Successful Anesthetic Management of an Adult Patient With Glycogen Storage Disease Type 1 During Liver Transplant: A Case Report.

Glycogen storage disease type 1 is a congenital abnormality of metabolism caused by the deficiency of the glucose-6-phosphatase enzyme, essential in glucose homeostasis. Patients with this disease are at high risk of developing hypoglycemia, hyperlipidemia, lactic acidemia, growth retardation, neutropenia, inflammatory bowel disease, and many other severe complications, such as hepatic adenomas converting into hepatocellular carcinomas. To prevent these complications, a liver transplant is the ultimate method of treatment. We present the successful anesthesia management for a 21-year-old man who had gross hepatomegaly, severe hypoglycemia, and hyperlactatemia and who received a liver transplant from his mother, which is a substantial challenge for anesthesiologists. Anesthesiologists should know the underlying pathophysiological condition and perform a comprehensive preoperative evaluation to determine the correct anesthesia plan in patients with glycogen storage disease type 1 who will undergo an orthotopic liver transplant due to multiple system disorders. Successful perioperative management of patients with glycogen storage disease type 1 relies on effective communication and collaboration between specialists through a multidisciplinary team approach.

Perioperative management in pediatric domino liver transplantation for metabolic disorders: A narrative review.

Domino liver transplantation and domino-auxiliary partial orthotopic liver transplantation are emerging techniques that can expand the liver donor pool and provide hope for children with liver disease. The innovative technique of domino liver transplantation has emerged as a pioneering strategy, capitalizing on structurally preserved livers from donors exhibiting single enzymatic defects within a morphologically normal context, effectively broadening the donor pool. Concurrently, the increasingly prevalent domino-auxiliary partial orthotopic liver transplantation method assumes a critical role in bolstering available donor resources. These advanced transplantation methods present a unique opportunity for pediatric patients who, despite having structurally and functionally intact livers and lacking early signs of portal hypertension or extrahepatic involvement, do not attain priority on conventional transplant lists. Utilizing optimal clinical conditions enhances posttransplant outcomes, benefiting patients who would otherwise endure extended waiting periods for traditional transplantation. The perioperative management of children undergoing these procedures is complex and requires careful consideration of some factors, including clinical and metabolic conditions of the specific metabolic disorder, and the need for tailored perioperative management planning. Furthermore, the prudent consideration of de novo disease development in the recipient assumes paramount significance when selecting suitable donors for domino liver transplantation, as it profoundly influences prognosis, mortality, and morbidity. This narrative review of domino liver transplantation will discuss the pathophysiology, clinical evaluation, perioperative management, and prognostic expectations, focusing on perioperative anesthetic considerations for children undergoing domino liver transplantation.

An Innovative Study Focused on Reducing Unnecessary Oxygen Exposure in Pediatric Patients.

PURPOSE: In the perioperative period, fractional-inspired oxygen is used at values up to 80% to stay within the safe range, even for a short time. A clear value for the safe range has not been specified, and therefore, clinicians prefer a high oxygen value. This study aims to reduce unnecessary oxygen exposure in pediatrice patients and to provide the optimum fractional inspired oxygen value. DESIGN: The study was designed as a prospective randomized controlled study, including 139 patients aged 1 to 8 years without comorbidity. METHODS: Three groups were formed by adjusting the fractional inspired oxygen to 30%, 50%, or 80% intraoperatively. In the intraoperative period, a strict inspired oxygen protocol (hypoxemia threshold was SpO2 < 90) and oxygen reserve index, fractional expired oxygen value, and peripheral oxygen saturation were used to maintain the balance of hypoxemia and hyperoxemia. FINDINGS: One hundred and nine children were included. The mean oxygen reserve index was significantly lower in the 30% group than in the other groups (0.09 ± 0.05, P < .0001). The mean arterial pressure in the 30% group was significantly lower than the 80% group but within the normal range (78 ± 6 mmHg, P < .003). There was no significant difference between the groups regarding delirium and pain in the recovery unit. CONCLUSIONS: Due to the known and unknown harmful effects of unnecessary oxygen exposure, it may be time to use optimal oxygen and to fear unnecessary oxygen, not less oxygen. As the next step, we think studies should be conducted with patient groups with lower oxygen concentrations (eg, %21 vs %24 vs %30), more patients, and arterial blood gas monitoring.

