BACKGROUND: IgG4-related disease (IgG4-RD) is a rare disease entity in India. We aimed at studying the clinical profile of IgG4-RD of gastrointestinal tract (GIT) from our centre, while systematically reviewing data from India.
METHODS: Retrospective review of IgG4-RD of GIT was done using electronic medical records between January 2013 and July 2022. Literature search was done for studies of IgG4-RD of the GIT reported from India from 2000 till January 2023. Case series, case reports of IgG4-RD of GIT and case reports describing GIT with multi-organ involvement were included in the review. Primary outcome measure was response to treatment. Secondary outcome measure was relapse after remission.
RESULTS: Thirty-one patients were included with 71% (22/31) having autoimmune pancreatitis. The diagnosis was achieved on surgical specimen in 35% (11/31) patients. Steroid was given to 64% (20/31) patients with remission achieved in 70% (14/20) patients. Four patients exhibitted response to prolonged course of steroids with maintenance azathioprine. Relapse was seen in four (20%) patients who achieved remission. Of 731 articles screened, 48 studies (four case series and 44 case reports) were included in the literature review. Of 95 patients described, steroids were given to 65.2% (62/95), while surgery was done in 33.6% (32/95). Remission was seen in 96.6% (85/88) with relapse occurring in 11.4% (10/88) patients on follow-up.
CONCLUSIONS: One-third patients of IgG4-RD of GIT are diagnosed after surgery. Response to steroids is good with relapse occurring in up to 12% patients.

Since the early 1980s, multiple researchers have contributed to the development of in vitro models of the human gastrointestinal system for the mechanistic interrogation of the gut microbiome ecology. Using a bioreactor for simulating all the features and conditions of the gastrointestinal system is a massive challenge. Some conditions, such as temperature and pH, are readily controlled, but a more challenging feature to simulate is that both may vary in different regions of the gastrointestinal tract. Promising solutions have been developed for simulating other functionalities, such as dialysis capabilities, peristaltic movements, and biofilm growth. This research field is under constant development, and further efforts are needed to drive these models closer to in vivo conditions, thereby increasing their usefulness for studying the gut microbiome impact on human health. Therefore, understanding the influence of key operational parameters is fundamental for the refinement of the current bioreactors and for guiding the development of more complex models. In this review, we performed a systematic search for operational parameters in 229 papers that used continuous bioreactors seeded with human feces. Despite the reporting of operational parameters for the various bioreactor models being variable, as a result of a lack of standardization, the impact of specific operational parameters on gut microbial ecology is discussed, highlighting the advantages and limitations of the current bioreactor systems.

Retrocecal hernia, a type of internal hernia, is a rare cause of small bowel obstruction. It can come as a surprise to the operating surgeon if not diagnosed preoperatively. We hereby report a case of retrocecal hernia presenting with small bowel obstruction. A man in his early 60s presented to the emergency department with recurrent episodes of vomiting associated with abdominal pain for 3 days and the inability to pass flatus for 1 day. Preoperative imaging revealed multiple air-fluid levels with a transition point in the terminal ileum suggestive of small bowel obstruction. After adequate resuscitation, emergency laparotomy was performed, and intraoperatively, herniated ileal loop through a retrocecal defect was identified as a cause of obstruction. Herniated ileal loop was reduced, followed by the closure of the defect and cecopexy. The postoperative course was uneventful, and the patient was discharged in a stable condition. He was asymptomatic on follow-up.

Noonan, Costello, and cardio-facio-cutaneous syndrome are neurodevelopmental disorders belonging to the RASopathies, a group of syndromes caused by alterations in the RAS/MAPK pathway. They are characterized by similar clinical features, among which feeding difficulties, growth delay, and gastro-intestinal disorders are frequent, causing pain and discomfort in patients. Hereby, we describe the main nutritional and gastrointestinal issues reported in individuals with RASopathies, specifically in Noonan syndrome, Noonan syndrome-related disorders, Costello, and cardio-facio-cutaneous syndromes. Fifty percent of children with Noonan syndrome may experience feeding difficulties that usually have a spontaneous resolution by the second year of life, especially associated to genes different than PTPN11 and SOS1. More severe manifestations often require artificial enteral nutrition in infancy are observed in Costello syndrome, mostly associated to c.34G>A substitution in the HRAS gene. In cardio-facio-cutaneous syndrome feeding issues are usually present (90-100% of cases), especially in individuals carrying variants in BRAF, MAP2K1, and MAP2K2 genes, and artificial enteral intervention, even after scholar age, may be required. Moreover, disorders associated with gastrointestinal dysmotility as gastro-esophageal reflux and constipation are commonly reported in all the above-mentioned syndromes. Given the impact on growth and on the quality of life of these patients, early evaluation and prompt personalized management plans are fundamental.

