The objective of our present study is to evaluate the impact of different PEEP levels on cerebral hemodynamic, gas exchanges and respiratory system mechanics in paediatric patients with ...post-traumatic brain swelling treated with decompressive craniectomy (DC).
A prospective physiologic study was carried out on 14 paediatric patients presenting with severe traumatic brain swelling treated with DC. Transcranial Doppler ultrasonography was performed on the middle cerebral artery bilaterally after DC. After assessment at ZEEP, intracranial pressure (ICP), cerebral perfusion pressure (CPP), mean arterial pressure (MAP), central venous pressure (CVP) and gas exchanges were recorded at PEEP 4 and PEEP 8.
From ZEEP to PEEP 8, the compliance of respiratory system indexed to the weight of the patient significantly increased (P = 0.02) without ICP modifications. No significant variation of the MAP, CPP, Vmed, the total resistance of respiratory system and ohmic resistance of the respiratory system indexed to the weight of the patients was observed. CVP significantly increased between ZEEP and PEEP 8 (P = 0.005), and between PEEP 4 and PEEP 8 (P = 0.05).
PEEP values up to 8 cmH20 seem to be safe in paediatric patients with a severe post-traumatic brain swelling treated with DC.
Background
Intrathecal nusinersen administration, a fundamental step in the treatment of spinal muscular atrophy, is challenging in children.
Aims
This retrospective monocentric analysis of ...prospectively collected data evaluated the feasibility of needleless general anesthesia exclusively with sevoflurane, without imaging guidance, for children undergoing nusinersen administration in a 24‐month period.
Methods
Clinical data included demographics, type of spinal muscular atrophy, presence and severity of scoliosis. Primary outcome was defined by the number of predefined sentinel adverse events related to anesthesia. Secondary outcomes were assessed by duration of the procedure, number of lumbar puncture attempts, and number of failures. Other measures included number and type of moderate, minor and minimal adverse events, as well as number and type of puncture‐related adverse events.
Results
116 patients (mean age: 8.7 (SD 6.9) years; with scoliosis: 49.1%) underwent 250 lumbar punctures; two cases of prolonged desaturation, considered as sentinel adverse events, (0.8%) were recorded during anesthesia (primary outcome). None of the patients underwent orotracheal intubation nor required an unplanned admission in the Pediatric Intensive Care Unit. No patient required an unplanned or prolonged hospitalization after the procedure. Mean number of puncture attempts was 1.6 (SD 1.3), and mean duration of the procedure was 14.1 (SD 8.3) minutes. No failure in the drug administration occurred (secondary outcomes).
Conclusion
In this single‐center experience, needleless general anesthesia with inhaled sevoflurane without imaging guidance has been shown to be feasible for children with spinal muscular atrophy undergoing lumbar puncture for nusinersen administration.
Background: Wernicke encephalopathy (WE) is an acute and potentially fatal neuropsychiatric disorder resulting from thiamine deficiency: its etiology and clinical presentation can be heterogeneous ...and arduously recognized, especially in children and adolescents. Case presentation: An 8-year-old girl arrived to the emergency room with ataxic gait, nystagmus, and mental confusion after a 10-day history of repeated severe vomiting; her recent clinical history was characterized by restricted nutrition due to a choking phobia, which caused substantial weight loss. Brain magnetic resonance imaging revealed a bilaterally increased T2 signal in the medial areas of the thalami and cerebral periaqueductal region. Diagnosis of WE based on clinical and neuroradiological findings was established and confirmed after labwork showing low serum thiamine. Following psychiatric evaluation, the patient was also diagnosed with avoidance-restrictive food intake disorder (ARFID), which required starting cognitive behavioral therapy and introducing aripiprazole. The patient displayed improvement of the radiological findings after one month and complete resolution of her neurological symptoms and signs. Conclusions: Eating disorders like ARFID might forerun acute signs of WE; this possibility should be considered even in pediatric patients, especially when atypical neurological pictures or feeding issues come out.
