Pharmacological management of prolonged seizures in Dravet syndrome including intravenous phenytoin.

Dravet syndrome (DS) is an infantile onset developmental and epileptic encephalopathy. Sodium channel blockers are known to exacerbate seizures in this syndrome. Due to its high incidence, the management of prolonged seizures is crucial for DS patients. There is still ambiguity regarding the use of intravenous phenytoin for prolonged seizure in DS patients mainly due to the lack of data, raising concern about the safety of it use. We conducted a retrospective study (from January 2009 to January 2020) aiming to assess the management of prolonged seizures in DS with a focus on the use of intravenous phenytoin. Data were collected for patients admitted to our hospital for seizures lasting >5 min. Among 52 identified patients in our database, 23 experienced 59 prolonged seizures managed in our hospital. Only four seizures ceased without rescue medication. Notably, the use of intravenous phenytoin was not associated with discernible adverse effects and was effective in stopping status epilepticus in 71% of cases. This study suggests the safety and efficacy of intravenous phenytoin for prolonged seizure in DS. There is a need for broader investigations of emergency treatments for evidence-based recommendations for the emergency plan of each patient.

Compliance with antibiotic therapy guidelines in french paediatric intensive care units: a multicentre observational study.

BACKGROUND: Bacterial infections (BIs) are widespread in ICUs. The aims of this study were to assess compliance with antibiotic recommendations and factors associated with non-compliance. METHODS: We conducted an observational study in eight French Paediatric and Neonatal ICUs with an antimicrobial stewardship programme (ASP) organised once a week for the most part. All children receiving antibiotics for a suspected or proven BI were evaluated. Newborns < 72 h old, neonates < 37 weeks, age ≥ 18 years and children under surgical antimicrobial prophylaxis were excluded. RESULTS: 139 suspected (or proven) BI episodes in 134 children were prospectively included during six separate time-periods over one year. The final diagnosis was 26.6% with no BI, 40.3% presumed (i.e., not documented) BI and 35.3% documented BI. Non-compliance with antibiotic recommendations occurred in 51.1%. The main reasons for non-compliance were inappropriate choice of antimicrobials (27.3%), duration of one or more antimicrobials (26.3%) and length of antibiotic therapy (18.0%). In multivariate analyses, the main independent risk factors for non-compliance were prescribing ≥ 2 antibiotics (OR 4.06, 95%CI 1.69-9.74, p = 0.0017), duration of broad-spectrum antibiotic therapy ≥ 4 days (OR 2.59, 95%CI 1.16-5.78, p = 0.0199), neurologic compromise at ICU admission (OR 3.41, 95%CI 1.04-11.20, p = 0.0431), suspected catheter-related bacteraemia (ORs 3.70 and 5.42, 95%CIs 1.32 to 15.07, p < 0.02), a BI site classified as "other" (ORs 3.29 and 15.88, 95%CIs 1.16 to 104.76, p < 0.03), sepsis with ≥ 2 organ dysfunctions (OR 4.21, 95%CI 1.42-12.55, p = 0.0098), late-onset ventilator-associated pneumonia (OR 6.30, 95%CI 1.15-34.44, p = 0.0338) and ≥ 1 risk factor for extended-spectrum ß-lactamase-producing Enterobacteriaceae (OR 2.56, 95%CI 1.07-6.14, p = 0.0353). Main independent factors for compliance were using antibiotic therapy protocols (OR 0.42, 95%CI 0.19-0.92, p = 0.0313), respiratory failure at ICU admission (OR 0.36, 95%CI 0.14-0.90, p = 0.0281) and aspiration pneumonia (OR 0.37, 95%CI 0.14-0.99, p = 0.0486). CONCLUSIONS: Half of antibiotic prescriptions remain non-compliant with guidelines. Intensivists should reassess on a day-to-day basis the benefit of using several antimicrobials or any broad-spectrum antibiotics and stop antibiotics that are no longer indicated. Developing consensus about treating specific illnesses and using department protocols seem necessary to reduce non-compliance. A daily ASP could also improve compliance in these situations. TRIAL REGISTRATION: ClinicalTrials.gov: number NCT04642560. The date of first trial registration was 24/11/2020.

