Weaning Off Research Articles

Pediatric Home Parenteral Nutrition in France: A six years national survey

Home Parenteral Nutrition (HPN) is the cornerstone management for children suffering from chronic intestinal failure (CIF). In France, HPN is organized from a network of 7 certified centers located in University Hospitals spread across the national territory. This study aims to review the data involving children on HPN over a 6-years period in France to outline the global and continuous improvement in care.This cross-sectional study included all children enrolled in any of the 7 French HPN certified centers from January 1st, 2014 to December 31st, 2019. Data was recorded from annual databases provided by each center regarding: age at inclusion, indication and duration of HPN, type of intravenous lipid emulsion (ILE), outcome [PN weaning off, transfer to adult center, death, intestinal transplantation (ITx)], rate of catheter-related bloodstream infections (CRSBIs) for 1000 days of HPN, Taurolidine lock procedure (TLP) use and prevalence of cholestasis defined as conjugated bilirubin ≥20 μmol/l.The number of patients increased by 43.6% from 268 in 2014 to 385 in 2019. According to the year of follow up, the indications for HPN were short bowel syndrome (SBS) (42.3-46.6%), congenital enteropathies (CE) (18.5-22.8%), chronic intestinal pseudo-obstruction syndrome (CIPOS) (13.0-16.3%), long segment Hirschsprung's disease (LSHD) (9.7-13.3%), Crohn's disease (CD) (1.6-2.6%) and other non-primary digestive diseases (NPDD) such as immune deficiency, cancer or metabolic disease (4.0-9.2%). The median age at discharge on HPN decreased from 11.7 months in 2014 to 8.3 months in 2019 (p < .001). By December 31st, 2019, 44.8% of children had left the HPN program after a median duration ranging between 39.9 and 66.4 months. Among these patients, 192 (74.2%) were weaned off PN (94.7% SBS), 41 (15.8%) were transferred to adult centers for CIPOS (42%), SBS (31%) or CE (27%), 21 died (8.1%) - mostly in relation to cancer or immune deficiency - and 5 were transplanted (1.9%): 4 underwent combined liver-intestine transplantation for LSHD (n = 2), SBS, CE and one multivisceral Tx for CIPOS. The use of a composite fish-oil based ILE increased from 67.4% in 2014 to 88.3% in 2019 (p < 0.001). CRBSIs dropped from 1.04 CRSBIs per 1000 days HPN in 2014 to 0.61 in 2019 (p < 0.001) while meantime, the percentage of children receiving TLP increased from 29.4% to 63.0% (p < 0.001). The prevalence of cholestasis (conjugated bilirubin ≥ 20 μmol/l) was low and stable between 4.1 and 5.9% of children during the study period.In France, the number of children enrolled in a HPN program continuously increased over a 6 years period. SBS is the leading cause of CIF requiring HPN. The rate of CRBSIs dropped dramatically as the use of TLP increased. Mortality rate was low and mainly in relation to the underlying disease (cancer, immune deficiency). Cholestasis and intestinal Tx remained very rare.

Variation of plasma citrulline as a predictive factor for weaning off long-term parenteral nutrition in children with neonatal short bowel syndrome

Long-term parenteral nutrition (PN) is the mainstay of the therapeutic strategy in intestinal failure (IF) due to neonatal short bowel syndrome (SBS). Our aim was to identify prognostic factors for PN weaning and to assess if measuring plasma citrulline concentrations over time could account for the intestinal adaptation in progress. This retrospective study included children with neonatal SBS with surgical measurement of the residual bowel length and repeated plasma citrulline assessments during a 4-year follow-up. The degree of IF was assessed by the PN dependency index (PN caloric intake/Resting energy expenditure). The analysis was carried out according to SBS anatomical groups: end-jejunostomy (type 1), jejuno-colic (type 2) and jejuno-ileal anastomosis (type 3). Fifty-five patients (8 type 1, 27 type 2, 20 type 3) were included. None of the patients with SBS type 1, 11 (41%) with type 2 and 11 (55%) with type 3 were weaned off during the follow-up period. Plasma citrulline levels significantly increased with time in patients who were finally weaned off PN; conversely, the levels did not consistently increase in patients who were still on PN at the end of the study period. There was an inverse relationship between plasma citrulline levels and the PN dependency index. The increasing citrulline levels had a positive effect on the probability of weaning, 2.7 times higher for each point increase in citrulline. No significant effect of age and residual bowel length at baseline was found. The increased plasma citrulline level over time in addition to the SBS anatomical type is a reliable marker for subsequent PN weaning. The prediction of PN weaning assessed solely by the residual bowel length or a single measurement of citrulline is insufficient and should also take into account the anatomical type of SBS and repeated measurements of plasma citrulline levels.

