Gallbladder dysfunction caused by MYPT1 ablation triggers cholestasis-induced hepatic fibrosis in mice.

BACKGROUND: The incidence of gallbladder diseases is as high as 20%, but whether gallbladder diseases contribute to hepatic disorders remains unknown. METHODS: Here, we established an animal model of gallbladder dysfunction and assessed the role of a diseased gallbladder in cholestasis-induced hepatic fibrosis (CIHF). RESULTS: Mice with smooth muscle-specific deletion of Mypt1, the gene encoding the main regulatory subunit of myosin light chain phosphatase (myosin phosphatase target subunit 1 [MYPT1]), had apparent dysfunction of gallbladder motility. This dysfunction was evidenced by abnormal contractile responses, namely, inhibited cholecystokinin 8-mediated contraction and nitric oxide-resistant relaxation. As a consequence, the gallbladder displayed impaired bile filling and biliary tract dilation comparable to the alterations in CIHF. Interestingly, the mutant animals also displayed CIHF features, including necrotic loci by the age of 1 month and subsequently exhibited progressive fibrosis and hyperplastic/dilated bile ducts. This pathological progression was similar to the phenotypes of the animal model with bile duct ligation and patients with CIHF. The characteristic biomarker of CIHF, serum alkaline phosphatase activity, was also elevated in the mice. Moreover, we observed that the myosin phosphatase target subunit 1 protein level was able to be regulated by several reagents, including lipopolysaccharide, exemplifying the risk factors for gallbladder dysfunction and hence CIHF. CONCLUSIONS: We propose that gallbladder dysfunction caused by myosin phosphatase target subunit 1 ablation is sufficient to induce CIHF in mice, resulting in impairment of the bile transport system.

Fasting gallbladder volume is increased in patients with Parkinson's disease.

INTRODUCTION: Autonomic denervation in patients with Parkinson's disease (PD) and isolated REM-sleep behavior disorder (iRBD) could impede gallbladder function leading to increased fasting gallbladder volume (fGBV) and higher risk of gallstones. We aimed to determine fGBV in patients with PD, iRBD, and healthy controls (HCs). METHODS: We included 189 subjects; 100 patients with PD, 21 with iRBD, and 68 HCs. fGBV was determined from abdominal CT scans, and radiopaque gallstone frequency was evaluated. RESULTS: Median fGBV was 35.7 ml in patients with PD, 31.8 ml in iRBD, and 27.8 ml in HCs (Kruskal-Wallis test: P = 0.0055). Post-tests adjusted for multiple comparison revealed a significant group difference between patients with PD and HCs (P = 0.0038). In the PD group, 23% had enlarged fGBV (cut-off at mean + 2 x standard deviation (SD) in the HC group). No difference in fGBV was observed between iRBD and the other two groups. The total prevalence of gallstones was 6.4% with no differences between the three groups. CONCLUSION: Almost a quarter of patients with PD in our cohort exhibited increased fGBV. This study illuminates a potentially overlooked topic in PD research and calls for more studies on biliary dysfunction.

Physical Activity Modulating Lipid Metabolism in Gallbladder Diseases.

Physical activity encompasses a series of overall benefits on cardiovascular health and metabolic disorders. Research has recently focused on the hepatobiliary tract, as an additional target of the health-related outcomes of different types of physical exercise. Here, we focus on the global features of physical activity with respect to exercise modality and intensity, and on studies linking physical activity to lipid metabolism, gallbladder diseases (gallstones, symptoms, complications and health-related quality of life), gallbladder motor-function, enterohepatic circulation of bile acids, and systemic metabolic inflammation. Additional studies need to unravel the pathophysiological mechanisms involved in both beneficial and harmful effects of physical activity in populations with different metabolic conditions.

Gallbladder and Biliary Disease in Pregnancy.