Perceived Quality of Life in Intensive Care Medicine Physicians: A French National Survey.

Purpose: There is a growing interest in the quality of work life (QWL) of healthcare professionals and staff well-being. We decided to measure the perceived QWL of ICU physicians and the factors that could influence their perception. Methods: We performed a survey coordinated and executed by the French Trade Union of Intensive Care Physicians (SMR). QWL was assessed using the French version of the Work-Related Quality of Life (WRQoL) scale, perceived stress using the French version of 10 item-Perceived Stress Scale (PSS-10) and group functioning using the French version of the Reflexivity Scale, the Social Support at Work Questionnaire (QSSP-P). Results: 308 French-speaking ICU physicians participated. 40% perceived low WRQoL, mainly due to low general well-being, low satisfaction with working conditions and low possibility of managing the articulation between their private and professional lives. Decreased QWL was associated with being a woman (p = .002), having children (p = .022) and enduring many monthly shifts (p = .022). Conclusions: This work highlights the fact that ICU physicians feel a significant imbalance between the demands of their profession and the resources at their disposal. Communication and exchanges within a team and quality of social support appear to be positive elements to maintain and/or develop within our structures.

Patient follow-up after discharge from the paediatric intensive care unit: A scoping review.

BACKGROUND: Most children admitted to a paediatric intensive care unit (PICU) now survive because of improvements in care. Many studies have identified the psychological, functional, cognitive and social impact of PICU admission on a child and their family. However, expert recommendations on follow-up are lacking. AIM: To identify the strategies of clinical follow-up after PICU discharge performed from 2001 to 2021. STUDY DESIGN: This scoping review was undertaken between January and April 2021 using three databases: PubMed, EMBASE and CINAHL. The search strategy consisted of a combination of keywords, including PICU, post-PICU discharge and follow-up in articles published between 2001 and 2021. The results are reported according to PRISMA-ScR guidelines. RESULTS: Six-hundred and fifty-two articles were identified and 68 were analysed. Median age was 4.5 years and the two main reasons for PICU admission were cardiorespiratory failure and sepsis. Median length of PICU stay was 8 days. Most follow-up was carried out by research units (88%), while 6% of studies reported follow-up by a multidisciplinary PICU team. The most common follow-up schedule included an assessment at PICU discharge, and then at 3, 6 and 12 months. Follow-up for >1 year was reported in 20% of studies. One third of studies focused on follow-up quality of life and neurological outcomes. Parental emotional impact was assessed in 7% of studies. CONCLUSION: Follow-up after PICU discharge was highly heterogeneous regarding timing, health care professionals involved and assessment methods. There is an urgent need for standardization and coordination of PICU follow-up because of the increasing number of patients impacted by a PICU stay. RELEVANCE TO CLINICAL PRACTICE: Although most patients admitted to a paediatric intensive care unit (PICU) now survive; they may develop paediatric post-intensive care syndrome (PICS-P). To our knowledge, there are currently no clinical guidelines regarding follow-up after PICU discharge. This review summarizes current approaches to follow-up after PICU discharge, including how it is carried out, who is involved and what the main aims of assessment are.

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.

Obstructive Apneas in a Mouse Model of Congenital Central Hypoventilation Syndrome.