Strategies for Continuous Renal Replacement Therapy De-escalation.

Strategies for Continuous Renal Replacement Therapy De-escalation.

Adaptation of a respiratory service to provide CPAP for patients with COVID-19 pneumonia, outside of a critical care setting, in a district general hospital

One-hundred and forty patients at Croydon University Hospital received continuous positive airway pressure (CPAP) on a specialist respiratory ward, as a bridge to invasive mechanical ventilation (IMV) or as a ceiling of care for COVID-19. This retrospective study aimed to outline service expansion, patient characteristics and explore risk factors in outcomes. Mean age of patients on CPAP was 64 years (standard deviation 12). The median number of days from admission to CPAP initiation was 1 day (interquartile range (IQR) 0-3), and time before successful wean off CPAP was 4 days (IQR 2-6). Twenty-eight-day mortality was 64%. Thirty-four per cent of patients went onto require IMV, 24% improved off CPAP and 41% were palliated. The 28-day non-survivor group were of older age, had statistically significant higher admission creatinine and higher peak oxygen requirement. Age above 65 years was associated with higher mortality (odds ratio 5.9; 95% confidence interval 2.63-13.3). CPAP is a viable ceiling-of-treatment option in those unsuitable for ventilation, and may even avoid the need for ventilation in others. Duration on CPAP may be useful for service provision to predict resource allocation. The rapidity from admission to CPAP initiation highlights the need for early ceilings of care to be established.

Difficulty Weaning off Cardiopulmonary Bypass in Patient Undergoing Aortic Valve Replacement and Concomitant CABG Surgery

The article's abstract is no available.

An unfortunate case of acute aortic regurgitation: Isolated aortic valve prolapse following a motor vehicle accident

We present an unfortunate case of severe acute aortic regurgitation (AR) following a motor vehicle accident (MVA) linked to isolated aortic valve prolapse, with no evidence of aortic root disruption or other valvular pathology missed on initial presentation. A 55-year-old gentleman, with known hypertension, was brought into the emergency department following a MVA, where he sustained severe intra-thoracic injuries. A bedside transthoracic echocardiogram (TTE) revealed a trileaflet aortic valve with evidence of mal-coaptation and severe AR. A computed tomography angiography of the thorax, however, failed to demonstrate evidence of dissection along the aortic root or ascending aorta. Following successful weaning off ventilatory support, the patient was discharged, but he presented back within a week with worsening dyspnoea and palpitations. Examination and investigation supported a diagnosis of acute heart failure with evident severe AR on repeat TTE. Transoesophageal echocardiography was performed, revealing prolapsed right and non-coronary cusps which were not seen in previous studies but absent evidence of the aortic root, ascending and descending aorta dilatation or dissection. Valvular complications rarely occur following blunt chest wall trauma, often involving right-sided valves due to their proximity to the sternum. Although aortic valve disruption can occur following MVAs, it is often associated with trauma to the aorta. Based on our literature search, there have been only a handful of reported cases of severe acute AR due to isolated prolapse or ruptured aortic valves in the absence of aortic valve perforation, aortic root disruption or dissection and other valvular abnormalities following trauma.

