Pediatric long-term noninvasive respiratory support in children with central nervous system disorders.

RATIONALE: The use of long-term noninvasive respiratory support is increasing in children along with an extension of indications, in particular in children with central nervous system (CNS) disorders. OBJECTIVE: The aim of this study was to describe the characteristics of children with CNS disorders treated with long-term noninvasive respiratory support in France. METHODS: Data were collected from 27 French pediatric university centers through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ≥3 months on 1st June 2019. MAIN RESULTS: The data of 182 patients (55% boys, median age: 10.2 [5.4;14.8] years old [range: 0.3-25]) were collected: 35 (19%) patients had nontumoral spinal cord injury, 22 (12%) CNS tumors, 63 (35%) multiple disabilities, 26 (14%) central alveolar hypoventilation and 36 (20%) other CNS disorders. Seventy five percent of the patients were treated with noninvasive ventilation (NIV) and 25% with continuous positive airway pressure (CPAP). The main investigations performed before CPAP/NIV initiation were nocturnal gas exchange recordings, alone or coupled with poly(somno)graphy (in 29% and 34% of the patients, respectively). CPAP/NIV was started in an acute setting in 10% of the patients. Median adherence was 8 [6;10] hours/night, with 12% of patients using treatment <4 h/day. Nasal mask was the most common interface (70%). Airway clearance techniques were used by 31% of patients. CONCLUSION: CPAP/NIV may be a therapeutic option in children with CNS disorders. Future studies should assess treatment efficacy and patient reported outcome measures.

Impact of time to diagnosis on the occurrence of cardiogenic shock in MIS-C post-COVID-19 infection.

BACKGROUND: In multisystem inflammatory syndrome in children (MIS-C), diagnostic delay could be associated with severity. This study aims to measure the time to diagnosis in MIS-C, assess its impact on the occurrence of cardiogenic shock, and specify its determinants. METHODS: A single-center prospective cohort observational study was conducted between May 2020 and July 2022 at a tertiary care hospital. Children meeting the World Health Organization MIS-C criteria were included. A long time to diagnosis was defined as six days or more. Data on time to diagnosis were collected by two independent physicians. The primary outcome was the occurrence of cardiogenic shock. Logistic regression and receiver operating characteristic curve analysis were used for outcomes, and a Cox proportional hazards model was used for determinants. RESULTS: Totally 60 children were assessed for inclusion, and 31 were finally analyzed [52% males, median age 8.8 (5.7-10.7) years]. The median time to diagnosis was 5.3 (4.2-6.2) days. In univariable analysis, age above the median, time to diagnosis, high C-reactive protein, and high N-terminal pro-B-type natriuretic peptide (NT-proBNP) were associated with cardiogenic shock [odds ratio (OR) 6.13 (1.02-36.9), 2.79 (1.15-6.74), 2.08 (1.05-4.12), and 1.70 (1.04-2.78), respectively]. In multivariable analysis, time to diagnosis ≥ 6 days was associated with cardiogenic shock [adjusted OR (aOR) 21.2 (1.98-227)]. Time to diagnosis ≥ 6 days had a sensitivity of 89% and a specificity of 77% in predicting cardiogenic shock; the addition of age > 8 years and NT-proBNP at diagnosis ≥ 11,254 ng/L increased the specificity to 91%. Independent determinants of short time to diagnosis were age < 8.8 years [aHR 0.34 (0.13-0.88)], short distance to tertiary care hospital [aHR 0.27 (0.08-0.92)], and the late period of the COVID-19 pandemic [aHR 2.48 (1.05-5.85)]. CONCLUSIONS: Time to diagnosis ≥ 6 days was independently associated with cardiogenic shock in MIS-C. Early diagnosis and treatment are crucial to avoid the use of inotropes and limit morbidity, especially in older children.

Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France.

