Indications and successes of intestinal transplantation in children in the 21st century: A retrospective cohort study.

AIMS: To report the results and successes of intestinal transplantation (ITx) in the most active European centres, to emphasize that, although it is a difficult procedure, it should remain a therapeutic option for children with total, definitive and complicated intestinal failure when intestinal rehabilitation fails. METHODS: We retrospectively collected data about all patients less than 18 receiving an ITx from 2010 to 2022 in 8 centres, and outcomes in July 2022. RESULTS: ITx was performed in 155 patients, median age 6.9 years, in 45% for short bowel syndromes, 22% congenital enteropathies, 25% motility disorders, and 15% re-transplantations. Indications were multiple in most patients, intestinal failure-associated liver disease in half. The graft was in 70% liver-containing. At last follow up 64% were alive, weaned from parenteral nutrition, for 7.9 years; 27% had died and the graft was removed in 8%, mostly early after ITx. DISCUSSION: ITx, despite its difficulties, can give a future to children with complicated intestinal failure. It should be considered among the therapeutic options offered to patients with a predicted survival rate lower than that after ITx. Patients should be early discussed within multidisciplinary teams in ITx centres, to avoid severe complications impacting the results of ITx, or even to avoid ITx.

Anesthetic Considerations for an Adult Patient with Freeman-Sheldon Syndrome Undergoing Open Heart Surgery.

Freeman-Sheldon syndrome (FSS) or "whistling face" syndrome is a rare congenital disorder complicated by characteristic facial deformities and muscular contractures. We report on a 64-year-old male patient presenting for surgical replacement of his aortic valve and review the available literature on anesthetic considerations and perioperative management principles. FSS frequently poses a significant challenge to airway management and gaining vascular access. Moreover, these patients are reportedly at risk for developing malignant hyperthermia (MH) or neuroleptic malignant syndrome.

Emergent transesophageal echocardiography in hemodynamically unstable obstetric patients.

BACKGROUND: The obstetric population has an increasing incidence of comorbid conditions. These, coupled with the possibility of acute embolic events involving air, amniotic fluid, and thrombus, increase the likelihood of hemodynamic instability. Although the utility of transesophageal echocardiography to guide management in cardiac and high-risk, non-cardiac surgical populations has been well established, the emergent use in critically-ill parturients has not been comprehensively evaluated. METHODS: Using our departmental transesophageal echocardiography database of 28 293 examinations, parturients were identified who underwent emergent transesophageal echocardiography for evaluation of hemodynamic instability, including cardiac arrest, between January 1999 and March 2014. Transesophageal echocardiography findings and their impact on patient management were analyzed. RESULTS: Ten peripartum patients were evaluated. Six patients became unstable during dilation and evacuation procedures; one after a forceps delivery; one during and one after cesarean delivery; and one during a postpartum laparotomy. Six patients proceeded to cardiac arrest; however, all women survived their initial operation and resuscitation. Transesophageal echocardiography was instrumental in determining the etiology and guiding resuscitation in all 10 patients including emergent cardiac surgical intervention with cardiopulmonary bypass (n=2). Seven patients survived to hospital discharge, but three died after experiencing neurologic complications. CONCLUSIONS: Severe hemodynamic instability and cardiac arrest can occur in previously healthy parturients in pregnancy. Our data suggest that emergent transesophageal echocardiography is a valuable tool in determining the etiology and directing therapy of refractory hypotension or cardiac arrest in obstetric patients.

Blood volume measurement by hemodilution: association with valve disease and re-evaluation of the Allen Formula.

BACKGROUND: Total blood volume (TBV) assessment is central to the management of cardiac surgical patients with cardiopulmonary bypass (CPB). The widely accepted Allen Formula lacks accuracy in estimating TBV in these patients. Moreover, the impact of commonly encountered cardiac disease states on TBV has not been systematically investigated. The aim of this study was to determine TBV by hemodilution (TBVHD) for patients with valve disease, compare TBVHD to algorithms frequently used during cardiac surgery and to modify the Allen Formula to better fit today's patient population. METHODS: TBVHD was prospectively measured upon initiation of CPB. Ninety-six patients were grouped into 4 cohorts by preoperative diagnosis and compared to Allen and weight-based formulae in a univariate analysis: mitral regurgitation (MR), coronary artery disease requiring bypass surgery (CABG) and aortic stenosis (AS) ± CABG. The independent effects of height and weight on TBV were correlated to the original Allen Formula by multiple linear regression. RESULTS: Patients with MR had significantly larger TBVHD compared to patients with AS, CABG or both. The smallest TBVHD was found in the patients with AS and CABG. The modified Allen Formula had an excellent model fit (R(2) = 0.88 and R(2) = 0.95 for males and females, respectively; p<0.001) while the classic formula overestimated TBV by 30% in males and females. For males, height impacted TBV calculations the most whereas weight was the predominant determinant in females. CONCLUSION: Blood volume assessment via the Allen Formula or bodyweight overestimated TBV in cardiac surgical patients, with potential implications on their management. The assumption that MR frequently presents with increased intravascular volume was confirmed whereas AS patients with coronary disease had a relatively smaller TBV. Lastly, a modified Allen Formula to better reflect today's patient population was derived to reproducibly improve accuracy in mathematical estimates of TBV.

