard analysis was performed. While the association between favipiravir use and length of hospital stay was determined using adjusted generalized linear model. This study was approved by the Central Institutional Review Board in The Saudi Ministry of Health (MoH) with the approval number IRB # 20-85-M. Results: This study included 598 moderate and severe COVID-19 patients, of whom 156 (26%) received favipiravir. Favipiravir treatment was associated with more extended hospital stays (14 vs. 10 median days, P = 0.034) and higher mortality rate (aHR 3.63; 95% CI 1.06-12.45) compared to no favipiravir regimen. Despite lack of effectiveness, favipiravir use was only associated with higher diarrhea adverse effects (12 vs. 5%, P = 0.002), but it did not affect the renal and liver profiles of patients. Conclusion: Favipiravir was ineffective in reducing the length of hospital stay and in-hospital mortality in patients with moderate and severe COVID-19.

Enhancing parental knowledge of childhood and adolescence safety: An interventional educational campaign.

BACKGROUND: Safeguarding children and adolescents from unintentional injuries is a significant concern for parents and caregivers. With them staying more at home during the coronavirus disease 2019 pandemic, more educational tools and valid educational programs are warranted to improve parental knowledge and awareness about childhood and adolescences' safety. This study aims to explore the effectiveness of childhood and adolescence safety campaigns on parents' knowledge and attitude toward preventable injuries. METHODS: This was a pre-post experimental study, in which the predesigned assessments were used as an evaluation tool before and after attending a childhood and adolescence safety campaign. The pre-post assessment question included questions to evaluate the socio-demographic status, followed by knowledge questions in line with the current childhood and adolescence safety campaign. The outcomes of interest were assessed before and after attending the campaign's stations. RESULTS: Three hundred eight parents volunteered to participate in this study. Their knowledge score improved from 36.2 [standard deviation (SD) 17.7] to 79.3 (SD 15.6) after attending the Campaign (t value = 34.6, P < .001). Both, perceptions on the preventability of accidents and the parents' perceived usefulness of educational campaigns showed improvements, with (t value = 6.3, P < .001) and (t value = 3.097, P < .001), respectively. CONCLUSION: The educational childhood and adolescence safety campaign for caregivers in Saudi Arabia resulted in a significant increase in the overall knowledge and attitudes toward childhood and adolescence's safety. As children and adolescents are currently staying at home more, additional educational tools and programs are warranted to promote safe practices among parents and caregivers.

ESPNIC clinical practice guidelines: intravenous maintenance fluid therapy in acute and critically ill children- a systematic review and meta-analysis.

PURPOSE: Intravenous maintenance fluid therapy (IV-MFT) prescribing in acute and critically ill children is very variable among pediatric health care professionals. In order to provide up to date IV-MFT guidelines, the European Society of Pediatric and Neonatal Intensive Care (ESPNIC) undertook a systematic review to answer the following five main questions about IV-MFT: (i) the indications for use (ii) the role of isotonic fluid (iii) the role of balanced solutions (iv) IV fluid composition (calcium, magnesium, potassium, glucose and micronutrients) and v) and the optimal amount of fluid. METHODS: A multidisciplinary expert group within ESPNIC conducted this systematic review using the Scottish Intercollegiate Guidelines Network (SIGN) grading method. Five databases were searched for studies that answered these questions, in acute and critically children (from 37 weeks gestational age to 18 years), published until November 2020. The quality of evidence and risk of bias were assessed, and meta-analyses were undertaken when appropriate. A series of recommendations was derived and voted on by the expert group to achieve consensus through two voting rounds. RESULTS: 56 papers met the inclusion criteria, and 16 recommendations were produced. Outcome reporting was inconsistent among studies. Recommendations generated were based on a heterogeneous level of evidence, but consensus within the expert group was high. "Strong consensus" was reached for 11/16 (69%) and "consensus" for 5/16 (31%) of the recommendations. CONCLUSIONS: Key recommendations are to use isotonic balanced solutions providing glucose to restrict IV-MFT infusion volumes in most hospitalized children and to regularly monitor plasma electrolyte levels, serum glucose and fluid balance.

Technical Aspects of Intensive Care Unit Management: A Single-Center Experience at a Tertiary Academic Hospital.

PURPOSE: Special technical issues associated with the function and maintenance of medical devices arise in intensive care units (ICUs). This study explored the level of comfort of ICU staff in dealing with selected equipment, the factors that are associated with the staff's ease of adaptation to new technologies, and the role of technical support staff. PATIENTS AND METHODS: This is a single-center cross-sectional questionnaire-based survey that was conducted in February 2018 and targeted nurses working in the ICUs of King Saud University Medical City in Riyadh, Saudi Arabia. RESULTS: Among the 297 nurses who completed the survey, almost all of the respondents (99.3%) were aware of the ICU equipment preventive maintenance program. Most of the nurses had received training on how to use infusion pumps (96.2%), cardiac monitoring systems (78.0%), and cardiac defibrillation devices (73.9%). Sixty nurses (20.2%) indicated that at least one super user was available for at least one device. About half of the staff reported one device whose user manual was available. Most nurses reported having no resources regarding updates on medical devices. CONCLUSION: Our findings revealed an alarming need to address technical issues related to medical devices used in the ICU and to design a framework for the safe operation of medical devices based on international practices. It is necessary to empower the role of the super user and medical device clinical educator as well as to optimize communication between the national regulatory body of medical devices and healthcare providers, especially those working in acute care areas.

2020 Update to Spinal Muscular Atrophy Management in Saudi Arabia.

Novel therapeutic strategies have shown some promise in treating spinal muscular atrophy (SMA). However, the outcomes and acceptance of these new strategies are yet to be explored. We aimed to investigate physicians' opinions and perceptions toward management strategies of SMA across Saudi Arabia. This is a cross-sectional survey using a self-administered, structured questionnaire sent to physicians who care for SMA patients during the Saudi Pediatric Neurology Society annual conference. A total of 72 clinicians of different neurological subspecialties were included. 48.6% prescribed nusinersen to their patients, with 39% of them having patients started on nusinersen. Though, 8.3% prescribed onasemnogene abeparvovec for 1-3 patients, while none of their patients started on the treatment. 64.3% stated that the only treatment available for SMA in their settings is supportive care. Around 69.4% described having a moderate to high knowledge on SMA gene therapy, and 79.2% would recommend it. 48.6% confirmed they would prescribe gene therapy at the age of 6 months, and 78.3% would prescribe it for type-I SMA. Pediatric neurologists are receptive to novel and innovative therapies for SMA in Saudi Arabia. However, the high treatment acquisition cost, strict regulations, logistical issues, and budget constraints delay their adoption and implementation.