The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.5 years) were treated with long term noninvasive ventilation/continuous positive airway pressure. Thirty three percent of patients had spinal muscular atrophy, 30% congenital myopathy/dystrophy, 20% Duchenne muscular dystrophy, 7% Steinert myotonic dystrophy, and 9% other neuromuscular diseases. Ninety-four percent of patients were treated with long term noninvasive ventilation and 6% with continuous positive airway pressure. Treatment was initiated electively for 85% of patients, mainly on an abnormal overnight gas exchange recording (38% of patients). Noninvasive ventilation/continuous positive airway pressure was initiated during a respiratory exacerbation in 15% of patients. Mean duration of noninvasive ventilation/continuous positive airway pressure was 3.3 ± 3.1 years. Mean objective long term noninvasive ventilation/continuous positive airway pressure use was 8.0 ± 3.1 h/24. Spinal muscular atrophy, congenital myopathy/dystrophy, and Duchenne muscular dystrophy represented 83% of children with neuromuscular diseases treated with long term noninvasive ventilation in France. Screening for nocturnal hypoventilation was satisfactory as noninvasive ventilation /continuous positive airway pressure was predominantly initiated electively.

ROHHAD syndrome without rapid-onset obesity: A diagnosis challenge.

Background: ROHHAD syndrome (Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation) is rare. Rapid-onset morbid obesity is usually the first recognizable sign of this syndrome, however a subset of patients develop ROHHAD syndrome without obesity. The prevalence of this entity is currently unknown. Alteration of respiratory control as well as dysautonomic disorders often have a fatal outcome, thus early recognition of this syndrome is essential. Material and methods: A retrospective, observational, multicenter study including all cases of ROHHAD without rapid-onset obesity diagnosed in France from 2000 to 2020. Results: Four patients were identified. Median age at diagnosis was 8 years 10 months. Median body mass index was 17.4 kg/m2. Signs of autonomic dysfunction presented first, followed by hypothalamic disorders. All four patients had sleep apnea syndrome. Hypoventilation led to the diagnosis. Three of the four children received ventilatory support, all four received hormone replacement therapy, and two received psychotropic treatment. One child in our cohort died at 2 years 10 months old. For the three surviving patients, median duration of follow-up was 7.4 years. Conclusion: ROHHAD syndrome without rapid-onset obesity is a particular entity, appearing later than ROHHAD with obesity. This entity should be considered in the presence of dysautonomia disorders without brain damage. Likewise, the occurrence of a hypothalamic syndrome with no identified etiology requires a sleep study to search for apnea and hypoventilation. The identification of ROHHAD syndrome without rapid-onset obesity is a clinical challenge, with major implications for patient prognosis.

Answer to Lynøe: Interesting data about confessions and abusive head trauma, but suboptimal analysis.

The authors of the cited paper respond to the critics formulated by a Swedish leading expert regarding methodology shortcomings of our study "Confessed versus denied inflicted head injuries in infants: similarities and differences." They admit some methodological limitations but maintain their conclusions that the diagnosis was correct in the confession and denial groups and that the denial was more difficult in the more severe cases.

Confessed versus denied inflicted head injuries in infants: similarities and differences.

BACKGROUND AND PURPOSE: Abusive head injuries (AHI), and in particular shaken baby syndrome (SBS), are common causes of mortality and morbidity in infants. Although SBS is a well-established entity, based on clinical experience and experimental data, and confirmed by the perpetrators' confessions, a growing number of publications challenge the diagnostic criteria, and even the validity of the perpetrators' confession. We decided to study AHI in infants and compare cases with and without confession. MATERIAL AND METHODS: We collected prospectively all cases of infantile traumatic head injuries hospitalized in our institution between 2001 and 2021. From this database, we selected victims of AHI, comparing cases for which the perpetrator confessed during police inquiry ("confession" group) versus cases without confession ("denial" group). RESULTS: We studied 350 cases of AHI in infants; 137 of these (39.1%) were confessed. We found no statistically significant difference between the two groups regarding the child's previous history, as well as the personality and previous history of the caretakers. However, the "confession" group showed significantly more severe clinical presentation, cerebral lesions, retinal hemorrhages, and a more pejorative outcome. CONCLUSIONS: We conclude that the diagnosis of AHI was confirmed by the confession in a large number of cases, indicating that the diagnostic criteria of AHI are robust. We also found that denial, although possibly sincere, was likely ill-founded, and that the perpetrators' decision to confess or deny was markedly influenced by the severity of the inflicted lesions.

Paediatric long term continuous positive airway pressure and noninvasive ventilation in France: A cross-sectional study.

OBJECTIVE: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France. DESIGN: Cross-sectional national survey. SETTING: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France. PATIENTS: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019. INTERVENTION: An anonymous questionnaire was filled in for every patient. RESULTS: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO2), while 23% had only a pulse oximetry (SpO2) with PtcCO2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO2 and PtcCO2 in 25% of patients, and AHI with PtcCO2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey. CONCLUSION: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular diseases.