Rationale: Congenital central hypoventilation syndrome (CCHS) is characterized by life-threatening sleep hypoventilation and is caused by PHOX2B gene mutations, most frequently the PHOX2B27Ala/+ mutation, with patients requiring lifelong ventilatory support. It is unclear whether obstructive apneas are part of the syndrome. Objectives: To determine if Phox2b27Ala/+ mice, which present the main symptoms of CCHS and die within hours after birth, also express obstructive apneas, and to investigate potential underlying mechanisms. Methods: Apneas were classified as central, obstructive, or mixed by using a novel system combining pneumotachography and laser detection of abdominal movement immediately after birth. Several respiratory nuclei involved in airway patency were examined by immunohistochemistry and electrophysiology in brainstem-spinal cord preparations. Measurements and Main Results: The median (interquartile range) of obstructive apnea frequency was 2.3 (1.5-3.3)/min in Phox2b27Ala/+ pups versus 0.6 (0.4-1.0)/min in wild types (P < 0.0001). Obstructive apnea duration was 2.7 seconds (2.3-3.9) in Phox2b27Ala/+ pups versus 1.7 seconds (1.1-1.9) in wild types (P < 0.0001). Central and mixed apneas presented similar significant differences. In Phox2b27Ala/+ preparations, the hypoglossal nucleus had fewer (P < 0.05) and smaller (P < 0.01) neurons, compared with wild-type preparations. Importantly, coordination of phrenic and hypoglossal motor activities was disrupted, as evidenced by the longer and variable delay of hypoglossal activity with respect to phrenic activity onset (P < 0.001). Conclusions: The Phox2b27Ala/+ mutation predisposed pups not only to hypoventilation and central apneas, but also to obstructive and mixed apneas, likely because of hypoglossal dysgenesis. These results thus demand attention toward obstructive events in infants with CCHS.

Pediatric Acute Alcohol Intoxication Admitted to the ICU: A Review of 102 Cases in France.

OBJECTIVES: To describe the profile and clinical outcomes of children (<18 yr) admitted to intensive care for acute alcohol intoxication, with special attention to complications and to the subgroup that required intubation. DESIGN: Retrospective observational study. SETTING: Seven pediatric and three adult ICUs in France. PATIENTS: Children 1-17 yr admitted to intensive care for acute alcohol intoxication between January 1, 2010, and December 30, 2017. INTERVENTIONS: The study was observational and patients received standard care. MEASUREMENTS AND MAIN RESULTS: We included 102 patients, with 71 males (69.6%) and 31 females (30.4%). Mean age was not different between males and females (14.0 ± 3.0 yr [range, 2-17 yr] and 14.2 ± 1.3 yr [range, 11-17 yr]; p = 0.67); six children were younger than 10 years. Mean blood alcohol concentration was not significantly different in males and females (2.42 ± 0.86 and 2.20 ± 0.54 g/L, respectively; p = 0.51). Of the 102 patients, 58 (57%) required intubation. Factors significantly associated with requiring intubation were lower Glasgow Coma Scale score (p = 0.002), lower body temperature (p = 0.045), and higher blood alcohol concentration (p = 0.012); vascular filling, and electrolyte disturbances were not associated with needing intubation. Mean intubation time was 9.7 ± 5.2 hours. Among the 59 patients with Glasgow Coma Scale score less than 8, 12 did not require intubation. The most common metabolic disturbance was a high lactate level (48%), followed by hypokalemia (27.4%); 59 (58.2%) patients had hyperglycemia and three had hypoglycemia. CONCLUSIONS: Male adolescents make up the majority of pediatric patients admitted to intensive care for acute alcohol intoxication. A need for intubation was associated with a worse Glasgow Coma Scale, lower body temperature, and higher blood alcohol concentration. Intubation was usually required for less than 12 hours. Other acute medical complications reported in adults with acute alcohol intoxication, such as electrolyte disturbances and aspiration pneumonia, were rare in our pediatric patients.

Mortality and Neurologic Sequelae in Influenza-Associated Encephalopathy: Retrospective Multicenter PICU Cohort in France.

OBJECTIVES: To describe and estimate the mortality rate of severe influenza-associated encephalopathy/encephalitis among children admitted to PICUs. DESIGN: Multicenter retrospective study. SETTING: Twelve French PICUs. PATIENTS: All children admitted for influenza-associated encephalopathy/encephalitis between 2010 and 2018 with no severe preexisting chronic neurologic disorders and no coinfection potentially responsible for the disease. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: We collected the clinical presentation; laboratory, electroencephalographic, and MRI findings; and treatments used in the PICU. The primary outcome was mortality. The secondary outcomes included sequelae at discharge and last follow-up. We included 41 patients with a median (interquartile range) age of 4.7 years (2.5-8.2 yr). The main reasons for admission were altered consciousness (59%) and status epilepticus (34%); 48% of patients had meningitis, and one third had acute necrotizing encephalopathy on MRI. Mechanical ventilation was required in 73% of patients and hemodynamic support in 24%. The use of specific treatments was variable; steroids were given to 49% of patients. Seven patients (17%) died in the PICU. Median (interquartile range) PICU stay length was 7 days (2-13 d), and total hospital length of stay was 23 days (7-33 d). On hospital discharge, 49% (n = 20) had neurologic sequelae, with 27% (n = 11) having severe disabilities defined by modified Rankin Score greater than or equal to 4. CONCLUSIONS: Children requiring PICU admission for influenza-associated encephalopathy/encephalitis have high mortality and morbidity rates. The management remains highly variable due to the lack of guidelines.

