-
Frontiers in Endocrinology 2024Hypertriglyceridemia (HTG) is a complex disorder caused by genetic and environmental factors that frequently results from loss-of-function variants in the gene encoding...
INTRODUCTION
Hypertriglyceridemia (HTG) is a complex disorder caused by genetic and environmental factors that frequently results from loss-of-function variants in the gene encoding lipoprotein lipase (LPL). Heterozygous patients have a range of symptoms, while homozygous LPL deficiency presents with severe symptoms including acute pancreatitis, xanthomas, and lipemia retinalis.
METHODS
We described the clinical characteristics of three Slovenian patients (an 8-year-old female, an 18-year-old man, and a 57-year-old female) and one Pakistani patient (a 59-year-old male) with LPL deficiency. We performed next-generation sequencing (NGS) targeting all coding exons and intron-exon boundaries of the gene, and Sanger sequencing for variant confirmation. In addition, we performed a systematic literature review of all cases with three identified variants and described their clinical characteristics.
RESULTS
Two Slovenian patients with a heterozygous pathogenic variant NM_000237.3:c.984G>T (p.Met328Ile) were diagnosed within the first three years of life and had triglyceride (TG) values of 16 and 20 mmol/L. An asymptomatic Pakistani patient with TG values of 36.8 mmol/L until the age of 44 years, was identified as heterozygous for a pathogenic variant NM_000237.3:c.724G>A (p.Asp242Asn). His TG levels dropped to 12.7 mmol/L on dietary modifications and by using fibrates. A Slovenian patient who first suffered from pancreatitis at the age of 18 years with a TG value of 34 mmol/L was found to be homozygous for NM_000237.3:c.337T>C (p.Trp113Arg).
CONCLUSIONS
Patients with LPL deficiency had high TG levels at diagnosis. Homozygous patients had worse outcomes. Good diet and medication compliance can reduce severity.
Topics: Humans; Male; Female; Slovenia; Adolescent; Middle Aged; Lipoprotein Lipase; Child; Pakistan; Hyperlipoproteinemia Type I; Mutation
PubMed: 38911039
DOI: 10.3389/fendo.2024.1387419 -
Cureus Oct 2023Endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy (EST) has been proven efficacious in the removal of CBD stones. Even after... (Review)
Review
Preventive Saline Irrigation of the Bile Duct to Reduce the Rate of Residual Common Bile Duct Stones Without Intraductal Ultrasonography: A Systematic Review and Meta-Analysis.
Endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy (EST) has been proven efficacious in the removal of CBD stones. Even after endoscopic stone removal, recurring cholangitis due to a residual common bile duct (CBD) stone is prevalent in clinical practice with a residual recurrence rate of 4-24% after successful stone retrieval. This comprehensive study and meta-analysis aimed to determine if preventive saline irrigation of the bile duct (PSIB) reduces the amount of residual CBD stones. Through a comprehensive search of PubMed, EMBASE, Cochrane Library, and Web of Science until November 20, 2022, we identified 164 articles comparing the efficacy of PSIB and non-PSIB post-endoscopic CBD stone removal. After stringent selection, three studies were included for meta-analysis using ReviewManager (ReVman version 5.4.1; Cochrane, London, UK). Using a random effect (RE) model, we derived a pooled odds ratio (OR) with confidence interval (CI) (95%CI). A total of three studies have been included in the analysis. Out of which, two are randomized controlled trials (RCTs) and one is a non-randomized study. Out of 323 patients, 157 underwent PSIB after an endoscopic stone removal of CBD stones to reduce the residual of CBD stones, whereas 166 did not undergo saline irrigation (non-PSIB). In our analysis, PSIB significantly reduced the risk of residual stones (OR: 0.22, 95%CI: 0.11-0.45). However, there was no notable link between PSIB and post-irrigation cholangitis (OR: 1.08, 95%CI: 0.21-2.21). Although not statistically significant, PSIB showed a trend toward lowered risks of post-procedural pancreatitis (OR: 0.65), bleeding (OR: 0.68), and other complications (OR: 0.64). PSIB effectively reduces residual CBD stones after endoscopy, offering a cost-effective alternative to invasive procedures such as intraductal ultrasound (IDUS). However, larger RCTs are needed to validate its definitive role.
PubMed: 38021516
DOI: 10.7759/cureus.46720 -
Cureus Apr 2024Chronic pancreatitis (CP), an inflammatory disease characterized by irreversible pancreatic changes and progressive fibrosis, significantly impairs patients' quality of... (Review)
Review
Chronic pancreatitis (CP), an inflammatory disease characterized by irreversible pancreatic changes and progressive fibrosis, significantly impairs patients' quality of life. This systematic review aims to assess the efficacy of antioxidant therapy in enhancing the quality of life of CP patients. Focusing on the role of oxidative stress in CP pathogenesis, we explored several databases for studies evaluating the impact of antioxidant supplementation. The review included randomized controlled trials and cohort studies reporting pain frequency, intensity, and overall quality of life measures. Findings from these studies present a mixed view of the efficacy of antioxidants in CP, with some suggesting benefits in symptom management, while others show inconsistency in improving patient outcomes. The review concludes that while antioxidant therapy holds potential, especially in symptom alleviation, there is a need for more rigorous, larger-scale studies to confirm its effectiveness in CP management and to establish standardized treatment protocols. The incorporation of antioxidants into CP treatment plans should be approached with personalized care, considering the varied responses observed in different patient populations.