Importance of pulse pressure after extracorporeal cardiopulmonary resuscitation

Recent reports have revealed better clinical outcomes for extracorporeal cardiopulmonary resuscitation (ECPR) than conventional cardiopulmonary resuscitation (CPR). In this retrospective study, we attempted to identify predictors associated with successful weaning off extracorporeal membrane oxygenation (ECMO) support after ECPR. The demographic and clinical data of 30 ECPR patients aged over 18 years treated between August 2016 and January 2019 were analyzed. All clinical data were retrospectively collected. The primary endpoint was successful weaning off ECMO support after ECPR. Patients were divided into two groups based on successful or unsuccessful weaning off ECMO support (Weaned (n = 14) vs. Failed (n = 16)). Univariate logistic regression analysis showed that age, CPR duration, ECMO complications, and loss of pulse pressure significantly predicted the results of weaning off ECMO support. However, multivariate logistic regression analysis showed that only CPR duration and loss of pulse pressure independently predicted unsuccessful weaning from ECMO support. We conclude that long CPR duration and loss of pulse pressure after ECPR predict unsuccessful weaning from ECMO. However, unlike CPR duration, loss of pulse pressure during post-ECPR was related to subsequent management. In patients with reduced pulse pressure after ECPR, careful management is warranted because this reduction is closely associated with unsuccessful weaning off ECMO support after ECPR.

Prediction of successful weaning off ECMO support after ECPR: Is pulse pressure crucial for success?

Extracorporeal cardiopulmonary resuscitation (ECPR) is shown to be associated with favorable outcomes. Next to the relevance of accurate patient selection, hemodynamic management and focused therapy after initiation of extracorporeal membrane oxygenation (ECMO) in cardiopulmonary resuscitation patients are gaining importance. Therapeutic options in patients with loss of pulse pressure during ECMO circulation should be established as early as possible to avoid additional adverse outcomes of ECPR patients.

Impact of Adjuvant Use of Midodrine to Intravenous Vasopressors: A Systematic Review and Meta-Analysis.

Purpose To evaluate the efficacy and safety of midodrine use in intensive care units (ICU) to facilitate weaning off intravenous vasopressors (IVV). Methods We searched PubMed/MEDLINE, Cochrane library, and Google Scholar (inception through October 18th, 2020) for studies evaluating adjuvant use of midodrine to IVV in the ICU. The outcomes of interest were ICU length of stay (LOS), hospital LOS, mortality, IVV reinstitution, ICU readmission, and bradycardia. Estimates were pooled using the random-effects model. We reported effect sizes as standardized mean difference (SMD) for continuous outcomes and risk ratios (RRs) for other outcomes with a 95% confidence interval (CI). Results A total of 6 studies were found that met inclusion criteria and had sufficient data for our quantitative analysis (1 randomized controlled trial and 5 retrospective studies). A total of 2,857 patients were included: 600 in the midodrine group and 2,257 patients in the control group. Midodrine use was not associated with a significant difference in ICU LOS (SMD 0.16 days; 95% CI −0.23 to 0.55), hospital LOS (SMD 0.03 days; 95% CI −0.33 to 0.0.39), mortality (RR 0.87; 95% CI 0.52 to 1.46), IVV reinstitution (RR 0.47; 95% CI 0.17 to 1.3), or ICU readmission (RR 1.03; 95% CI 0.71 to 1.49) when compared to using only IVV. However, there were higher trends of bradycardia with midodrine use that did not reach significance (RR 7.64; 95% CI 0.23 to 256.42). Conclusion This meta-analysis suggests that midodrine was not associated with a significant decrease in ICU LOS, hospital LOS, mortality, or ICU readmissions.

