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.

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.

Safety of early norepinephrine infusion through peripheral vascular access during transport of critically ill children.

STUDY OBJECTIVE: In prehospital and emergency settings, vasoactive medications may need to be started through a peripheral intravenous catheter. Fear of extravasation and skin injury, with norepinephrine specifically, may prevent or delay peripheral vasopressor initiation, though studies from adults suggest the actual risk is low. We sought to study the risk of extravasation and skin injury with peripheral administration of norepinephrine in children in the prehospital setting. METHODS: We performed a retrospective study of pediatric patients (≤18 years) who received a vasopressor during prehospital transport. We collected data from retrieval and hospital records from 2 pediatric medical retrieval teams in the Paris/Ile-de-France region. Patients were eligible if they had documentation of distributive or obstructive shock and administration of norepinephrine through a peripheral catheter (intravenous or intraosseous) during retrieval. The primary outcomes were the occurrence of extravasation and evidence of skin injury. We also examined approach to norepinephrine administration (concentration, duration, proximal vs distal site) and hospital outcomes. RESULTS: Over a 3-year-period, 37 pediatric patients received norepinephrine through a peripheral catheter (33 intravenous, 4 intraosseous). Median patient age was 1.8 years. Thirty-two patients (86.5%) had septic shock. The median total duration of norepinephrine infusion was almost 4 hours. One patient (2.7%, 95% confidence interval 0.5%, 13.8%) had suspected extravasation from a 24-gauge intravenous catheter in the hand, with local skin hypoperfusion. Skin changes were noted after 135 minutes of norepinephrine infusion. Perfusion normalized after catheter removal, and there were no other sequelae. CONCLUSIONS: In a 3-year sample of pediatric patients from a large metropolitan area, we found only 1 patient with evidence of any harm with peripheral administration of norepinephrine. This finding is consistent with the adult literature but requires multicenter and multiyear investigation before a firm recommendation for this practice can be made.

Buccal Midazolam Compared With Rectal Diazepam Reduces Seizure Duration in Children in the Outpatient Setting.

BACKGROUND: Seizures are very common in children. They frequently happen in outpatient settings, in the presence of caregivers who are not always trained in their management. First-line rescue therapy is based on benzodiazepine, historically diazepam. Recent studies have investigated the use of other benzodiazepines in the treatment of acute seizures. OBJECTIVES: The aims of this study were to evaluate the management of pediatric seizures carried out by parents or caregivers in an outpatient setting and to evaluate the differences in terms of immediate management and subsequent outcome when comparing the use of rectal diazepam versus buccal midazolam. METHODS: In this retrospective study, medical records of children consulting for seizures at the Robert Debré Pediatric Emergency Department of Paris, France, over 18 months were analyzed to evaluate seizure characteristics, management by caregivers, received treatments, and the admission rate. RESULTS: Five hundred ninety-four patients resulted eligible for the study. The interview was completed for 135 children who presented a further episode of seizure after inclusion. In the subgroup of children receiving buccal midazolam, compared with the subgroup receiving intrarectal diazepam, seizure duration was significantly shorter (10.3 vs 48.4 minutes, P = 0.0004), and the risk of a status epilepticus decreased (1 vs 11, P = 0.0008). The admission rate was not different between the 2 subgroups. CONCLUSIONS: Based on our results, buccal midazolam seems to have some advantages compared with rectal diazepam in terms of feasibility in an outpatient setting and in terms of reduced seizure duration.

[Paediatric mobile emergency and intensive care services, objectives and missions]. / Les services mobiles d'urgence et de réanimation pédiatriques, objectifs et missions.

The paediatric mobile emergency and intensive care service care teams have expertise in taking care of children in life-threatening circumstances. At the Robert-Debré Hospital in Paris, the paediatric Smur is multi-skilled, specialising particularly in transporting neonates and infants with severe cardiac or respiratory difficulties. The pathologies handled are very varied and include both neonatal pathologies and trauma pathologies in older children.

Recovery position significantly associated with a reduced admission rate of children with loss of consciousness.

BACKGROUND: Loss of consciousness (LOC) is often seen in children. The response of caregivers to a child with LOC has been poorly investigated. Potential caregivers (parents, teachers) seem to have a poor knowledge of the recovery position (RP)-that is, the position into which an unconscious child should be placed in order to protect the airway. OBJECTIVES: To report the management and diagnoses of LOC in childhood, and to evaluate variables associated with an increased hospital admission rate. METHODS: We conducted a prospective cohort study of consecutive children aged between 0 and 18 years diagnosed with LOC at 11 paediatric emergency departments (PEDs) of 6 European countries. The enrolment period was 3 months. Data were obtained from parental interviews, PED reports and clinical examination. RESULTS: 553 children were enrolled. The most frequent final diagnoses causing LOC were seizures (n=278, 50.3%), and vasovagal syncope (n=124, 22.4%). Caregivers put the child in the RP in 145 cases (26.2%). The RP was independently associated with a significant decrease in the admission rate (aOR=0.28; 95% CI 0.17 to 0.48; p<0.0001). CONCLUSIONS: Our study demonstrates for the first time that the RP may reduce the admission rate of infants with LOC. Caregivers often perform inadequate manoeuvres when a child becomes unconscious. Campaigns aiming at increasing knowledge of the RP should be promoted.

