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The patient's brother had died at the same age, from pulmonary veno-occlusive disease associated with hypertrophic cardiomyopathy. 1 year previously he had been diagnosed with end-stage kidney disease of unknown origin; he had had no history of cognitive impairment. The most frequent and severe presentation occurs in neonates.1,2 Pulmonary artery hypertension (PAH) and renal TMA have been described in early-onset cblC deficiency, whereas isolated neuropsychiatric symptoms prevail in adult-onset forms.1,2 A case of adult-onset renal TMA had similar renal outcomes to our patient.3 The family history and coexistence of a specific glomerulopathy in our patient suggest that PAH, renal TMA, and the glomerular lesions are consequences of the variants in MMACHC.

Acute exacerbation of interstitial lung disease (AE-ILD) may lead to acute respiratory failure with poor prognosis. Little is known about the optimal oxygenation strategy in this indication, in particular since the development of high-flow nasal oxygen therapy (HFNO). Therefore, we conducted a nationwide survey among respiratory and intensive care unit (ICU) physicians to assess their real-life practices. Between January and June 2024, physicians were questioned, using an online survey (11 questions), regarding the non-invasive oxygenation strategy they used in the management of patients with AE-ILD: face mask (FM), HFNO, continuous positive airway pressure (CPAP) or non-invasive ventilation (NIV). The response rate was 119/270 (44.1%), including 59.5% of ICU and 40.5% of respiratory physicians.=

Increasing numbers of studies in chronic diseases have been published showing the relationship between body composition (BC) parameters (i.e. skeletal muscle mass (SMM) and adipose tissue (AT)) and outcomes. For patients admitted to intensive care unit (ICU), BC parameters have rarely been described as a prognostic marker of outcome. The primary objective was to evaluate the relationship between body composition at ICU admission and major clinical outcomes. Secondary objectives were to assess the relationship between BC parameters and other parameters (systemic inflammatory markers, Sequential Organ Failure Assessment (SOFA) score, albumin level) at ICU admission, and between BC alterations during ICU stay and outcomes. This retrospective study enrolled 25 adult patients who had two abdominal CT scans for clinical indication: first, within 48 hours of ICU admission (initial assessment), and second, 7 to 14 days later (late assessment). Skeletal Muscle radiodensity (SMD), cross-sectional area of SMM, Visceral Adipose Tissue (VAT) and Subcutaneous Adipose Tissue (SAT) were measured at the third lumbar vertebra. Cox regression analysis was used to determine the association between these parameters and mortality. Patients' mean age was 64.6 years. Their mean BMI was 27.7 kg/m2 (SD = 6.0). ICU mortality was 36%. There was no correlation between BC parameters at initial assessment and ICU outcomes. We observed a negative correlation between SMM index and SOFA score at initial assessment (r = -0.458, p = 0.037). There was a significant loss of VAT between two CT assessments which was associated with mortality (-22.34cm2 / m2 in non-survivors versus -6.22 cm2 / m2 in survivors, p = 0.039). Loss of SMD was greater with the occurrence of an infection than without (Delta SMD = -5.642 vs +1.957, p = 0.04). Our results show alterations in body composition during ICU stay with a loss of muscle quality (decreased SMD) and adipose tissue. These findings require confirmation in future studies but already show that BC assessments at ICU admission and BC alterations during ICU stay are important factors for outcome in critically ill patients.

Background The ventilatory physiopathology of patients with interstitial lung disease (ILD) remains poorly understood. We aimed to personalize a mechanical simulator to model healthy and ILD profiles ventilation, and to evaluate the effect of spontaneous breathing on respiratory mechanics at rest and during exercise. Methods In a 2-compartment lung simulator (ASL 5000 ® ), we modeled 1 healthy and 3 ILD profiles, at rest and during exercise, based on physiological data from literature and patients. Measurements were: tidal volume, end-expiratory lung volume, driving pressure, transpulmonary driving pressure, dynamic alveolar strain, mechanical power, and time lag of inspiratory flow between compartments 1 and 2. Results Healthy and ILD models were validated: maximum differences between real and simulated tidal volume were 5% (96 ml) and 6% (54 ml) at rest and during exercise respectively, considered clinically negligible. When we simulated lung inhomogeneity (compliance in compartment 1 > compartment 2), tidal volume, end-expiratory lung volume, driving pressure and mechanical power increased in compartment 1 and decreased in compartment 2. Driving transpulmonary pressure and dynamic alveolar strain increased in compartment 2 and decreased in compartment 1. Time lag of inspiratory flow between compartments 1 and 2 was positively correlated with a difference of compliance between compartments ( r  = 0.98, CI95% (0.9106; 0.9962), p  < 0.0001). Conclusion In this bench study, we personalized a mechanical simulator thatmodels the lung inhomogeneity and spontaneous breathing of healthy subjects and ILD patients at rest and during exercise. Our results suggest that lung inhomogeneity could increase lung vulnerability to volo-atelec-trauma mechanisms in ILD. Further physiological studies are needed to evaluate the impact of this vulnerability on acute or chronic ILD worsening.

