Clinical and Immunologic Features of a Patient With Homozygous FNIP1 Variant.

Agammaglobulinemia represents the most profound primary antibody deficiency, stemming from early cessation of B-cell development. Deficiency in folliculin-interacting protein 1 (FNIP1) is a novel inborn error of immunity characterized by a severe defect in B-cell development, agammaglobulinemia, variable neutropenia, and hypertrophic cardiomyopathy. FNIP1 plays a critical role in B-cell development and metabolic homeostasis, establishing a metabolic checkpoint that ensures pre-B cells possess sufficient metabolic capacity to undergo division while concurrently limiting lymphogenesis due to abnormal growth. Disruption of FNIP1 functionality affects the fundamental metabolic regulators adenosine monophosphate-activated protein kinase and mTOR, culminating in a severe B-cell deficiency alongside hypogammaglobulinemia, hypertrophic cardiomyopathy, preexcitation syndrome, and intermittent neutropenia. This case report presents an 11-month-old male patient with FNIP1 deficiency who, in addition to classical features, exhibited posterior cerebellar hypoplasia.

A new hematologic predictor of major adverse events after cardiac surgery: red cell distribution width to lymphocyte ratio.

BACKGROUND: The perioperative risk factors that cause severe morbidity and prolongation of postoperative hospital stay after cardiac surgery should be determined. Various scores have been used to predict morbidity and mortality. Preoperative blood counts are considered potential biomarkers of inflammation and oxidative stress. Inflammatory and immune imbalances may have a significant impact on postoperative adverse events. The present study aimed to investigate the association and potential predictive properties of red cell distribution width/ lymphocyte ratio (RLR) for major adverse events in adult patients who underwent coronary surgery with cardiopulmonary bypass. METHODS: After approval from the ethics committee, pre- and post-operative data of 700 patients were obtained from the electronic database of the hospital, intra- and post-operative anesthesia, and intensive care unit follow-up charts. We performed a stepwise multiple logistic regression analysis to investigate the association of RLR with major adverse events in adult patients who underwent coronary surgery with cardiopulmonary bypass. RESULTS: Among 700 patients, 47 (6.7%) had major adverse events after surgery. Multivariate logistic regression analysis showed that age (odds ratio [OR], 1.08; 95% confidence interval [CI], 1.03-1.12; P < 0.001), mean platelet volume (OR, 1.49; 95% CI, 1.07-2.06; P = 0.017), and RLR (OR, 1.21; 95% CI, 1.02-1.43; P = 0.026) were significantly associated with major adverse events. CONCLUSIONS: RLR indicates the balance between inflammatory and immune responses. Therefore, it can be used to predict adverse events following coronary surgery.

Preoperative ultrasound-guided bilateral thoracic erector spinae plane block within an enhanced recovery program is associated with decreased intraoperative lactate levels in cardiac surgery.

INTRODUCTION: In the perioperative period, regional analgesia techniques may play an increasingly important role in "Enhanced Recovery After Surgery (ERAS)" programs, as they can facilitate recovery. We hypothesized that Erector Spinae Plane (ESP) block could improve regional perfusion, thereby limiting blood lactate increase. Therefore, we aimed to evaluate the effect of ESP block on intraoperative blood lactate levels in patients scheduled for elective on-pump cardiac surgery with ERAS protocol. METHODS: A total of 68 adult patients scheduled for on-pump cardiac surgery were included. All patients were randomized to the ESP group and the non-ESP group. Blood lactate analyses were performed at intraoperative five-time points. C-Reactive protein (CRP) values were also measured. RESULTS: Blood lactate values were significantly lower in the ESP group than in the Non-ESP group, at the end of CPB [1.78 (1.23-2.78) mmol L-1 to 2.63 (1.70-3.12) mmol L-1] and during the sternal closure period [1.78 (1.27-2.42) mmol L-1 to 2.40 (2.14-2.80) mmol L-1] (p = 0.039, p = 0.009). In addition, CRP values were significantly lower in the ESP group in the postoperative period [0.048 (0.036-0.105) g L-1 to 0.090 (0.049-0.154) g L-1] (p = 0.035). CONCLUSIONS: This study showed that preoperative bilateral single-shot ESP block significantly reduces intraoperative final blood lactate and postoperative CRP values. We consider that these results are related to the attenuation of intraoperative hypoperfusion and the alleviation of surgery-related postoperative inflammation. ERAS programs aim to achieve the rapid recovery of patients, a decrease in inflammation, and high-quality analgesia with less opioid consumption. Therefore, our results also prove that it is easier to reach the primary goals of ERAS programs with the application of ESP block in cardiac surgery.

