The present study explores the therapeutic potential of Tiliacora acuminata, a traditional medicinal plant widely used in the treatment of jaundice. The root extract has antimicrobial efficacy, and the phytochemical analysis of the root extract showed the occurrence of proteins, alkaloids, flavonoids, phenols, saponins, steroids, tannins, and terpenoids. A cytotoxicity assay on HepG2 cells showed no significant cytotoxic effects, even at concentrations up to 30 µg/mL. GC-MS analysis identified bioactive compounds with high binding affinity for UDP-glucuronosyltransferase 1A1 (UGT1A1), a key enzyme involved in bilirubin glucuronidation. 3,5,24-trimethyltetracontane demonstrated the strongest binding affinity, suggesting its potential to enhance bilirubin glucuronidation. Tiliacora acuminata root extract was utilized to synthesise silver nanoparticles, to investigate their efficacy in the photocatalytic degradation of reactive red 120, reactive black 5, reactive brown 2, and mixed dyes. UV-DRS, FT-IR, XRD, SEM, and TEM analyses were employed to characterise the synthesised nanoparticles. Silver nanoparticles had a strong cytotoxic impact on HepG2 cells, with an IC50 value of 39.34 µg/mL using the MTT assay. The silver nanoparticle has antibacterial efficacy against Klebsiella pneumoniae (MTCC 109), Escherichia coli (MTCC 443), and Staphylococcus aureus (MTCC 7443), as well as two fungal strains, Candida albicans (MTCC 183), and Aspergillus brasiliensis (MTCC 1344). Degradation efficiencies of reactive red 120 were 52.63% ± 1.15 in 150 min, reactive black 5 was 64.47% ± 1.17 in 150 min, reactive brown 2 was 48.44% ± 0.66 in 120 min, and mixed dyes were 49.48% ± 1.15 in 90 min. Silver nanoparticles synthesized from Tiliacora acuminata exhibit antimicrobial and textile degradation properties, playing a crucial role in environmental remediation and biomedical applications.

Primary sclerosing cholangitis (PSC) is a rare cholestatic disease that causes fibrosis and strictures of the bile ducts. It is primarily diagnosed in males between 30 and 40 years of age. Its presentation in elderly females is uncommon and represents a diagnostic challenge. The objective of this study is to present a clinical case of PSC with an atypical presentation in an elderly female with associated cholelithiasis. A 74-year-old female with no medical history presented with jaundice, dark urine, acholia, and pruritus. She was admitted to the emergency department with jaundice and a cholestatic pattern. Initial imaging studies identified cholecystitis and possible extrinsic compression of the bile duct by the gallbladder, mimicking Mirizzi syndrome. Magnetic resonance cholangiopancreatography identified a focal biliary stricture, but serological markers for PSC were negative. Due to persistent pain, a cholecystectomy and liver biopsy were performed. The liver biopsy was positive for PSC. PSC should be considered in elderly patients with persistent cholestatic jaundice despite concomitant gallstone disease. Characteristic cholangiographic findings, together with histopathological evaluation and exclusion of secondary causes, support the diagnosis in atypical presentations.

Objective: Metagenomic sequencing was employed to analyze the pathogen detection profile in pediatric severe acute non-A-E hepatitis. Methods: Based on the platform of the China Childhood Severe Acute Hepatitis Collaborative Group, a case series study was conducted. This study enrolled 36 children with severe acute non-A-E hepatitis, who were admitted to 17 hospitals between April and July 2022. Clinical data, including etiological test results and liver function tests, were collected, and peripheral blood and nasopharyngeal swab specimens were obtained. Metagenomic next-generation sequencing (mNGS) was performed to detect potential infectious pathogens. Results: Among 36 children, there were 24 males and 12 females, with an onset age of 3.5 (1.1, 9.0) years. Common clinical symptoms were fever in 22 cases (61%), jaundice in 13 cases (36%), vomiting in 12 cases (33%), abdominal pain in 10 cases (28%), rash in 10 cases (28%), and diarrhea in 3 cases (8%). Serum alanine aminotransferase and aspartate aminotransferase levels were 950 (826, 1 404) and 811 (498, 1 295) U/L, respectively. Using PCR, plasma Epstein-Barr virus (EBV)-DNA was tested in 31 cases (86%) and plasma cytomegalovirus (CMV)-DNA in 25 cases (69%), and all results were below 5×10[5] copies/L. Plasma mNGS was performed on all 36 patients, detecting 11 viruses. These included EBV in 14 cases, CMV in 12 cases, human adenovirus in 2 cases, herpes simplex virus type 1 in 2 cases, adeno-associated virus type 2 (AAV2) in 1 case, and 6 other viruses. No patient tested positive for both human adenovirus and AAV2 simultaneously. Plasma mNGS results showed a viral read count of 4 (2, 10) per 1×10[8] reads. Nasopharyngeal swab mNGS was performed on 8 cases (22%), detecting 7 viruses. These included human herpesvirus 7 in 4 cases, EBV in 3 cases, and CMV in 3 cases, as well as 4 other viruses. Human adenovirus and AAV2 were not detected. Among the 4 children with human herpesvirus 7, 2 cases were also positive for human herpesvirus 6B, but neither virus was detected in their plasma mNGS. Conclusions: The detection rates of human adenovirus and AAV2 are both low among children with severe acute non-A-E hepatitis, and there are no cases of co-infection with both viruses.

