Acute Hepatic Porphyria – Minireview

Acute Hepatic Porphyria (AHP) is an uncommon and hereditary illness that belongs to a group of disorders known as porphyries. This condition results from a deficiency of the porphobilinogen deaminase enzyme, which plays a role in heme production, a crucial component of haemoglobin in the bloodstream. This deficiency leads to the accumulation of substances called porphyrins in the body, which can trigger the appearance of severe and potentially life-threatening symptoms. In the following, we will discuss classifications - with a focus on the similarities and differences between subtypes of porphyria, the pathophysiology of acute hepatic porphyria, risk factors – and their influence on the onset of the disease, clinical manifestations, diagnosis, and management – both curative and symptomatic, all of which play a very important role in understanding this rare condition

Complications of Diverticular Disease – a Quick Overview

Diverticulosis is a chronic acquired disease defined by the presence of diverticular protrusions throughout the wall layers of the digestive tract. Colonic diverticular disease is defined as clinically manifest or symptomatic diverticulosis, either by inflammation, diverticular bleeding or segmental colitis. It is a frequent cause of hospitalization in industrialized countries and also makes a major contribution to healthcare costs. Due to the spread of the Western-style diet, low in fiber and high in processed foods, the prevalence of diverticulosis is now increasing globally. Obesity is a significant risk factor contributing to the increased prevalence of both diverticular disease and diverticulitis and its complications, particularly in the younger population, previously considered to be at much lower risk than the geriatric population. Diverticulitis occurs when one or more diverticula, together with adjacent colonic tissue, undergo an inflammatory process. Approximately 15% of patients who suffer an episode of acute diverticulitis will experience complications, the most common of which is peridiverticular abscess, which can be complicated by peritonitis. Less common complications are colonic lumen stenosis and fistulae. Being a relatively common disease in the general population, with a constantly increasing prevalence, and also a disease with potentially reducible complications, especially in the case of frail patients with multiple comorbidities, it is necessary not only to update the therapeutic strategies, but also to set up multidisciplinary medical teams in which communication between the specialists involved results in a personalized (personalized) approach to each case

Effect of omeprazole on patient-reported outcome measures in uninvestigated heartburn: a multi-country, multi-center observational study

Background: Heartburn occurs predominantly in the upper gastrointestinal tract and is associated with gastroesophageal reflux disease (GERD) and gastritis. Omeprazole is the most prescribed proton pump inhibitor class of medication to treat heartburn related clinical conditions. To compare the efficacy of omeprazole 40 mg (as a total daily dose) and 20 mg using patient-reported outcome measures (PROMs) in patients with heartburn due to various aetiologies like non-erosive reflux disease, GERD, gastritis, dyspepsia, functional heartburn, gastro-duodenal ulcer.Methods: Naïve patients presenting heartburn symptoms were treated with omeprazole. PROMs were assessed based on short-form-leeds dyspepsia questionnaires (SF-LDQ), work productivity activity impairment (WPAI), relief obtained using medication and, treatment satisfactory questionnaires (TSQ).Results: A total of 18,724 patients with heartburn (GERD and gastritis; n=10,509) were treated with omeprazole (Dr. Reddy’s omeprazole [DO]/generic omeprazole [GO]/branded omeprazole [BO]) 40 mg (as a total daily dose) and 20 mg. Statistical comparative analysis showed significant improvement with omeprazole 40 mg (as a total daily dose) compared to omeprazole 20 mg in SF-LDQ, relief obtained using medication among patients with heartburn. DO 20 mg showed a greater improvement under the ‘a lot’ and ‘complete’ relief category.Conclusions: Omeprazole 40 mg (as a total daily dose) presented better efficacy as compared to omeprazole 20 mg in patient reported outcomes. This study highlights omeprazole 40 mg as the preferred intervention for improving PROMs and quality of life in the treatment of heartburn related clinical conditions

Effects of a high-dose 24-h infusion of tranexamic acid on death and thromboembolic events in patients with acute gastrointestinal bleeding (HALT-IT): an international randomised, double-blind, placebo-controlled trial