Anesthetic Management of a Patient with MIRAGE Syndrome: A Case Report.

MIRAGE syndrome consists of Myelodysplasia, Infection, Growth restriction, Adrenal hypoplasia, Genital phenotypes, and Enteropathy. We report the uneventful anesthesia management of a 6-year-old female patient with MIRAGE syndrome. We think it can guide anesthesiologists caring for patients with this syndrome to find the appropriate method for them.

Successful Anesthesia Management of Pediatric Living Donor Liver Transplant With Mild Bilateral Pulmonary Artery Stenosis Due to Alagille Syndrome: A Case Report.

Alagille syndrome is an autosomal-dominantinherited disease characterized by intrahepatic bile duct involvement, congenital heart disease, eye anomalies, skeletal and central nervous system involvement, kidney anomalies, and facial appearance. Liver transplant is the only treatment option for patients with end-stage liver disease and Alagille syndrome. Bilateral peripheral pulmonary artery stenosis is a contraindication for liver transplant due to high mortality, and the decision for liver transplant in patients with bilateral peripheral pulmonary artery stenosis is extremely challenging for anesthesiologists andtransplant surgeons.Wepresent a 2-year-oldfemale patient with successful anesthetic management of a pediatric living donor liver transplant with mild bilateral pulmonary artery stenosis, mild aortic stenosis, and mitral regurgitation due to Alagille syndrome. Anesthesiologists should know the underlying pathophysiological condition and perform a comprehensive preoperative evaluation to determine the correct anesthesia plan in patients with Alagille syndrome who will undergo liver transplants to treat multiple system disorders. Successful perioperative management of Alagille syndrome requires effective communication and collaboration between specialists through a multidisciplinary team approach.

Anaesthesia management for liver transplantation: A narrative review.

Orthotopic liver transplantation is the definitive standard treatment for end-stage liver disease. Orthotopic liver transplantation anaesthesia management is a complex procedure that requires a multidisciplinary team approach. Understanding the complex pathophysiology of end-stage liver disease and its complications in the affected systems is essential for proper anaesthesia management in orthotopic liver transplantation. Orthotopic liver transplantation is a dynamic process, and preoperative optimisation is essential in these patients. Therefore, anaesthesiologists should focus on rapidly fluctuating physiology, haemodynamics, metabolic, and coagulation status in the anaesthesia management of these patients. Perioperative care and anaesthesia for orthotopic liver transplantation can be divided into preoperative evaluation, anaesthesia induction and management, dissection, anhepatic, neo-hepatic, and postoperative care, with essential anaesthetic considerations at each point. Considering the clinical situation, haemodynamic changes, misapplications, knowledge, attitude, and multimodal and multidisciplinary approach are vital in anaesthesia and the perioperative period. In our review, in line with the literature, we aimed to present the perioperative and anaesthesia management in orthotopic liver transplantation patients.

Vasoplegic Syndrome and Anaesthesia: A Narrative Review.

Vasoplegic syndrome (VS) is defined as low systemic vascular resistance, normal or high cardiac output, and resistant hypotension unresponsive to vasopressor agents and intravenous volume. VS is a frequently encountered complication in cardiovascular and transplantation surgery, burns, trauma, pancreatitis, and sepsis. The basis of the pathophysiology is associated with an imbalance of vasodilator and vasoconstrictive structure in vascular smooth muscle cells and is highly complex. The pathogenesis of VS has several mechanisms, including overproduction of iNO, stimulation of ATP-dependent K+ channels and NF-κB, and vasopressin receptor 1A (V1A-receptor) down-regulation. Available treatments involve volume and inotropes administration, vasopressin, methylene blue, hydroxocobalamin, Ca++, vitamin C, and thiamine, and should also restore vascular tone and improve vasoplegia. Other treatments could include angiotensin II, corticosteroids, NF-κB inhibitor, ATP-dependent K+ channel blocker, indigo carmine, and hyperbaric oxygen therapy. Despite modern advances in treatment, the mortality rate is still 30-50%. It is challenging for an anaesthesiologist to consider this syndrome's diagnosis and manage its treatment. Our review aims to review the diagnosis, predisposing factors, pathophysiology, treatment, and anaesthesia approach of VS during anaesthesia and to suggest a treatment algorithm.

