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Sitosterolemia is a lipid disorder characterized by the accumulation of dietary xenosterols in plasma and tissues caused by mutations in either or . encodes a pair of ABC half transporters that form a heterodimer (G5G8), which then traffics to the surface of hepatocytes and enterocytes and promotes the secretion of cholesterol and xenosterols into the bile and the intestinal lumen. We review the literature from the initial description of the disease, the discovery of its genetic basis, current therapy, and what has been learned from animal, cellular, and molecular investigations of the transporter in the twenty years since its discovery. The genomic era has revealed that there are far more carriers of loss of function mutations and likely pathogenic variants of than previously thought. The impact of these variants on G5G8 structure and activity are largely unknown. We propose a classification system for mutants based on previously published systems for diseases caused by defects in ABC transporters. This system establishes a framework for the comprehensive analysis of disease-associated variants and their impact on G5G8 structure-function.

BackgroundLaparoscopic surgeries are a risk factor for raised intracranial **pressure and neurological complications. Even though rare, the consequences may be severe.MethodsOne hundred and one patients of laparoscopic cholecystectomy were enrolled and were randomized into two groups: low-pressure 8 mm Hg (Group A) and high-pressure 14 mm Hg (Group B) carbon dioxide pneumoperitoneum during surgery. Fifty patients were in group A and 51 patients were in group B. Intracranial pressure was measured by measuring the optic nerve sheath diameter (ONSD) using ultrasound examination. Baseline ONSD was recorded followed by ONSD recording at various intervals: at the induction of anesthesia; 30 min, 45 min, at the end of surgery; and 30 min post surgery.ResultsThe groups were comparable in terms of demographics and comorbidities. The mean age of group A was 45 years and for group B it was 45.75 years. Most common indication for surgery was symptomatic gall stone disease. Baseline ONSD in group A was 0.427 ± 0.0459 mm, whereas it was 0.412 ± 0.0412 mm in group B. There was a significant rise of ONSD (p < 0.05) 30 min after induction of pneumoperitoneum and up to 30 min post anesthesia. In the low-pressure group 7 (14%) patients had a significant rise of ICP, whereas in the high-pressure group 20 (39%) patients had a significant rise of ICP (p < 0.05).ConclusionsHigh-pressure pneumoperitoneum causes significant rise in intracranial pressure in comparison to low-pressure pneumoperitoneum during laparoscopic cholecystectomy, which can be monitored by ONSD measurement by ultrasound examination and is totally non-invasive.

Cholecystectomy is considered the standard treatment for acute cholecystitis and symptomatic gallstones. An increasing number of frail elderly patients are being referred for this surgical treatment. A better understanding of surgical outcome in the elderly is needed to improve quality of care. A retrospective analysis of 565 patients who underwent cholecystectomy was performed. Focus of the analyses was on postoperative complications and its predictors. The study population was divided in two cohorts; aged <70. More complications were found in patients aged ≥70 years. More elderly patients were admitted to the intensive care, respectively 4.0% and 14.1% (P = 0.045). Hospital mortality was 6% in patients aged ≥70 years vs 0.6% in patients <70. In elderly patients, the complication and mortality rate following cholecystectomy is higher than previously reported. For high-risk patients aged ≥70 with cholecystitis, alternative therapies should be considered as a bridge to surgery or definite treatment. •Cholecystectomy in elderly patients is associated with a higher complication rate (CCI).•Complications were accompanied by a significantly longer HLOS and a higher ICU admittance rate in the elderly.•More pre-operative factors than age alone are predictors for postoperative complications.