Blood volumes in cardiac surgery with cardiopulmonary bypass.

PURPOSE: Total blood volume (TBV) estimation potentially impacts various aspects of cardiac surgical care, including pharmacological and transfusion interventions, hemodynamic and volume management and perfusion equipment selection. TBV is commonly computed during cardiopulmonary bypass (CPB), using standardized formulae. We hypothesized that these equations fail to accurately predict individual blood volume variability. The aim of this study was to determine TBV with a dilution technique and compare the results to commonly utilized TBV calculations. METHODS: After institutional review board approval, data was prospectively collected and analyzed for 101 patients undergoing open-heart surgery. Hematocrits (Hct) just prior to and immediately after the initiation of CPB were used to calculate the TBV. Results were compared to (1) the Allen formula and (2) weight-based standards (70 ml/kg for males (SM); 65 ml/kg for females (SF)). RESULTS: The average dilution TBV (male: 4684 ± 1641 ml; female: 3027 ± 1067 ml; total: 4175 ± 1617 ml) was significantly smaller (p<0.05) than TBV estimated by Allen's formula (male: 6328 ± 973 ml; female: 4167 ± 643 ml; total: 5665 ± 1134 ml) and weight-based standards (male: 6278 ± 1256 ml; female: 4924 ± 1064 ml; total: 5862 ± 1350 ml). Allen's formula and the weight-based standards correlated strongly (R(2) = 0.821, p<0.001), suggesting similar estimates of TBV when using these methods. In contrast, hemodilution correlated poorly with the estimates by Allen (R(2) = 0.221, p<0.001) and weight-based formulae (R(2) = 0.122, p<0.001), suggesting different TBV computation. CONCLUSIONS: The dilution method during CPB for TBV estimation is applicable and reproducible in the cardiac surgical arena and can be utilized to calculate TBV. Our results suggest that traditional TBV assessment in cardiac surgical patients by Allen's and weight-based formulae lacks the desired accuracy in estimating true TBV.

Aspirin-triggered resolvin D1 reduces mucosal inflammation and promotes resolution in a murine model of acute lung injury.

Acute lung injury (ALI) is a severe illness with excess mortality and no specific therapy. Protective actions were recently uncovered for docosahexaenoic acid-derived mediators, including D-series resolvins. Here, we used a murine self-limited model of hydrochloric acid-induced ALI to determine the effects of aspirin-triggered resolvin D1 (AT-RvD1; 7S,8R,17R-trihydroxy-4Z,9E,11E,13Z,15E,19Z-docosahexaenoic acid) on mucosal injury. RvD1 and its receptor ALX/FPR2 were identified in murine lung after ALI. AT-RvD1 (~0.5-5 µg kg(-1)) decreased peak inflammation, including bronchoalveolar lavage fluid (BALF) neutrophils by ~75%. Animals treated with AT-RvD1 had improved epithelial and endothelial barrier integrity and decreased airway resistance concomitant with increased BALF epinephrine levels. AT-RvD1 inhibited neutrophil-platelet heterotypic interactions by downregulating both P-selectin and its ligand CD24. AT-RvD1 also significantly decreased levels of BALF pro-inflammatory cytokines, including interleukin (IL)-1ß, IL-6, Kupffer cells, and tumor necrosis factor-α, and decreased nuclear factor-κB-phosphorylated p65 nuclear translocation. Taken together, these findings indicate that AT-RvD1 displays potent mucosal protection and promotes catabasis after ALI.

Zero balance ultrafiltration for the correction of acute acidosis after a period of prolonged deep hypothermic circulatory arrest.

A 36-year-old woman with normal renal function underwent a total arch replacement requiring 110 minutes of deep hypothermic circulatory arrest. Prior to rewarming, a pH of 7.063 with a base deficit of 10.8 was obtained. Zero-balance ultrafiltration (Z-BUF) was initiated during rewarming 7 minutes after resumption of cardiopulmonary bypass. After one hour (10L) of Z-BUF, all electrolyte and acid-base disturbances were fully corrected. Our case illustrates that Z-BUF offers a valuable option for the correction of severe electrolyte and acid-base disturbances in adults undergoing cardiac surgery. Its use should be considered in addition to the more standard pharmacologic approaches to such derangements as it offers rapid and predictable electrolyte and acid-base correction and potential anti-inflammatory benefits.