Evaluation of coronavirus diseases (COVID-19) in terms of epidemiological and clinical features, comorbidities, diagnostic methods, treatment, and mortality.

BACKGROUND: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) causes COVID-19. On March 11, 2020, the WHO declared it a pandemic. SARS-CoV-2 indicates that it poses a significant threat to public health and global economy. The aim of the study was to determine (a) patient characteristics, (b) demographic characteristics, (c) comorbidities, diagnostic methods used, treatment, and outcomes, and (d) mortality rates of patients. MATERIALS AND METHODS: This retrospective cohort study included 352 hospitalized adult patients from Baskent University Hospital in Ankara who were confirmed cases of COVID-19 between March 2020 and March 2021. SPSS v. 14.0 was used for statistical analysis. RESULTS: Out of 352 patients, 55 died (males: 37, females: 18), while 297 survived (males: 162, females: 135). The most common comorbidities were hypertension (HT), diabetes mellitus (DM), coronary artery disease (CAD), cancer, Vitamin D deficiency, and chronic obstructive pulmonary disease. Comorbidities associated with mortality rate were obesity (33%) (P = 0.118), Vitamin D deficiency (28%) (P = 0.009), DM (25%) (P = 0.004), CAD (21.2%) (P = 0.142), cancer (20.9%) (P = 0.084), and HT (16.6%) (P = 0.90). Normal ward admission resulted in death in 67.3% and survival in 93.9% (P = 0.001), intensive care unit (ICU) admission resulted in death in 69.1% and survival in 18.5% (P = 0.001), and oxygen therapy was used in 80% death and survival in 39.4% (P = 0.001). CONCLUSIONS: Our study shows that male gender, advanced age, and presence of comorbidities in COVID 19 patients are at higher risk for severe disease, ICU admission, and death. We emphasize that morbidity and mortality can be reduced by early and comprehensive identification of risk factors and the warning systems that will meet the ICU needs of these patients.

Anesthetic management for dental surgery in a child with glycogen storage disease type IIIa: a case report.

Glycogen storage disease (GSD) is a group of inherited disorders, which result in the deficiency of enzymes involved in glycogen metabolism, leading to an accumulation of glycogen in various organs. Deficiency of amylo-1-6-glicosidase (debranching enzyme) causes glycogen storage disease type III (GSD III). The main problems that anesthesiologists face in patients with GSD III include hypoglycemia, muscle weakness, delayed awakening due to abnormal liver function, possible difficulty in airway, and cardiomyopathy. In the face of these difficulties, airway preparation and appropriate glucose monitoring and support during the fasting period are important. The doses of the drugs to be used should be calculated considering the increased volume of distribution and decreased metabolic activity of the liver. We present the case of a child with GSD IIIa who underwent dental prosedation under general anesthesia. She was also being prepared for liver transplantation. This case was additionally complicated by the patient's serious allergic reaction to eggs and milk.

Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report.

INTRODUCTION: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. PATIENT CONCERNS: A 13-year-old female patient had a history of chronic renal failure, hypothyroidism, mental retardation, hypogonadotropic hypogonadism, obesity, and retinitis pigmentosa and was undergoing 4-hour hemodialysis 3 days a week. DIAGNOSIS: We diagnosed Bardet-Biedl syndrome based on the results of genetic tests. INTERVENTIONS: We performed renal transplantation under general anesthesia while considering the perioperative risks of airway obstruction and hypothermia. OUTCOMES: Multidisciplinary preoperative evaluation is crucial to avoid perioperative complications. The risk of an obstructed airway should be considered. Hypothyroidism is a rare consequence of Bardet-Biedl syndrome. Rocuronium and sugammadex are safe for anesthetic management during renal transplantation to address Bardet-Biedl syndrome. CONCLUSION: Safe anesthetic management can be achieved with the rigorous preoperative assessment of perioperative complications.

