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Current Opinion in Gastroenterology Mar 2018Gallstone disease is a major epidemiologic and economic burden worldwide, and the most frequent form is cholesterol gallstone disease. (Review)
Review
PURPOSE OF REVIEW
Gallstone disease is a major epidemiologic and economic burden worldwide, and the most frequent form is cholesterol gallstone disease.
RECENT FINDINGS
Major pathogenetic factors for cholesterol gallstones include a genetic background, hepatic hypersecretion of cholesterol, and supersaturated bile which give life to precipitating cholesterol crystals that accumulate and grow in a sluggish gallbladder. Additional factors include mucin and inflammatory changes in the gallbladder, slow intestinal motility, increased intestinal absorption of cholesterol, and altered gut microbiota. Mechanisms of disease are linked with insulin resistance, obesity, the metabolic syndrome, and type 2 diabetes. The role of nuclear receptors, signaling pathways, gut microbiota, and epigenome are being actively investigated.
SUMMARY
Ongoing research on cholesterol gallstone disease is intensively investigating several pathogenic mechanisms, associated metabolic disorders, new therapeutic approaches, and novel strategies for primary prevention, including lifestyles.
Topics: Animals; Bile; Biliary Dyskinesia; Cholelithiasis; Cholesterol; Disease Models, Animal; Epigenesis, Genetic; Gallstones; Gastrointestinal Microbiome; Gene-Environment Interaction; Genetic Predisposition to Disease; Humans; Intestines; Mice
PubMed: 29283909
DOI: 10.1097/MOG.0000000000000423 -
Journal of Nuclear Medicine : Official... Jun 2014Cholescintigraphy with (99m)Tc-hepatobiliary radiopharmaceuticals has been an important, clinically useful diagnostic imaging study for almost 4 decades. It continues to... (Review)
Review
Cholescintigraphy with (99m)Tc-hepatobiliary radiopharmaceuticals has been an important, clinically useful diagnostic imaging study for almost 4 decades. It continues to be in much clinical demand; however, the indications, methodology, and interpretative criteria have evolved over the years. This review will emphasize state-of-the-art methodology and diagnostic criteria for various clinical indications, including acute cholecystitis, chronic acalculous gallbladder disease, high-grade and partial biliary obstruction, and the postcholecystectomy pain syndrome, including sphincter-of-Oddi dysfunction and biliary atresia. The review will also emphasize the use of diagnostic pharmacologic interventions, particularly sincalide.
Topics: Biliary Tract; Biliary Tract Diseases; False Positive Reactions; Humans; Liver; Radionuclide Imaging; Reference Standards
PubMed: 24744445
DOI: 10.2967/jnumed.113.131490 -
Frontiers in Pediatrics 2020While functional gallbladder disorder is a well-recognized and defined condition in adults, its pediatric analog, biliary dyskinesia, lacks uniformity in diagnosis.... (Review)
Review
While functional gallbladder disorder is a well-recognized and defined condition in adults, its pediatric analog, biliary dyskinesia, lacks uniformity in diagnosis. Yet, biliary dyskinesia is among the most common conditions resulting in cholecystectomy in youth and its frequency continues to rise. The primary aims of the current review were assess the efficacy of cholecystectomy in children diagnosed with biliary dyskinesia and the utility of cholescintigraphy in predicting outcomes. All previous studies assessing outcomes in youth with biliary dyskinesia have been retrospective and therefore of low quality. There is a lack of uniformity in patient selection. Short term follow-up reveals partial response in 63.4-100% with complete resolution in 44.2-100%. Only 4 studies have reported long-term outcomes with complete symptom resolution in 44-60.7%. The published research generally indicates that the gallbladder ejection fraction (GBEF) as determined by cholescintigraphy lacks utility in predicting cholecystectomy outcome utilizing the commonly used cut-off values. There are data suggesting that more extreme cut-off values may improve the predictive value of GBEF. There is a lack of consensus on the symptom profile defining biliary dyskinesia in youth and current literature does not support the use of cholescintigraphy to select patients for cholecystectomy. There is a substantial portion of pediatric patients diagnosed with biliary dyskinesia who do not experience long-term benefit from cholecystectomy. Well-designed prospective studies of surgical outcomes are lacking. Increasing the uniformity in patient selection, including both symptom profiles and cholescintigraphy results, will be key in understanding the utility of cholecystectomy for this condition.
PubMed: 32266192
DOI: 10.3389/fped.2020.00122 -
Orphanet Journal of Rare Diseases Sep 2022Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a... (Review)
Review
Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.
Topics: Anti-Bacterial Agents; Bronchiectasis; Child; Humans; Intestinal Volvulus
PubMed: 36085154
DOI: 10.1186/s13023-022-02515-2 -
Journal of Nuclear Medicine Technology Dec 2014Cholescintigraphy with (99m)Tc-hepatobiliary radiopharmaceuticals has been an important, clinically useful diagnostic imaging study for almost 4 decades. It continues to...