Biallelic PPA2 Mutations Cause Sudden Unexpected Cardiac Arrest in Infancy.

Sudden unexpected death in infancy occurs in apparently healthy infants and remains largely unexplained despite thorough investigation. The vast majority of cases are sporadic. Here we report seven individuals from three families affected by sudden and unexpected cardiac arrest between 4 and 20 months of age. Whole-exome sequencing revealed compound heterozygous missense mutations in PPA2 in affected infants of each family. PPA2 encodes the mitochondrial pyrophosphatase, which hydrolyzes inorganic pyrophosphate into two phosphates. This is an essential activity for many biosynthetic reactions and for energy metabolism of the cell. We show that deletion of the orthologous gene in yeast (ppa2Δ) compromises cell viability due to the loss of mitochondria. Expression of wild-type human PPA2, but not PPA2 containing the mutations identified in affected individuals, preserves mitochondrial function in ppa2Δ yeast. Using a regulatable (doxycycline-repressible) gene expression system, we found that the pathogenic PPA2 mutations rapidly inactivate the mitochondrial energy transducing system and prevent the maintenance of a sufficient electrical potential across the inner membrane, which explains the subsequent disappearance of mitochondria from the mutant yeast cells. Altogether these data demonstrate that PPA2 is an essential gene in yeast and that biallelic mutations in PPA2 cause a mitochondrial disease leading to sudden cardiac arrest in infants.

Breathing under Anesthesia: A Key Role for the Retrotrapezoid Nucleus Revealed by Conditional Phox2b Mutant Mice.

BACKGROUND: Optimal management of anesthesia-induced respiratory depression requires identification of the neural pathways that are most effective in maintaining breathing during anesthesia. Lesion studies point to the brainstem retrotrapezoid nucleus. We therefore examined the respiratory effects of common anesthetic/analgesic agents in mice with selective genetic loss of retrotrapezoid nucleus neurons (Phox2b mice, hereafter designated "mutants"). METHODS: All mice received intraperitoneal ketamine doses ranging from 100 mg/kg at postnatal day (P) 8 to 250 mg/kg at P60 to P62. Anesthesia effects in P8 and P14 to P16 mice were then analyzed by administering propofol (100 and 150 mg/kg at P8 and P14 to P16, respectively) and fentanyl at an anesthetic dose (1 mg/kg at P8 and P14 to P16). RESULTS: Most mutant mice died of respiratory arrest within 13 min of ketamine injection at P8 (12 of 13, 92% vs. 0 of 8, 0% wild type; Fisher exact test, P < 0.001) and P14 to P16 (32 of 42, 76% vs. 0 of 59, 0% wild type; P < 0.001). Cardiac activity continued after terminal apnea, and mortality was prevented by mechanical ventilation, supporting respiratory arrest as the cause of death in the mutants. Ketamine-induced mortality in mutants compared to wild types was confirmed at P29 to P31 (24 of 36, 67% vs. 9 of 45, 20%; P < 0.001) and P60 to P62 (8 of 19, 42% vs. 0 of 12, 0%; P = 0.011). Anesthesia-induced mortality in mutants compared to wild types was also observed with propofol at P8 (7 of 7, 100% vs. 0 of 17,7/7, 100% vs. 0/17, 0%; P < 0.001) and P14 to P16 (8 of 10, 80% vs. 0 of 10, 0%; P < 0.001) and with fentanyl at P8 (15 of 16, 94% vs. 0 of 13, 0%; P < 0.001) and P14 to P16 (5 of 7, 71% vs. 0 of 11, 0%; P = 0.002). CONCLUSIONS: Ketamine, propofol, and fentanyl caused death by respiratory arrest in most mice with selective loss of retrotrapezoid nucleus neurons, in doses that were safe in their wild type littermates. The retrotrapezoid nucleus is critical to sustain breathing during deep anesthesia and may prove to be a pharmacologic target for this purpose.