Does early removal of double J stents reduce urinary infection in living donor renal transplantation?

INTRODUCTION: Prophylactic use of double J (DJ) stents in recipients is highly accepted in renal transplantation. In this study, the association between the frequency of urologic complications (UC) and urinary tract infections (UTI), and the early or late removal of DJ stents was investigated. MATERIAL AND METHODS: A total of 116 live-donor renal transplant patients were included in the study during a 4-year period, with a mean follow-up of 29.2 ±15.3 months. In all, DJ stents were used. All patients were clinically monitored for graft function by assessment of the complete blood count, renal biochemistry, urine analysis and blood drug level according to our follow-up protocol. RESULTS: The patients were divided into 2 groups according to the time of their stent removal: group I (n = 44), removal within the first 14 days; and group II (n = 72), removal after 14 days. No urinary leaks were detected in either of the groups. Three patients suffered from anastomotic stricture (group I, n = 1; group II, n = 2). The rates of UTI were similar in groups I and II (13.6% vs. 16.6%, respectively, p = 0.79). The rate of UTI in women was found to be 3.8 times higher than in men. CONCLUSIONS: The results of our study demonstrated that DJ stent removal within 14 days did not reduce the risk of UTI when compared to stent removal after 14 days. Similar effects on complication rates for ureteral stenting for these 2 removal periods were observed.

Fast-track anesthesia in patients undergoing outpatient laparoscopic cholecystectomy: comparison of sevoflurane with total intravenous anesthesia.

BACKGROUND: The use of short-acting anesthetics has introduced a "fast-track anesthesia" concept in outpatient surgery which provides discharge of the patients from operation room directly to the phase II recovery area without entering into postanesthesia care unit. The aim of this prospective and randomized study was to compare general anesthesia using sevoflurane with propofol-remifentanil-based total intravenous anesthesia (TIVA) for fast-track eligibility in patients undergoing outpatient laparoscopic cholecystectomy. The secondary aim was to compare 2 discharge scoring systems: White's Fast-Tracking Scoring System (WFTSS) and Modified Aldrete Scoring Systems (MASS) with regard to postanesthesia care unit bypass rate and postoperative problems. METHODS: After obtaining ethical approval and written informed patient consent, 80 patients were randomly assigned into 2 groups: group sevoflurane (n=40) and group TIVA (n=40). Anesthesia was induced with propofol, fentanyl, and rocuronium in both groups and maintained with sevoflurane in group sevoflurane and with remifentanil-propofol in group TIVA. Fast-track eligibility was evaluated using both WFTSS and MASS while patients were discharged from operation room according to WFTSS. Recovery times, number of fast-track eligible patients, factors related to fast-track ineligibility, and perioperative complications were evaluated. RESULTS: The ratio of fast-track eligible patients was higher and times to fast-track eligibility were shorter in group TIVA compared with group sevoflurane (82.1% vs 57.5% and 8 minutes vs 12 minutes; P<.05). The primary factors that have inhibited fast-tracking were desaturation, hemodynamic instability, pain, and postoperative nausea and vomiting, respectively. Postoperative nausea and vomiting presented a major difference in the rate of fast-track ineligibility between groups (4 patients in group sevoflurane, whereas none in group TIVA; P<.05). The fast-track ratio was lower with the WFTSS compared with MASS in group sevoflurane (57.5% vs 77.5%, P<.05), but similar in group TIVA.

Do the patients read the informed consent?