Preoperative red cell distribution width to lymphocyte ratio as biomarkers for prolonged intensive care unit stay among older patients undergoing cardiac surgery: a retrospective longitudinal study.

Introduction: Our aim was to use the red cell distribution width-lymphocyte ratio (RLR) as a novel biomarker to predict prolonged intensive care unit (ICU) length of stay (LOS) among older patients undergoing cardiovascular surgery. Methods: This longitudinal study included older patients admitted to a tertiary cardiovascular surgery hospital between January 2017 and January 2022. Results: A total of 574 patients were studied, including 83 patients (14.5%) who had prolonged ICU LOS and 471 (85.5%) control subjects. After adjustment for the European System for Cardiac Operative Risk Evaluation 2, the RLR score showed a 10% increased risk of prolonged ICU LOS (odds ratio: 1.10; CI: 1.05-1.16; p = 0.01). Conclusion: Preoperative RLR can be used to predict the risk of long-term intensive care stay in older cardiac surgery patients.

Low-Dose Heparin Protocol in Type A Aortic Dissection Surgeries.

OBJECTIVE: We aim to compare the heparin dose regimen in terms of bleeding, reoperation rate due to severe bleeding, and the amount of transfusion of the blood products in patients who underwent surgery for type A aortic dissection (TAAD). MATERIALS AND METHODS: Between January 2018 and August 2021, 90 adult patients who underwent for TAAD were included. Primary outcome measures were postoperative bleeding amount and blood product transfusion requirements. Two different protocols performed in TAAD surgery in our clinic. In this pre- and postimplementation study, before October 2019, the standard-dose heparin protocol (SH group) was used and after November 2019, the low-dose heparin protocol (LH group) was used and two groups were compared. Mechanical ventilation duration, length of intensive care unit and hospital stay, postoperative drainage volumes, blood product transfusions, reoperations due to bleeding, and in-hospital mortality rates were recorded. RESULTS: The dosages of heparin and activated clotting time values, as well as the additional heparin requirement, were significantly different between the two groups (p < 0.001). Standard-dose heparinization was needed only in 33.3% of patients in the LH group. In the SH group, postoperative total drainage and red blood cell (RBC) transfusion were significantly higher than the LH group (p = 0.036 and p = 0.046, respectively). CONCLUSION: We found that the low-dose heparin regimen resulted in significantly less postoperative total drainage and RBC transfusion requirement in patients who underwent for TAAD.

Evaluation of supply sustainability of vaccine alternatives with multi-criteria decision-making methods.

BACKGROUND: The treatment of the COVID-19 epidemic, whose contagious features are changing day by day, is the most current problem today throughout of the world. In order to be protected from COVID-19 and reduce its spread, it is of great importance to follow the rules such as mask, distance and hygiene. In addition, one of the most important ways to prevent the epidemic is to develop population immunity. The most important tool in having population immunity is vaccination. AIMS: During the COVID-19 pandemic, there have been problems in the supply process of many products in food and health sectors. Vaccine is also one of the most difficult tools to supply. In this context, the study focused on the selection of the vaccine provided by the countries within the scope of population vaccination studies. MATERIALS & METHODS: At the selection point, the criteria affecting the purchasing process were determined and the weights of these criteria were calculated using the AHP method. Then, the criteria weights obtained were used to rank the alternatives in an integrated manner in the Preference Ranking Organization Method for Enrichment of Evaluations (PROMETHEE) and Technique for Order Preference by Similarity to Ideal Solution (TOPSIS) methods. The results of both methods were analyzed comparatively. RESULTS: According to the TOPSIS Method, the first alternative is Oxford-AstraZeneca for all countries, and BioNTech for all countries in the PROMETHEE method. DISCUSSION: The vaccine storage conditions criterion is the most important in vaccine supply. The criterion with the lowest importance is Supply Cost. It has been revealed that cost elements remain in the background under pandemic conditions. CONCLUSION: Vaccine evaluation studies and policy recommendations are presented by considering public health in the selection of vaccine alternatives.