Severe traumatic brain injury (TBI) is one of the most dramatic events in pediatric age and, despite advanced neuro-intensive care, the survival rate of these patients remains low. Children suffering ...from severe TBI show long-term sequelae, more pronounced in behavioral, neurological and neuropsychological functions leading to, in the most severe cases, an unresponsive wakefulness syndrome (UWS). Currently, no effective treatments can restore neuronal loss or produce significant improvement in these patients. In experimental animal models, human- recombinant Nerve Growth Factor (hr-NGF) promotes neural recovery supporting neuronal growth, differentiation and survival of brain cells and up-regulating the neurogenesis-associated processes. Only a few studies reported the efficacy of intranasal hr-NGF administration in children with post- traumatic UWS. Children with the diagnosis of post-traumatic UWS were enrolled. These patients underwent a treatment with intranasal hr-NGF administration, at a total dose of 50 gamma/kg, three times a day for 7 consecutive days. The treatment schedule was performed for 4 cycles, at one month distance each. Neuroradiogical evaluation by Positron Emission Tomography scan (PET), Single Photon Emission Computed Tomography (SPECT), Electroencephalography (EEG), and Power Spectral Density (PSD) was determined before the treatment and one month after the end. Neurological assessment was also deepened by using modified Ashworth Scale, Gross Motor Function Measure, and Disability Rating Scale. Three children with post-traumatic UWS were treated. hr-NGF administration improved functional (PET and SPECT) and electrophysiological (EEG and PSD) assessment. Also clinical conditions improved, mainly for the reduction of spasticity and with the acquisition of voluntary movements, facial mimicry, attention and verbal comprehension, ability to cry, cough reflex, oral motility, and feeding capacity, with a significant improvement of their neurological scores. No side effects were reported. These promising results and the ease of administration of this treatment make it worthwhile to be investigated further, mainly in the early stages from severe TBI and in patients with better baseline neurological conditions, to explore more thoroughly the benefits of this new approach on neuronal function recovery after traumatic brain damage.
The role of cytokines in relation to clinical manifestations, disease severity, and outcome of children with H1N1 virus infection remains thus far unclear. The aim of this study was to evaluate ...interleukin IL-1β and IL-6 plasma expressions and their association with clinical findings, disease severity, and outcome of children with H1N1 infection. We prospectively evaluated 15 children with H1N1 virus infection and 15 controls with lower respiratory tract infections (LRTI). Interleukin plasma levels were measured using immunoenzymatic assays. Significantly higher levels of IL-1β and IL-6 were detected in all patients with H1N1 virus infection compared to controls. It is noteworthy to mention that in H1N1 patients with more severe clinical manifestations of disease IL-1β and IL-6 expressions were significantly upregulated compared to H1N1 patients with mild clinical manifestations. In particular, IL-6 was significantly correlated with specific clinical findings, such as severity of respiratory compromise and fever. No correlation was found between interleukin expression and final outcome. In conclusion, H1N1 virus infection induces an early and significant upregulation of both interleukins IL1β and IL-6 plasma expressions. The upregulation of these cytokines is likely to play a proinflammatory role in H1N1 virus infection and may contribute to airway inflammation and bronchial hyperreactivity in these patients.
Botulism is a neuroparalytic syndrome caused by a neurotoxin produced by Clostridium botulinum. We describe a patient with neurological symptoms associated with intoxication by Clostridium botulinum ...and infection by SARSCoV2. This report underlines that it is mandatory, even in case of SARS-CoV-2 positivity, to investigate all the causes of a clinical pattern.
•Botulism is a neuroparalytic syndrome caused by a neurotoxin produced by the Clostridium Botulinum.•Considering the SARS-CoV-2 pandemic, in patients with neurological symptoms, this infection must be considered.•Considering neurological manifestations of COVID-19, some of them are associated also to a botulinum-like clinical pattern.•If there's overlap of clinical signs a detailed medical history should be collected in order to make differential diagnosis.
The use of peripheral blood hematopoietic stem cells for bone marrow reconstitution after myeloablative therapy is well established in children with malignant disorders. However, the peripheral blood ...hematopoietic stem cells collection in very low-body weight (≤10 kg) children remains a significant challenge because of technical and clinical issues. A male newborn affected by atypical teratoid rhabdoid tumor, diagnosed prenatally, received two cycles of chemotherapy following surgical resection. After an interdisciplinary discussion, it was decided to intensify the treatment with high-dose chemotherapy followed by autologous stem cell transplantation. After 7 days of G-CSF administration the patient underwent hematopoietic progenitor cells-apheresis collection. The procedure was performed in the pediatric intensive care unit, using two central venous catheters and Spectra Optia device. The cell collection procedure was completed in 200 min, during which time 3.9 total blood volumes were processed. During apheresis we did not observe electrolyte alterations. No adverse events were recorded during or immediately following the cell collection procedure. Our report describes the feasibility of performing large volume leukapheresis without complications in an extremely low-body weight patient weighing 4.5 kg using the Spectra Optia apheresis device. No catheter-related problems occurred, and apheresis was completed without any adverse event. In conclusion, we believe that very low-body weight pediatric patients need a multidisciplinary approach to manage central venous access, hemodynamic monitoring, cell collection, prevention of metabolic complications to improve safety, feasibility, and efficiency of stem cell collection procedures.
Objective
To verify the feasibility of non-invasive ventilation (NIV) in immunocompromised children affected by ARDS.
Setting
University Hospital PICU.
Patients
Twenty-three consecutive ...immunocompromised children treated with NIV for ARDS.