BACKGROUND: Informed consent is a process which consists of informing the patient about the medical interventions planned to be applied to the patient's body and making the patient active in the decision making process. AIMS: The aim of this study was to evaluate whether the patients read the informed consent document or not and if not, to determine why they did not read it. This was achieved via a questionnaire administered at the pre-anaesthetic visit to assess the perception of patients to the informed consent process. STUDY DESIGN: Survey study. METHODS: The patients were given a questionnaire after signing the informed consent document at the pre-anaesthetic visit. We studied whether the patients read the informed consent document or not and asked for their reasons if they did not. RESULTS: A total of 522 patients were included during the two month study (mean age: 38.1 years; 63.8% male, 36.2% female). Overall, 54.8% of patients reported that they did not read the informed consent. Among them, 50.3% did not care about it because they thought they would have the operation anyway, 13.4% did not have enough time to read it, 11.9% found it difficult to understand, 5.9% could not read because they had no glasses with them, and 5.2% found it frightening and gave up reading. Inpatients, older patients and patients with co-morbidities were less likely to read the informed consent document than outpatients, and younger and healthy patients (p<0.05). Also, 57.9% of parents whose children would be operated on had read the document. CONCLUSION: This study shows that the majority of our patients did not understand the importance of the informed consent. It is therefore concluded that informed consent documents should be rearranged to be easily read and should be supported with visual elements such as illustrations or video presentations, as informed consent is a process rather than just simply signing a form.

Severe gastrointestinal burn with hydrochloric acid.

Inadvertantly or purposely, an oral intake of corrosive substances may cause life-threatening problems. Early admission to the hospital, clinical and endoscopic evaluation, and early surgery when required, may reduce morbidity and mortality. We report the case of a 49-year-old male patient, who had attempted suicide, by drinking about 800 mL of 25% hydrochloric acid, and who had severe intra-abdominal damage. The aim of this report is to state the fact that a good outcome is possible in severe burns caused by oral intake of corrosive substances, when fast, multidisciplinary, and appropriate management is provided on time.

Preoperative carbohydrate nutrition reduces postoperative nausea and vomiting compared to preoperative fasting.

BACKGROUND: The aim of this prospective, randomized, single-blinded study was to compare the effects of a carbohydrate drink 400 mL given 2 h before the surgery with preoperative overnight fasting on the gastric pH and residual volume, postoperative nausea and vomiting (PONV) and antiemetic consumption in patients undergoing laparoscopic cholecystectomy. MATERIALS AND METHODS: Forty American Society of Anesthesiologists physical status I-II patients who underwent elective laparoscopic cholecystectomy. Randomized, prospective, controlled study, Gulhane Medical Faculty and Guven Hospital Department of Anesthesiology and Reanimation. Patients were randomly assigned into two groups: Pre-operative carbohydrate drink group (group C, n = 20) and preoperative fasting group (group F, n = 20). Group C was given a 400 mL carbohydrate drink 2 h before to the surgery. The patients of group F were fasted 8 h before the surgery. Both groups were operated under general anesthesia with volatile anesthetics. RESULTS: Hemodynamic parameters, demographic data, gastric acidity and residual volumes were similar for both groups. No complications were observed. PONV and antiemetic consumption was lower in group C compared to group F (P = 0.001). Patient's satisfaction was higher in group C (P < 0.001). CONCLUSION: This study showed that pre-operative carbohydrate drink may be used safely and also improves patient's satisfaction and comfort in patients undergoing laparoscopic cholecystectomy.

A pulmonary aspergillosis case with fatal course in a patient with SIRS clinic.

A 77-year-old male patient with a history of tuberculosis applied to emergency service with complaints of confusion, shortness of breath, tachycardia, hypothermia and hypotension. A bronchoalveolar lavage culture was collected because a fungus ball was seen on repeat chest X-ray and thoracic CT of the patient. Aspergillus fumigatus grew and voricona-zole treatment was started, but the patient was lost from multiple organ failure (MOF). In diagnosis of patients with SIRS clinic, causative factor may be aspergillus located in an old tuberculosis cavity, and this may have a fatal course in an old patient having previous pulmonary and systemic diseases.