Diseases of the gallbladder and biliary tract are extremely common in developed nations. Because of the physiology of pregnancy, their incidence increases during gestation. This article represents a review of the existing literature on the entire spectrum of biliary disease. The physiology, clinical presentation, and diagnostic evaluation of a variety of conditions are reviewed. Historical and contemporary data regarding pregnancy implications and treatment options are discussed.

The association between eating difficulties and biliary sludge in the gallbladder in older adults with advanced dementia, at end of life.

OBJECTIVES: In clinical settings, untreatable biliary sludge in the gallbladder can be observed in older adults with advanced dementia. The underlying cause of biliary sludge existence in patients with dementia is currently unknown. Therefore, we aimed to investigate the prevalence, risk factors, and related outcomes of biliary sludge formation in the gallbladder of older adults with dementia. DESIGN: Cross-sectional study. SETTING: Geriatric ward of University Hospital in Japan. PARTICIPANTS: Inpatients aged 80 and older living with dementia. MEASUREMENTS: We evaluated the presence of biliary sludge by diagnostic ultrasonography and collected data regarding patient demographic information, cognition (mini-mental state examination [MMSE]), physical activity (Barthel Index), oral food intake (food intake level scale [FILS]), clinical stage of dementia (functional assessment staging [FAST] of dementia), and patient performance status (Zubrod/ Karnofsky score). RESULTS: Male sex, larger gallbladder volume and calories from oral intake were significantly associated with the presence of biliary sludge (P = .02, .02, .002, respectively). There was a significant negative correlation between the FAST stage and the FILS level in all patients (P < .001). More advanced dementia and dysphagia was more likely to be found in patients with Alzheimer disease (AD) with biliary sludge, compared to patients with AD without biliary sludge (FAST 7a, FILS II and FAST 6c, FILS V, respectively, P = .06, 04). A logistic regression analysis revealed that the eating status of FILS I and II, generally called "fasting or anorexia", was a significant risk factor for forming biliary sludge in older adults with dementia (P = .031, odds ratio: 5.25, 95% confidence interval: 1.16-23.72). CONCLUSIONS: Fasting status may be associated with the existence of biliary sludge in older adults with dementia. Therefore, supportive care for eating might be an important solution to comfortable end-of-life care for older adults with advanced dementia.

Investigation of adrenal and thyroid gland dysfunction in dogs with ultrasonographic diagnosis of gallbladder mucocele formation.

Gallbladder mucocele formation is an emerging disease in dogs characterized by increased secretion of condensed granules of gel-forming mucin by the gallbladder epithelium and formation of an abnormally thick mucus that can culminate in obstruction of the bile duct or rupture of the gallbladder. The disease is associated with a high morbidity and mortality and its pathogenesis is unknown. Affected dogs have a significantly increased likelihood of concurrent diagnosis of hyperadrenocorticism, hypothyroidism, and hyperlipidemia. Whether these endocrinopathies represent coincidental primary disease processes that exacerbate gallbladder mucocele formation in predisposed dogs or reflect a concurrent disruption of endocrine and lipid metabolism is unclear. In this study, we investigated a hypothesis that dogs with gallbladder mucocele formation would have a high prevalence of occult and atypical abnormalities in adrenal cortical and thyroid gland function that would suggest the presence of endocrine disruption and provide deeper insight into disease pathogenesis. We performed a case-control study of dogs with and without ultrasonographic diagnosis of gallbladder mucocele formation and profiled adrenal cortical function using a quantitative mass spectrometry-based assay of serum adrenal-origin steroids before and after administration of synthetic cosyntropin. We simultaneously profiled serum thyroid hormone concentrations and evaluated iodine sufficiency by measurement of urine iodine:creatinine ratios (UICR). The studies were complemented by histological examination of archival thyroid tissue and measurements of thyroid gland organic iodine from dogs with gallbladder mucocele formation and control dogs. Dogs with gallbladder mucocele formation demonstrated an exaggerated cortisol response to adrenal stimulation with cosyntropin. A prevalence of 10% of dogs with gallbladder mucocele formation met laboratory-based criteria for suspect or definitive diagnosis of hyperadrenocorticism. A significantly greater number of dogs with gallbladder mucocele formation had basal serum dehydroepiandrosterone (DHEAS) increases compared to control dogs. A high percentage of dogs with gallbladder mucocele formation (26%) met laboratory-based criteria for diagnosis of hypothyroidism, but lacked detection of anti-thyroglobulin antibodies. Dogs with gallbladder mucocele formation had significantly higher UICRs than control dogs. Examination of thyroid tissue from an unrelated group of dogs with gallbladder mucocele formation did not demonstrate histological evidence of thyroiditis or significant differences in content of organic iodine. These findings suggest that dogs with gallbladder mucocele formation have a greater capacity for cortisol synthesis and pinpoint DHEAS elevations as a potential clue to the underlying pathogenesis of the disease. A high prevalence of thyroid dysfunction with absent evidence for autoimmune thyroiditis suggest a disrupted thyroid hormone metabolism in dogs with gallbladder mucocele formation although an influence of non-thyroidal illness cannot be excluded. High UICR in dogs with gallbladder mucocele formation is of undetermined significance, but of interest for further study.

