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Medicina (Kaunas, Lithuania) Jan 2023: Although laparoscopic cholecystectomy is the preferred treatment method in patients who experience typical biliary pain with or without gallstones, medical treatment... (Observational Study)
Observational Study
: Although laparoscopic cholecystectomy is the preferred treatment method in patients who experience typical biliary pain with or without gallstones, medical treatment has not been extensively studied. Rowachol is a potent choleretic agent, comprising six cyclic monoterpenes. This study aimed to investigate the clinical improvement and changes in gallbladder ejection fraction (GBEF) by Rowachol treatment in patients with typical biliary pain. : We retrospectively reviewed 138 patients with typical biliary pain who underwent cholescintigraphy from July 2016 to April 2022. We included patients who received Rowachol for more than 2 months and underwent follow-up GBEF measurements. Finally, we analyzed pre- and post-treatment symptoms and GBEF. GBEF was calculated using the fatty meal-stimulated cholescintigraphy. : This retrospective observational study included 31 patients; their median age was 46.0 (range, 26.0-72.7) years, and 22 (71.0%) were female. Overall, 9 (29.0%) patients had gallbladder stones or sludges (maximum size: 2 mm) on initial transabdominal ultrasonography. During a median follow-up of 23.3 months, the symptoms of 21 (67.7%) patients were resolved after a median Rowachol treatment of 10.0 months. The mean GBEF was significantly improved after Rowachol treatment (initial cholescintigraphy: 42.6% ± 16.2%; follow-up cholescintigraphy: 53.0% ± 18.1%, = 0.012). In patients with a GBEF ≤35% (n = 9), Rowachol significantly increased the GBEF from 21.3% ± 8.3% to 49.1% ± 20.7% ( = 0.008). : Rowachol may have beneficial medical effects that can improve gallbladder dysfunction and treatment response.
Topics: Humans; Female; Middle Aged; Male; Retrospective Studies; Stroke Volume; Gallbladder Diseases; Monoterpenes; Pain
PubMed: 36676729
DOI: 10.3390/medicina59010105 -
Mathematical Biosciences and... Sep 2022This study explored the chemical and pharmacological mechanisms of Shao Yao Gan Cao decoction (SYGC) in the treatment of Sphincter of Oddi Dysfunction (SOD) through...
This study explored the chemical and pharmacological mechanisms of Shao Yao Gan Cao decoction (SYGC) in the treatment of Sphincter of Oddi Dysfunction (SOD) through ultra-high-performance liquid chromatography coupled with Quadrupole Exactive-Orbitrap high-resolution mass spectrometry (UHPLC-Q Exactive-Orbitrap HR-MS), network pharmacology, transcriptomics, molecular docking and in vivo experiments. First, we identified that SYGC improves SOD in guinea pigs by increased c-kit expression and decreased inflammation infiltration and ring muscle disorders. Then, a total of 649 SOD differential genes were found through RNA sequencing and mainly enriched in complement and coagulation cascades, the B cell receptor signaling pathway and the NF-kappa B signaling pathway. By combining UHPLC-Q-Orbitrap-HRMS with a network pharmacology study, 111 chemicals and a total of 52 common targets were obtained from SYGC in the treatment of SOD, which is also involved in muscle contraction, the B cell receptor signaling pathway and the complement system. Next, 20 intersecting genes were obtained among the PPI network, MCODE and ClusterOne analysis. Then, the molecular docking results indicated that four active compounds (glycycoumarin, licoflavonol, echinatin and homobutein) and three targets (AURKB, KIF11 and PLG) exerted good binding interactions, which are also related to the B cell receptor signaling pathway and the complement system. Finally, animal experiments were conducted to confirm the SYGC therapy effects on SOD and verify the 22 hub genes using RT-qPCR. This study demonstrates that SYGC confers therapeutic effects against an experimental model of SOD via regulating immune response and inflammation, which provides a basis for future research and clinical applications.
Topics: Animals; Guinea Pigs; Glycyrrhiza uralensis; Network Pharmacology; Tandem Mass Spectrometry; Molecular Docking Simulation; Sphincter of Oddi Dysfunction; Inflammation; Receptors, Antigen, B-Cell
PubMed: 36654051
DOI: 10.3934/mbe.2022626 -
Frontiers in Cellular and Infection... 2022Biliary calculi, a common benign disease of the gastrointestinal tract, are affected by multiple factors, including diet, lifestyle, living environment, and personal and...