Interventions
All consecutive patients received NIV through a face-mask or a helmet.
Results
No differences were found regarding admission data and severity scores between NIV responders and non-responders. Early and sustained improvement in PaO
2
/FiO
2
ratio were observed in 82 and 74% of cases, respectively. 13 out of 23 patients (54.5%) avoided intubation and were discharged from the PICU; ten patients required intubation: two of them survived and eight patients died (two refractory hypoxemia, three septic shock, three multi-organ failure). PICU and intra-hospital mortality was significantly higher for NIV-nonresponders (
P
< 0.001). PICU stay was significantly shorter for NIV responders (
P
= 0.03). NIV responders had significantly lower heart and respiratory rate at the end of treatment (
P
< 0.001 and
P
= 0.048, respectively).
Conclusions
NIV administration is feasible and well tolerated in immunocompromised children with ARDS. A short NIV trial can be used to verify the usefulness of the technique. A randomized controlled trial is needed to confirm the efficacy of NIV in immunocompromised children requiring ventilatory support for ARDS.
Cardio-facio-cutaneous syndrome (CFC) is a genetic disorder due to variants affecting genes coding key proteins of the Ras/MAPK signaling pathway. Among the different features of CFC, neurological ...involvement, including cerebral malformations and epilepsy, represents a common and clinically relevant aspect. Status epilepticus (SE) is a recurrent feature, especially in a specific subgroup of CFC patients with developmental and epileptic encephalopathy (DEE) and history of severe pharmacoresistant epilepsy. Here we dissect the features of SE in CFC patients with a particular focus on longitudinal magnetic resonance imaging (MRI) findings to identify clinical-radiological patterns and discuss the underlying physiopathology.
We retrospectively analyzed clinical, electroencephalogram (EEG), and MRI data collected in a single center from a cohort of 23 patients with CFC carrying pathogenic BRAF variants who experienced SE during a 5-year period.
Seven episodes of SE were documented in 5 CFC patients who underwent EEG and MRI at baseline. MRI was performed during SE/within 72 hours from SE termination in 5/7 events. Acute/early post-ictal MRI findings showed heterogenous abnormalities: restricted diffusion in 2/7, focal area of pcASL perfusion change in 2/7, focal cortical T2/FLAIR hyperintensity in 2/7. Follow-up images were available for 4/7 SE. No acute changes were detected in 2/7 (MRI performed 4 days after SE termination).
Acute focal neuroimaging changes concomitant with ictal EEG focus were present in 5/7 episodes, though with different findings. The heterogeneous patterns suggest different contributing factors, possibly including the presence of focal cortical malformations and autoinflammation. When cytotoxic edema is revealed by MRI, it can be followed by permanent structural damage, as already observed in other genetic conditions. A better understanding of the physiopathology will provide access to targeted treatments allowing to prevent long-term adverse neurological outcome.
Cardio-facio-cutaneous syndrome is a genetic disorder that often causes prolonged seizures known as status epilepticus. This study has a focus on electroclinical and neuroimaging patterns in patients with cardio-facio-cutaneous syndrome. During these status epilepticus episodes, we found different abnormal brain imaging patterns in patients, indicating various causes like brain malformations and inflammation. Understanding these patterns could help doctors find specific treatments, protecting cardio-facio-cutaneous syndrome patients from long-term brain damage.
Abstract
Objectives
To investigate how life-sustaining treatment (LST) decisions are made and identify problematic ethical concerns confronted by physicians and nurses in pediatric intensive care ...within Italy.
Methods
An 88-question online survey was created, based on a previous qualitative study conducted by this team. The survey was designed to identify how LST decisions were managed; contrasting actual practices with what participants think practices should be. Replies from physicians and nurses were compared, to identify potential inter-professional ethical tensions. The study also identified participants’ principal ethical concerns. Moreover, open-ended questions elicited qualitative perspectives on participants’ views. The survey was pilot-tested and refined before initiation of the study.
Results
31 physicians and 65 nurses participated in the study. Participants were recruited from pediatric intensive care units across five Italian cities; i.e., Florence, Milan, Padua, Rome, Verona. Statistically significant differences were identified for (a) virtually all questions contrasting actual practices with what participants think practices should be and (b) 14 questions contrasting physician replies with those of nurses. Physicians and nurses identified the absence of legislative standards for LST withdrawal as a highly problematic ethical concern. Physicians also identified bearing responsibility for LST decisions as a major concern. Qualitative descriptions further demonstrated that these Italian pediatric intensive care clinicians encounter significantly distressing ethical problems in their practice.
Conclusions
The results of this study highlight a need for the development of (a) strategies for improving team processes regarding LST decisions, so they can be better aligned with how clinicians think decisions should be made, and (b) Italian LST decision-making standards that can help ensure optimal ethical practices.
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