Systematic review with meta-analysis: cholecystectomy for biliary dyskinesia-what can the gallbladder ejection fraction tell us?

BACKGROUND: Gallbladder dyskinesia (gallbladder spasm, biliary dyskinesia or chronic acalculous cholecystitis) is a poorly defined entity which presents as biliary-type pain without any identifiable organic pathology. Abnormal gallbladder ejection fraction (GBEF) is used by some to select those likely to benefit from cholecystectomy. The validity of this approach has been questioned. AIM: To systematically review the literature and summarise the evidence surrounding the practice of cholecystectomy based on GBEF for gallbladder dyskinesia. METHODS: We conducted a systematic search of PubMed/MEDLINE and SCOPUS from 1980 to 2016 to identify the relevant literature. RESULTS: Twenty-nine studies including 2891 patients were included in the final analysis. In comparing cholecystectomy with medical management, patients with a normal GBEF did not benefit from cholecystectomy; whereas those with low GBEF had a higher chance (RR, relative risk = 2.37) of symptomatic improvement following surgery. When those classified as "low" and "normal" GBEF were compared in terms of outcome following cholecystectomy, the rate of improvement following surgery was similar in the two groups (RR 1.09) which suggests a placebo effect of surgery. CONCLUSIONS: While a low GBEF may provide some guidance in identifying those with gallbladder dyskinesia who may benefit from cholecystectomy, the available data are inconsistent and based on studies of poor quality which are often subject to bias and the impact of confounding factors. For these reasons, we conclude that the role of scintigraphy and cholecystectomy in the definition and management of this disorder remain unclear pending definitive study.

Effect of prednisolone administration on gallbladder emptying rate and gallbladder bile composition in dogs.

OBJECTIVE To investigate effects of prednisolone administration on gallbladder emptying rate and gallbladder bile composition in dogs. ANIMALS 6 healthy Beagles. PROCEDURES Prednisolone was administered (2 mg/kg, SC, once daily for 2 weeks) to each dog and tapered over 2 weeks. Gallbladder emptying rate and bile composition were evaluated before and after administration of prednisolone for 2 weeks as well as 1 week after cessation of prednisolone administration. RESULTS Gallbladder emptying rate decreased significantly after prednisolone administration (median, 27%; range, 0% to 38%), compared with rate before administration (median, 59%; range, 29% to 68%), but then increased 1 week after cessation of administration (median, 45%; range, 23% to 48%). Gallbladder bile mucin concentration decreased significantly after prednisolone administration (median, 8.8 mg/dL; range, 6.2 to 11.3 mg/dL), compared with concentration before administration (median, 13.1 mg/dL; range, 10.7 to 21.7 mg/dL), but then increased 1 week after cessation of administration (median, 14.3 mg/dL; range, 9.6 to 26.7 mg/dL). Gallbladder taurochenodeoxycholic acid concentration decreased significantly after prednisolone administration (8.1 mmol/L; range, 6.8 to 15.2 mmol/L), compared with concentration before administration (median, 27.2 mmol/L; range, 22.0 to 31.9 mmol/L), but then increased 1 week after cessation of administration (median, 26.4 mmol/L; range, 15.1 to 31.5 mmol/L). CONCLUSIONS AND CLINICAL RELEVANCE A lower gallbladder emptying rate caused by prednisolone administration may be involved in the pathogenesis of gallbladder disease in dogs. Further studies are required to determine the clinical importance of lower gallbladder bile mucin concentrations caused by glucocorticoid administration in the pathogenesis of gallbladder disease in dogs.