Assessment of Procedural Distress in Sedated/Intubated Children Under 3 Years Old Using the Newborn Infant Parasympathetic Evaluation: A Diagnostic Accuracy Pilot Study.

OBJECTIVES: Newborn infant parasympathetic evaluation index is based on heart rate variability and is related to the autonomic response to pain or stress. The Comfort Behavior Scale is used to assess distress intensity in sedated intubated children. The objective of this study was to assess the validity and performance of newborn infant parasympathetic evaluation as a distress indicator during procedural distress. DESIGN: Monocentric, prospective, noninterventional pilot study of diagnostic accuracy between October 1, 2017, and April 30, 2019. SETTING: PICU in a tertiary care university hospital. PATIENTS: Sedated intubated children under 3 years old. INTERVENTIONS: We continuously obtained mean newborn infant parasympathetic evaluation and instantaneous newborn infant parasympathetic evaluation scores and compared them to Comfort Behavior scores obtained before (T1 period), during (T2 period), and after (T3 period) care procedures. MEASUREMENTS AND MAIN RESULTS: We obtained 54 measurements from 32 patients. The median age was 4 months (23 d to 31 mo). Between T1 and T2, there was a significant decrease in the instantaneous newborn infant parasympathetic evaluation and mean newborn infant parasympathetic evaluation scores (64 ± 2 to 42 ± 1 [p 0.0001] and 64 ± 1 to 59 ± 1 [p = 0.007], respectively) and a significant increase in the Comfort Behavior scores (from 12 ± 0 to 16 ± 1; p 0.0001). Comfort Behavior scores and instantaneous newborn infant parasympathetic evaluation and mean newborn infant parasympathetic evaluation scores were significantly inversely correlated (r = -0.44, p 0.0001 and r = -0.19, p = 0.01, respectively). With a instantaneous newborn infant parasympathetic evaluation score threshold of 53, the sensitivity, specificity, positive predictive, and negative predictive values to predict a Comfort Behavior Scale up to 17 were 80.0%, 73.5%, 43.8%, and 93.5%, respectively. CONCLUSIONS: Instantaneous newborn infant parasympathetic evaluation is valid for assessing distress in sedated/intubated children in the PICU. Further studies are needed to confirm these results and for newborn infant parasympathetic evaluation-based comparisons of sedation-analgesia protocols.

Use of paediatric early warning scores in intermediate care units.

OBJECTIVE: Paediatric early warning scores (EWS) were developed to detect deterioration in paediatric wards or emergency departments. The aim of this study was to assess the relationship between three paediatric EWS and clinical deterioration detected by the nurse in paediatric intermediate care units (PImCU). METHODS: This was a prospective, observational, multicentre study at seven French regional hospitals that included all children <18 years of age. Clinical parameters included in three EWS (Paediatric Advanced Warning Score, Paediatric Early Warning Score and Bedside Paediatric Early Warning System) were prospectively recorded every 8 hours or in case of deterioration. The outcome was a call to physician by the nurse when a clinical deterioration was observed. The cohort was divided into derivation and validation cohorts. An updated methodology for repeated measures was used and discrimination was estimated by the area under the receiver-operating curve. RESULTS: A total of 2636 children were included for 14 708 observations to compute a posteriori the EWS. The discrimination of the three EWS for predicting calls to physicians by nurses was good (range: 0.87-0.91) for the derivation cohort and moderate (range: 0.71-0.76) for the validation cohort. Equations for probability thresholds of calls to physicians, taking into account the time t, the score at time t and the score at admission, are available. CONCLUSION: These three EWS developed for children in paediatric wards or emergency departments can be used in PImCU to detect a clinical deterioration and predict the need for medical intervention.

Dilated Cardiomyopathy Caused by Wilms Tumor.

The authors report the case of a 7-month-old girl with unilateral Wilms tumor with dilated cardiomyopathy, leading to cardiac failure and requiring intensive care. After chemotherapy and tumor removal, cardiac function has improved. This is a rare report on the association between nephroblastoma and dilated cardiomyopathy without hypertension.

[Fatal aorto-esophageal fistula due to accidental ingestion of button battery. Algorithm for management of disk-battery ingestion in patients younger than 6 years old]. / Ingestion d'une pile bouton compliquée d'une fistule œso-aortique fatale. Proposition d'un algorithme de prise en charge chez l'enfant de moins de 6ans.

