Best Practice & Research. Clinical Gastroenterology

Malnutrition in patients with chronic pancreatitis is common, but its evaluation is often missed in clinical practice. Pancreatic exocrine insufficiency is the single most important cause of malnutrition; therefore, it needs to be screened for and treated appropriately. Specific diet regimens in patients suffering from chronic pancreatitis are rarely reported in the literature. Patients suffering from chronic pancreatitis have a higher demand for energy but a lower caloric intake secondary to pancreatic exocrine insufficiency, combined with the malabsorption of liposoluble vitamin and micronutrients, which needs be corrected by appropriate dietary counselling...

There are three major hereditable syndromes that affect primarily the stomach: hereditary diffuse gastric cancer (HDGC), gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) and familial intestinal gastric cancer (FIGC). HDGC is caused by germline mutations in CDH1 gene that occur in 10-40% of HDGC families and, in a minority of cases, by mutations in CTNNA1 gene. GAPPS is caused by germline mutations in the promoter 1B of APC gene, and the genetic cause of FIGC is not fully elucidated. Gastric cancer can also be observed as part of other inherited cancer disorders, namely in familial adenomatous polyposis, MUTYH-associated polyposis, Peutz-Jeghers syndrome, juvenile polyposis syndrome, Lynch syndrome, Li-Fraumeni syndrome, Cowden syndrome, and hereditary breast and ovarian cancer syndrome...

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There are two main problems in the clinical management of the gastrointestinal (GI) tract in patients with Peutz-Jeghers syndrome (PJS), namely long-term cancer risk and managing polyp related complications (of which the most important clinically is intussusception). Given the rarity of this condition, the evidence base upon which to make recommendations is small. Furthermore, controversies persist regarding the relationship between PJ polyps, cancer development and cancer risk. In this article we will explore some of these controversies, to put into context the recommendations for clinical management of these patients...

No abstract text is available yet for this article.

Congenital inherited hepatic disorders (CIHDs) are a set of diverse and heterogeneous group of genetic disorders leading to a defect in an enzyme or transporter. Most of these disorders are currently treated by liver transplantation as standard of care. Improved surgical techniques and post-operative care has led to a wider availability and success of liver transplantation program worldwide. However liver transplantation has its own limitations due to invasive surgery and lifelong use of immunosuppressive agents...

Œsophageal atresia is a rare neonatal malformation consisting in an interruption of the continuity of the œsophagus, with or without a tracheo-œsophageal fistula. Although mortality rate is now low and most cases can benefit from successful surgical repair soon after birth, morbidity -specially digestive and nutritional-remains high. Many of the adults born with œsophageal atresia will suffer from dysphagia, gastro-œsophageal reflux and/or œsophageal dysmotility, leading to nutritional consequences and quality of life impairment...

Several paediatric gastrointestinal diseases result in life-shortening organ failure. For many of these conditions, current therapeutic options are suboptimal and may not offer a cure. Regenerative medicine is an inter-disciplinary field involving biologists, engineers, and clinicians that aims to produce cell and tissue-based therapies to overcome organ failure. Exciting advances in stem cell biology, materials science, and bioengineering bring engineered gastrointestinal cell and tissue therapies to the verge of clinical trial...

Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI) motility. HSCR is a developmental disorder of the enteric nervous system invariably affecting the large intestine, whereas the majority of PIPO conditions represent congenital disorders of one or more components of the neuromusculature and more diffusely affect the GI tract. Histopathology is deemed the gold standard for the diagnosis of HSCR and, arguably, of PIPO, but, other diagnostic modalities such as manometric and genetic studies have seen recent advances that may increase their utility...

No abstract text is available yet for this article.

Hereditary pancreatitis (HP) encompasses two distinct disease groups: the first manifests as congenital exocrine pancreatic insufficiency (EPI), and the second includes hereditary forms of pancreatitis. EPI represents the ultimate expression of gland function loss. Cystic fibrosis is by far the most frequent aetiology of early-onset EPI; genetics and a growing understanding of the disease mechanisms have paved the way for innovative and personalized treatment approaches. Efforts are ongoing to further decipher the pathophysiology and explore new therapies for other causes of EPI...

