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Case Report
Unmasking Alagille Syndrome in Adulthood- A Complete Clinical Portrait of a Rare Syndrome
Issue:
Volume 10, Issue 1, June 2026
Pages:
1-4
Received:
20 December 2025
Accepted:
27 January 2026
Published:
14 February 2026
Abstract: Alagille syndrome (ALGS) is a rare autosomal dominant multisystem disorder characterized by marked phenotypic variability, and the diagnosis is sometimes very challenging. We describe a 26-year-old man who presented with progressive jaundice, pruritus, and weight loss over three months, with a history of similar episodes in early childhood. He had previously been diagnosed with atrial septal defect with severe pulmonary stenosis, myopia, and hypothyroidism. After a thorough Clinical examination which revealed icterus, pedal edema, stigmata of chronic liver disease, and characteristic facial dysmorphism with café-au-lait spots, a possibility of Alagille syndrome was considered. Laboratory evaluation showed cholestatic liver dysfunction and pancytopenia. Whole-exome sequencing identified a likely pathogenic JAG1 variant, and liver biopsy confirmed bile duct paucity. This case highlights the diagnostic challenges of ALGS and underscores the importance of meticulous clinical evaluation and comprehensive longitudinal history-taking, as multiple healthcare encounters preceded the correct diagnosis as far as this patient is concerned.
Abstract: Alagille syndrome (ALGS) is a rare autosomal dominant multisystem disorder characterized by marked phenotypic variability, and the diagnosis is sometimes very challenging. We describe a 26-year-old man who presented with progressive jaundice, pruritus, and weight loss over three months, with a history of similar episodes in early childhood. He had ...
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Case Report
An Unusual Case of Ascites: POEMS Syndrome Presenting as High SAAG Ascites
Issue:
Volume 10, Issue 1, June 2026
Pages:
5-8
Received:
4 February 2026
Accepted:
26 February 2026
Published:
12 March 2026
DOI:
10.11648/j.ijg.20261001.12
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Abstract: Castleman disease is a rare histopathological entity associated with a variety of lymphoproliferative disorders and autoimmune conditions. The disease can occur in a unicentric form (involving single lymph node group) or multicentric form. While sometimes associated with POEMS syndrome, ascites is not an uncommon association with these disorders. However, the ascites described in association with Castleman disease or POEMS is usually a low SAAG ascites. We report a case with high SAAG ascites as the presenting feature in a patient with POEMS syndrome and multicentric Castleman disease which is quite rare as evidenced by scarcity of literature. Our patient who had presented with ascites, was found to have polyneuropathy, organomegaly (liver and spleen), endocrinopathy (thyroid hormone abnormality), monoclonal gammopathy and skin lesions on evaluation. The scenario was complicated due to a misdiagnosis of tuberculosis from elsewhere and an improper course of antituberculosis treatment. Things fell into place when the diagnosis was confirmed with the histopathological examination of the cervical lymph node biopsy showing hyaline vascular variant of Castleman disease. Thalidomide and its congeners along with steroids have been the mainstay of treatment of this along with stem cell transplantation, while anti IL-6 agents like siltuximab are emerging treatment options. In view of its complexity and the multisystem involvement of this syndrome, it may be easily overlooked or missed if one is not aware or actively looking for the various clinical clues.
Abstract: Castleman disease is a rare histopathological entity associated with a variety of lymphoproliferative disorders and autoimmune conditions. The disease can occur in a unicentric form (involving single lymph node group) or multicentric form. While sometimes associated with POEMS syndrome, ascites is not an uncommon association with these disorders. H...
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Research Article
Clinical Profile and Predictors of Hospital Outcomes in Acute-on-chronic Liver Failure
Shreyansh Goyal*
,
Prabhat Sinha,
Sharad Malhotra
Issue:
Volume 10, Issue 1, June 2026
Pages:
9-19
Received:
15 February 2026
Accepted:
9 March 2026
Published:
19 March 2026
Abstract: Acute-on-chronic liver failure (ACLF) is a distinct clinical syndrome characterized by acute hepatic deterioration in patients with underlying chronic liver disease and is associated with multiorgan dysfunction and high short-term mortality. In India, alcohol remains the predominant etiology of chronic liver disease, with infectious causes and alcoholic hepatitis being common acute precipitating events. However, clinical profiles and predictors of in-hospital outcomes show variability across populations. This hospital-based observational study included 50 patients diagnosed with ACLF according to the APASL criteria and admitted to Aakash Healthcare Super Speciality Hospital, New Delhi, between February 2024 and January 2026. Detailed clinical evaluation and laboratory investigations were performed at admission. Disease severity was assessed using Child–Turcotte–Pugh, Model for End-Stage Liver Disease (MELD), and MELD-Na scores. Precipitating factors, complications, organ system involvement, and outcomes during hospitalization were documented. Outcomes were categorized as survival with discharge, in-hospital mortality, or referral for liver transplantation. The mean age of patients was 49.96 ± 11.30 years, with a male predominance (84%). Alcohol-related liver disease was the most common underlying etiology (60%). Alcoholic hepatitis (38%) and sepsis (30%) were the leading precipitating events. Jaundice, abdominal distension, and altered sensorium were the most frequent presenting symptoms. Ascites (96%), portal hypertension (92%), and hepatic encephalopathy (78%) were the predominant complications. In-hospital mortality was observed in 46% of patients, while 22% required referral for liver transplantation. Elevated international normalized ratio, serum creatinine, ammonia, lactate, serum potassium levels, and higher MELD and MELD-Na scores were significantly associated with poor outcomes (p < 0.05). Hepatic encephalopathy was the only clinical complication significantly associated with mortality on univariate analysis (p = 0.021). ACLF predominantly affects middle-aged males with alcohol-related chronic liver disease and is associated with high in-hospital mortality. Early identification of high-risk patients and timely escalation of care, including prompt evaluation for liver transplantation, are crucial to improving outcomes.