Gallstone-related disease comprises a spectrum of conditions resulting from biliary stone formation, leading to obstruction and inflammatory complications. These can significantly impact patient quality of life and carry high morbidity if not accurately detected. Appropriate imaging is essential for evaluating the extent of gallstone disease and assuring appropriate clinical management. Magnetic Resonance Imaging (MRI) techniques (including Magnetic Resonance Cholangiopancreatography (MRCP) are increasingly used for diagnosis of gallstone disease and its complications and provide high contrast resolution and facilitate tissue-level assessment of gallstone disease processes. In this review we seek to delve deep into the spectrum of MR imaging in diagnose of gallstone-related disease within the gallbladder and complications related to migration of the gallstones to the gall bladder neck or cystic duct, common hepatic duct or bile duct (choledocholithiasis) and beyond, including gallstone pancreatitis, gallstone ileus, Bouveret syndrome, and dropped gallstones, by offering key examples from our practice. Furthermore, we will specifically highlight the crucial role of MRI and MRCP for enhancing diagnostic accuracy and improving patient outcomes in gallstone-related disease and showcase relevant surgical pathology specimens of various gallstone related complications.

AimsThe rate of acute laparoscopic cholecystectomy remains low due to operational constraints. The purpose of this study is to evaluate a service model of index admission cholecystectomy with referral protocols, refined logistics and targeted job planning.MethodsA prospectively maintained dataset was evaluated to determine the processes of care and outcomes of patients undergoing emergency biliary surgery. The lead author has maintained a 28 years prospective database capturing standard demographic data, intraoperative details including the difficulty of cholecystectomy as well as postoperative outcome parameters and follow up data.ResultsOver five thousand (5555) consecutive laparoscopic cholecystectomies were performed. Only patients undergoing emergency procedures (2399,43.2% of entire group) were analysed for this study. The median age was 52 years with 70% being female. The majority were admitted with biliary pain (34%), obstructive jaundice (26%) and acute cholecystitis (16%). 63% were referred by other surgeons. 80% underwent surgery within 5 days (40% within 24 h). Cholecystectomies were performed on scheduled lists (44%) or dedicated emergency lists (29%). Two thirds had suspected bile duct stones and 38.1% underwent bile duct exploration. The median operating time was 75 min, median hospital stay 7 days, conversion rate 0.8%, morbidity 8.9% and mortality rate 0.2%.ConclusionIndex admission cholecystectomy for biliary emergencies can have low rates of morbidity and mortality. Timely referral and flexible theatre lists facilitate the service, optimising clinical results, number of biliary episodes, hospital stay and presentation to resolution intervals. Cost benefits and reduced interval readmissions need to be weighed against the length of hospital stay per episode.

Intestinal obstruction is a common surgical emergency with high morbidity and mortality. Patients presenting with features of small bowel obstruction need urgent evaluation to avoid complications such as bowel gangrene, perforation, or peritonitis. Imaging is necessary in most cases of suspected bowel obstruction, to take an appropriate decision, for apt patient management. Among the common causes of small bowel obstruction, adhesions, external herniae, malignancies, and Crohn's disease top the chart. Imaging helps in determining the presence of obstruction, the severity of obstruction, transition point, cause of obstruction, and associated complications such as strangulation, bowel gangrene, and peritonitis. This review is based on the cases with unusual causes of bowel obstruction encountered during our routine practice and also on the extensive literature search through the standard textbooks and electronic databases. Through this review we want our readers to have sound knowledge of the imaging characteristics of the uncommon yet important causes of bowel obstruction. We have also revisited and structured a checklist to simplify the approach while reporting a suspected case of small bowel obstruction. Imaging plays a key role in the diagnosis of small bowel obstruction and in determining the cause and associated complications. Apart from the common causes of small bowel obstruction, we should also be aware of the uncommon causes of small bowel obstruction and their imaging characteristics to make an accurate diagnosis and for apt patient management.