Autoimmune hepatitis (AIH) is a chronic immune-mediated liver disease diagnosed by the clustering of compatible biochemical, serological, and histological features after exclusion of competing causes. Concurrent biliary obstruction or drug-induced injury can obscure the underlying autoimmune process and misdirect the initial diagnosis toward a purely obstructive or toxic cause. A 65-year-old woman with a remote cholecystectomy presented with one week of painless jaundice, dark urine, fatigue, and pruritus on a background of a year of slowly progressive aminotransferase elevation. She had also completed an unprescribed two-week course of oral ivermectin shortly before presentation. Admission studies showed marked hepatocellular injury (aspartate aminotransferase (AST): 2,109 U/L, alanine aminotransferase (ALT): 1,652 U/L), conjugated hyperbilirubinemia (total bilirubin: 8.4 mg/dL), mildly elevated alkaline phosphatase (ALP) (253 U/L), hypergammaglobulinemia (globulin: 5 g/dL; subsequent immunoglobulin G (IgG): 2,925 mg/dL), and an international normalized ratio (INR) of 1.6. With a negative viral hepatitis panel, a normal acetaminophen level, and bland cross-sectional imaging, empiric intravenous corticosteroids were begun on hospital day 2. Aminotransferases and bilirubin fell briskly over the next 72 hours, before any mechanical relief of obstruction. Magnetic resonance cholangiopancreatography (MRCP) on hospital day 4 revealed a 7-mm common bile duct stone, extracted at endoscopic retrograde cholangiopancreatography on hospital day 5. Smooth muscle antibody (SMA) returned at a titer of ≥1:640, with negative antinuclear antibodies (ANA) and antimitochondrial antibodies (AMA), a profile consistent with type 1 AIH. By a simplified International Autoimmune Hepatitis Group (IAIHG) score of 6, the combination of high-titer SMA, markedly elevated IgG, and a corticosteroid response that preceded biliary decompression identified AIH as the most likely dominant driver of injury, with choledocholithiasis as a concurrent second insult and ivermectin as a possible contributor of indeterminate magnitude. Obstructive imaging findings can anchor clinicians away from a concurrent, treatable autoimmune process, and a corticosteroid response that precedes mechanical decompression carries genuine diagnostic weight. Because histology was not obtained during the index admission, the relative contributions of the biliary stone and ivermectin cannot be fully excluded; long-term follow-up, including confirmatory biopsy and a structured trial of corticosteroid withdrawal, will clarify whether the disease is best classified as idiopathic AIH unmasked by concurrent insults or as a drug-related variant.

Bile duct injuries (BDI) after cholecystectomy often coexist with vascular injuries, but hepatic artery pseudoaneurysms may be missed on initial imaging. We report a right hepatic artery (RHA) pseudoaneurysm that manifested as haemobilia only after biliary decompression by Roux-en-Y hepaticojejunostomy for a Strasberg type E3 injury. A 28-year-old man presented 4 weeks after open cholecystectomy with obstructive jaundice. Magnetic resonance imaging confirmed a Strasberg type E3 stricture and identified a missed 8-mm RHA pseudoaneurysm. Following hepaticojejunostomy, haematemesis occurred on postoperative day 3. Computed tomography angiography demonstrated enlargement of the pseudoaneurysm to 12-mm with arteriobiliary communication. Covered stenting was followed by recurrent bleeding requiring glue-lipiodol embolization, complicated by non-target splenic embolization and an abscess requiring percutaneous drainage. The patient recovered and remained asymptomatic at three months. This case suggests that relief of biliary hypertension can unmask contained pseudoaneurysms and reinforces the importance of routine preoperative arterial-phase imaging in complex BDI.