Clinical Pearls

Health care and professional organizations have made much effort to standardize and improve critical care through evidence-based protocols and guidelines. However, intensive care unit (ICU) survivors are at risk for various cognitive, functional, and mental health impairments that can persist during their hospitalization.To characterize the early intensive care unit aftercare period, Kim and colleagues conducted a retrospective study of patients who transferred from a medical ICU to an acute medical unit. They found that the following practices and syndromes (with percentage affected) continued after transfer from the ICU: Findings show that the complexity of ICU care continues after transfer to the acute medical unit. The authors emphasize the urgent need for engagement of non–critical care providers to improve the quality of patient care during early ICU aftercare.See Article, pp 193-200Survivors of acute respiratory distress syndrome (ARDS) can experience long-term problems such as physical limitations, cognitive impairment, and emotional difficulties. Although patients and their family members have reported these problems, there are no specific proven treatments that prevent or treat such problems.Hauschildt and colleagues interviewed adult ARDS survivors 9 to 16 months after their illness to explore how they adapted. They found Although these findings contribute to the knowledge base of ongoing support needed by patients and families in the immediate postdischarge period, the authors recommend future research to assess the effectiveness of various strategies.See Article, pp 221-229About 14% to 16% of all hospitalized patients with COVID-19 require intensive care. Although uncertainties persist about the disease and treatment, some clinical patterns of care have emerged, including difficulties in weaning off mechanical ventilation, challenges in sedation, and use of off-label therapeutics.Bose and colleagues surveyed intensivists’ interest in use of a disease-focused checklist embedded in the electronic medical record to streamline rounds. They found Although overall efficiency and utility of the tool need further examination, the authors suggest that institutions modify the checklist to focus on their own priorities and needs.See Article, pp 238-241Medical errors are the third leading cause of death in the United States. A recent national study indicated that poor physical and mental health of nurses was related to increased medical errors; however, that study included nurses from all specialties.Melnyk and colleagues surveyed critical care nurses (CCNs) to examine the relationship between their health, professional quality of life, perception of wellness support, and self-reported medical errors. They found Nurses who perceived high workplace wellness support reported better health on all measures. Health care system leaders are encouraged to create and sustain cultures of wellness to improve the quality of patient care.See Article, pp 176-184

Celiac Disease in Children: An Association With Drug-Resistant Epilepsy

BackgroundNeurological manifestations are commonly reported in patients with celiac disease (CD). We aimed to characterize epilepsy features in a pediatric population with CD and the effect of a gluten-free diet (GFD) on seizure burden. MethodsA retrospective chart review was performed on pediatric patients treated at the University of Utah and Primary Children's Hospital in Salt Lake City, Utah, with both epilepsy and CD and compared with a control group with epilepsy only. ResultsWe identified 56 patients with epilepsy and biopsy-confirmed CD (n = 36, 64%) or elevated tissue transglutaminase antibodies (tTG-Ab) without biopsy-confirmed CD (n = 20, 36%). Age- and gender-matched controls were selected from patients with epilepsy only (n = 168). Patients with biopsy-proven CD or positive tTG-Ab had high percentage of drug-resistant epilepsy (DRE) compared with the control group (P < 0.05). Age at seizure onset preceded the diagnosis of CD on average by 5.9 years for patients with DRE (P < 0.01) compared with 2.2 years for those with drug-responsive epilepsy. Adhering to a GFD reduced seizure frequency or resulted in weaning dosage or weaning off of one or more antiseizure medications in a majority of patients with DRE. ConclusionsDRE was more prevalent in pediatric patients with biopsy-confirmed CD and positive tTG-Ab compared with the control group (which included childhood epilepsy syndromes), but comparable with the prevalence of DRE in the general population. Adherence to a GFD in combination with antiseizure medications appears to reduce seizure burden for those with CD and DRE.

Prone Position Improve Physiological Parameters of Preterm Infants Weaning from CPAP: A Randomized Control Trial

The need for continuous positive airway pressure (CPAP) was vital for preterm infant with respiratory distress, but the impact of long-term use was very harmful. The effort to do weaning must be done and facilitate the infant to adapt wean off CPAP. The effect of positioning on stabilized physiological parameters may help preterm infant to adapt under gradual weaning from CPAP. Aim in this study to determine the effect of prone positioning on physiological parameters in preterm infants under gradual weaning CPAP. From Mei to September 2020 total sample 60 preterm infants on non-invasive ventilation or CPAP were randomized into prone position (intervention group, n = 30) and supine position (control group, n = 30). Oxygen saturation, respiratory and heart rate between groups were compared. In the prone position was significant higher than supine position at 30 th minute. The mean oxygen saturation in the prone position (99.87±0.35) was higher than in the supine position (97.63±1.45; p=0.001), the mean respiratory rate in the prone position (42.10±8.59 breaths/min) was lower than in the supine position (53.20±6.24 breaths/min; p=0.001), and the mean heart rate in the prone position (144.63±13.07/beats/min) was lower than in the supine position (153.53±10.02/beats/min; p=0.001), so that the prone position can be applied to increase oxygen saturation and to maintain the stability of respiratory rate in preterm infants during gradual weaning. The failure to wean off CPAP (re-CPAP) in the prone group was less frequent which is 3 respondents (5% versus 10%). However, there is no relation between positioning and re-CPAP (p=0.472).