Endoscopic ultrasound (EUS) was born from the combination of a high-frequency ultrasound probe with an endoscope to assess in detail the walls of the upper and lower gastrointestinal tract and surrounding organs and structures. The subsequent possibility of EUS-guided tissue acquisition has rapidly established the irreplaceable role of EUS in the management of a wide range of benign and malignant gastrointestinal diseases. The actual diagnostic armamentarium involving fine-Doppler, elastography, and contrast enhancement has significantly improved its diagnostic yield, which could be even more refined by newer ways of interrogating data and images, such as artificial intelligence...

The role of endoscopic ultrasound in the diagnosis and management of chronic liver disease is rapidly increasing. It forms one of the major backbones of endo-hepatology and brings us a step closer to personalized medicine. This review will focus on the particular use of EUS in the diagnosis and management of cirrhotic portal hypertension and potential complications hereof, such as ascites and gastrooesophageal varices. More specifically, EUS-guided Porto-systemic Pressure Gradient (EUS-PPG) measurement, EUS-guided coil and glue embolization of gastric varices, EUS-guided paracentesis and EUS-guided intrahepatic portosystemic shunt creation (IPSS) will be discussed in-depth with regard to clinical status, available data and technical considerations...

EUS-guided treatments for focal tumor lesions has been developed since 20 years using at onset of the technique mainly local and guided alcohol injection [1-4]. Pancreatic tumors are the most assessed targeted lesions for EUS treatment because of their accessibility and because EUS management could be a safe alternative to surgery. More and more pancreatic tumors are discovered mainly fortuitously due to the advances in conventional imaging (abdominal ultrasound, CT, MRI) resulting in the question of surgical management of an asymptomatic pancreatic lesion ("incidentaloma") [5-8]...

No abstract text is available yet for this article.

Endoscopic drainage requires transpapillary access to the pancreatic duct during ERCP. When ERCP failed, EUS-guided pancreatico-gastro or bulbostomy and/or rendez-vous technique offers an alternative to surgery. Although data has demonstrated that the procedure can be safe and effective, EUS-guided PD drainage remains one of the most technically challenging therapeutic EUS interventions, as evidenced by the multiple considerations on device selection and the risk of severe complications.

With the introduction of EUS, endoscopy was no longer limited to luminal indications. However, the method was unable to distinguish malignant from benign lesions. Consequently, needles designed for tissue acquisition under EUS-guidance was designed. Initially, the needles were designed for fine needle aspiration (FNA); nevertheless, with increased requirement for the precured tissue in terms of quality and quantity, newly design needles aimed at obtaining tissue cores for histological assessment were developed...

Patients with pancreas cancer must deal frequently with intractable and refractory pain. Endoscopic ultrasound guided-celiac plexus neurolysis (EUS-CPN) has been the most studied and used therapeutic technique aimed to destroy the pain fibres that allow the pancreas to communicate with the central nervous system. A neurolytic agent, most commonly ethanol, is optimally spread around the celiac axis in order to reduce pain and mitigate narcotic requirements. This can be performed early to prevent the spiral of pain and medication use, or more historically as salvage therapy...

EUS-guided therapeutic procedures have emerged in the recent years as a minimally invasive option for the management of complex hepatobiliary disorders. Gastrointestinal surgeries, e.g., pancreaticoduodenectomy, Roux-en-Y Gastric Bypass, Roux-en-Y hepaticojejunostomy, and partial gastrectomy with different reconstructions are commonly performed for a wide range of indications that include tumour resections and weight loss. Conventional endoscopic procedures are challenging in those patients due to inaccessible or unreachable ampulla...

Endoscopic Retrograde Cholangiopancreatography (ERCP), even in expert hands, may fail in 5-10% of cases, especially in cases of papillary infiltration, malignant gastric outlet obstruction, or surgically altered anatomy. Percutaneous transhepatic biliary drainage (PTBD) has represented the traditional rescue therapy, despite associated with high rate of adverse events, need for re-interventions and an inferior quality of life. The evolution of Endoscopic Ultrasound (EUS) from a diagnostic to a therapeutic tool offers an effective and safe alternative for internal biliary drainage (BD) into the stomach or the duodenum...