Factors associated with adverse outcome among children with sickle cell disease admitted to the pediatric intensive care unit: an observational cohort.

BACKGROUND: Sickle cell disease (SCD) is one of the most frequent inherited diseases in the world. Over the last decades, in high-income countries, an important decrease in mortality have been observed due to the improvement of care. However, children with SCD can become critically ill and require admission in Pediatric Intensive Care Units (PICU). The purpose of this study was to describe the epidemiology of children with SCD admitted to PICU for acute crisis and to identify factors associated with adverse outcome (AO). METHODS: We conducted a retrospective study in a Tertiary Hospital in France including all consecutive children with SCD admitted to PICU between January 1st, 2009 and December 31, 2019. We collected baseline patient's characteristics, clinical and biological data as well as treatments and life sustaining therapies used in the PICU. Patients were defined as experiencing AO in case of death during stay and/or need for invasive mechanical ventilation (MV) and/or for non-invasive ventilation (NIV) for more than 3 days and/or need for vasopressors and/or need for renal replacement therapy. RESULTS: We included 579 admissions in 395 patients, mainly of SS genotype (90%) with a median age of 9.2 years [5.5-13.4] and a median baseline hemoglobin of 8.0 g/dl (7.5-8.8). The two main reasons for admission were acute chest syndrome (ACS) (n = 331, 57%) and vaso-occlusive crisis refractory to first line therapy (n = 99, 17%). Half of patients required NIV and 47 (8%) required MV. The overall length of stay was 3 days [1-4] and seven (1%) patients died during PICU stay.There was a total of 113 (20%) admissions with AO and on multivariable analysis, baseline hemoglobin < 8 g/dL, history of bronchial obstruction and admission for ACS were associated with AO. There was no difference in the proportion of hydroxyurea treatment or exchange transfusion program between patients with AO and the other patients. CONCLUSIONS: Baseline hemoglobin < 8 g/dL, history of bronchial obstruction and admission for ACS were the strongest risk factors for severe evolution in SCD children admitted to PICU. These factors could be taken into consideration when choosing the adequate therapeutic options.

Gasping in Dying Children: Health Care Professionals' Feelings and Knowledge.

Purpose: To assess the feelings and knowledge of health care professionals (HCPs) about gasping in dying patients in neonatal intensive care units (NICUs) and pediatric intensive care units (PICUs). Methods: A 9-item questionnaire addressed to 488 HCPs (physicians, nurses, and nursing assistants) of 2 NICUs and 2 PICUs. Questions were about HCPs' feelings when confronted with gasping, their knowledge, and their opinions on what to tell family members. Results: Responses were obtained from 248 staff members. Of the respondents, 43% felt that gasping was painful to the patient and most felt that witnessing gasps was distressing for the parents, and 77% reported being distressed by witnessing gasps. Conclusions: Efforts are needed to educate HCPs about the physiology of gasping, to help them to cope with gasping, and to give better support to parents.

Visiting Policies and Parental Presence During PICU Admission: A Survey in French Units.

OBJECTIVES: To describe the policies about parent visiting and involvement in care during admission to French PICUs. DESIGN: A structured questionnaire was emailed to the chief of each of 35 French PICUs. Data about visiting policies, involvement in care, evolution of policies, and general characteristics were collected from April 2021 to May 2021. A descriptive analysis was conducted. SETTING: Thirty-five PICUs in France. PATIENTS: None. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty-nine of 35 (83%) PICUs replied. Twenty-four-hour access for parents was reported for all PICUs responding. Other allowed visitors were grandparents (21/29, 72%) and siblings (19/29, 66%) with professional support. Simultaneous visits were restricted to two visitors in 83% (24/29) of PICUs. Family presence was always permitted during medical rounds for 20 of 29 (69%) PICUs. Most of the units rarely or never allowed parental presence during the most invasive procedures, such as central venous catheter placement (18/29, 62%) and intubation (22/29, 76%). CONCLUSIONS: Unrestricted access to the PICU, for both parents, was available in all responding French units. There were, however, restrictions on the number of visitors and the presence of other family members at the bedside. Moreover, permission for parental presence during care procedures was heterogenous, and mainly restricted. National guidelines and educational programs are needed to support family wishes and promote acceptance by healthcare providers in French PICUs.

Factors linked to Staphylococcus aureus healthcare-associated infections among pediatric intensive care unit colonized patients.