A pooled RT-PCR testing strategy for more efficient COVID-19 pandemic management.

OBJECTIVES: Reverse transcription polymerase chain reaction (RT-PCR) testing is indispensable in management of the coronavirus disease 2019 (COVID-19) pandemic. However, with the emergence of new variants of severe acute respiratory syndrome coronavirus-2, the cause of COVID-19, the screening capacity of RT-PCR testing is overburdened, and new strategies and capabilities need to be established. One option is pooled RT-PCR testing. DESIGN: This study used various mixtures of COVID-19 samples known to be negative and positive, and investigated the impact of pool size and mixture level on final cycle threshold (Ct) values. More specifically, 5, 10 and 20 negative samples were combined with one, two or three low Ct or high Ct positive samples. RESULTS: Average baseline Ct and numbers of high and low Ct samples in the pool were found to be the main drivers of the final Ct value, making detectability easier. Pool size was not significantly associated with final Ct, but was suggestive. CONCLUSIONS: A pooled RT-PCR testing strategy does not reduce the sensitivity of RT-PCR, and thus provides a practical way to expand RT-PCR screening capacity in pandemic management. The pool size was not found to be significant, so it is recommended that a pool size of 20 would be a practical number to reduce the time taken to obtain the results and the cost of RT-PCR testing.

Retrograde autologous priming method reduces plasma free hemoglobin level in aortic surgery.

Background: Although conventional cardiopulmonary bypass (cCPB) is still the most widely used method in open heart surgery, methods such as retrograde autologous priming (RAP) are increasingly popular in terms of limiting hemodilution. Our hypothesis is that the use of the RAP method in aortic surgery may result in a limitation of hemodilution and a decrease in fHb levels. For this purpose, plasma free hemoglobin (fHb) levels were investigated in adult open aortic arch repair with axillary artery cannulation patients using cCPB and rRAP methods. Materials and Methods: In this study, a total of 36 patients undergoing aortic surgery using rRAP and standard cCPB were investigated. Measurements were performed at five time points: After induction of anesthesia, 5th minute of CPB, 10th minute of antegrade cerebral perfusion, 30th minute after declamping of aorta, and at sternum closure. Besides hemodynamic variables, arterial blood gas analysis and postoperative variables, patients were assessed for fHb levels. Results: The rRAP group had a significantly lower increase in fHb levels in T3, T4, and T5 time points, when compared to the cCPB group (p = 0.002, 0.047, 0.009, respectively). There was no significant difference between the rRAP and cCPB groups in other intraoperative, and postoperative variables. Also, it was observed that rRAP did not make a difference in terms of blood and blood product transfusion. Conclusion: In this study, in patients undergoing aortic surgery, a reduction in the increase of fHb was observed with the rRAP method which is a simple procedure that does not require high cost or advanced technology.

All in one: Multiple coronary stents, history of stent thrombosis, pancreatic carcinoma, postoperative bleeding and thrombocytosis.

Although the management of a stent patient may appear in the guidelines, some patients may be unique and out of class. In this presentation, the patient had multiple thrombotic risk factors for perioperative myocardial injury. These factors included coronary artery disease with multiple implantations of drug-eluting stent (DES), stent thrombosis history, implantation of a new stent 11months ago and hypercoagulability (due to malignancy and surgical procedure). The patient's history of DES presented a dilemma for the anesthesiology, surgery, and cardiology teams in considering the optimal method to minimize the risk of perioperative bleeding and stent re-thrombosis.

Building a patient blood management program in a large-volume tertiary hospital setting: Problems and solutions.

Successful implementation of a patient blood management program necessitates the collaboration of a strong organization and a multidisciplinary approach. We organized a meeting with broad participation in our center to establish a consensus for implementation of a specific patient blood management program. International and domestic experiences were shared, the importance of coordination and execution of different pillars in patient blood management were discussed, and the problems about the blood transfusion system were also investigated with the proposal for solutions. The data obtained from this meeting are presented to be a guide for similar large-volume tertiary hospitals for integration of a patient blood management protocol.