PubMed: 38694657
DOI: 10.7759/cureus.57402 -
Frontiers in Endocrinology 2024[This corrects the article DOI: 10.3389/fendo.2023.1214334.].
[This corrects the article DOI: 10.3389/fendo.2023.1214334.].
PubMed: 38549766
DOI: 10.3389/fendo.2024.1342543 -
Scientific Reports Mar 2024Endoscopic Retrograde Cholangiopancreatography (ERCP) is the primary therapeutic procedure for pancreaticobiliary disorders, and studies highlighted the impact of... (Meta-Analysis)
Meta-Analysis
Endoscopic Retrograde Cholangiopancreatography (ERCP) is the primary therapeutic procedure for pancreaticobiliary disorders, and studies highlighted the impact of papilla anatomy on its efficacy and safety. Our objective was to quantify the influence of papilla morphology on ERCP outcomes. We systematically searched three medical databases in September 2022, focusing on studies detailing the cannulation process or the rate of adverse events in the context of papilla morphology. The Haraldsson classification served as the primary system for papilla morphology, and a pooled event rate with a 95% confidence interval was calculated as the effect size measure. Out of 17 eligible studies, 14 were included in the quantitative synthesis. In studies using the Haraldsson classification, the rate of difficult cannulation was the lowest in type I papilla (26%), while the highest one was observed in the case of type IV papilla (41%). For post-ERCP pancreatitis, the event rate was the highest in type II papilla (11%) and the lowest in type I and III papilla (6-6%). No significant difference was observed in the cannulation failure and post-ERCP bleeding event rates between the papilla types. In conclusion, certain papilla morphologies are associated with a higher rate of difficult cannulation and post-ERCP pancreatitis.
Topics: Humans; Cholangiopancreatography, Endoscopic Retrograde; Catheterization; Ampulla of Vater; Sphincterotomy, Endoscopic; Pancreatitis
PubMed: 38538734
DOI: 10.1038/s41598-024-57758-9 -
Clinical Hematology International 2023Asparaginase-associated pancreatitis complicates 2-10% of patients treated for acute lymphoblastic leukemia, causing morbidity and discontinuation of asparaginase...
Asparaginase-associated Pancreatitis Complicated by Pancreatic Fluid Collection Treated with Endoscopic Cistogastrostomy in Pediatric Acute Lymphoblastic Leukemia: A Case Report and Systematic Review of the Literature.
Asparaginase-associated pancreatitis complicates 2-10% of patients treated for acute lymphoblastic leukemia, causing morbidity and discontinuation of asparaginase administration. Among acute complications, pancreatic fluid collections can be managed conservatively, but intervention is indicated when associated with persistent insulin therapy need and recurrent abdominal pain. Endoscopic treatment has become the standard approach in adult patients, with increasing favorable evidence in children. This work compares the characteristics of a pediatric oncology patient treated at our institution with reported literature experiences, showing feasibility, safety and effectiveness of endoscopic approach.
PubMed: 38817959
DOI: 10.46989/001c.90958 -
World Journal of Gastrointestinal... Aug 2023Post-operative pancreatic fistula (POPF) is the primary cause of morbidity following pancreaticoduodenectomy. Rates of POPF have remained high despite well known risk...
BACKGROUND
Post-operative pancreatic fistula (POPF) is the primary cause of morbidity following pancreaticoduodenectomy. Rates of POPF have remained high despite well known risk factors. The theory that hypoperfusion of the pancreatic stump leads to anastomotic failure has recently gained interest.
AIM
To define the published literature with regards to intraoperative pancreas perfusion assessment and its correlation with POPF.
METHODS
A systematic search of available literature was performed in November 2022. Data extracted included study characteristics, method of assessment of pancreas stump perfusion, POPF and other post-pancreatic surgery specific complications.
RESULTS
Five eligible studies comprised two prospective non-randomised studies and three case reports, total 156 patients. Four studies used indocyanine green fluorescence angiography to assess the pancreatic stump, with the remaining study assessing pancreas perfusion by visual inspection of arterial bleeding of the pancreatic stump. There was significant heterogeneity in the definition of POPF. Studies had a combined POPF rate of 12%; intraoperative perfusion assessment revealed hypoperfusion was present in 39% of patients who developed POPF. The rate of POPF was 11% in patients with no evidence of hypoperfusion and 13% in those with evidence of hypoperfusion, suggesting that not all hypoperfusion gives rise to POPF and further analysis is required to analyse if there is a clinically relevant cut off. Significant variance in practice was seen in the pancreatic stump management once hypoperfusion was identified.
CONCLUSION
The current published evidence around pancreas perfusion during pancreaticoduodenectomy is of poor quality. It does not support a causative link between hypoperfusion and POPF. Further well-designed prospective studies are required to investigate this.