OBJECTIVE
Biliary calculi, a common benign disease of the gastrointestinal tract, are affected by multiple factors, including diet, lifestyle, living environment, and personal and genetic background. Its occurrence is believed to be related to a change in biliary microbiota. Approximately 10%-20% of symptomatic patients with cholecystolithiasis have choledocholithiasis, resulting in infection, abdominal pain, jaundice, and biliary pancreatitis. This study aimed to determine whether a dysfunction in the sphincter of Oddi, which controls the outflow of bile and separates the bile duct from the intestine, leads to a change in biliary microbiota and the occurrence of biliary calculi.
METHODS
Forty patients with cholecystolithiasis and choledocholithiasis were prospectively recruited. Bile specimens were obtained, and biliary pressure was measured during and after surgery. The collected specimens were analyzed with 16S rRNA gene to characterize the biliary microbiota. The risk factors of common bile duct calculi were analyzed numerically combined with the pressure in the sphincter of Oddi.
RESULTS
Different biliary microbiota were found in all cases. Patients with sphincter of Oddi dysfunction had significantly increased biliary microbiota as well as significantly higher level of systemic inflammation than patients with normal sphincter of Oddi.
CONCLUSIONS
The systemic inflammatory response of patients with sphincter of Oddi dysfunction is more severe, and their microbial community significantly differs from that of patients with normal sphincter of Oddi, which makes biliary tract infection more likely; furthermore, the biliary tract of patients with sphincter of Oddi dysfunction has more gallstone-related bacterial communities.
Topics: Humans; Gallstones; Choledocholithiasis; Sphincter of Oddi Dysfunction; RNA, Ribosomal, 16S; Sphincter of Oddi; Common Bile Duct Diseases; Biliary Tract
PubMed: 36569207
DOI: 10.3389/fcimb.2022.1001441 -
The American Journal of Case Reports Nov 2022BACKGROUND Langerhans cell histiocytosis (LCH) is a rare condition caused by a neoplastic proliferation of myeloid cells. It can present as a single-system or...
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare condition caused by a neoplastic proliferation of myeloid cells. It can present as a single-system or multi-system disorder. Worse prognosis is associated with the hematopoietic system (spleen, liver, bone marrow), which is routinely observed in multi-system disease. Because of the varied presentation of this disorder, diagnosis can be difficult, and therefore suitable treatment can be delayed. CASE REPORT We report a case of hepatic LCH in a 51-year-old man who presented with epigastric abdominal pain, with imaging demonstrating a hepatic nodule. A low ejection fraction on hepatobiliary iminodiacetic acid scan suggested chronic cholecystitis. Therefore, the patient underwent a cholecystectomy for biliary dyskinesia, in which liver nodules were noted, and biopsies were taken. The biopsies demonstrated characteristic findings of LCH along with positive immunohistochemical markers and negative BRAF V600E mutation. Radiologic and pathologic findings were consistent with LCH within the liver, associated with bile duct injury and mild biliary obstruction. The patient was placed on a cladribine regimen. His abdominal pain improved. CONCLUSIONS LCH limited to the liver is uncommon and can appear as chronic biliary disease, as was suspected in this case. Despite the poor prognosis of hematopoietic LCH, early recognition can lead to better outcome and chemotherapy susceptibility. This patient was most likely in the first stage of liver LCH, given his presentation, which could have aided his response to chemotherapy. The lack of BRAF V600E mutation could have contributed to a positive prognosis and more possibilities for treatment.
Topics: Male; Humans; Middle Aged; Proto-Oncogene Proteins B-raf; Mutation; Histiocytosis, Langerhans-Cell; Liver; Abdominal Pain
PubMed: 36404611
DOI: 10.12659/AJCR.937628 -
Cureus Sep 2022Background Biliary dyskinesia is a functional gallbladder disorder in which there is an absence of a structural or mechanical cause for biliary pain. A...
Background Biliary dyskinesia is a functional gallbladder disorder in which there is an absence of a structural or mechanical cause for biliary pain. A cholecystokinin-hepatobiliary iminodiacetic acid (CCK-HIDA) scan is typically performed during workup, and cholecystectomy is the accepted treatment for low ejection fraction (EF) (less than 33%, as defined by the literature). However, few studies have examined the role of cholecystectomy in hyperkinetic gallbladder (EF ≥80%). The aim of our study was to examine symptom resolution following minimally invasive cholecystectomy in patients with hyperkinetic gallbladder. Methodology A retrospective chart review was conducted at Robert Packer Hospital in Sayre, PA. Patients who underwent minimally invasive cholecystectomy for biliary colic with EF ≥80% and who were without cholelithiasis on preoperative imaging or on final pathology were included in this study. The main outcome was symptom resolution at the postoperative visit. Data collected included age, gender, EF, body mass index, symptoms with CCK infusion, and pathology. Results A total of 48 patients were included. The mean age of patients was 41.2 years (standard deviation = 14.4), and the median age of patients was 42.2 years, with a range of 17-71 years. The majority of patients were female (83.3%). Overall, 58.3% of patients had replication of symptoms with CCK infusion. The mean gallbladder EF was 87.3%, with a median of 87.0 and a range of 80-98. In total, 33 (68.8%) patients had chronic cholecystitis on final pathology reports. There was a 95.9% symptom resolution rate among our patients two weeks postoperatively. Conclusions The overwhelming majority of patients experienced symptom resolution prior to their two-week postoperative visit following minimally invasive cholecystectomy for hyperkinetic gallbladder. These results strongly suggest a role of surgical management in patients with high EF.