Pediatric gastroenteritis in the emergency department: practice evaluation in Belgium, France, The Netherlands and Switzerland.

BACKGROUND: Based on European recommendations of ESPGHAN/ESPID from 2008, first line therapy for dehydration caused by acute gastroenteritis (AGE) is oral rehydration solution (ORS). In case of oral route failure, nasogastric tube enteral rehydration is as efficient as intra-venous rehydration and seems to lead to fewer adverse events. The primary objective was to describe rehydration strategies used in cases of AGE in pediatric emergency departments (PEDs) in Belgium, France, The Netherlands, and Switzerland. METHODS: An electronic survey describing a scenario in which a toddler had moderate dehydration caused by AGE was sent to physicians working in pediatric emergency departments. Analytical data were analyzed with descriptive statistics and Kruskal -Wallis Rank test. RESULTS: We analyzed 68 responses, distributed as follows: Belgium N = 10, France N = 37, The Netherlands N = 7, and Switzerland N = 14. Oral rehydration with ORS was the first line of treatment for 90% of the respondents. In case of first line treatment failure, intravenous rehydration was preferred by 95% of respondents from France, whereas nasogastric route was more likely to be used by those from Belgium (80%), The Netherlands (100%) and Switzerland (86%). Serum electrolyte measurements were more frequently prescribed in France (92%) and Belgium (80%) than in The Netherlands (43%) and Switzerland (29%). Racecadotril was more frequently used in France, and ondansetron was more frequently used in Switzerland. No respondent suggested routine use of antibiotics. CONCLUSION: We found variations in practices in terms of invasiveness and testing. Our study supports the need for further evaluation and implementation strategies of ESPGHAN/ESPID guidelines. We plan to extend the study throughout Europe with support of the Young ESPID Group.

Headache with focal neurologic signs in children at the emergency department.

OBJECTIVE: To identify predictors of secondary headache in children consulting at the pediatric emergency department (ED) for headache with a focal neurologic deficit. STUDY DESIGN: In this prospective cohort study, we enrolled children aged 6-18 years presenting to the ED of a tertiary care hospital with moderate to severe headache and focal neurologic deficit. Enrollment took place between March 2009 and February 2012. Children with a history of trauma, fever, or neurosurgical intervention were excluded from the study. The final diagnosis was made after 1 year of follow-up. Our primary aim was to identify any differences in the frequency of clinical signs between children with a final diagnosis of primary headache and those with a final diagnosis of secondary headache. RESULTS: Of the 101 patients included in the study, 66% received a final diagnosis of primary headache (94% migraine with aura), and 34% received a final diagnosis of secondary headache (76.5% focal epilepsy). On multivariate analysis, children with bilateral localization of pain had a higher likelihood (aOR, 8.6; 95% CI, 3.2-23.2; P<.001) of having secondary headache. CONCLUSION: Among children presenting to the ED with focal neurologic deficits, a bilateral headache location was associated with higher odds of having a secondary cause of headache. Additional longitudinal studies are needed to investigate whether our data can aid management in the ED setting.

Lung function, diagnosis, and treatment of sleep-disordered breathing in children with achondroplasia.

Children with achondroplasia are at risk of sleep-disordered breathing. The aim of the study was to evaluate lung function and sleep-disordered breathing in children with achondroplasia. An interview, clinical examination, lung function tests with blood gases, and a polygraphic sleep study were obtained as part of routine annual evaluation in consecutive children with achondroplasia. We included 30 children (median age 3.0 years, range: 0.4-17.1) over a period of 21 months. Habitual snoring and witnessed apneas were observed in 77% and 33% of the patients, respectively. Prior to the sleep study, 10/29 (34%) patients had undergone upper airway surgery and 5/29 (17%) craniocervical decompression operation. Arterial blood gases were abnormal in two (7%) patients. Sleep findings were abnormal in 28/30 (93%) patients. Eleven (37%) patients had an apnea index≥1 event/hr and 26 (87%) had an apnea-hypopnea index≥5 events/hr. The ≥3% desaturation index was >5/hr in 22 (73%) patients. Sixteen (53%) patients had a minimal pulse oximetry<90% but only two (7%) patients had a maximal transcutaneous carbon dioxide pressure>50 mmHg during sleep. As a consequence, the following therapeutic interventions were performed: upper airway surgery in four patients and noninvasive positive pressure ventilation (NPPV) in five other patients, resulting in an improvement in sleep studies in all nine patients. Systematic sleep studies are recommended in children with achondroplasia because of the high prevalence of sleep-disordered breathing. Upper airway surgery and NPPV are effective treatments of sleep-disordered breathing.