The ingestion of disc battery is a common problem in children and current treatment may be sometime inadequate. Ingested button batteries have the potential to cause significant morbidity and mortality. Ingestion of button batteries has been seen with increasing frequency over the last decade, particularly for children aged younger than 6 years. If most cases of disc battery ingestion run uneventful courses, however, harmful outcomes are more common with ingestion of lithium batteries (3V) with a diameter greater than or equal to 16 mm. These young children have to benefit from a chest radiograph within 2hours which follow the ingestion. If the battery impacts in the esophagus, emergency endoscopic management is necessary. We report the case of one young child died followed an unknown lithium disk-battery ingestion complicated with an aorto-œsophageal fistula. We propose a protocol of specific coverage for patients aged younger than 6 years old.

Prognostic value of shock index in children with septic shock.

OBJECTIVES: Septic shock is frequent in children and is associated with high mortality and morbidity rates. Early recognition of severe sepsis improve outcome. Shock index (SI), ratio of heart rate (HR) and systolic blood pressure (SBP), may be a good noninvasive measure of hemodynamic instability that has been poorly studied in children. The aim of the study was to explore the usefulness of SI as an early index of prognosis for septic shock in children. METHODS: The study was retrospective and performed in 1 pediatric intensive care unit at a university hospital. The following specific data were collected at 0, 1, 2, 4, and 6 hours after admission: HR and SBP for SI calculation and lactate concentration. Patients were divided into 2 groups according to their outcome (death/survival). RESULTS: A total of 146 children admitted with septic shock between January 2000 and April 2010 were included. Shock index was significantly different between survivors and nonsurvivors at 0, 4, and 6 hours after admission (P = 0.02, P = 0.03, and P = 0.008, respectively). Age-adjusted SIs were different between survivors and nonsurvivors at 0 and 6 hours, with a relative risk of death at these time points of 1.85 (1.04-3.26) (P = 0.03) and 2.17 (1.18-3.96) (P = 0.01), respectively. Moreover, an abnormal SI both at admission and at 6 hours was predictive of death with relative risk of 1.36 (1.05-1.76). CONCLUSIONS: In our population of children with septic shock, SI was a clinically relevant and easily calculated predictor of mortality. It could be a better measure of hemodynamic status than HR and SBP alone, allowing for the early recognition of severe sepsis.

Assessment of the Pediatric Index of Mortality 2 with the Pao2/Fio2 ratio derived from the Spo2/Fio2 ratio: a prospective pilot study in a French pediatric intensive care unit.

OBJECTIVE: The Pediatric Index of Mortality 2 is a mortality prediction tool used in pediatric intensive care units. Arterial blood gas sampling that is required to calculate the Pao2/Fio2 ratio is often unavailable. Several authors have proposed mathematical relations between the Pao2/Fio2 and Spo2/Fio2 ratios. The main objective of this study was to assess the validity of the Pediatric Index of Mortality 2 score and three modified Pediatric Index of Mortality 2 scores with the Pao2/Fio2 ratio calculated from the Spo2/Fio2 ratio. DESIGN: Prospective observational study of consecutive patients admitted during a 23-month period. SETTING: A multidisciplinary French pediatric intensive care unit. PATIENTS: One thousand forty-three patients, 80 of whom died (7.7%). INTERVENTIONS: None. MEASUREMENTS AND MAINS RESULTS: Only 15 of 1043 patients had a Pao2 measurement. Spo2/Fio2 ratio was determined for 338 patients (33%) for whom Fio2 was known and Spo2 was ≤ 97%. However, for mathematical reasons, Pao2/FIo2 ratio could be calculated from the three Spo2/Fio2 equations for 328 (31%), 295 (28%), and 100 (10%) patients, respectively. Discrimination assessed by the area under the receiver operating characteristic curve was 0.86 for Pediatric Index of Mortality 2 and the modified scores. Calibration assessed by the Hosmer-Lemeshow goodness-of-fit test was poor for Pediatric Index of Mortality 2 (p = .04) and one modified score (p = .03) and good for two other modified scores (p = .06, p = .09). CONCLUSION: This study suggests that the Spo2/Fio2 ratio could be used in place of Pao2/Fio2 for calculating Pediatric Index of Mortality 2. This must nonetheless be confirmed by a larger prospective multicenter study.