Hereditary Pancreatitis – A Review of Current Concepts and Management

Hughes, Daniel; Gordon-Weeks, Alex; Silva, Michael

doi:10.21926/obm.hg.1903028

Cited by 2 publications

(1 citation statement)

References 86 publications

(128 reference statements)

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“…Therefore, a marked time delay was implemented between the onset of the disease and definitive treatment, with only patients with advanced and well-established disease being listed for surgery. The nature of surgery is dependent on multifactorial aspects: notably the disease distribution throughout the gland and the size and the morphology of the main pancreatic duct [ 13 , 14 ]. In general, surgery adopts one of the three different approaches: resection of diseased tissue, ductal drainage and a combination of resection and drainage [ 13 ].…”

Section: Introductionmentioning

confidence: 99%

A meta-analysis of the long-term outcomes following surgery or endoscopic therapy for chronic pancreatitis

Hughes

Silva

2022

Langenbecks Arch Surg

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Purpose Refractory abdominal pain is a cardinal symptom of chronic pancreatitis (CP). Management strategies revolve around pain mitigation and resolution. Emerging evidence from observational studies highlights that surgery may result in superior pain relief when compared to endoscopic therapy; however, its impact on long-term quality of life or functional outcome has yet to be determined. Methods A search through MEDLINE, PubMed and Web of Science was performed for RCTs that compared endoscopic treatment with surgery for the management of CP. The main outcome measure was the impact on pain control. Secondary outcome measures were the effect on quality of life and the incidence rate of new onset exocrine and endocrine failure. Data was pooled for analysis using either an odds ratio (OR) or mean difference (MD) with a random effects model. Results Three RCTs were included with a total of 267 patients. Meta-analysis demonstrated that operative treatment was associated with a significantly higher rate of complete pain control (37%) when compared to endoscopic therapy (17%) [OR (95% confidence interval (CI)) 2.79 (1.53–5.08), p = 0.0008]. No difference was noted in the incidence of new onset endocrine or exocrine failure between treatment strategies. Conclusion Surgical management of CP results in a greater extent of complete pain relief during long-term follow-up. Further research is required to evaluate the impact of the time interval between diagnosis and intervention on exocrine function, combined with the effect of early up-front islet auto-transplantation in order to determine whether long-term endocrine function can be achieved.

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Section: Introductionmentioning

confidence: 99%

A meta-analysis of the long-term outcomes following surgery or endoscopic therapy for chronic pancreatitis

Hughes

Silva

2022

Langenbecks Arch Surg

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Transcriptional co-activators: emerging roles in signaling pathways and potential therapeutic targets for diseases

Talukdar,

Chatterji

2023

Sig Transduct Target Ther

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Specific cell states in metazoans are established by the symphony of gene expression programs that necessitate intricate synergic interactions between transcription factors and the co-activators. Deregulation of these regulatory molecules is associated with cell state transitions, which in turn is accountable for diverse maladies, including developmental disorders, metabolic disorders, and most significantly, cancer. A decade back most transcription factors, the key enablers of disease development, were historically viewed as ‘undruggable’; however, in the intervening years, a wealth of literature validated that they can be targeted indirectly through transcriptional co-activators, their confederates in various physiological and molecular processes. These co-activators, along with transcription factors, have the ability to initiate and modulate transcription of diverse genes necessary for normal physiological functions, whereby, deregulation of such interactions may foster tissue-specific disease phenotype. Hence, it is essential to analyze how these co-activators modulate specific multilateral processes in coordination with other factors. The proposed review attempts to elaborate an in-depth account of the transcription co-activators, their involvement in transcription regulation, and context-specific contributions to pathophysiological conditions. This review also addresses an issue that has not been dealt with in a comprehensive manner and hopes to direct attention towards future research that will encompass patient-friendly therapeutic strategies, where drugs targeting co-activators will have enhanced benefits and reduced side effects. Additional insights into currently available therapeutic interventions and the associated constraints will eventually reveal multitudes of advanced therapeutic targets aiming for disease amelioration and good patient prognosis.

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Hereditary Pancreatitis – A Review of Current Concepts and Management

Cited by 2 publications

References 86 publications

A meta-analysis of the long-term outcomes following surgery or endoscopic therapy for chronic pancreatitis

A meta-analysis of the long-term outcomes following surgery or endoscopic therapy for chronic pancreatitis

Transcriptional co-activators: emerging roles in signaling pathways and potential therapeutic targets for diseases

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