Abstract: Acute-on-chronic liver failure (ACLF) is a distinct clinical syndrome characterized by acute hepatic deterioration in patients with underlying chronic liver disease and is associated with multiorgan dysfunction and high short-term mortality. In India, alcohol remains the predominant etiology of chronic liver disease, with infectious causes and alco...
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Research Article
Clinical, Endoscopic, Histological, and Therapeutic Aspects of Gastric Polyps at the Sikasso Regional Hospital, Mali: A Study of 39 Cases
Issue:
Volume 10, Issue 1, June 2026
Pages:
20-24
Received:
21 March 2026
Accepted:
7 April 2026
Published:
16 April 2026
DOI:
10.11648/j.ijg.20261001.14
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Abstract: Introduction: Gastric polyps are tissue lesions that develop within the gastric lumen. Their clinical, endoscopic, histopathological, and therapeutic features remain variable and poorly described in sub-Saharan Africa. This study aimed to characterize these aspects in patients at Sikasso Regional Hospital, Mali. Methods: We conducted a retrospective study over an eight-year period (2017–2024) including all histologically confirmed gastric polyps. Data were collected from medical records and analyzed using Microsoft Excel. Results: A total of 39 patients were included (24 men, 15 women; sex ratio 1.6), with a mean age of 52 years (range: 15–87). Polyps were sessile in 71% and pedunculated in 29% of cases. Lesions were single in 51% and multiple in 49%, most frequently located in the antrum. The mean polyp size was 6.2 mm (range: 2–29 mm). Histologically, hyperplastic polyps were observed in 30.8%, adenomatous in 23.7%, fibro-inflammatory in 15.4%, juvenile in 12.8%, fundic gland in 12.8%, and papillomatous in 10.3%. Helicobacter pylori infection was present in 35.9% of cases, with 15.4% showing associated gastritis. Mean follow-up duration of patients was 31 months. Conclusion: This study provided valuable insights into gastric polyps in the population of Sikasso, highlighting the diversity of their clinical and endoscopic characteristics. Helicobacter pylori infection remains endemic in this setting and is often chronic, frequently evolving asymptomatically while potentially leading to significant gastric lesions. These findings emphasize the importance of strengthening preventive strategies, including public awareness on screening, early management, and regular surveillance of patients with gastric polyps.
Abstract: Introduction: Gastric polyps are tissue lesions that develop within the gastric lumen. Their clinical, endoscopic, histopathological, and therapeutic features remain variable and poorly described in sub-Saharan Africa. This study aimed to characterize these aspects in patients at Sikasso Regional Hospital, Mali. Methods: We conducted a retrospectiv...
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Review Article
The Beneficial Role of Early-Life Gut Microbiota Development for Improving Intestinal Health Integrity and Gut-Immune Function in Infants of Bangladesh
Issue:
Volume 10, Issue 1, June 2026
Pages:
25-31
Received:
12 March 2026
Accepted:
25 March 2026
Published:
24 April 2026
DOI:
10.11648/j.ijg.20261001.15
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Abstract: Early life represents a critical window for the establishment of the gut microbiome, a complex microbial ecosystem that plays a central role in intestinal health, immune system development, physical growth, and long-term disease risk. In low- and middle-income countries such as Bangladesh, infants are exposed to a range of nutritional and environmental stressors—including undernutrition, recurrent infections, and inadequate sanitation—that can significantly disrupt normal microbiome development. These challenges make the study of early-life microbiome assembly particularly important in this context. This review synthesizes recent research on gut microbiome development in Bangladeshi infants and explores how deviations from normal patterns—such as microbiome immaturity, dysbiosis, and the overrepresentation of pathogenic organisms—are associated with adverse outcomes. These include impaired intestinal function, chronic inflammation, weakened immune responses, and growth faltering. Studies consistently show that healthy infants typically undergo a predictable succession of microbial colonization, with beneficial taxa such as Bifidobacterium infantis and other members of the Bifidobacterium longum group dominating early life, particularly in breastfed infants. These microbes are crucial for metabolizing human milk oligosaccharides and supporting immune maturation. In contrast, malnourished infants often exhibit delayed or altered microbiome maturation. Their gut microbial communities resemble those of younger infants, indicating immaturity, and are often enriched with harmful or less beneficial bacteria. Importantly, conventional nutritional interventions alone have been shown to only partially restore a healthy microbiome composition, suggesting that more targeted approaches are necessary. Recent advances highlight the potential of microbiota-directed complementary foods (MDCF) and specific probiotic supplementation in addressing these issues. These interventions are designed to promote the growth of beneficial microbes and restore microbial balance. Evidence indicates that MDCF can accelerate microbiome repair, enhance gut barrier integrity, reduce intestinal inflammation, and contribute to improved growth outcomes in undernourished children. In conclusion, targeting gut microbiome development during early infancy offers a promising strategy to improve health outcomes among Bangladeshi children. However, further research is needed to determine the optimal timing, duration, and sustainability of such interventions, as well as to better understand the underlying biological mechanisms driving these benefits.
Abstract: Early life represents a critical window for the establishment of the gut microbiome, a complex microbial ecosystem that plays a central role in intestinal health, immune system development, physical growth, and long-term disease risk. In low- and middle-income countries such as Bangladesh, infants are exposed to a range of nutritional and environme...
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