PubMed: 37701689
DOI: 10.4240/wjgs.v15.i8.1799 -
Frontiers in Medicine 2024This study aimed to systematically evaluate the efficacy and safety of the double-guidewire technique along with other methods (persistent standard cannulation...
OBJECTIVE
This study aimed to systematically evaluate the efficacy and safety of the double-guidewire technique along with other methods (persistent standard cannulation techniques, transpancreatic sphincterotomy, and pancreatic stent-assisted technique) for difficult biliary cannulation.
METHODS
Two researchers searched for literature on the efficacy and safety of the double-guidewire technique and other techniques in difficult biliary cannulation in databases, including PubMed, Embase, Cochrane, China National Knowledge Infrastructure, and Wanfang Data, based on the inclusion and exclusion criteria. The success rate of cannulation, duration of cannulation, post-ERCP pancreatitis, and overall postoperative complications were also analyzed using RevMan 5.4 software.
RESULTS
In total, 20 randomized controlled trial (RCT) studies involving 2008 participants were identified. The success rate of cannulation in the double-guidewire technique was much higher than that in persistent standard cannulation techniques [RR = 1.37, 95%CI (1.05, 1.79), = 0.02]. However, it was lower than the success rate observed with transpancreatic sphincterotomy [RR = 0.89, 95%CI (0.81, 0.97), = 0.01]. There was no significance in post-ERCP pancreatitis [RR = 1.09, 95% CI (0.85, 1.40), = 0.49], overall postoperative complications [RR = 0.90, 95% CI (0.56, 1.45), = 0.66], and duration of cannulation [SMD = -0.14, 95%C I (-1.43, 1.15), = 0.83] between the double-guidewire technique and other techniques.
CONCLUSION
This study demonstrated that the success rate of cannulation ranged from transpancreatic sphincterotomy to the double-guidewire technique and then to persistent standard cannulation techniques.
PubMed: 38716417
DOI: 10.3389/fmed.2024.1344644 -
Frontline Gastroenterology Mar 2024Most studies have assessed the impact of elevated serum amylase levels in clinical practice, but only a few have investigated the significance of low serum amylase. We...
OBJECTIVE
Most studies have assessed the impact of elevated serum amylase levels in clinical practice, but only a few have investigated the significance of low serum amylase. We therefore, aimed to review the literature to understand the conditions associated with low serum amylase and its clinical relevance.
METHOD
This systematic review was performed in accordance with the criteria established in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The search was conducted on Medline and Embase databases until November 2022. After identifying relevant titles, abstracts were read and data of eligible articles retrieved. The conditions associated with low serum amylase were evaluated. The quality of the studies was assessed using the Newcastle-Ottawa Score.
RESULTS
Our search strategy identified 19 studies including a total of 15 097 patients for systematic review. All the studies were observational including two studies which used secretin-induced test. The main conditions associated with low serum amylase were diabetes mellitus (n=9), metabolic syndrome (n=3), chronic pancreatitis (CP) (n=3), non-alcoholic fatty liver disease (n=2) and obesity (n=1). Low serum amylase showed a high specificity (94%) with low sensitivity (38.7%-59%) in diagnosing chronic pancreatitis.
CONCLUSION
This systematic review revealed a unique insight into the relevance of low serum amylase in clinical practice. Low serum amylase can be a useful adjunct test in the assessment of patients with CP, pancreatic exocrine insufficiency, diabetes mellitus and metabolic syndrome.
PubMed: 38779473
DOI: 10.1136/flgastro-2023-102405 -
Anatomy & Cell Biology Mar 2024The exocrine part of the pancreas has a duct system called the pancreatic ductal system (PDS). Its mechanism of development is complex, and any reorganization during...
The exocrine part of the pancreas has a duct system called the pancreatic ductal system (PDS). Its mechanism of development is complex, and any reorganization during early embryogenesis can give rise to anatomical variants. The aim of this study is to collect, classify, and analyze published evidence on the importance of anatomical variants of the PDS, addressing gaps in our understanding of such variations. The MEDLINE, Web of Science, Embase, and Google Scholar databases were searched to identify publications relevant to this review. R studio with meta-package was used for data extraction, risk of bias estimation, and statistical analysis. A total of 64 studies out of 1,778 proved suitable for this review and metanalysis. The meta-analysis computed the prevalence of normal variants of the PDS (92% of 10,514 subjects). Type 3 variants and "descending" subtypes of the main pancreatic duct (MPD) predominated in the pooled samples. The mean lengths of the MPD and accessory pancreatic duct (APD) were 16.53 cm and 3.36 cm, respectively. The mean diameters of the MPD at the head and the APD were 3.43 mm and 1.69 mm, respectively. The APD was present in only 41% of samples, and the long type predominated. The pancreatic ductal anatomy is highly variable, and the incorrect identification of variants may be challenging for surgeons during ductal anastomosis with gut, failure to which may often cause ductal obstruction or pseudocysts formation.
PubMed: 38351473
DOI: 10.5115/acb.23.148