We report the case of an 18-year-old female with cholestatic hepatopathy of unknown origin, presenting with therapy-resistant jaundice and pruritus following oral contraceptive use and gastrointestinal infections. In another hospital, drug-induced autoimmune hepatitis (DILI-AIH) was suspected, and corticosteroid therapy was initiated but remained unresponsive. Initiation of colestyramine led to a worsening of symptoms and increasing liver transaminases. After referral to our centre, a mini-laparoscopic liver biopsy was performed, and histopathology revealed cholestatic DILI and mild fibrosis, with no signs of AIH. After a repeated precise history from the mother, a hereditary fructose intolerance was highly suggestive, and genetic testing confirmed a homozygous mutation in the aldolase B gene (A149P). Additionally, no pathogenic mutations in genes encoding bile acid and phospholipid transporters were detected. The treatment was adjusted to sugar-free colestyramine, leading to complete resolution of symptoms. In the management of hereditary fructose intolerance, not only dietary ingredients but also the supplements and medications may contain fructose excipients that could exacerbate and aggravate liver injuries.

Erythropoietic protoporphyria (EPP) is a disease caused by an inborn error of heme biosynthesis. It manifests as painful photosensitivity in childhood. Liver disease occurs due to the deposition of protoporphyrin. Cholestasis due to protoporphyrin leads to a vicious cycle of worsening, which can lead to liver failure. A 29-year-old woman presented with a three-month history of intermittent low-grade fever and a one-month history of jaundice. She had a history of painful blisters over the skin in childhood that started at two years of age and progressed to multiple contractures from healed ulcers by five years of age. She also had splenomegaly on examination. Her blood tests showed pancytopenia, hyperbilirubinemia, and elevated liver enzymes. Tropical fever workup was negative. Autoimmune hepatitis was considered, as antinuclear antibodies (ANA) were positive with elevated immunoglobulin G (IgG). Skin biopsy showed features suggestive of lipoid proteinosis. However, on review with Periodic Acid-Schiff-diastase (PAS-D) staining, a close mimic, EPP was considered a possibility. Liver biopsy showed features of EPP with infiltration with refractile brown pigment showing bright-red birefringence on polarizing microscopy. Next-generation sequencing (NGS) confirmed EPP with a ferrochelatase enzyme coding gene mutation. The patient developed pneumonia during hospitalization and developed acute on chronic liver failure (ACLF) grade 3a, as defined by the European Association for the Study of the Liver (EASL), with ascites and encephalopathy. She was treated with photoprotective measures, ursodeoxycholic acid, antibiotics, and supportive care. The family was counseled regarding options for sequential hepatic and bone marrow transplants. She was managed with the best supportive care.

We describe the case of a woman in her 40s with heavy alcohol use who presented with jaundice, persistent fever and severe liver dysfunction initially managed as alcohol-associated hepatitis. Despite broad-spectrum antibiotics and negative septic screening, she remained febrile. Further investigation, prompted by rural livestock exposure and recent family exposure to Q fever, revealed acute Coxiella burnetii infection, supported by phase II-predominant Q fever serology, including phase II IgG IFA titre 1:2048, and positive serum/blood PCR. Liver biopsy demonstrated moderate-to-severe alcohol-associated steatohepatitis with numerous fibrin-ring granulomas, confirming dual pathology. Fever resolved after doxycycline was commenced; however, severe hepatic dysfunction persisted. Prednisolone was initiated after doxycycline had started, fever had resolved and no uncontrolled bacterial focus was identified, resulting in marked biochemical improvement and a Lille score of 0.054. This case highlights the challenge of distinguishing infection from sterile inflammation in alcohol-associated hepatitis and emphasises the importance of considering atypical zoonotic infection before immunosuppressive therapy.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome characterized by dysregulated immune activation and cytokine storm, frequently triggered by infections, autoimmune disorders, and malignancies. Among adults, lymphoma-associated HLH represents one of the most aggressive subtypes and carries significant mortality if not recognized early. We report the case of a 24-year-old male who initially presented with constitutional symptoms, progressive jaundice, and biochemical features suggestive of acute hepatitis. Initial evaluation focused on hepatic and autoimmune etiologies; however, progressive pancytopenia, marked hyperferritinemia (>15,000 ng/mL), hypofibrinogenemia, hypertriglyceridemia, and hepatosplenomegaly raised suspicion for HLH. Further imaging revealed diffuse lymphadenopathy and infiltrative hepatic lesions. Histopathological examination of a supraclavicular lymph node confirmed diffuse large B-cell lymphoma (DLBCL). The patient fulfilled HLH diagnostic criteria with an HScore of 200, indicating high probability of disease. Despite prompt initiation of dexamethasone and immunomodulatory therapy, the patient deteriorated rapidly, necessitating transfer to a tertiary oncology center. This case highlights the diagnostic challenge posed by hepatic-predominant HLH and emphasizes the importance of considering HLH in patients presenting with unexplained hepatitis, cytopenias, and hyperferritinemia.