Scintigraphic Findings Beyond Ejection Fraction on Hepatobiliary Scintigraphy: Are They Correlated With Chronic Gallbladder Disease?

OBJECTIVE: To determine if classically reported findings associated with chronic cholecystitis on hepatobiliary scintigraphy (HBS), such as delayed small bowel (SB) transit, slow gallbladder (GB) filling, and reversal of the normal GB and SB transit, are associated with a depressed GB ejection fraction (GBEF). The secondary objectives were to determine whether GBEF is correlated with the time of GB filling, time of SB transit, or reversal of normal GB/SB filling sequence. We hypothesize that an association between a depressed GBEF and these classical HBS findings could validate them as surrogate markers for chronic GB disease. PATIENTS AND METHODS: We reviewed all HBS exams over a retrospective 16-month period. Data from 221 patients (mean age, 45.3 ± 15.2 years; 152 female and 69 male subjects) who underwent HBS with GBEF determination for evaluation of chronic symptoms concerning for biliary etiology met inclusion criteria. Classically reported findings for cholecystitis were recorded for each patient. Comparisons were made using t test and Fisher test analysis. RESULTS: Comparing exams with normal and abnormal GBEF values, there were no significant differences based on age, sex, GB fill time, normal versus delayed SB transit, and reversal of normal GB/SB filling sequence. Additionally, we did not see a correlation between the measured GBEF and GB fill time, SB transit time, or reversal of normal GB/SB filling sequence. CONCLUSIONS: Delayed SB transit, slow GB filling time, and reversal of the normal GB and SB filling sequence on HBS imaging are not associated with the measured GBEF and not predictive of chronic GB disease.

A comprehensive review on Primary gallbladder tuberculosis.

Tuberculosis (TB) is an infectious disease that can affect any organ system of the body. Abdominal TB can be gastrointestinal, lymph nodal, visceral or peritoneal. The gallbladder (GB) is rarely involved in abdominal TB as a primary organ. Extensive research literature on gallbladder TB is limited to case reports. There has been no review on this rare abdominal pathology. GB tuberculosis is a difficult diagnosis preoperatively. It is a rare differential among the more common gallbladder pathologies such as cholelithiasis, or a gallbladder malignancy. Typical histopathology of the resected specimen helps to establish this rare diagnosis. Subjecting every specimen to histopathological examination followed by medical treatment offers the chance of cure. Through this review, the authors attempt to provide an insight into this disease entity.

Gallbladder Dysfunction: Cholecystitis, Choledocholithiasis, Cholangitis, and Biliary Dyskinesia.

The prevalence of gallstones is 10% to 15% in adults. Individuals with acute cholecystitis present with right upper quadrant pain, fever, and leukocytosis. Management includes supportive care and cholecystectomy. The prevalence of choledocholithiasis is 10% to 20%, and serious complications include cholangitis and gallstone pancreatitis. The goal of management in individuals with choledocholithiasis consists of clearing common bile duct stones. Acute ascending cholangitis is a life-threatening condition involving acute inflammation and infection of the common bile duct. Treatment includes intravenous fluids, analgesia, intravenous antibiotics, and biliary drainage and decompression. Biliary dyskinesia includes motility disorders resulting in biliary colic in the absence of gallstones.

