The care of critically ill adults with COVID-19 in Ontario pediatric intensive care units.

PURPOSE: To describe and review the experience of two pediatric intensive care units (PICUs) in Ontario, Canada, adapting and providing care to critically ill adults during the COVID-19 pandemic. CLINICAL FEATURES: At a time of extreme pressure to adult intensive care unit (ICU) capacity, two PICUs provided care to critically ill adults with COVID-19 pneumonia. Substantial yet rapid planning was required to facilitate safe delivery of critical care to adult patients while maintaining PICU services, including thoughtful development of care pathways and patient selection. To prepare clinical staff, several communication strategies, knowledge translation, skill consolidation, and system-adaptation mechanisms were developed. There was iterative adaptation of operational processes, including staffing models, specialist consultation, and the pharmacy. Care provided by the interprofessional teams was reoriented as appropriate to the needs of critically ill adults in close collaboration with adult ICU teams. Forty-one adults were admitted to the two PICUs over a 12-week period. In total, 36 patients (88%) received invasive ventilation, eight patients (20%) were supported with venovenous extracorporeal membrane oxygenation, and six patients (15%) received continuous renal replacement therapy. Four died in the PICU during this period. Feedback from staff included anxiety around reorienting practice to the care of critically ill adults, physical exhaustion, and psychological distress. Importantly, staff also reported a renewed sense of purpose with participation in the program. CONCLUSION: Though challenging, the experience has provided opportunity to enhance collaboration with partner institutions and improve the care of older children and adolescents in the PICU.

RéSUMé: OBJECTIF: Nous avions pour objectif de décrire et examiner l'expérience de deux unités de soins intensifs pédiatriques (USIP) en Ontario, au Canada, qui se sont adaptées et ont fourni des soins aux adultes gravement malades pendant la pandémie de COVID-19. CARACTéRISTIQUES CLINIQUES: À l'époque où la capacité des unités de soins intensifs (USI) pour adultes était extrêmement sollicitée, deux USIP ont prodigué des soins à des adultes gravement malades souffrant de pneumonie liée à la COVID-19. Une planification substantielle mais rapide a été nécessaire pour faciliter la prestation sécuritaire de soins intensifs à une patientèle adulte tout en maintenant les services de l'USIP, y compris l'élaboration réfléchie des trajectoires de soins et la sélection de la patientèle. Pour préparer le personnel clinique, plusieurs stratégies de communication, d'application des connaissances, de consolidation des compétences et de mécanismes d'adaptation du système ont été élaborées. Il y a eu une adaptation progressive des processus opérationnels, y compris des modèles de dotation, des consultations de spécialistes et de la pharmacie. Les soins fournis par les équipes interprofessionnelles ont été réorientés en fonction des besoins des adultes gravement malades en étroite collaboration avec les équipes adultes de soins intensifs. Quarante et une personnes adultes ont été admises dans les deux unités de soins intensifs pédiatriques sur une période de 12 semaines. Au total, 36 adultes (88 %) ont bénéficié d'une ventilation invasive, huit personnes (20 %) ont reçu une oxygénation par membrane extracorporelle veino-veineuse, et six (15 %) ont reçu un traitement substitutif de l'insuffisance rénale en continu. Quatre personnes sont décédées à l'USIP au cours de cette période. Les commentaires du personnel comprenaient de l'anxiété liée à la réorientation de leur pratique vers des soins à des adultes gravement malades, l'épuisement physique et la détresse psychologique. Fait important, le personnel a également signalé un regain de motivation lié à la participation de leur USIP à ce programme. CONCLUSION: Bien que difficile, l'expérience a permis d'améliorer la collaboration avec les établissements partenaires et d'améliorer les soins aux enfants plus âgé·es et aux adolescent·es de l'USIP.

Identifying and Treating Severe Bone Marrow Necrosis and Fat Embolism Syndrome in Pediatric Patients With Sickle Cell Disease: A Case Report.