Diagnosed in operating room with hypertension: a widespread but unseen affliction.

OBJECTIVE: The present study aims to describe a widely held misconception in the literature concerning preoperative hypertension diagnosis. The blood pressure elevation occurring in the operative room is seen commonly even in subjects considered hitherto fully normotensive. As these patients have a condition which - similar to White Coat Hypertension (WCH) - indicates the presence of hypertension, and thus necessitates more frequent intraoperative checks. METHODS: We have named a condition 'Diagnosed in Operating Room (DIOR) with Hypertension', following the preoperative stage at which it is detected. RESULT: Our observational study evaluated 718 elective noncardiac surgery adult patients, finding 28% of them (n = 204) to be 'DIOR-tensive' and thus at risk for suboptimal intraoperative care. CONCLUSION: In addition to recommending a modification to the domain's best practices, we provide a preliminary description of DIOR hypertension patient identifying characteristics (older, higher body weight and BMI, and higher rates of chronic obstructive pulmonary disease, hypothyroidism and obesity), so that DIOR hypertension patients may be more readily identified, and that future research may build on the findings, and that the operating team may remain generally aware that this problem can occur and be dealt with regardless of the patient's medical history.

Does uncontrolled diabetes mellitus affect cerebral hemodynamics in heart surgery?

BACKGROUND: In this study, we aimed to investigate the effects of poor blood glucose control on the intraoperative cerebral system in patients undergoing coronary artery bypass grafting using various neuromonitors. METHODS: Between January 2011 and December 2011, a total of 40 adult patients (31 males, 9 females; mean age 58.8±9.2 years; range, 38 to 78 years) who were scheduled for elective coronary artery bypass grafting were included in the study. The patients were divided into four groups according to hemoglobin A1c levels as follows: Group 1 including non-diabetic controls (n=11); Group 2 including those with a hemoglobin A1c value of <7% (n=10); Group 3 including those with a hemoglobin A1c value of 7 to 10% (n=11); and Group 4 including those with a hemoglobin A1c value of ≥10% (n=8). Cerebral monitoring was performed with near-infrared spectroscopy and transcranial Doppler. Measurement periods were defined as follows: Before anesthesia induction (period 1), 10 min after anesthesia induction (period 2), during cannulation (period 3), 10 min after cardiopulmonary bypass (period 4), at 32°C temperature during cardiopulmonary bypass (period 5), at 36°C temperature during cardiopulmonary bypass (period 6), and at the end of the operation (period 7). RESULTS: There was a significant difference in the near-infrared spectroscopy values in the cannulation period for both right (p<0.001) and left (p=0.002) sides and the mean transcranial Doppler flow velocity (p=0.002) in Group 4, compared to Group 1. The heart rate was found to be significantly lower in Group 4 in the cannulation period. The near-infrared spectroscopy values and transcranial Doppler blood flow velocity decreased in Group 4 in all measurement periods. CONCLUSION: The results of our study show that, in patients with severe diabetes undergoing open heart surgery, heart rate decreases in the cannulation period due to possible autonomic neuropathy, and cerebral blood flow and oxygenation decrease. For these patients, particularly in the cannulation period, perfusion of both cerebral and other organs should be closely monitored and necessary interventions should be performed.

Anaesthesia Management in Idiopathic Pulmonary Artery Aneurysm Surgery.

Pulmonary artery (PA) aneurysm is a very rare disease. Surgical treatment is important in symptomatic patients. Due to its anatomical condition, the rupture of such aneurysms may lead to sudden right ventricular failure or sudden death. The aim of this report is to present the use of balanced general anaesthesia management in a patient who underwent PA aneurysm repair surgery. Surgical repair was planned in a 55-year-old man with chest and back pain as well as haemoptysis. With the help of balanced anaesthesia, stress on the aneurysm wall was prevented by avoiding sudden blood pressure increases. With appropriate ventilation methods, we attempted to avoid an increase in pulmonary vascular resistance, and therefore, the pressure on the aneurysm, as well as an increase in the postoperative right ventricle. With the help of appropriate anaesthesia and ventilation techniques, uncomplicated and successful anaesthetic management was effected in the repair of a PA aneurysm.