BACKGROUND: Staphylococcus aureus (SA) is one of the main pathogens responsible for healthcare-associated infection (HCAI) in pediatrics. The aim of this study was to describe the prevalence of SA-HCAI among colonized patients and the factors associated with it in the pediatric intensive care unit (PICU). METHODS: We designed a 6-year retrospective cohort study of a PICU in a French university children's hospital including all children admitted to the PICU from January 1, 2011, to December 31, 2016, who had SA colonization on PICU admission. For each patient, the past medical history and the hospitalization data were collected. HCAIs related to SA were verified according to the criteria of the United States Centers for Disease Control and Prevention. RESULTS: Among all patients colonized with SA (n = 1381, 26%), 105 (8%) had methicillin-resistant SA carriage and 41 (3%) developed an HCAI caused by SA. The main HCAIs were ventilator-associated pneumonia (51%) and central line-associated bloodstream infections (27%). Patients developing HCAI caused by SA had a significantly longer length of hospital stay and a higher mortality rate than the rest of the population. Using a multivariate logistic regression model, the presence of mechanical ventilation, the implementation of a surgical procedure during the PICU stay, and the onset of at least one episode of anemia during the PICU stay were significantly associated with the occurrence of HCAI due to SA. CONCLUSION: HCAIs linked to SA carriage are rare but severe. Mechanical ventilation, surgery during the PICU stay, and anemia are factors associated with SA-HCAI.

Perceptible Signs of End of Life in Pediatric Intensive Care Patients.

Background: How children die in pediatric intensive care units (PICUs) has been poorly described, and support for parents during this traumatic experience could be improved. Better information on perceptible signs of the end of life (EOL) in children may help mitigate the trauma. Objective: To describe the most common perceptible EOL signs in PICU patients. Methods: A prospective study in a PICU. Health care providers observed 28 children expected to die and noted the perceptible signs. Results: The most common perceptible signs were desaturation and bradycardia. Twenty-seven patients had at least one change in physical appearance. Gasping was noted in 12 patients. Conclusion: Perceptible signs of the EOL were different from those reported in children dying in other units or in adults. Sharing information about signs of EOL with parents may prepare them and enable them to better support their child.

Management of severe inaugural diabetic ketoacidosis in paediatric intensive care: retrospective comparison of two protocols.

The best protocol for severe inaugural diabetic ketoacidosis (DKA) in children remains unclear. We compared two protocols by assessing effects during the first 24 h on osmolality, serum sodium, and glucose variations, which are associated with the risk of cerebral oedema, the most dreaded complication of DKA. We also recorded complications. We retrospectively included children aged 28 days to 18 years and admitted for severe DKA to either of two paediatric intensive care units (PICUs) in Paris (France). The two protocols differed regarding hydration volume, glucose intake, and sodium intake. From 17 June 2010 to 17 June 2015, 93 patients were included, 29 at one PICU, and 64 at the other. We compared severe glycaemic drops (> 5.5 mmol/L/h), mean glycaemia variations, serum sodium, serum osmolality, and the occurrence of cerebral oedema (CE) during the first 24 h after PICU admission. Severe glycaemic drops occurred in 70% of patients, with no between-group difference. Blood glucose, serum sodium, and serum osmolality variations were comparable. Seven (7.5%) patients were treated for suspected CE, (4 [10.3%)] and 3 [6.3%]) in each PICU; none had major residual impairments. CONCLUSION:  The two paediatric DKA-management protocols differing in terms of fluid-volume, glucose, and sodium intakes had comparable effects on clinical and laboratory-test changes within 24 h. Major drops in glycaemia and osmolality were common with both protocols. No patients had residual neurological impairments. WHAT IS KNOWN: • Cerebral oedema is the most severe complication of diabteic ketoacidosis in children.The risk of cerebral oedema is dependant on both patient related and treatment-related factors. • The optimal protocol for managing severe inaugural diabetic ketoacidosis in children remains unclear, and few studies have targeted this specific population. WHAT IS NEW: • Two management protocols that complied with ISPAD guidelines but differed regarding the amounts of fluids, glucose, and sodium administered produced similar outcomes in children with severe inaugural diabetic ketoacidosis. • Cerebral oedema was rare with both protocols and caused no lasting impairments.

Emergence of Kawasaki disease related to SARS-CoV-2 infection in an epicentre of the French COVID-19 epidemic: a time-series analysis.

BACKGROUND: Kawasaki disease is an acute febrile systemic childhood vasculitis, which is suspected to be triggered by respiratory viral infections. We aimed to examine whether the ongoing COVID-19 epidemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with an increase in the incidence of Kawasaki disease. METHODS: We did a quasi-experimental interrupted time series analysis over the past 15 years in a tertiary paediatric centre in the Paris region, a French epicentre of the COVID-19 outbreak. The main outcome was the number of Kawasaki disease cases over time, estimated by quasi-Poisson regression. In the same centre, we recorded the number of hospital admissions from the emergency department (2005-2020) and the results of nasopharyngeal multiplex PCR to identify respiratory pathogens (2017-2020). These data were compared with daily hospital admissions due to confirmed COVID-19 in the same region, recorded by Public Health France. FINDINGS: Between Dec 1, 2005, and May 20, 2020, we included 230 patients with Kawasaki disease. The median number of Kawasaki disease hospitalisations estimated by the quasi-Poisson model was 1·2 per month (IQR 1·1-1·3). In April, 2020, we identified a rapid increase of Kawasaki disease that was related to SARS-CoV-2 (six cases per month; 497% increase [95% CI 72-1082]; p=0·0011), starting 2 weeks after the peak of the COVID-19 epidemic. SARS-CoV-2 was the only virus circulating intensely during this period, and was found in eight (80%) of ten patients with Kawasaki disease since April 15 (SARS-CoV-2-positive PCR or serology). A second peak of hospital admissions due to Kawasaki disease was observed in December, 2009 (six cases per month; 365% increase ([31-719]; p=0.0053), concomitant with the influenza A H1N1 pandemic. INTERPRETATION: Our study further suggests that viral respiratory infections, including SAR-CoV-2, could be triggers for Kawasaki disease and indicates the potential timing of an increase in incidence of the disease in COVID-19 epidemics. Health-care providers should be prepared to manage an influx of patients with severe Kawasaki disease, particularly in countries where the peak of COVID-19 has recently been reached. FUNDING: French National Research Agency.