Prone Position Improve Physiological Parameters of Preterm Infants Weaning from CPAP: A Randomized Control Trial

The need for continuous positive airway pressure (CPAP) was vital for preterm infant with respiratory distress, but the impact of long-term use was very harmful. The effort to do weaning must be done and facilitate the infant to adapt wean off CPAP. The effect of positioning on stabilized physiological parameters may help preterm infant to adapt under gradual weaning from CPAP. Aim in this study to determine the effect of prone positioning on physiological parameters in preterm infants under gradual weaning CPAP. From Mei to September 2020 total sample 60 preterm infants on non-invasive ventilation or CPAP were randomized into prone position (intervention group, n = 30) and supine position (control group, n = 30). Oxygen saturation, respiratory and heart rate between groups were compared. In the prone position was significant higher than supine position at 30th minute. The mean oxygen saturation in the prone position (99.87±0.35) was higher than in the supine position (97.63±1.45; p=0.001), the mean respiratory rate in the prone position (42.10±8.59 breaths/min) was lower than in the supine position (53.20±6.24 breaths/min; p=0.001), and the mean heart rate in the prone position (144.63±13.07/beats/min) was lower than in the supine position (153.53±10.02/beats/min; p=0.001), so that the prone position can be applied to increase oxygen saturation and to maintain the stability of respiratory rate in preterm infants during gradual weaning. The failure to wean off CPAP (re-CPAP) in the prone group was less frequent which is 3 respondents (5% versus 10%). However, there is no relation between positioning and re-CPAP (p=0.472).

Brain auditory evoked potentials in pediatric Intensive Care Unit: diagnostic role on encephalopathy and central respiratory failure on infants.

Encephalopathy of different etiologies in infants is often the reason for central respiratory insufficiency which eventually leads patients to the Pediatric Intensive Care Unit. Magnetic resonance imaging (MRI) and brainstem auditory evoked potentials (BAEPs) may be useful to identify brainstem alterations among patients with respiratory insufficiency of central origin. MRI is a compulsory technique to identify brain abnormalities, but may fail to detect brainstem lesions of small dimensions. BAEPs play a highly sensitive role on brainstem dysfunction identification because of the generators of different peaks which are related to specific brainstem structures. The study included ten infants affected by encephalopathy of different etiologies and early neurological respiratory failure. To evaluate BAEPs, the surface electrodes were placed at the vertex (Cz) and on each mastoid side. All subjects presented alteration of BAEPs. The brain MRI revealed selective injury of the brainstem in four patients and supratentorial alterations in six patients. The early identification of brainstem lesions in mechanically ventilated infants with encephalopathy may reduce the weaning off mechanical ventilation's attempt numbers and provide early informative discussions with families and clinical caregivers about treatment options, such as tracheostomy, long term ventilation and the reduction of their length of PICU stay. Furthermore, this would support the evaluation process concerning the affected children, their families and the needs of other social groups, including health systems.

Acute-onset delirium in intensive care COVID patients: association of imperfect brain repair with foodborne micro-pollutants.