Comparison of new generation baska mask with i-gel and classical laryngeal mask in outpatient urological interventions.

OBJECTIVES: To compare the clinical performance of the baska mask (PTY Ltd, Australia), i-gel (Intersurgical Ltd, UK) and classic laryngeal mask airway (cLMA) in adult patients undergoing outpatient urologic interventions. METHODS: One hundred fifty patients with American Society of Anesthesiologists I-III physical status were enrolled between January 2017 and September 2017 in Yuksek Ihtisas Research and Educational Hospital, Ankara, Turkey for elective urological surgery for this prospective randomized controlled trial. There were 50 patients in each of the following groups: baska mask, i-gel, and cLMA. In each group, the insertion times, ventilation times, 'first attempt' success rates, airway dynamics-complications and hemodynamic variables were evaluated. RESULTS: No statistically significant values were observed in means of demographic data, airway dynamics, complications, and hemodynamic variables. Insertion and ventilation times were different between groups (p less than 0.001 for each). In cLMA group, insertion and ventilation times were found to be shorter than others (insertion times 5.78±1.72 seconds and ventilation times 11.72±4.72 seconds). The longest insertion and ventilation times were observed in baska mask with 12.04±6.25 and 21.26±8.53 seconds. The 'first attempt' success rates were 98% for cLMA, 92% for i-gel, and 88% for baska mask. The addition maneuvering requirements in baska mask group was  20% (40/10). Conclusion: When cLMA, i-gel and baska mask are compared regarding insertion and ventilating times, first attempt success rates, and additional maneuvers, cLMA and i-gel are superior to baska mask in urological ambulatory surgical cases.

Visceral oxidative stress during antegrade cerebral perfusion and lower body circulatory arrest.

Background: Antegrade cerebral perfusion (ACP) is the standard neuroprotection method in aortic surgery. Visceral ischemia during this modality brings out some controversies. We aimed to investigate the level of oxidative stress at the lower part of body during ACP. Methods: Thirty consecutive patients underwent elective ascending aorta and hemiarch repair with ACP (without distal perfusion) were enrolled to study. The patients were enrolled into two groups which were based on 50th percentile of ACP duration (15 patients in each group). Blood samples from inferior vena cava at the end of ACP were collected to assess oxidative stress with biochemical parameters such as lactate, advanced oxidative protein products (AOPP) and thiol levels. Clinical follow-up parameters regarding to visceral and spinal cord ischemia were recorded. There were no clinical complications at both groups. Results: Mean ACP duration for the study group was found to be 15 min (10-28 min). Lactate, AOPP, and thiol levels were found to be similar between two groups. Furthermore, correlation analysis revealed only low level of correlation between ACP duration and lactate levels. Renal and liver function tests were found to be similar between groups. Conclusions: Immediate parameters (such as lactate, AOPP, and thiol) that show alterations in response to oxidative stress were not affected by the duration of ACP. Therefore, ACP without distal perfusion may not be harmful when conducted for short duration.

AuraGain and i-Gel laryngeal masks in general anesthesia for laparoscopic cholecystectomy. Performance characteristics and effects on hemodynamics.

OBJECTIVES:   To evaluate and compare the performances of new types of supraglottic airway devices (SADs) with endotracheal intubation regarding their ease of insertions, perioperative complications, and effects on hemodynamic parameters and peak airway pressures in laparascopic cholecystectomy (LC).  Methods: One hundred and fourteen patients with ASA 1-2 physical status scheduled for elective LC were recruited for this prospective randomized controlled trial. The study was completed between January  2016 and January 2017 in Adiyaman University Research and Educational Hospital, Adiyaman, Turkey. The patients were divided into AuraGain(Ambu, Ballerup, Denmark) (n=38), i-Gel® (Intersurgical Ltd, UK) (n=35), and endotracheal tube (ETT)(n=32) groups. Ease of insertion, airway pressures, complications, and hemodynamic variables were compared. Results: The trial was completed with 105 patients. Ease of insertion for SADs which was evaluated with insertion procedure duration, attempts, first insertion success rates, and oropharyngeal leak pressures were similar between the groups. Heart rate, systolic and diastolic arterial pressures, and peak airway pressures were significantly lower in the AuraGain and i-Gel® groups, compared with the ETT, p less than 0.017. Conclusion: Both AuraGain and i-Gel® SADs are comparable with ETT used for airway control in general anesthesia for LC, regarding application ease and perioperative complications. Favorable hemodynamic responses to AuraGain and i-Gel® SADs may put them in a better place than ETT.