Premature birth is a major cause of mortality and morbidity in the pediatric population. Because their immune, gastrointestinal and nervous systems are not fully developed, preterm infants (<37 weeks of gestation) and especially very preterm infants (VPIs, <32 weeks of gestation) are more prone to infectious diseases, tissue damage and future neurodevelopmental impairment. The aim of this narrative review is to report the immaturity of VPI systems and examine the role of Human Breast Milk (HBM) in their development and protection against infectious diseases, inflammation and tissue damage. For this purpose, we searched and synthesized the data from the existing literature published in the English language. Studies revealed the significance of HBM and indicate HBM as the best dietary choice for VPIs.

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Background: The role of purinergic P2X7 receptor (P2X7R) is of interest due to its involvement in inflammation and mediating immune cell responses. P2X7R is particularly implicated in the development of inflammatory bowel disease (IBD). However, the extent of the actions of P2X7R in the gastrointestinal (GI) system under physiological and pathophysiological conditions remains to be elucidated. This systematic review aimed to identify, summarize and evaluate the evidence for a critical role of P2X7R in the GI system. Methods: We searched PubMed, Embase and Scopus with search terms pertained to P2X7R in the GI system in disease or physiological state, including \"P2X7 or P2X7 receptor or purinergic signaling\" in combination with any of the terms \"intestine or colon or gut or gastrointestinal,\" \"pathology or inflammation or disease or disorder,\" and \"physiology or expression.\" Titles and abstracts were screened for potentially eligible full texts, and animal and human studies published in English were included in this study. Data were extracted from papers meeting inclusion criteria. Meta-analysis was not feasible given the study diversity. Results: There were 48 papers included in this review. We identified 14 experimental colitis models, three sepsis models and one ischemia-reperfusion injury model. Among them, 11 studies examined P2X7R in GI infections, six studies on immune cell regulation, four studies on GI inflammation, two studies on GI malignancies, three studies involving intestinal injury due to various causes, two studies on ATP-activated P2X7R in the GI system and two studies on metabolic regulation. Conclusion: Evidence supports P2X7R mediating inflammation and immune cell responses in GI inflammation, infections and injury due to IBD and other challenges to the intestinal wall. P2X7R inhibition by gene knockout or by application of P2X7R antagonists can reduce tissue damage by suppressing inflammation. P2X7R is also implicated in GI malignancies and glucose and lipid homeostasis. P2X7R blockade, however, did not always lead to beneficial outcomes in the various pathological models of study.

Hydralazine is a commonly prescribed antihypertensive agent. Some of its labelled adverse reactions include lupus-like syndrome, tachycardia, headache and fever. Despite its well-known side effects, little is known about hydralazine\'s hepatotoxic effects. We report the case of a 54-year-old female patient who was started on hydralazine for hypertension management but later presented with hydralazine-induced liver injury. Her initial presentation consisted of non-specific symptoms and a hepatocellular injury pattern. Liver biopsy revealed hepatic steatosis. Three weeks after discontinuation of hydralazine, the patient\'s liver enzymes normalised, and her symptoms resolved. Few studies have examined the incidence and mechanism by which hydralazine induces a liver injury pattern. With this case, we review the literature, the pathogenesis involved and the eventual management of hydralazine-induced liver injury. We propose close monitoring of liver enzymes for patients on hydralazine throughout their treatment course.