Fat embolism syndrome after bone marrow necrosis is an extremely rare complication in sickle cell disease associated with significant morbidity and mortality. A high index of suspicion is required for diagnosis. This case report will assist pediatric clinicians and hematologists to recognize this severe complication in patients with sickle cell disease and to promptly initiate treatment. Red flags include severe bone pain, respiratory distress, neurological impairment, decreasing platelet count, peripheral leukocyte left shift, elevated nucleated red blood cells, and significant elevation in plasma ferritin and lactate dehydrogenase. We report a pediatric patient who was diagnosed early, received urgent red cell exchange transfusion and plasma exchange, and ultimately survived this devastating complication.

The role of high density lipoprotein in Type 1 Gaucher disease.

Type I Gaucher Disease (GD1) is known to be associated with hypocholesterolemia and reduced levels of low density lipoprotein (LDL) and high density lipoprotein (HDL). In this study we aimed to correlate disease severity with HDL levels and to evaluate the effect of enzyme replacement therapy (ERT) on HDL levels as well as estimating the frequency of cardiovascular events in GD. Two groups of GD1 patients were evaluated: 30 untreated and 36 patients on ERT. Disease severity, biomarkers of GD and lipid levels were evaluated in the two groups. The Zimran Severity Score Index (SSI) was used to estimate disease severity and the effect of ERT on HDL levels was evaluated, as well as the frequency of cardiovascular disease. GD1 patients with more severe disease (SSI median 11) had significantly lower levels of HDL (median 23mg/dL), compared to patients with milder (SSI median 4.5) disease (median 37mg/dL p=0.001). HDL levels increased after ERT. Despite lower HDL levels in patients with more severe disease, a low frequency of cardiovascular events was detected. HDL level should be used in GD as a biomarker for diagnosis, monitoring and estimation of ERT effect.

Response to erythropoietin in pediatric patients with chronic kidney disease: insights from an in vitro bioassay.

BACKGROUND: Decreased production of erythropoietin (EPO) is a major cause of anemia associated with chronic kidney disease (CKD). Treatment with recombinant human EPO (rHuEPO) improves patients' quality of life and survival; however, there is a marked variability in response to rHuEPO. At present, no available laboratory test is capable of evaluating responsiveness to EPO treatment. The aim of the present study was to use an in vitro bioassay to estimate the effect of uremic environment on EPO-dependent erythroid cell proliferation. METHODS: EPO-dependent human erythroleukemia cells (UT-7) were incubated with exogenous EPO (2 u/ml) and sera obtained from 60 pediatric patients (aged 1-23 years). Three groups were studied: (1) 12 children on dialysis (4 peritoneal, 8 hemodialysis); (2) 28 patients with CKD 1-5 (not on dialysis), and (3) 20 healthy children. RESULTS: Sera from dialysis patients inhibited UT-7 cell growth compared to the CKD group and healthy controls at 48 h (p = 0.003 and p = 0.04, respectively) and 72 h of culture (p = 0.02 and p = 0.07, respectively). In 18 patients treated with rHuEPO, a significant inverse correlation was found between the EPO resistance index and cell proliferation at 48 h (p = 0.007, r = - 0.63) and 72 h (p = 0.03, r = - 0.52). CONCLUSIONS: Our findings support the presence of erythropoiesis inhibitory substances in uremic sera. EPO/EPO-R-dependent mechanisms may play a role in inhibiting erythropoiesis. The in vitro bioassay described herein may serve as an indicator of rHuEPO responsiveness which may encourage further investigation of underlying mechanisms of EPO resistance.

Positive IgM in Congenital CMV Infection.

Neonatal serum detection of cytomegalovirus (CMV) immunoglobulin M (IgM) has low sensitivity in identifying congenital cytomegalovirus (cCMV). Several reports have endeavored to associate the presence/absence of IgM to disease severity. Data were collected for all infants with cCMV followed in our clinic. Infant outcome after birth was compared between infants who tested positive or negative. Sensitivity of positive IgM in diagnosing cCMV was 40.7%. The rate of symptomatic disease in those who tested positive was statistically higher (67.7%, P < .001). Odds ratio for symptomatic disease in infants with positive IgM born after a maternal primary infection was 3.47 (95% confidence interval = 1.7-7.1). Positive IgM was found in only 48.8% of symptomatic and 22.1% of asymptomatic children. Our results demonstrated a low sensitivity of IgM in diagnosing cCMV. However, while a positive IgM antibody for CMV is associated with a more symptomatic disease, it does not serve as a precise laboratory marker for a severity.