[Expression profile of long non-coding RNA in non-alcoholic fatty liver disease].

OBJECTIVE: To analyze the characteristics of long non-coding RNA (lncRNA) expression in non-alcoholic fatty liver disease (NAFLD).
 Methods: lncRNA-mRNA microarray was conducted on the liver tissue samples from 10 patients with simple gallbladder stone (5 NAFLD liver samples and 5 normal liver samples), and the differentially expressed lncRNA was analyzed by bioinformatics technology.
 Results: Compared with the normal liver samples, there were abnormal expression of 1 735 lncRNAs and 1 485 mRNAs in NAFLD liver samples. Among them, 535 lncRNAs and 760 mRNAs were up-regulated, 1 200 lncRNAs and 725 mRNAs were down-regulated.
 Conclusion: Compared with normal liver, the expression of lncRNA in NAFLD tissues is obviously abnormal. These lncRNAs may play an important role in the occurrence and development of NAFLD.

Bile acid composition of gallbladder contents in dogs with gallbladder mucocele and biliary sludge.

OBJECTIVE To examine bile acid composition of gallbladder contents in dogs with gallbladder mucocele and biliary sludge. ANIMALS 18 dogs with gallbladder mucocele (GBM group), 8 dogs with immobile biliary sludge (i-BS group), 17 dogs with mobile biliary sludge (m-BS group), and 14 healthy dogs (control group). PROCEDURES Samples of gallbladder contents were obtained by use of percutaneous ultrasound-guided cholecystocentesis or during cholecystectomy or necropsy. Concentrations of 15 bile acids were determined by use of highperformance liquid chromatography, and a bile acid compositional ratio was calculated for each group. RESULTS Concentrations of most bile acids in the GBM group were significantly lower than those in the control and m-BS groups. Compositional ratio of taurodeoxycholic acid, which is 1 of 3 major bile acids in dogs, was significantly lower in the GBM and i-BS groups, compared with ratios for the control and m-BS groups. The compositional ratio of taurocholic acid was significantly higher and that of taurochenodeoxycholic acid significantly lower in the i-BS group than in the control group. CONCLUSIONS AND CLINICAL RELEVANCE In this study, concentrations and fractions of bile acids in gallbladder contents were significantly different in dogs with gallbladder mucocele or immobile biliary sludge, compared with results for healthy control dogs. Studies are needed to determine whether changes in bile acid composition are primary or secondary events of gallbladder abnormalities.

Clinical Relationship between Cholestatic Disease and Pituitary-Dependent Hyperadrenocorticism in Dogs: A Retrospective Case Series.

BACKGROUND: A high prevalence of cholestatic disease, including gallbladder mucocele (GBM), has been reported in dogs with naturally occurring pituitary-dependent hyperadrenocorticism (PDH). HYPOTHESIS/OBJECTIVES: Differences exist in the clinical features of dogs with PDH and concurrent cholestatic disease, and also is the management of these dogs with trilostane. ANIMALS: Sixty-five client-owned dogs with naturally occurring PDH. METHODS: This was a retrospective, observational case series. Each dog was treated with trilostane for at least 3 months before the study, and had a good clinical response, as determined by owners. Statistical comparisons of clinical signs, results of routine blood tests, basal and post-ACTH cortisol concentration, and optimal trilostane dosage were made after dogs were separated into the following 3 groups by ultrasonographic imaging: normal on ultrasound (NOU) group, cholestasis group, and GBM group. RESULTS: The GBM group had more severe clinical signs and significantly different total serum cholesterol concentration and post-ACTH stimulation cortisol concentration at the time of diagnosis. Dogs that weighed <6 kg had a significantly higher prevalence of cholestatic disease than did the other dogs (P = .003). The optimal trilostane dosages for the GBM and cholestasis groups were 2.5 and 1.5 times the dosage of the NOU group, respectively (P < .001). CONCLUSIONS AND CLINICAL IMPORTANCE: Gallbladder disease associated with cholestatic disease is correlated with PDH in dogs, in both its clinical features and drug management. These findings may be associated with hypercholesterolemia, unidentified genetic factors, and the hydrophobic nature of trilostane.