Hyponatremia in children under 100 days old: incidence and etiologies.

Hyponatremia is one of the most common electrolyte disorders in hospitalized children. The underlying mechanisms are poorly understood and potentially multifactorial, making management difficult, particularly in neonates. This retrospective study aimed to determine the incidence and etiologies of hyponatremia in hospitalized children under the age of 100 days, in our pediatric tertiary care hospital over a 1-year period. The etiology of hyponatremia was determined by reviewing the data noted in each patient's medical reports. Neonatal hyponatremia had a prevalence of 4.3% (86/2012 patients) and was mostly hospital-acquired (74/86 patients). Fifty-nine patients (68.9%) were preterm neonates. The etiology was iatrogenic in 26 cases (30.2%). In other cases, hyponatremia was due to transient (23 patients, 26.7%) or genetic abnormalities of the renal mineralocorticoid pathway (3 patients, 3.4%), SIADH (12 patients, 14%), digestive disease (3 patients, 3.5%), acute renal failure (3 patients, 3.5%), or heart failure (1 patient, 1.2%).Conclusion: Our findings confirm that hyponatremia is a frequent electrolyte disorder in neonates. Various mechanisms underlie this condition, most of which could be prevented by optimized management. The prevalence of genetic hypoaldosteronism and pseudohypoaldosteronism was higher than expected. We provide a simple diagram to help physicians identify the mechanisms underlying neonatal hyponatremia. What is Known: • In neonates, hyponatremia may be multifactorial, making it difficult to treat. • Newborns display partial resistance to aldosterone, and preterms have a defect in aldosterone secretion. What is New: • Four percent of hospitalized neonates had hyponatremia, 86% hospital-acquired. Hyponatremia was due to a transient or constitutional defect of the mineralocorticoid pathway in 26/86 patients (30%) which is higher than expected. • We propose a tree diagram for improving the management of hyponatremia in neonates.

Intraoperative hemodynamic factors predicting early postoperative renal function in pediatric kidney transplantation.

BACKGROUND: The anesthetic management of kidney transplantation in children remains somewhat empirical. The goal of the present study was to investigate intraoperative hemodynamic factors affecting posttransplantation kidney function. METHODS: We performed a retrospective analysis of data from patients undergoing kidney transplantation in our pediatric teaching hospital from 2000 to 2014. Data collected included: donor and recipient demographic data, recipient comorbidities, fluids administered intraoperatively, and intraoperative blood pressure and central venous pressure. The main outcome of the study was the creatinine clearance at day 1 corrected to a body surface area of 1.73 m². Analysis was performed using Classification Tree Analysis with 10-fold cross-validation. RESULTS: One hundred and two patients were included. The following predictors of increased postoperative creatinine clearance at day 1 were identified: decreasing recipient weight, mean blood pressure-to-weight ratio 10 minutes after reperfusion, reduced cold ischemia duration, and increased intraoperative albumin infusion. Increased creatinine clearance was observed when mean blood pressure-to-weight ratio 10 minutes after reperfusion was ≥4.3 in patients weighing 13-21 kg and ≥2.5 in those ≥22 kg. Overall, the model explained 64% (and at cross-validation 60%) of creatinine clearance variability at day 1. CONCLUSION: Intraoperative hemodynamics during kidney transplantation should be optimized in order to increase mean blood pressure according to values indicated by our analyses. Cold ischemia duration should be shortened as far as possible.

Assessing fluid balance in critically ill pediatric patients.