[Pro-resolving lipid mediators in inflammatory lung diseases]. / Die Rolle neuer "pro-resolving" Lipidmediatoren bei entzündlichen Lungenerkrankungen.

Uncontrolled inflammation of the lung contributes to the major medical and economic burden on healthcare, and the need for therapeutics to dampen pathological inflammation is largely unmet. Recently, a new genus of anti-inflammatory/ pro-resolving lipid mediators has been identified: Lipoxins, resolvins, protectins and maresins. These compounds are enzymatically derived from the polyunsaturated fatty acids (PUFAs) arachidonic acid (AA), docosahexaenoic acid (DHA), and eicosapentaenoic acid (EPA) that have long been known to have beneficial health properties. These mediators have potent anti-inflammatory effects IN VITRO and IN VIVO in murine models of lung inflammation. Therefore, this group of compounds carries considerable therapeutic potential for the treatment of many inflammatory lung diseases including asthma, cystic fibrosis and acute lung injury.

Lung injury following thoracic aortic occlusion: comparison of sevoflurane and propofol anaesthesia.

BACKGROUND: Halogenated anaesthetics have been shown to reduce ischaemia-reperfusion injuries in various organs due to pre- and post-conditioning mechanisms. We compared volatile and total intravenous anaesthesia with regard to their effect on remote pulmonary injury after thoracic aortic occlusion and reperfusion. METHODS: Eighteen pigs were randomized after sternotomy and laparotomy (fentanyl-midazolam anaesthesia) to receive either sevoflurane or propofol in an investigator-blinded fashion. Ninety minutes of thoracic aortic occlusion was induced by a balloon catheter. During reperfusion, a goal-directed resuscitation protocol was performed. After 120 min of reperfusion, the anaesthetic regimen was changed to fentanyl-midazolam again for another 180 min. The oxygenation index and intra-pulmonary shunt fractions were calculated. After 5 h of reperfusion, a bronchoalveolar lavage was performed. The total protein content and lactate dehydrogenase activity were measured in epithelial lining fluid (ELF). Alveolar macrophage oxidative burst was analysed. The wet to dry ratio was calculated and tissue injury was graded using a semi-quantitative score. Ten animals (n=5 for each anaesthetic) without aortic occlusion served as time controls. RESULTS: The oxygenation index decreased and the intra-pulmonary shunt fraction increased significantly in both occlusion groups. There were no significant differences between sevoflurane and propofol with respect to the oxygenation index, ELF composition, morphologic lung damage, wet to dry ratio and alveolar macrophage burst activity. Differences were, however, seen in terms of systemic haemodynamic stability, where catecholamine requirements were less pronounced with sevoflurane. CONCLUSION: We conclude that the severity of remote lung injury was not different between sevoflurane and propofol anaesthesia in this porcine model of severe lower-body ischaemia and reperfusion injury.

Effects of sevoflurane and propofol on ischaemia-reperfusion injury after thoracic-aortic occlusion in pigs.

BACKGROUND: Thoraco-abdominal-aneurysm surgery predicts high mortality. Propofol and sevoflurane are commonly used anaesthetics for this procedure. Halogenated anaesthetics induce organ protection similar to ischaemic preconditioning. We investigated which anaesthetic regimen would lead to a better protection against ischaemia-reperfusion injury induced by temporary thoracic-aortic occlusion. METHODS: Following initial fentanyl-midazolam anaesthesia for surgical preparation, 18 pigs were randomly assigned to two groups: group one received propofol (n=9) and group two sevoflurane (n=9) before, during, and after lower body ischaemia in an investigator blinded fashion. Ten animals without aortic occlusion served as time controls (propofol, n=5; sevoflurane, n=5). For induction of ischaemia, the thoracic aorta was occluded by a balloon-catheter for 90 min. After 120 min of reperfusion, the study anaesthetics were discontinued and fentanyl-midazolam re-established for an additional 180 min. Goal-directed therapy was performed during reperfusion. Fluid and catecholamine requirements were assessed. Serum samples and intestinal tissue specimens were obtained. RESULTS: Severe declamping shock occurred in both study groups. While norepinephrine requirements in the sevoflurane group were significantly reduced during reperfusion (P<0.05), allowing cessation of catecholamine support in 4/9 animals, all 9/9 animals were still catecholamine dependent at the end of the experiment in the propofol group. Serum activities of lactate dehydrogenase, aspartate transaminase, and alanine aminotransferase were lower with sevoflurane (P<0.05). Small intestine tissue specimens did not differ histologically. CONCLUSIONS: Use of sevoflurane compared with propofol attenuated the haemodynamic sequelae of reperfusion injury in our model. Release of serum markers of cellular injury was also attenuated.