Can Pre-Operative HbA1c Values in Coronary Surgery be a Predictor of Mortality?

OBJECTIVE: One of the most frequently studied parameters in terms of outcome estimation in cardiac surgery is HbA1c. Several studies in literature suggest that high HbA1c value increases mortality and morbidity, but there is no relation between them. The primary aim of the present study is to investigate whether HbA1c value in diabetic patients undergoing coronary bypass graft surgery is an independent predictor for post-operative mortality and morbidity, and our secondary goal was to determine independent risk factors that cause mortality and morbidity in the same patient population. METHODS: 380 diabetic patients diagnosed with diabetes who underwent coronary surgery with cardiopulmonary bypass in Mersin State hospital between July 2014 to December 2016 after the approval of the Mersin University Faculty of medicine ethics committee were included in this retrospective, observational, and cross-sectional study. Patient demographic and perioperative information were obtained from the electronic information operating system and from anesthesia-intensive care follow-up forms. The HbA1c threshold was accepted as 7%, which was reported to be more appropriate for evaluating high-risk groups. RESULTS: Three hundred and fifty-four patients with complete access to the data were included in the study. The mean age of the patients was 60.8±9.4 years. 37% of the patients (131 patients) were female. The number of patients with HbA1c≥7 was 194 (54,8%) in the entire patient population. In this study, high HbA1c (≥7) values in diabetic patients undergoing isolated coronary bypass graft surgery were not found to be independent predictors of post-operative mortality and morbidity. Mortality was seen in 28 patients (7.9%). Ejection fraction (EF) was found to be an independent predictor factor for pre-operative factors in logistic regression models constructed according to mortality predictors (OR:0.94; 95% CI: 0.90-0.99; p=0.016). Complications were seen in 50 patients (14.1%). In the models formed from the point of view of the complication predicators, only EF was found to be independent predictor (OR:0.95; 95% CI: 0.92-0.98; p=0.004). It was found that HbA1c was not predictive in all models for mortality and complication (p>0.05). CONCLUSION: There are reports in the literature that mortality increases 4-fold when HbA1c value is higher than 8.6% in coronary surgery. However, there is a view that HbA1c alone cannot predict mortality in coronary surgery if diabetes associated factors are excluded. In this study, high HbA1c (≥7) values in diabetic patients undergoing isolated coronary bypass graft surgery were not found to be independent predictors of post-operative mortality and morbidity. Pre-operative low ejection fraction was found as an independent risk factor for post-operative mortality and morbidity in the general patient population.

Fast-Track Anaesthesia in Off-Pump Coronary Surgery: A Comparison of Normotensive and Hypertensive Patients.

OBJECTIVE: In this study, our aim was to investigate the efficacy and sufficiency of bispectral indeks (BIS) guided remifentanil-desflurane anaesthesia on intraoperative haemodynamic stability in both normotensive and hypertensive patients undergoing off-pump coronary artery bypass surgery. METHODS: Thirty adult, ASA I-III patients undergoing elective off-pump coronary surgery were included in the study. According to the presence of essential hypertension preoperatively, patients were divided into two groups. Haemodynamic parameters were recorded at 11 time points during the operation. RESULTS: There were no differences in the demographic data, heart rate and intraoperative and postoperative parameters between the groups. Arterial blood pressure and additional requirement of remifentanil were found to be significantly higher in the hypertensive group intraoperatively. CONCLUSION: In patients undergoing off-pump coronary revascularisation surgery, intraoperative haemodynamic stabilisation with remifentanil-desflurane anaesthesia under BIS guidance was safely provided, but higher remifentanil doses were required in hypertensive patients.