Treatment for bile duct stones (BDS) depends largely on anatomical circumstances; yet, whether the outcome of cholecystectomies is impacted by the localization of intraoperatively discovered BDS remains largely unknown. A population-based registry study using data from the national Swedish Registry for Gallstone Surgery and Endoscopic Retrograde Cholangiopancreatography (GallRiks). 115,084 cholecystectomies 2006–2016 with the indications gallstone colic or complications were included. The surgical outcome between patients with distal BDS and those with at least one stone above the confluence was compared. 10,704 met the inclusion criteria. Patients with stones above the confluence had 16% longer operation times and significantly higher rates of intraoperative complications (OR 1.47), gut perforation (OR 4.60), and cholangitis (OR 1.96) compared to patients with distal BDS. The highest clearance rate (96%), as reflected by the need for re-ERCP, was seen after intraoperative ERCP, regardless of the localization of the BDS. Stones located above the confluence are associated with increased complication risks. These findings stress the importance of carefully considering the optimal methods for BDS removal during surgery. •A nationwide, population-based registry study of 115,084 cholecystectomies.•Intraoperative removal of BD stones high in the biliary tree have more complications.•Open choledochotomy was the most used method to remove high BD stones (28.6%).•The overall most used method to remove BD-stones, regardless of position is ERCP.•The importance of choosing the optimal methods to remove BD-stones.

The author outlines his philosophy and practice of management of gall bladder cancer based on his more than three-decade experience at a large tertiary level super-specialty referral hospital attached to a university-status teaching institution at Lucknow in northern India where GBC is very common.

Introduction Endoscopic and open surgical interventions are widely implemented as the standard practice in common bile duct exploration. However, the laparoscopic approach has been also reported to have comparative/superior outcomes in this concept. This has created an ongoing debate about the ideal approach to adopt in practice. Methods A systematic review of the published evidence over the last decade that has looked into the outcomes of laparoscopic exploration of the common bile duct through transductal and transcystic approaches. Results Our systematic review included 36 relevant papers. The majority were based on nonrandomized protocols conducted in single centers with high expertise. The data analysis showed that laparoscopic common bile duct exploration through both approaches was successful in more than 84% of the patients, with an average length of hospital stay of 5.6 days. Conversion to open surgery was reported in 5%-8% of the patients, and the bile leak rates from the more recent studies was 0%-12%, with mortality figures of 0%-1.3%. The outcomes were statistically in favor of the transcystic route when compared to the transductal route from the viewpoints of bile leak rates, mean operative time, duration of hospital stay and morbidity. Conclusion In experienced hands, both laparoscopic approaches in common bile duct exploration are safe in patients who are clinically fit to have this intervention. It is associated with a statistically significant lower overall morbidity and shorter duration of hospital stay when compared to open surgery. Relevant up-to-date high-quality randomized trials are unavailable.

Background Acute pancreatitis is a potentially life‐threatening inflammation of the pancreas, with a rising incidence in most countries. Recent studies have suggested that acute pancreatitis is associated with increased long‐term mortality. However, the extent to which this association is influenced by the development of chronic pancreatitis or comorbid conditions, such as malignant disease, remains unclear. Objective To assess the association between acute pancreatitis and long‐term all‐cause mortality. Methods The Swedish Pancreatitis Cohort (SwePan) was used, including all individuals with a first‐time episode of acute pancreatitis in Sweden between 1990 and 2019 who survived the index hospital stay and 1:10 matched pancreatitis‐free individuals from the general population. Multivariable conditional Cox proportional hazard models were used to compare mortality among individuals with acute pancreatitis compared with the matched pancreatitis‐free control group. Results In total, 89,465 individuals discharged from hospital with acute pancreatitis and 890,837 matched pancreatitis‐free individuals were followed up for 10,155,039 person‐years (mean 10.0 years). There were 33,764 (37.7%) deaths among individuals with acute pancreatitis and 265,403 (29.8%) deaths among controls. In multivariable adjusted models, mortality was increased in individuals with acute pancreatitis throughout the follow‐up period, particularly among those with severe and non‐gallstone‐related acute pancreatitis as compared to the matched controls. These results remained statistically significant after censoring the follow‐up time for recurrent acute pancreatitis or a diagnosis of chronic pancreatitis. Conclusions Acute pancreatitis was associated with increased long‐term mortality, even after adjusting for comorbidities, including cancer, and censoring for recurrent acute pancreatitis or chronic pancreatitis. Future research should assess causes of death and focus on understanding long‐term morbidity to facilitate prevention through tailored follow‐up strategies.