Long-term Metabolic and Socioeducational Outcomes of Transient Neonatal Diabetes: A Longitudinal and Cross-sectional Study.

OBJECTIVE: Transient neonatal diabetes mellitus (TNDM) occurs during the 1st year of life and remits during childhood. We investigated glucose metabolism and socioeducational outcomes in adults. RESEARCH DESIGN AND METHODS: We included 27 participants with a history of TNDM currently with (n = 24) or without (n = 3) relapse of diabetes and 16 non-TNDM relatives known to be carriers of causal genetic defects and currently with (n = 9) or without (n = 7) diabetes. Insulin sensitivity and secretion were assessed by hyperinsulinemic-euglycemic clamp and arginine-stimulation testing in a subset of 8 TNDM participants and 7 relatives carrying genetic abnormalities, with and without diabetes, compared with 17 unrelated control subjects without diabetes. RESULTS: In TNDM participants, age at relapse correlated positively with age at puberty (P = 0.019). The mean insulin secretion rate and acute insulin response to arginine were significantly lower in TNDM participants and relatives of participants with diabetes than in control subjects (median 4.7 [interquartile range 3.7-5.7] vs. 13.4 [11.8-16.1] pmol/kg/min, P < 0.0001; and 84.4 [33.0-178.8] vs. 399.6 [222.9-514.9] µIU/mL, P = 0.0011), but were not different between participants without diabetes (12.7 [10.4-14.3] pmol/kg/min and 396.3 [303.3-559.3] µIU/mL, respectively) and control subjects. Socioeducational attainment was lower in TNDM participants than in the general population, regardless of diabetes duration. CONCLUSIONS: Relapse of diabetes occurred earlier in TNDM participants compared with relatives and was associated with puberty. Both groups had decreased educational attainment, and those with diabetes had lower insulin secretion capacity; however, there was no difference in insulin resistance in adulthood. These forms of diabetes should be included in maturity-onset diabetes of the young testing panels, and relatives of TNDM patients should be screened for underlying defects, as they may be treated with drugs other than insulin.

Methicillin-sensible Staphylococcus aureus causing endocarditis, with cerebral and orthopaedic complications, in a 17-month-old child with no risk factor.

We report a case of Staphylococcus aureus endocarditis, with large vegetation, in a 17-month-old male infant, complicated with meningitis, ischaemic strokes and osteoarthritis leading to haemorrhagic stroke by aneurysm rupture. He did not present any risk factor for endocarditis. The final course was favourable through, after valve replacement. The strain was sensible to methicillin and belongs to complex clonal 398, with accessory gene regulator I. We did not found immunodeficiency.

Successful off-label sulfonylurea treatment of neonatal diabetes mellitus due to chromosome 6 abnormalities.

Chromosome 6 abnormalities such as paternal uniparental isodisomy, paternal 6q24 duplication, and maternal DMR (differentially methylated region) hypomethylation are a common cause of transient neonatal diabetes mellitus (TNDM). Oral sulfonylurea (SU) is used off-label to treat permanent neonatal diabetes mellitus owing to potassium channel mutation but has not been evaluated in TNDM. Our objective was to evaluate the efficacy and safety of SU therapy in chromosome 6-related TNDM. Description of 3 case reports and literature review was the subject of the study. SU therapy was successful in 2 patients (initiated during neonatal life in 1 patient and during relapse in the other) but failed in the other despite the use of high dosage. The literature review identified 11 cases of patients with chromosome 6-related TNDM treated with SU, including 4 treated before remission and 7 after the relapse. SU therapy was consistently effective, although 4 patients treated after the relapse required multiple oral medications. None of the patients needed associated insulin therapy. No side effects of SU or complications of diabetes were reported. SU seems effective and safe in chromosome 6-related TNDM treatment when used to treat the initial episode of diabetes or the relapse. It improves patients' and families' quality of life. SU is available only as oral tablets. A pediatric dosage form would facilitate the treatment of neonates and infants.