IntroductionA wide range of recent ventilators, dedicated or not, is available for non-invasive ventilation (NIV) in respiratory or intensive care units (ICU). We conducted a bench study to compare their technical performances.MethodsVentilators, including five ICU ventilators with NIV mode on, two dedicated NIV ventilators and one transport ventilator, were evaluated on a test bench for NIV, consisting of a 3D manikin head connected to an ASL 5000 lung model via a non-vented mask. Ventilators were tested according to three simulated lung profiles (normal, obstructive, restrictive), three levels of simulated air leakage (0, 15, 30 L/min), two levels of pressure support (8, 14 cmH2O) and two respiratory rates (15, 25 cycles/min).ResultsThe global median Asynchrony Index (AI) was higher with ICU ventilators than with dedicated NIV ventilators (4% (0; 76) vs 0% (0; 15), respectively; p<0.05) and different between all ventilators (p<0.001). The AI was higher with ICU ventilators for the normal and restrictive profiles (p<0.01) and not different between ventilators for the obstructive profile. Auto-triggering represented 43% of all patient-ventilator asynchrony. Triggering delay, cycling delay, inspiratory pressure-time product, pressure rise time and pressure at mask were different between all ventilators (p<0.01). Dedicated NIV ventilators induced a lower pressure-time product than ICU and transport ventilators (p<0.01). There was no difference between ventilators for minute ventilation and peak flow.ConclusionDespite the integration of NIV algorithms, most recent ICU ventilators appear to be less efficient than dedicated NIV ventilators. Technical performances could change, however, according to the underlying respiratory disease and the level of air leakage.

CD19-specific chimeric antigen receptor T (CAR-T) cell therapy has recently been shown to improve the prognosis of refractory diffuse large B-cell lymphoma (DLBCL). However, CAR-T cells may induce numerous adverse events, in particular cytokine release syndrome (CRS) which is frequently associated with cardiovascular manifestations. Among the latter, acute pericardial effusion represents less than 1% of cases and cardiac tamponade has only been reported once. The management and outcome of these severe complications are not well established. We report here, a case of cardiac tamponade associated with CRS in a context of CAR-T cell therapy, which required urgent pericardiocentesis.

Severity of hypoxaemia can be assessed using the partial pressure of arterial oxygen to fraction of inspired oxygen ratio (FiO2). However, in patients breathing through non-rebreather reservoir bag oxygen mask, accuracy of bedside FiO2 estimation methods remains to be tested. In a post-hoc analysis of a multicentre clinical trial, three FiO2 estimation methods were compared with FiO2 measured with a portable oxygen analyser introduced in the oxygen mask. Among 262 patients analysed, mean (SD) measured FiO2 was 65% (13). The 3%-formula (21% + oxygen flow rate in L/min × 3) was the most accurate method to estimate FiO2. Other methods overestimated FiO2 and hypoxaemia severity, so they should be avoided.