Colesterolosis vesicular: algunos aspectos de su fisiopatología / Vesicular cholesterolosis: some aspects of its pathophysiology

Gallbladder cholesterolosis is a nosological clinical entity where the central element is the deposit of lipids in immune cells that reside under the gallbladder epithelium. The mechanisms involved in its development are not entirely clear, but they seem to have some resemblances that are observed in the wall of the arteries with atherosclerosis. The lipid-laden cells observed in the gallbladder wall appear to share many of the characteristics of atherosclerosis foam cells, which by means of scavenger receptors have endocited oxidized low-density lipoproteins and accumulate them in their cytoplasm. Foam cells, in themselves, are not dangerous, but in atherosclerosis at least they can become a problem when they are located in vessels and specific anatomic sites. The role they may have in the gallbladder is not known to date. We will review some considerations that seem relevant to us to elucidate if these entities share the same protagonist: macrophages transformed by modified lipids. (AU)

Functions of the Gallbladder.

The gallbladder stores and concentrates bile between meals. Gallbladder motor function is regulated by bile acids via the membrane bile acid receptor, TGR5, and by neurohormonal signals linked to digestion, for example, cholecystokinin and FGF15/19 intestinal hormones, which trigger gallbladder emptying and refilling, respectively. The cycle of gallbladder filling and emptying controls the flow of bile into the intestine and thereby the enterohepatic circulation of bile acids. The gallbladder also largely contributes to the regulation of bile composition by unique absorptive and secretory capacities. The gallbladder epithelium secretes bicarbonate and mucins, which both provide cytoprotection against bile acids. The reversal of fluid transport from absorption to secretion occurs together with bicarbonate secretion after feeding, predominantly in response to an adenosine 3',5'-cyclic monophosphate (cAMP)-dependent pathway triggered by neurohormonal factors, such as vasoactive intestinal peptide. Mucin secretion in the gallbladder is stimulated predominantly by calcium-dependent pathways that are activated by ATP present in bile, and bile acids. The gallbladder epithelium has the capacity to absorb cholesterol and provides a cholecystohepatic shunt pathway for bile acids. Changes in gallbladder motor function not only can contribute to gallstone disease, but also subserve protective functions in multiple pathological settings through the sequestration of bile acids and changes in the bile acid composition. Cholecystectomy increases the enterohepatic recirculation rates of bile acids leading to metabolic effects and an increased risk of nonalcoholic fatty liver disease, cirrhosis, and small-intestine carcinoid, independently of cholelithiasis. Among subjects with gallstones, cholecystectomy remains a priority in those at risk of gallbladder cancer, while others could benefit from gallbladder-preserving strategies. © 2016 American Physiological Society. Compr Physiol 6:1549-1577, 2016.

Laparoscopic Cholecystectomy for Gallbladder Calculosis in Fibromyalgia Patients: Impact on Musculoskeletal Pain, Somatic Hyperalgesia and Central Sensitization.