Background and purposeCOVID‐19 affects the brain in various ways, amongst which delirium is worrying. An assessment was made of whether a specific, long‐lasting, COVID‐19‐related brain injury develops in acute respiratory distress syndrome patients after life‐saving re‐oxygenation.MethodsTen COVID+ patients (COVID+) with unusual delirium associated with neuroimaging suggestive of diffuse brain injury and seven controls with non‐COVID encephalopathy were studied. The assessment took place when the intractable delirium started at weaning off ventilation support. Brain magnetic resonance imaging (MRI) was performed followed by standard cerebrospinal fluid (CSF) analyses and assessment of CSF erythropoietin concentrations (as a marker for the assessment of tissue repair), and of non‐targeted CSF metabolomics using liquid chromatography high resolution mass spectrometry.ResultsPatients were similar as regards severity scores, but COVID+ were hospitalized longer (25 [11.75; 25] vs. 9 [4.5; 12.5] days, p = 0.03). On admission, but not at MRI and lumbar puncture performance, COVID+ were more hypoxic (p = 0.002). On MRI, there were leptomeningeal enhancement and diffuse white matter haemorrhages only in COVID+. In the latter, CSF erythropoietin concentration was lower (1.73 [1.6; 2.06] vs. 3.04 [2.9; 3.91] mIU/ml, p = 0.01), and CSF metabolomics indicated (a) increased compounds such as foodborne molecules (sesquiterpenes), molecules from industrialized beverages and micro‐pollutants (diethanolamine); and (b) decreased molecules such as incomplete breakdown products of protein catabolism and foodborne molecules (glabridin). At 3‐month discharge, fatigue, anxiety and depression as well as MRI lesions persisted in COVID+.ConclusionsSome COVID+ are at risk of a specific delirium. Imperfect brain repair after re‐oxygenation and lifestyle factors might influence long‐lasting brain injuries in a context of foodborne micro‐pollutants.

Arrhythmia-induced cardiomyopathy: A potentially reversible cause of refractory cardiogenic shock requiring venoarterial extracorporeal membrane oxygenation

The most severe form of arrhythmia-induced cardiomyopathy in adults- refractory cardiogenic shock requiring mechanical circulatory support-has rarely been reported. The purpose of this study was to describe the management of critically ill patients admitted for acute, nonischemic, or worsening of previously known cardiac dysfunction and recent-onset supraventricular arrhythmia who developed refractory cardiogenic shock requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO). This study is a retrospective analysis of prospectively collected data. Between 2004 and 2018, 35 patients received VA-ECMO for acute, nonischemic cardiogenic shock and recent supraventricular arrhythmia (77% atrial fibrillation [AF]). Cardiogenic shock was the first disease manifestation in 21 patients (60%). Characteristics at ECMO implantation [median (interquartile range)] were Sequential Organ Failure Assessment score 10 (7-13); inotrope score 29 (11-80); left ventricular ejection (LVEF) fraction 10% (10%-15%); and lactate level 8 (4-11) mmol/L. For 12 patients, amiodarone and/or electric cardioversion successfully reduced arrhythmia, improved LVEF, and enabled weaning off VA-ECMO; 11 had long-term survival without transplantation or long-term assist device. Eight patients experiencing arrhythmia-reduction failure underwent ablation procedures (7 atrioventricular node [AVN] with pacing, 1 atrial tachycardia) and were weaned off VA-ECMO; 7 survived. Of the remaining 15 patients without arrhythmia reduction or ablation, only the 6 bridged to heart transplantation or left ventricular (LV) assist device survived. Arrhythmia-induced cardiomyopathy, mainly AF-related, is an underrecognized cause of refractory cardiogenic shock and should be considered in patients with nonischemic cardiogenic shock and recent-onset supraventricular arrhythmia. VA-ECMO support allowed safe arrhythmia reduction or rate control by AVN ablation while awaiting recovery, even among those with severe LV dilation.

Adaptive Support Ventilation (ASV). Beneficial or not?

Ventilators functions and features have evolved with the advancement of technology along with the addition of microprocessors. It is important to understand and examine the benefits and risks associated with these advanced automated modes. Adaptive Support Ventilation (ASV) is a mode that is unique to the Hamilton Medical ventilators, thereby limiting the number of clinicians who have experience with using this mode. ASV can make changes to respiratory rate and tidal volume and adjusting the driving pressure in the absence of a professional. ASV changes ventilator strategies when it detects changes to a patient’s lung dynamics. The scope of ASV mode is not universally understood. Respiratory therapists may feel their position would be threatened with the use of smart automated modes. This paper will aim to review the literature on the ASV mode of ventilation. The literature review will address the following research questions to broaden the understanding of the risks and benefits of the ASV mode. 1) Is the ASV mode effective for weaning patients? 2) Is ASV a safe mode of ventilation for patients with COPD and ARDS? 3) Is ASV a safe mode of ventilation with changes in lung dynamics? 4) Does ASV impact the bedside respiratory therapist? Conclusions: ASV appears to be at least effective or even more superior to other modes especially during weaning off mechanical ventilation, and in other forms of respiratory failure. More studies in different clinical conditions and head-to-head with other modes. Keywords: ASV, COPD, ARDS, Weaning