Background: Tranexamic acid reduces surgical bleeding and reduces death due to bleeding in patients with trauma. Meta-analyses of small trials show that tranexamic acid might decrease deaths from gastrointestinal bleeding. We aimed to assess the effects of tranexamic acid in patients with gastrointestinal bleeding. Methods: We did an international, multicentre, randomised, placebo-controlled trial in 164 hospitals in 15 countries. Patients were enrolled if the responsible clinician was uncertain whether to use tranexamic acid, were aged above the minimum age considered an adult in their country (either aged 16 years and older or aged 18 years and older), and had significant (defined as at risk of bleeding to death) upper or lower gastrointestinal bleeding. Patients were randomly assigned by selection of a numbered treatment pack from a box containing eight packs that were identical apart from the pack number. Patients received either a loading dose of 1 g tranexamic acid, which was added to 100 mL infusion bag of 0·9% sodium chloride and infused by slow intravenous injection over 10 min, followed by a maintenance dose of 3 g tranexamic acid added to 1 L of any isotonic intravenous solution and infused at 125 mg/h for 24 h, or placebo (sodium chloride 0·9%). Patients, caregivers, and those assessing outcomes were masked to allocation. The primary outcome was death due to bleeding within 5 days of randomisation; analysis excluded patients who received neither dose of the allocated treatment and those for whom outcome data on death were unavailable. This trial was registered with Current Controlled Trials, ISRCTN11225767, and ClinicalTrials.gov, NCT01658124. Findings: Between July 4, 2013, and June 21, 2019, we randomly allocated 12 009 patients to receive tranexamic acid (5994, 49·9%) or matching placebo (6015, 50·1%), of whom 11 952 (99·5%) received the first dose of the allocated treatment. Death due to bleeding within 5 days of randomisation occurred in 222 (4%) of 5956 patients in the tranexamic acid group and in 226 (4%) of 5981 patients in the placebo group (risk ratio [RR] 0·99, 95% CI 0·82–1·18). Arterial thromboembolic events (myocardial infarction or stroke) were similar in the tranexamic acid group and placebo group (42 [0·7%] of 5952 vs 46 [0·8%] of 5977; 0·92; 0·60 to 1·39). Venous thromboembolic events (deep vein thrombosis or pulmonary embolism) were higher in tranexamic acid group than in the placebo group (48 [0·8%] of 5952 vs 26 [0·4%] of 5977; RR 1·85; 95% CI 1·15 to 2·98). Interpretation: We found that tranexamic acid did not reduce death from gastrointestinal bleeding. On the basis of our results, tranexamic acid should not be used for the treatment of gastrointestinal bleeding outside the context of a randomised trial

Gastrointestinal stromal tumor

doi:10.3748/wjg.14.628

Defining the Failure of Medical Therapy for Inflammatory Bowel Disease in the Era of Advanced Therapies: A Systematic Review

Background: The expansion of advanced therapies for inflammatory bowel disease created a lag between the development of these new therapies and their incorporation and use in daily practice. At present, no clear definitions for treatment optimization, treatment failure or criteria to abandon therapy are available. We aimed to centralize criteria for a nonresponse to all available molecules and to summarize guideline principles for treatment optimization. Methods: We conducted a systematic review of studies that reported criteria for the treatment response to all advanced therapies (infliximab, adalimumab, golimumab, ustekinumab, vedolizumab and tofacitinib) in patients with inflammatory bowel disease. Results: Across trials, criteria for a response of both patients with ulcerative colitis and Crohn’s disease are heterogenous. Investigators use different definitions for clinical and endoscopic remission, and endoscopic response and outcomes are assessed at variable time points. Current society guidelines provide heterogenous recommendations on treatment optimization. Most available data on loss of response concern anti-TNF molecules, and newer therapies are not included in the guidelines. Conclusion: The lack of clear definitions and formal recommendations provide the premise for empirical treatment strategies and premature abandonment of therapies

Influence of the characteristics of biogas generated in the leather industry on combustion quality

The treatment of protein waste in the leather industry also includes transformation into biogas through anaerobic digestion. A pilot plant producing biogas with a 45-60% CH4 concentration was designed for this purpose. Starting from primary experimental tests in a Bunsen burner, the paper presents the theoretical aspects of choosing the best combustion technique for this peculiar gaseous fuel in order to calculate the combustion velocity and checking the conditions for the flame stability. The applicability of the kinetic combustion (with pre-mix) will be attempted, because low combustion rates for this fuel also involve low air velocities. Diffusive combustion seems to not be affected by this impediment, as there is always an equality condition of flame velocity with burning speed, so a theoretical spot of a stable flame. After selecting the best combustion technique, a pilot burner will be designed and tested

Interstitial cells of Cajal in the gut - A gastroenterologist’s point of view

Alterations of normal function of interstitial cells of Cajal (ICC) are reported in many intestinal disorders. Diagnosis of their involvement is rare (infrequent), but necessary to propose a specific treatment. This article reviews the place of ICC in the pathogenesis of achalasia, gastroesophageal reflux disease, infantile hypertrophic pyloric stenosis, chronic intestinal pseudo-obstruction and slow transit constipation. Moreover we discuss the role of the Cajal cells in the development of stromal tumors of the gastrointestinal tract

Analytical model for estimating the production and composition of gas resulted through gasification

For the modelling of gasification processes, several models have been developed over the years. It is remarked that gasification calculation models of very high complexity entail some complications. Therefore, simpler mathematical representations of gasification characteristics and process behavior are required as a first step in addressing such systems. The preliminary calculation simplicity is needed form two perspectives: First – the pre-sizing of gasification installations, and second – the estimation of experimental or functional results. For this kind of topics, an adequate simplified model should be defined. Further, to validate the results it will be necessary to use complex calculation models. The model proposed in this paper addresses gasification with distributive air in the air distribution current, considering general concurrent flow of air fuel. Previous successful investigations, conducted by the present research team, are taken into account within model definition stages. Thus, the work presented here provides useful advances in the field of mathematical modeling of gasification processes. The originality of the model consists in its easy computational accessibility, which allows the approach of technological optimizations, such as the variation of excess air and fuel composition