Cholescintigraphy with (99m)Tc-hepatobiliary radiopharmaceuticals has been an important, clinically useful diagnostic imaging study for almost 4 decades. It continues to be in much clinical demand; however, the indications, methodology, and interpretative criteria have evolved over the years. This review will emphasize state-of-the-art methodology and diagnostic criteria for various clinical indications, including acute cholecystitis, chronic acalculous gallbladder disease, high-grade and partial biliary obstruction, and the postcholecystectomy pain syndrome, including sphincter-of-Oddi dysfunction and biliary atresia. The review will also emphasize the use of diagnostic pharmacologic interventions, particularly sincalide.
PubMed: 25472513
DOI: 10.2967/jnumed.113.131490 -
Translational Gastroenterology and... 2021Biliary dyskinesia generally refers to a hypofunctioning gallbladder with an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid scan with cholecystokinin...
BACKGROUND
Biliary dyskinesia generally refers to a hypofunctioning gallbladder with an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid scan with cholecystokinin stimulation (CCK-HIDA testing). In adults, biliary hyperkinesia has a defined association with biliary colic symptoms and can be relieved with surgical intervention. This clinical entity has not been well described in children or adolescents. In fact, only recently have we seen biliary hyperkinesia on HIDA at our centers. To that end, we reviewed our recent experience with adolescents who have presented and been treated for this unusual clinical entity.
METHODS
With IRB approval, we retrospectively reviewed the records of all patients with abnormally high HIDA EFs (>80%) cared for by the pediatric surgery services at two tertiary care centers over the span of a three-year period. Age, sex, BMI, CCK-HIDA results, and preoperative testing and post-operative pathology were noted. Resolution of symptoms was determined by subjective patient self-reporting at postoperative visit.
RESULTS
Eighteen patients met inclusion criteria. Average age 15.7 (range, 10-17 years), median BMI 27.3 (±8.2). Fifteen patients were female and 3 were male. Average CCK-HIDA EF was 91.6% (±5.2), 82.4% of the patients had evidence of chronic cholecystitis and/or cholesterolosis on pathology. Postoperatively, 82.4% of the patients available for follow up (n=17) reported complete or near complete resolution of symptoms.
CONCLUSIONS
Biliary hyperkinesia is an emerging clinical entity in children and adolescents and has a similar presentation to biliary hypokinesia. While the pathophysiologic mechanism of pain is not fully elucidated, laparoscopic cholecystectomy appears to provide a surgical cure for these patients and should be considered in the differential for the patient with an unremarkable workup and history suggestive of biliary colic.
PubMed: 34423157
DOI: 10.21037/tgh-20-258 -
Cureus Dec 2023Objective Our goal is to describe and report the outcomes of patients undergoing robotic single-site cholecystectomy at a single institution. Background Cholecystectomy...
Objective Our goal is to describe and report the outcomes of patients undergoing robotic single-site cholecystectomy at a single institution. Background Cholecystectomy is a common procedure performed to remove the gallbladder. Robotic single-site cholecystectomy (RSSC) is rapidly emerging as a safe and feasible alternative to conventional 4-port laparoscopic techniques. Patients who undergo RSSC procedures may also have a decreased need for postoperative analgesics and a lower postoperative pain score. Methods From September 2020 to August 2023, there were 33 cases of RSSC performed by a single surgeon at a single institution. We recorded demographic data including sex, age, and BMI as well as postoperative outcome data including wound dehiscence, postoperative infection, biliary leakage, wound herniation, blood loss, and conversion to open procedure. Results The patient group included 24 females (72.7%) and nine males (27.3%) with a median age of 32 (Range: 9-70) and a median BMI of 24.2 kg/m (Range: 18.1-30.7). The majority of these patients were receiving cholecystectomies for benign conditions such as symptomatic cholelithiasis (n = 18, 54.5%), biliary dyskinesia (n = 13, 39.4%), acute cholecystitis (n = 1, 0.03%), and biliary colic (n = 1, 0.03%). The average estimated blood loss was 5.91 mL. Thirty-two patients (96.9%) were discharged home the same day of surgery. One patient was admitted overnight for observation due to severe biliary dyskinesia diagnosed preoperatively. The patient had no complications and was discharged the following day. One patient presented with acute abdominal wall cellulitis and omphalitis with no underlying abscess four weeks after the operation. They were treated with therapeutic antibiotics. No patients underwent conversion to an open procedure. There were no incidences of postoperative wound dehiscence or biliary leakages. One patient was admitted overnight for observation of biliary dyskinesia and another experienced abdominal wall cellulitis four weeks post-operation. Conclusions Although conventional multi-incision laparoscopic cholecystectomy remains the gold standard treatment for benign gallbladder disease, our study demonstrates the practicality and safety of Robotic Single-Site Cholecystectomy procedures.
PubMed: 38196423
DOI: 10.7759/cureus.50271