PubMed: 36340559
DOI: 10.7759/cureus.29778 -
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 Clinical Medicine Jun 2022Refractory functional dyspepsia (RFD) is diagnosed when symptoms persist for at least 6 months despite at least two medical treatments. No consensus treatment guidelines...
Refractory functional dyspepsia (RFD) is diagnosed when symptoms persist for at least 6 months despite at least two medical treatments. No consensus treatment guidelines exist. The implicated causes of functional biliary dyspepsia are a narrowed cystic duct, Sphincter of Oddi dysfunction, microlithiasis, and gallbladder dyskinesia. We investigated the treatment effects of litholytic agents. RFD patients were prospectively enrolled in six tertiary medical centers. All subjects took chenodeoxycholic and ursodeoxycholic acids (CNU) twice daily for 12 weeks. We monitored their medication adherence, laboratory results, and complications. The 7-point global symptom scale test scores were determined before and after treatment. Of the 52 patients who were prospectively screened, 37 were included in the final analysis. The mean age was 51.3 years: 14 were males, and 23 were females. Before treatment, the mean number and duration of symptoms were 2.4 and 48.2 months, and a mean of 3.3 FD-related drugs were taken. The mean CNU adherence was 95.3%. The mean global symptom scale score decreased from 5.6 pretreatment to 2.6 posttreatment. The symptom improvement rate was 94.6% (35 out of 37 patients). The only adverse event was mild diarrhea (10.8%) that was resolved after conservative management. Conclusions: CNU improved the symptoms of RFD patients who did not respond to conventional medications. Litholytic agents are good treatment options for patients with RFD and biliary dyspepsia secondary to biliary microlithiasis. Further prospective, large-scale mechanistic studies are warranted.
PubMed: 35683573
DOI: 10.3390/jcm11113190 -
European Journal of Human Genetics :... Aug 2022The birth prevalence of laterality defects is about 1.1/10,000 comprising different phenotypes ranging from situs inversus totalis to heterotaxy, mostly associated with...
The birth prevalence of laterality defects is about 1.1/10,000 comprising different phenotypes ranging from situs inversus totalis to heterotaxy, mostly associated with complex congenital heart defects (CHD) and situs abnormalities such as intestinal malrotation, biliary atresia, asplenia, or polysplenia. A proportion of laterality defects arise in the context of primary ciliary dyskinesia (PCD) accompanied by respiratory symptoms or infertility. In this study, exome sequencing (ES) was performed in 14 case-parent trios/quattros with clinical exclusion of PCD prior to analysis. Moreover, all cases and parents underwent detailed clinical phenotyping including physical examination, echocardiography by a skilled paediatric cardiologist and abdominal ultrasound examinations not to miss mildly affected individuals. Subsequent survey of the exome data comprised filtering for monoallelic de novo, rare biallelic, and X-linked recessive variants. In two families, rare variants of uncertain significance (VUS) in PKD1L1 and ZIC3 were identified. Both genes have been associated with laterality defects. In two of the remaining families, biallelic variants in LMBRD1 and DNAH17, respectively, were prioritized. In another family, an ultra-rare de novo variant in WDR47 was found. Extensive exome survey of 2,109 single exomes of individuals with situs inversus totalis, heterotaxy, or isolated CHD identified two individuals with novel monoallelic variants in WDR47, but no further individuals with biallelic variants in DNAH17 or LMBRD1. Overall, ES of 14 case-parent trios/quattros with cardiovascular laterality defects identified rare VUS in two families in known disease-associated genes PKD1L1 and ZIC3 and suggests DNAH17, LMBRD1, and WDR47 as potential genes involved in laterality defects.
Topics: Exome; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Membrane Proteins; Nucleocytoplasmic Transport Proteins; Phenotype; Situs Inversus; Exome Sequencing
PubMed: 35474353
DOI: 10.1038/s41431-022-01100-2