Fibromyalgia, a chronic syndrome of diffuse musculoskeletal pain and somatic hyperalgesia from central sensitization, is very often comorbid with visceral pain conditions. In fibromyalgia patients with gallbladder calculosis, this study assessed the short and long-term impact of laparoscopic cholecystectomy on fibromyalgia pain symptoms. Fibromyalgia pain (VAS scale) and pain thresholds in tender points and control areas (skin, subcutis and muscle) were evaluated 1week before (basis) and 1week, 1,3,6 and 12months after laparoscopic cholecystectomy in fibromyalgia patients with symptomatic calculosis (n = 31) vs calculosis patients without fibromyalgia (n. 26) and at comparable time points in fibromyalgia patients not undergoing cholecystectomy, with symptomatic (n = 27) and asymptomatic (n = 28) calculosis, and no calculosis (n = 30). At basis, fibromyalgia+symptomatic calculosis patients presented a significant linear correlation between the number of previously experienced biliary colics and fibromyalgia pain (direct) and muscle thresholds (inverse)(p<0.0001). After cholecystectomy, fibromyalgia pain significantly increased and all thresholds significantly decreased at 1week and 1month (1-way ANOVA, p<0.01-p<0.001), the decrease in muscle thresholds correlating linearly with the peak postoperative pain at surgery site (p<0.003-p<0.0001). Fibromyalgia pain and thresholds returned to preoperative values at 3months, then pain significantly decreased and thresholds significantly increased at 6 and 12months (p<0.05-p<0.0001). Over the same 12-month period: in non-fibromyalgia patients undergoing cholecystectomy thresholds did not change; in all other fibromyalgia groups not undergoing cholecystectomy fibromyalgia pain and thresholds remained stable, except in fibromyalgia+symptomatic calculosis at 12months when pain significantly increased and muscle thresholds significantly decreased (p<0.05-p<0.0001). The results of the study show that biliary colics from gallbladder calculosis represent an exacerbating factor for fibromyalgia symptoms and that laparoscopic cholecystectomy produces only a transitory worsening of these symptoms, largely compensated by the long-term improvement/desensitization due to gallbladder removal. This study provides new insights into the role of visceral pain comorbidities and the effects of their treatment on fibromyalgia pain/hypersensitivity.

A Newly-Discovered Mutation in the RFX6 Gene of the Rare Mitchell-Riley Syndrome.

Mitchell-Riley syndrome is a genetic disorder characterized by neonatal diabetes, pancreatic hypoplasia, intestinal atresia and/or malrotation, biliary atresia, and gallbladder aplasia or hypoplasia. It was considered a variant of the Martinez-Frias syndrome with similar phenotypic characteristics, except for neonatal diabetes and tracheoesophageal fistula. However, the genetic mutation in (regulatory factor X on chromosome 6) RFX6 was only detected in babies who had diabetes, making it different from the previously known mutations for the disease. This is the first reported case of a classical Mitchell-Riley syndrome in the Arab peninsula along with additional features and novel mutations in the RFX6 gene.

The role of cholescintigraphy in demonstrating delayed post prandial gallbladder motility in cirrhotic patients.

OBJECTIVE: The purpose of this study was to investigate the postprandial gallbladder motility of cirrhotic patients using cholescintigraphy by technetium-99m-ethylhydroiminodiacetic acid ((99m)Tc-EHIDA). SUBJECTS AND METHODS: Sixty two cirrhotic patients (Child-Pugh A: 28 patients; Child-Pugh B: 21 and Child-Pugh C: 13 patients) and 24 normal subjects were included in this study. All patients underwent cholescintigraphy. Mean gallbladder ejection fraction (GBEF) and mean ejection period (EP) were acquired by the region of interest method. In order to evaluate gallbladder contractility of cirrhotic patients, their mean GBEF and EP were compared with the same tests of normal subjects using an independent sample t test. RESULTS: The mean GBEF of cirrhotic patients was lower and their mean ER was longer than that of normal subjects. The means of GBEF and EP of cirrhotic patients were different among different Child-Pugh grade groups. All these differences showed statistical significance. CONCLUSIONS: This study suggests that post prandial cholescintigraphy by (99m)Tc EHIDA demonstrated slower gallbladder motility in cirrhotic patients. The ejection period of cholescintigraphy by (99m)Tc-EHIDA can be used as an index of abnormal gallbladder motility.