UNLABELLED: Monitoring fluid balance (FB) in a pediatric intensive care unit (PICU) is crucial to assess fluid overload. Pediatric intensivists (PI) frequently use the fluid intake minus output (FIMO) or FIMO with adjustments for insensible fluid loss (AFIMO). However, the accuracy of FIMO/AFIMO has never been tested in critically ill children. We designed a prospective, monocentric cohort study in a PICU of a university hospital. Body weight (BW) was measured in all children consecutively admitted to PICU and 24 h later. Every 12 h, the nurses calculated FIMO/AFIMO. Time burden and convenience of each procedure (median; [interquartile range]) were recorded and compared using a Wilcoxon test. Data were analysed using linear regression (r (2) coefficient) and the Bland-Altman plot (mean difference ± standard deviation; absolute mean difference), with a 300-ml variation of FB considered clinically relevant. Sixty consecutive patients, 304-day [39-1,565] old with admission weight of 9.2 kg [4.4-17.8] were included. Although correlations between FIMO/AFIMO and BW changes (BWC) were strong (r (2) FIMO = 0.63, p < 0.0001 and r (2) AFIMO = 0.72, p < 0.0001, respectively), agreement between FIMO/AFIMO and BWC were over 300 mL (-0.305 ± 0.451, 0.382 L and -0.007 ± 0.447, 0.302 L, respectively). No significant differences were noted between FIMO/AFIMO and BWC measurements for time burden (5 min [5-10] vs. 5 min [5-10], p = 0.84) or convenience (1 min [1-2] vs. 1 min [0-1.3], p = 0.13). CONCLUSION: Because agreement between FIMO/AFIMO and BWC is poor during the first 24 h after admission into PICU, PIs may reserve FIMO/AFIMO to monitor FB in patients with absolute contraindications of BW measurements.

Invasive pneumococcal disease in children can reveal a primary immunodeficiency.

BACKGROUND: About 10% of pediatric patients with invasive pneumococcal disease (IPD) die from the disease. Some primary immunodeficiencies (PIDs) are known to confer predisposition to IPD. However, a systematic search for these PIDs has never been carried out in children presenting with IPD. METHODS: We prospectively identified pediatric cases of IPD requiring hospitalization between 2005 and 2011 in 28 pediatric wards throughout France. IPD was defined as a positive pneumococcal culture, polymerase chain reaction result, and/or soluble antigen detection at a normally sterile site. The immunological assessment included abdominal ultrasound, whole-blood counts and smears, determinations of plasma immunoglobulin and complement levels, and the evaluation of proinflammatory cytokines. RESULTS: We included 163 children with IPD (male-to-female ratio, 1.3; median age, 13 months). Seventeen children had recurrent IPD. Meningitis was the most frequent type of infection (87%); other infections included pleuropneumonitis, isolated bloodstream infection, osteomyelitis, endocarditis, and mastoiditis. One patient with recurrent meningitis had a congenital cerebrospinal fluid fistula. The results of immunological explorations were abnormal in 26 children (16%), and a PID was identified in 17 patients (10%), including 1 case of MyD88 deficiency, 3 of complement fraction C2 or C3 deficiencies, 1 of isolated congenital asplenia, and 2 of Bruton disease (X-linked agammaglobulinemia). The proportion of PIDs was much higher in children aged >2 years than in younger children (26% vs 3%; P < .001). CONCLUSIONS: Children with IPD should undergo immunological investigations, particularly those aged >2 years, as PIDs may be discovered in up to 26% of cases.

Infant deaths in slings.

Although the incidence of sudden unexpected death in infancy (SUDI) decreased markedly after campaigns to promote supine positioning during sleeping, it has remained unchanged over the last decade. Epidemiological data suggest a role for new causes such as suffocation, asphyxia, and entrapment. Health authorities in several countries have issued warnings about slings used to carry infants. However, few reports of infant deaths in slings have been published in medical journals. Our paediatric intensive care unit has admitted two infants who experienced cardiorespiratory arrest while carried in a sling. Diagnostic investigations including a post-mortem examination established asphyxia as the mechanism of death. In conclusion, baby slings may carry a risk of SUDI, either by compression of the baby into a forward-flexed position or by direct suffocation. European recommendations for the cautious use of baby slings should be disseminated to families and professionals involved in caring for infants, as done recently in Australia, Canada, and the USA.

[Pediatric necrotizing soft-tissue infections]. / Infections cutanées nécrosantes de l'enfant.