Endobronchial metastasis of a primary transitional-cell and signet-ring cell carcinoma of the urinary bladder.

Endobronchial metastasis is generally a late finding of primary tumor or may be determined before the diagnosis of primary tumor. We present a rare case of primary transitional and signet-ring cell carcinoma of the urinary bladder that occurred with malignant pleural effusion and endobronchial metastasis. A 71-year-old man complained of dyspnea and hematuria. He was admitted to the intensive care unit (ICU) with a prediagnosis of acute respiratory failure. He had decreased respiratory sounds and fine rales bilaterally at the lung bases. Respiratory failure worsened, and he was placed on mechanical ventilation. Radiograph and computed tomogram revealed bilateral effusion and metastatic nodules in the lung parenchyma. Subsequent abdominal computed tomogram revealed a mass in the urinary bladder, and transurethral biopsy indicated transitional epithelial carcinoma (modified Bergvist grade IIIB, and World Health Organization/International Society of Urological Pathology higher-grade urothelial papillary carcinoma) and signet-ring cell carcinoma, with lymphovascular invasion, consistent with the pathology findings. Our treatment plan was radical cysto-prostatectomy, followed by chemotherapy and radiotherapy, but because of his very poor medical status, the operation was not performed. On the 5th ICU day he died from severe respiratory failure, despite intensive supportive therapy. This case highlights the need to rule out malignancies in other organs in patients admitted with severe respiratory symptoms.

Effects of the general anaesthetic agent, propofol, on erythrocyte deformability.

OBJECTIVES: Propofol is an anesthetic agent frequently used for sedation and general anesthesia. The purpose of our study was to investigate the effect of propofol, a general anesthetic, on erythrocyte deformability in rats. METHODOLOGY: The study was performed on 20 male and 20 female rats, with 10 rats in each study group and 10 rats in each control group. The rats in the study group were administered propofol (150 mg.kg(-1)) intraperitoneally, and the rats in the control group were administered SF. Erythrocyte packs were prepared using heparinized total blood samples. Deformability measurements were done by erythrocyte suspensions in PBS buffer. A constant flow filtrometer system was used to measure erythrocyte deformability, and the relative resistance was calculated. RESULTS: The use of propofol resulted in the increase in the relative resistance, which is an indicator for the erythrocyte deformability in both male and female rats (p = 0.002, p = 0.042, respectively). CONCLUSION: A negative change in the erythrocyte deformability may cause a functional deterioration in blood flow and tissue perfusion (Fig. 1, Ref. 23).

Tracheal compression during shoulder arthroscopy in the beach-chair position.

BACKGROUND: Respiratory distress is a rare complication of outpatient shoulder arthroscopy and mostly associated with general anesthesia, pneumothorax, anaphylaxis, or phrenic nerve paralysis. OBJECTIVE: We report on a shoulder arthroscopy complicated by tracheal compression caused by extravasation of irrigation fluid into soft tissues of the upper airway while the patient was in the beach-chair position under general anesthesia. CASE SUMMARY: A 33-year-old male was scheduled for shoulder arthroscopy for impingement syndrome of the right shoulder under general anesthesia combined with interscalene brachial plexus block. During the operation, the patient's neck, right chest, and shoulder were observed to be swollen and tense on palpation. A fiberoptic bronchoscopic evaluation through the endotracheal tube revealed that the trachea was compressed to the left, but not completely obstructed. It was determined that the irrigation fluid had leaked subcutaneously from the shoulder joint to the neck. Vital signs were stable and the patient could be adequately ventilated despite the airway obstruction. The patient was transferred to the ward 16 hours after the operation with stable vital signs and discharged from the hospital on the second day. CONCLUSIONS: We report a case of airway obstruction due to tracheal compression from extravasation of irrigation fluid during shoulder arthroscopy under general anesthesia combined with peripheral nerve block in the beach-chair position. General anesthesia with endotracheal intubation protected the patient from a possibly fatal complication.