Transcranial Doppler Ultrasonography in Children With Acute Liver Failure and Severe Hepatic Encephalopathy.

OBJECTIVES: To report our single-center use of transcranial Doppler (TCD) for noninvasive neuromonitoring in pediatric patients with acute liver failure (ALF). DESIGN: Retrospective cohort from January 2016 to June 2019. SETTING: PICU in Bicêtre Hospital, Assistance Publique- Hôpitaux de Paris (AP-HP), a national referral center for pediatric liver transplantation. PATIENTS: Pediatric patients with severe ALF (prothrombin time < 30% and Hepatic Encephalopathy score ≥ 3), on continuous venovenous high-flow hemofiltration. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Ten children were identified, six were transplanted (1/6 died) and four were not (3/4 died). TCD was performed several times per patient and the evolution of cerebral perfusion parameters was followed. Of interest, zero of six patients who survived lost end-diastolic velocity (EDV), whereas four of four patients who died did (difference, 100%; 95% CI, 37-100%; χ 2 , 9; degrees of freedom, 1; p = 0.0027). We failed to identify an association between pulsatility index (PI) or EDV, and severity of hepatic encephalopathy. CONCLUSIONS: TCD was a noninvasive and bedside available tool to detect and screen for presence of abnormal cerebral flow in children with ALF, according to age-related reference values. TCD detected reduced EDV and elevated PI in children with ALF awaiting transplant who died compared with those who survived.

Ultrasound guided percutaneous catheterization of the brachiocephalic vein by small caliber catheter: An alternative to epicutaneo-caval catheter in newborn and premature infants.

BACKGROUND: Umbilical Venous Catheter (UVC) and Epicutaneo-Caval Catheters (ECC) are reference catheters in the neonatal period. However, many factors such as the corpulence of neonates, poor venous capital, and anatomical variants can complicate ECC insertion or make it impossible. In newborns with failed ECC insertion, we developed an hybrid technique that combines the insertion of a long-lasting silicone or polyurethane small caliber catheter, usually used as a ECC in newborns, with the ease and speed of ultrasound guided puncture of the brachiocephalic vein (BCV). METHODS: Three years retrospective single center experience of ultrasound guided BCV insertion of silicon or polyurethane small caliber central catheter in a tertiary neonatal intensive care in case of insertion fail of ECC. RESULTS: Twenty-one echo guided BCV-ECC insertions were performed in 20 newborns. Median age was 16 days (range: 0-110 days), median weight was 1700 g (range: 605-4960 g) at insertion. In most cases, insertion was on the left side (17/21). No failures were noted. Only one attempt was necessary in all cases. Insertion time, when noted, was always of <45 min. The median duration of use of these catheters was 11 days (range 3-35 days). No complication was noted during insertion or catheter use, including catheter-related infections and thrombosis. CONCLUSION: Echo guided percutaneous catheterization of the brachiocephalic vein with a long lasting silicone or polyurethane small caliber catheter is a safe alternative to the ECC if insertion has failed. However, it requires a mastery of ultrasound-guided insertion technique in term and premature neonates.

High flow nasal cannula therapy versus continuous positive airway pressure and nasal positive pressure ventilation in infants with severe bronchiolitis: a randomized controlled trial.

INTRODUCTION: non-invasive ventilation is widely used in the respiratory management of severe bronchiolitis. METHODS: a randomized controlled trial was carried out in a tertiary pediatric university hospital´s PICU over 3 years to compare between continuous positive airway pressure/nasal positive pressure ventilation (CPAP/NPPV) and high flow nasal cannula (HFNC) devices for severe bronchiolitis. The trial was recorded in the national library of medicine registry (NCT04650230). Patients aged from 7 days to 6 months, admitted for severe bronchiolitis were enrolled. Eligible patients were randomly chosen to receive either HFNC or CPAP/NPPV. If HFNC failed, the switch to CPAP/NPPV was allowed. Mechanical ventilation was the last resort in case of CPAP/NPPV device failure. The primary outcome was the success of the treatment defined by no need of care escalation. The secondary outcomes were failure predictors, intubation rate, stay length, serious adverse events, and mortality. RESULTS: a total of 268 patients were enrolled. The data of 255 participants were analyzed. The mean age was 51.13 ± 34.43 days. Participants were randomized into two groups; HFNC group (n=130) and CPAP/NPPV group (n=125). The success of the treatment was significantly higher in the CPAP/NPPV group (70.4% [61.6%- 78.2%) comparing to HFNC group (50.7% [41.9%- 59.6%])- (p=0.001). For secondary outcomes, lower baseline pH was the only significant failure predictor in the CPAP/NPPV group (p=0.035). There were no differences in intubation rate or serious adverse events between the groups. CONCLUSION: high flow nasal cannula was safe and efficient, but CPAP/ NPPV was better in preventing treatment failure. The switch to CPAP/NPPV if HFNC failed, avoided intubation in 54% of the cases.

Renal Involvement in 2 Siblings With Cockayne Syndrome.

Renal involvement in Cockayne syndrome is rare and its pathogenesis is yet unknown. We report herein 2 cases (siblings) with Cockayne syndrome type A confirmed by biochemical and molecular assays. The first case was a 13-year-old girl who presented with nephritic syndrome and a rapidly progressive kidney failure. Her younger sister, 7 years old, exhibited hypertension, hyperfiltration, and microalbuminuria. She had hyperreninemia and hyperaldosteronemia without kidney failure or renal arterial stenosis. Renal biopsy, performed the older sister, revealed cystic focal segmental glomerulosclerosis, arteriosclerosis, tubulointerstitial fibrosis, and tubular atrophy. The different clinical phenotypes in the two siblings support the absence of an obvious genotype-phenotype correlation in Cockayne syndrome type A patients. In the older sister, the particular focal glomerular sclerosis and senile lesions assume that kidney disease in Cockayne syndrome may be related to prematurely aging secondary to a defective nucleotide repair.

Predictors of Mortality in Mechanically Ventilated Critical Pertussis in a low Income Country.

BACKGROUND: Critical pertussis is characterized by severe respiratory failure, important leukocytosis, pulmonary hypertension, septic shock and encephalopathy. AIM: To describe the clinical course of critical pertussis, and identify predictors of death at the time of presentation for medical care. METHODOLOGY: Retrospective study conducted in children's hospital Tunisian PICU between 01 January and 31 October 2013. Patients with critical pertussis confirmed by RT-PCR and requiring mechanical ventilation were included. Predictors of death were studied. RESULTS: A total of 17 patients was studied. Median age was 50 days. Mortality was 23%. Predictors risk of mortality were : high PRISM score (Pediatric Risk of Mortality Score) (p=0,007), shock (p=0,002), tachycardia (p=0,005), seizures (p=0,006), altered mental status (p=0,006), elevated WBC count (p=0,003) and hemodynamic support (p=0022). However, the difference did not reach statistical significance in comorbidity, pneumoniae, high pulmonary hypertension or exchange transfusion. Concomitant viral or bacterial co-infection was not related to poor outcome. CONCLUSION: Young infants are at high risk to have critical pertussis. Despite advances in life support and the treatment of organ failure in childhood critical illness, critical pertussis remains difficult to treat.