BACKGROUND: Whether noninvasive ventilation should be administered in patients with acute hypoxemic respiratory failure is debated. Therapy with high-flow oxygen through a nasal cannula may offer an alternative in patients with hypoxemia. METHODS: We performed a multicenter, open-label trial in which we randomly assigned patients without hypercapnia who had acute hypoxemic respiratory failure and a ratio of the partial pressure of arterial oxygen to the fraction of inspired oxygen of 300 mm Hg or less to high-flow oxygen therapy, standard oxygen therapy delivered through a face mask, or noninvasive positive-pressure ventilation. The primary outcome was the proportion of patients intubated at day 28; secondary outcomes included all-cause mortality in the intensive care unit and at 90 days and the number of ventilator-free days at day 28. RESULTS: A total of 310 patients were included in the analyses. The intubation rate (primary outcome) was 38% (40 of 106 patients) in the high-flow-oxygen group, 47% (44 of 94) in the standard group, and 50% (55 of 110) in the noninvasive-ventilation group (P=0.18 for all comparisons). The number of ventilator-free days at day 28 was significantly higher in the high-flow-oxygen group (24+/-8 days, vs. 22+/-10 in the standard-oxygen group and 19+/-12 in the noninvasive-ventilation group; P=0.02 for all comparisons). The hazard ratio for death at 90 days was 2.01 (95% confidence interval [CI], 1.01 to 3.99) with standard oxygen versus high-flow oxygen (P=0.046) and 2.50 (95% CI, 1.31 to 4.78) with noninvasive ventilation versus high-flow oxygen (P=0.006). CONCLUSIONS: In patients with nonhypercapnic acute hypoxemic respiratory failure, treatment with high-flow oxygen, standard oxygen, or noninvasive ventilation did not result in significantly different intubation rates. There was a significant difference in favor of high-flow oxygen in 90-day mortality. (Funded by the Programme Hospitalier de Recherche Clinique Interregional 2010 of the French Ministry of Health; FLORALI ClinicalTrials.gov number, NCT01320384.).

In this narrative review, we discuss the relevant issues of therapeutic plasma exchange (TPE) in critically ill patients. For many conditions, the optimal indication, device type, frequency, duration, type of replacement fluid and criteria for stopping TPE are uncertain. TPE is a potentially lifesaving but also invasive procedure with risk of adverse events and complications and requires close monitoring by experienced teams. In the intensive care unit (ICU), the indications for TPE can be divided into (1) absolute, well-established, and evidence-based, for which TPE is recognized as first-line therapy, (2) relative, for which TPE is a recognized second-line treatment (alone or combined) and (3) rescue therapy, where TPE is used with a limited or theoretical evidence base. New indications are emerging and ongoing knowledge gaps, notably regarding the use of TPE during critical illness, support the establishment of a TPE registry dedicated to intensive care medicine.

Background In immunocompromised patients with acute hypoxemic respiratory failure (ARF), initial management aims primarily to avoid invasive mechanical ventilation (IMV). Methods To assess the impact of initial management on IMV and mortality rates, we performed a multinational observational prospective cohort study in 16 countries (68 centers). Results A total of 1611 patients were enrolled (hematological malignancies 51.9%, solid tumors 35.2%, systemic diseases 17.3%, and solid organ transplantation 8.8%). The main ARF etiologies were bacterial (29.5%), viral (15.4%), and fungal infections (14.7%), or undetermined (13.2%). On admission, 915 (56.8%) patients were not intubated. They received standard oxygen ( N  = 496, 53.9%), high-flow oxygen (HFNC, N  = 187, 20.3%), noninvasive ventilation (NIV, N  = 153, 17.2%), and NIV + HFNC ( N  = 79, 8.6%). Factors associated with IMV included age (hazard ratio = 0.92/year, 95% CI 0.86–0.99), day-1 SOFA (1.09/point, 1.06–1.13), day-1 PaO 2 /FiO 2 (1.47, 1.05–2.07), ARF etiology ( Pneumocystis jirovecii pneumonia (2.11, 1.42–3.14), invasive pulmonary aspergillosis (1.85, 1.21–2.85), and undetermined cause (1.46, 1.09–1.98). After propensity score matching, HFNC, but not NIV, had an effect on IMV rate (HR = 0.77, 95% CI 0.59–1.00, p  = 0.05). ICU, hospital, and day-90 mortality rates were 32.4, 44.1, and 56.4%, respectively. Factors independently associated with hospital mortality included age (odds ratio = 1.18/year, 1.09–1.27), direct admission to the ICU (0.69, 0.54–0.87), day-1 SOFA excluding respiratory score (1.12/point, 1.08–1.16), PaO 2 /FiO 2  < 100 (1.60, 1.03–2.48), and undetermined ARF etiology (1.43, 1.04–1.97). Initial oxygenation strategy did not affect mortality; however, IMV was associated with mortality, the odds ratio depending on IMV conditions: NIV + HFNC failure (2.31, 1.09–4.91), first-line IMV (2.55, 1.94–3.29), NIV failure (3.65, 2.05–6.53), standard oxygen failure (4.16, 2.91–5.93), and HFNC failure (5.54, 3.27–9.38). Conclusion HFNC has an effect on intubation but not on mortality rates. Failure to identify ARF etiology is associated with higher rates of both intubation and mortality. This suggests that in addition to selecting the appropriate oxygenation device, clinicians should strive to identify the etiology of ARF.