COVID-19 is a recent pandemic that is still a major health problem of modern times and already more than 17.5 lakhs people succumbed to this deadly disease. This disease is caused by novel coronavirus which is named SARS-COV-2 by the International Committee on Taxonomy of Viruses. This virus originated from Wuhan city in Hubei province of China in December 2019 and within a short period spread across the many countries in the globe. There are a lot of basic as well as clinical research is going on to study the mode of transmission and the mechanism of action of SARS-COV-2 infection and its therapeutics. SARS-COV-2 is not only known to infect lungs, but it also infects other organs in the human body including the gastrointestinal (GI) tract, the liver, and the pancreas via the angiotensin-converting enzyme (ACE) 2, an important component of the renin-angiotensin system. In this short review, we are mainly discussing the mode of SARS-COV-2 transmission, physiological counterbalancing roles of ACE2 and ACE and the tissue patterns of ACE2 expression, and the overall effect of COVID19 on human gastrointestinal System. Therefore, this review sheds light on the possible mechanism of SARS-COV-2 infection in the GI system and its pathological symptoms raising a potential possibility of GI tract acting as a secondary site for SARS-CoV-2 tropism and infection. Finally, future studies to understand the fecal-oral transmission of the virus and the correlation of viral load and severity of GI symptoms are proposed to gain knowledge of the GI symptoms in COVID-19 to aid in early diagnosis and prognosis.

Although multiple pathophysiologic changes develop within the gastrointestinal (GI) system in the setting of malnutrition, the etiology of the reported multitude of symptoms in those with anorexia nervosa and avoidant restrictive intake disorder, as well as their contribution toward disordered eating, remain poorly understood. This systematic review seeks to better understand how these physiologic changes of malnutrition of the esophagus, stomach, intestines, and pancreas contribute toward the reported GI symptoms, as well as better understand how celiac disease, inflammatory bowel disease, pelvic floor dysfunction, and Ehlers-Danlos syndrome contribute toward disordered eating.
Studies of any design exploring the pathogenesis of complications and treatment strategies were included. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used to structure and complete the review.
A total of 146 articles were used for the review. The majority of studies were observational or case reports/case series.
Pathophysiologic changes of the esophagus, stomach, and intestines develop with malnutrition, although these changes do not consistently correlate with expressed GI symptoms in patients with restrictive eating disorders. Celiac disease and inflammatory bowel disease also contribute to disordered eating through the associated somatic GI complaints, while pelvic floor dysfunction and Ehlers-Danlos syndrome contribute through both somatic symptoms and functional symptoms. Indeed, functional GI symptoms remain problematic during the course of treatment, and further research is required to better understand the extent to which these symptoms are functional in nature and remit or remain as treatment ensues.
Aunque en el contexto de la desnutrición se desarrollan múltiples cambios fisiopatológicos dentro del sistema gastrointestinal (GI), la etiología de la multitud de síntomas reportados en aquellos que padecen anorexia nerviosa y trastorno evitativo restrictivo de la ingesta, así como su contribución a la alimentación disfuncional, siguen siendo poco entendidos. Esta revisión sistemática busca entender mejor cómo estos cambios fisiológicos de la desnutrición del esófago, estómago, intestinos y páncreas contribuyen a los síntomas gastrointestinales reportados, así como entender mejor cómo la enfermedad celíaca, la enfermedad inflamatoria intestinal, la disfunción del piso pélvico y el síndrome de Ehlers Danlos contribuyen a la alimentación disfuncional. MÉTODOS: Se incluyeron estudios de cualquier diseño que exploraran la patogénesis de las complicaciones y las estrategias de tratamiento. Los lineamientos PRISMA se utilizaron para estructurar y completar el examen.
Se utilizaron un total de 146 artículos para la revisión. La mayoría de los estudios fueron observacionales o reporte de caso o series de casos. DISCUSIÓN: Los cambios fisiopatológicos del esófago, el estómago y los intestinos se desarrollan con la desnutrición, aunque estos cambios no se correlacionan consistentemente con los síntomas gastrointestinales expresados en pacientes con trastornos de la conducta alimentaria restrictivos. La enfermedad celíaca y la enfermedad inflamatoria intestinal también contribuyen a la alimentación disfuncional a través de las quejas gastrointestinales somáticas asociadas, mientras que la disfunción del piso pélvico y el síndrome de Ehlers Danlos contribuyen a través de síntomas somáticos y síntomas funcionales. De hecho, los síntomas gastrointestinales funcionales siguen siendo problemáticos durante el curso del tratamiento, y se requiere más investigación para comprender mejor hasta qué punto estos síntomas son de naturaleza funcional y se remiten o permanecen a medida que se produce el tratamiento.