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
. 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.).
OBJECTIVESTo 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. METHODSA retrospective chart review of children 0-14 years old admitted to PICU with a viral infection. RESULTSOf 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). CONCLUSIONViral 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.
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.
To explore the changing patterns of long-stay patients (LSP) to improve the utilization of pediatric intensive care units (PICUs) resources.
This is a 2-points cross-sectional study (5 years apart; ...2014-2019) conducted among PICUs and SCICUs in Riyadh, Saudi Arabia. Children who have stayed in PICU for more than 21 days were included.
Out of the 11 units approached, 10 (90%) agreed to participate. The prevalence of LSP in all these hospitals decreased from 32% (48/150) in 2014 to 23.4% (35/149) in 2019. The length of stay ranged from 22 days to 13.5 years. The majority of LSP had a neuromuscular or cardiac disease and were admitted with respiratory compromise. Ventilator-associated pneumonia was the most prevalent complication (37.5%). The most commonly used resources were mechanical ventilation (93.8%), antibiotics (60.4%), and blood-products transfusions (35.4%). The most common reason for the extended stay was medical reasons (51.1%), followed by a lack of family resources (26.5%) or lack of referral to long-term care facilities (22.4%).
A long-stay is associated with significant critical care bed occupancy, complications, and utilization of resources that could be otherwise utilized as surge capacity for critical care services. Decreasing occupancy in this multicenter study deserves further engagement of the healthcare leaders and families to maximize the utilization of resources.
With the evolving COVID-19 pandemic and the emphasis on social distancing to decrease the spread of SARS-CoV-2 among healthcare workers (HCWs), our pediatric intensive care unit (PICU) piloted the ...integration of Zoom meetings into clinical rounds. We aimed to explore the feasibility of these hybrid virtual and physical clinical rounds for PICU patients.
Mixed quantitative and qualitative deductive thematic content analysis of narrative responses.
PICU, single tertiary-care academic center.
Multidisciplinary PICU HCWs.
Integration of Zoom meeting into clinical daily PICU rounds.
For the quantitative part, we gathered the details of daily PICU hybrid rounds in terms of times, number of HCWs, and type of files shared through Zoom. For the qualitative part, open-ended questions were used.
The physical round took statistically significantly less time (34.68 ± 14.842 min) as compared with the Zoom round (72.45 ± 22.59 min),
< 0.001. The most shared component in the virtual round was chest X-rays (93.5%). Thirty-one HCWs participated in focus group discussions and were included in the analysis. Some of the HCWs' perceived advantages of the hybrid rounds were enabling multidisciplinary discussions, fewer round interruptions, and practicality of virtual discussions. The perceived challenges were the difficulty of the bedside nurse attending the virtual round, decreased teaching opportunities for the trainees, and decreased interactions among the team members, especially if video streaming was not utilized.
Multidisciplinary hybrid virtual and physical clinical rounds in the PICU were perceived as feasible by HCWs. The virtual rounds decreased the physical contact between the HCWs, which could decrease the possibility of SARS-CoV-2 spread among the treating team. Still, several components of the hybrid round should be optimized to facilitate the virtual team-members' interactions and enhance the teaching experience.
Cytomegalovirus (CMV) can rarely cause severe manifestations in immunocompetent individuals. Hereby, we report a twelve-year-old boy who presented with tachycardia, tachypnea, fever and leukocytosis, ...which progressed to hypoxemic respiratory failure and severe acute respiratory distress syndrome (ARDS). Subsequently, he developed multi-organ failure despite the ongoing full supportive care and empiric broad spectrum antibiotics. Cytomegalovirus infection was diagnosed by Polymerase Chain Reaction (PCR) in blood and histopathological examination of lung biopsy. Immunological work up for the child was unremarkable. Ganciclovir therapy was introduced and showed significant improvement until full recovery. However, our patient developed transient heart block as a rare complication for Ganciclovir therapy throughout his course. We present this case with literature review for the CMV infection associated morbidity and mortality among immunocompetent children.
To describe two young Saudi brothers with bilateral progressive retinal arterial aneurysms and a subtotal exudative retinal detachment with Coats-like presentation in the older sibling as the initial ...presentation of Familial Retinal Arterial Macroaneurysms (FRAM).
Two young Saudi brothers with a family history of consanguinity presented with the classic clinical presentation and genetic identification of FRAM. In this report, we describe the presence of prominent peripheral retinal capillary changes mimicking Coats' disease.
FRAM can present similar to bilateral Coats' disease and should be considered in the differential diagnosis of Coats-like retinopathy. The diagnosis of FRAM may have a significant implication because of the associated cardiac abnormality, such as supravalvular pulmonary stenosis, which should be evaluated by echocardiography and managed accordingly.
Objectives: The objective of the study wasto describe the effect of nusinersen therapy and its 1st-year associated outcomes, weaning of ventilator support, and length of stay in pediatric intensive ...care among ventilator-dependent spinal muscular atrophy (SMA) patients. Methods: This is a multicenter, retrospective case series study that was conducted in four tertiary care centers in Saudi Arabia. We report all the genetically diagnosed SMA ventilator-dependent patients who were admitted to the pediatric intensive care unit (PICU) and received a course of nusinersen therapy from (January 2017 to March 2018). The study endpoints were success to wean or extubate the patient and discharge from the PICU. Results: Five patients from four tertiary PICUs were included in this report. All five patients received a minimum of four doses of nusinersen intrathecally as per the manufacturer. At 12 months postinitiation of therapy, only one patient showed clinical improvement, while the other four patients did not experience improvement in their respiratory status following the administration of nusinersen. These four patients are still admitted in the PICU due to the inability to wean them from respiratory support. The remaining patient was discharged to home successfully and neither require any home ventilatory support nor oxygen therapy. Conclusion: In conclusion, our clinical experience and data suggest that the response to nusinersen therapy is very likely but mostly dependent on the time of initiation of nusinersen therapy and the cumulative number of doses among other variables related to disease severity. However, further studies targeting a larger group of patients with better standardized respiratory care are needed to investigate the effect of nusinersen on particularly the long-term outcomes.
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.
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.
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.
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.
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.
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.
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).
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.
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