PEDIATRIC NECROTIZING SOFT-TISSUE INFECTIONS. Necrotizing soft-tissue infections (NSTI) include necrotizing forms of fasciitis, myositis, and cellulitis. In children, these are extremely rare conditions with an estimated annual incidence of less than 0.1/100,000 patients aged 0-18 years in France. Nevertheless, the evolution can be very serious (6% mortality, higher than the mortality observed in paediatric intensive care units [PICU]), whereas the initial local symptoms are poor and can be falsely reassuring. The monitoring of a skin infection must be close in order not to ignore the evolution towards a NSTI. In this case, prompt transfer to a PICU with all the necessary technical facilities and used to the management of these rare conditions must be done. Early initiation of antibiotic treatment and aggressive haemodynamic resuscitation according to the latest Surviving Sepsis Campaign guidelines should be a priority. The paediatric surgeon should be called upon as soon as clinical suspicion arises and participate in the frequent clinical reassessment to determine the optimal time to perform the surgical treatment.

INFECTIONS CUTANÉES NÉCROSANTES DE L'ENFANT. Les infections cutanées nécrosantes comprennent les dermo- hypodermites bactériennes nécrosantes (DHBN) et les fasciites nécrosantes (FN). Chez l'enfant, ce sont des pathologies extrêmement rares, avec une incidence annuelle en France estimée inférieure à 0,1/100 000 patients âgés de 0 à 18 ans. Néanmoins, leur évolution peut être gravissime (mortalité de 6 %, supérieure à la mortalité observée habituellement dans les unités de réanimation pédiatrique [URP]) alors que la symptomatologie locale initiale est pauvre et peut faussement rassurer. La surveillance d'une infection cutanée doit être rapprochée afin de ne pas méconnaître l'évolution vers une DHBN-FN. Dans ce cas, une orientation vers une URP disposant de l'ensemble du plateau technique nécessaire, et surtout habituée à gérer ces situations cliniques, est justifié. L'initiation précoce du traitement antibiotique et la prise en charge hémodynamique agressive en suivant les dernières recommandations de la Surviving Sepsis Campaign doivent être une priorité. Le chirurgien pédiatrique doit être appelé dès la suspicion clinique et participer à la réévaluation pluriquotidienne afin de déterminer le moment optimal pour réaliser le traitement chirurgical.

The pediatric intensive care unit in France: What happens afterwards?

Although pediatric post-intensive care syndrome is frequent and impacts the child's quality of life in various aspects, there are currently no guidelines regarding post-pediatric intensive care unit (PICU) follow-up. The aim of this study was to describe post-PICU follow-up in France. Among the 37 French PICUs, only 67 % had a consultation service, mostly performed by pediatric intensivists (95 %). Post-intensive care evaluation was the main objective for 46 % of these centers, whereas others focused on specific patient populations. Post-intensive care follow-up is highly heterogeneous and developing such consultation services appears to be a main challenge for PICU teams.

Excessive pickle consumption: beware of adrenal crisis.

Adrenal insufficiency (AI) is one of the most life-threatening disorders resulting from adrenal cortex dysfunction. Symptoms and signs of AI are often nonspecific, and the diagnosis can be missed and lead to the development of AI with severe hypotension and hypovolemic shock. We report the case of a 13-year-old child admitted for cardiac arrest following severe hypovolemic shock. The patient initially presented with isolated mild abdominal pain and vomiting together with unexplained hyponatremia. He was discharged after an initial short hospitalization with rehydration but with persistent hyponatremia. After discharge, he had persistent refractory vomiting, finally leading to severe dehydration and extreme asthenia. He was admitted to pediatric intensive care after prolonged hypovolemic cardiac arrest with severe anoxic encephalopathy leading to brain death. After re-interviewing, the child's parents reported that he had experienced polydipsia, a pronounced taste for salt with excessive consumption of pickles lasting for months, and a darkened skin since their last vacation 6 months earlier. A diagnosis of autoimmune Addison's disease was made. Primary AI is a rare life-threatening disease that can lead to hypovolemic shock. The clinical symptoms and laboratory findings are nonspecific, and the diagnosis should be suspected in the presence of unexplained collapse, hypotension, vomiting, or diarrhea, especially in the case of hyponatremia.