Autologous Hematopoietic Stem Cell Transplantation for Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to conventional therapy can lead to marked disability and represents a therapeutic challenge. To report five cases of treatment-refractory disabling CIDP treated with autologous hematopoietic stem cell transplantation (AHSCT). This was a retrospective cohort study from a tertiary care referral center for both neuromuscular disease and AHSCT. Patients with CIDP treated with AHSCT between 2008 and 2020 were included. All patients had major persistent and disabling neuropathic deficits despite combinations of intensive immunosuppressive therapy. The primary outcome measures were: Medical Research Council sum score, Overall Neuropathy Limitations Scale and requirement for ongoing CIDP immunotherapy after transplantation. We also analyzed safety outcomes by documenting all severe AHSCT-related complications. Five patients with refractory CIDP underwent AHSCT. Three were classified as manifesting a typical syndrome, two were classified as the multifocal Lewis Sumner variant. The mean age at time of CIDP diagnosis was 33.4 years (range 24-46 years), with a median delay of 46 months (range 21-135 months) between diagnosis and AHSCT. The median follow-up period was 41 months. All five patients were able to wean off CIDP-related immunotherapy. Marked improvements in Medical Research Council scale and overall Neuropathy Limitations Scale were noted in 4/5 patients. One patient with longstanding neurogenic atrophy showed no improvement in disability scales. There were no treatment-related deaths or critical illnesses. AHSCT can achieve marked sustained clinical improvement of refractory CIDP and may allow for weaning off long-term complex immunotherapies.

Itolizumab in addressing symptoms of acute respiratory distress syndrome (ARDS) with weaning off oxygen requirements in a COVID-19 patient: A case study

Itolizumab in addressing symptoms of acute respiratory distress syndrome (ARDS) with weaning off oxygen requirements in a COVID-19 patient: A case study - IJIRM- Print ISSN No: - 2581-4214 Online ISSN No:- 2581-4222 Article DOI No:- 10.18231/j.ijirm.2021.013, IP Indian Journal of Immunology and Respiratory Medicine-IP

Weaning from neonatal and pediatric ECMO with stand-by cannula.

The precise moment for weaning a patient off extracorporeal membrane oxygenation (ECMO) is not always easy to establish. Also, mechanical causes may obligate to disconnect the patient from the circuit before the optimal weaning off. In these selected cases, the patient can be disconnected from the circuit and the cannula can be left in place (stand-by cannula) until the patient's stability without ECMO is assured. The aim was to describe our experience with the stand-by cannula. Single-institution, long-term retrospective study in a pediatric tertiary care hospital. Neonatal and pediatric patients who were under ECMO and needed stand-by cannula before definitive de-cannulation were included. During 18years, 166 children required ECMO. In 31 patients (18.7%), stand-by cannula was performed before the weaning off. Twenty patients (64.5%) were newborn. The main reason for requiring ECMO in these newborn was persistent pulmonary hypertension. Eleven patients were pediatric and their main cause for requiring ECMO was cardiogenic shock (six patients, 54.4%). The reasons for requiring stand-by cannula were the uncertainty of a successful weaning off in 17 patients (54.8%), to undergo surgery in 10 patients (32.3%) and to replace the circuit in four cases (12.9%). The median duration of stand-by cannula was 12h (IQR 6-24). Heparinized saline serum was the main maintenance perfusion (28 patients, 90.3%). Three patients needed to restart support with ECMO. Only one mechanical complication was detected. Stand-by cannula is a safe technique, which allows performing a quick re-entrance on ECMO if the weaning off fails.