Postpartum autoimmune hepatitis (AIH) is a rare but serious condition triggered by immune rebound after delivery. Diagnosis is typically supported by elevated immunoglobulins and positive autoimmune markers; however, seronegative presentations remain diagnostically challenging. We present the fourth reported worldwide case of seronegative postpartum AIH in a 36-year-old woman who developed jaundice, pruritus, pale stools, and dark urine 4 months after delivery. Laboratory evaluation revealed severe hepatocellular injury with aspartate aminotransferase 1373 U/L, alanine aminotransferase 2137 U/L, and marked hyperbilirubinemia. Viral, toxic, metabolic, and rheumatologic investigations were negative, while autoimmune serologies and immunoglobulin levels remained normal. Imaging demonstrated hepatomegaly without biliary obstruction or vascular abnormalities. Liver biopsy revealed acute-on-chronic autoimmune-mediated hepatitis with necrosis, confirming seronegative postpartum AIH. This case emphasizes seronegative AIH as a critical consideration in postpartum acute hepatitis, with liver biopsy essential for diagnosis and early treatment.

Congenital dyserythropoietic anemia type IV (CDA IV) is a rare inherited erythroid disorder within the broad phenotypic spectrum associated with pathogenic variants in Krüppel-like factor 1 (KLF1), a master transcriptional regulator of erythropoiesis. This study aimed to describe the clinical picture, genetic causes, global distribution, and treatment of CDA IV. We retrospectively reviewed three pediatric patients diagnosed and treated at the Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, since December 2014. Demographic, clinical, genetic, laboratory, treatment, transplantation, and follow-up data were collected, including conditioning regimens, graft-versus-host disease (GVHD) prophylaxis, engraftment, complications, and donor chimerism. We also conducted a literature review of CDA IV cases reported worldwide between January 1991 and December 2024. All three children developed symptoms within the first month of life. They had neonatal jaundice and anemia. One of them needed intrauterine transfusion. Gene testing found four KLF1 variants: c.525_526insCGGCGCC, c.1012C > T, c.1012C > A, and c.973G > A. Before hematopoietic stem cell transplantation (HSCT), all three patients needed regular red blood cell transfusions and had iron overload. All three then received HSCT from a parent or sibling. Neutrophils and platelets engrafted in every case. Donor chimerism stayed above 95% during follow-up, and no graft failure happened. No patient had acute or chronic GVHD. Viral reactivation after HSCT was mainly cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infection, and no severe infection was seen. At the last follow-up, all three patients were free of transfusion. Serum ferritin levels went down after HSCT, but liver or heart iron overload did not fully go away in some patients. For carefully chosen children with severe transfusion-dependent KLF1-related red cell disease, allogeneic HSCT may be a feasible curative option. Stable donor chimerism and lasting freedom from transfusion can be achieved. CDA IV has many clinical forms and can be mistaken for thalassemia or Evans syndrome, so early gene testing is very important for correct diagnosis. In areas where thalassemia is common, KLF1 mutation screening should be considered when the cause of microcytic anemia is unclear. Bigger studies with longer follow-up are still needed to better judge the long-term results of HSCT.

ObjectiveNeonatal hyperbilirubinemia (NHB) is a common clinical condition that may lead to long-term neurodevelopmental impairment due to bilirubin-induced neurotoxicity. The development of reliable methods for neurological monitoring in affected newborns remains a major challenge in neonatal care. This study aimed to evaluate the association between treatment and EEG changes on cerebral activity in infants with hyperbilirubinemia using conventional EEG.Methods & MaterialsThis prospective before-and-after study was conducted on neonates aged over 35 weeks of gestation who were diagnosed with hyperbilirubinemia. Demographic and clinical data, including neonatal age, sex, birth weight, presence of ABO incompatibility, and treatment modalities, were collected. Maternal data, including parity, mode of delivery, and maternal and neonatal blood groups, were also recorded.Conventional EEG was performed for all neonates during the first eight hours of hospitalization and repeated after therapeutic interventions, including phototherapy and, when indicated, exchange transfusion.ResultsA total of 26 neonates with hyperbilirubinemia were included in this study. The mean total serum bilirubin level before treatment was 23.01 ± 2.99 mg/dL.A statistically significant change in the EEG spectral power was observed before and after treatment in the frontal regions Fp1, Fp2, and Fz, particularly within the delta and beta frequency bands.Furthermore, the findings demonstrated that delta activity was significantly dominant in the Fp1, Fp2, and Fz regions both before and after treatment. In contrast, no dominant frequency bands were observed in the C3, O1, T3, C4, T4, Cz, and O2 regions either before or after treatment.ConclusionHyperbilirubinemia in neonates may be associated with alterations in cortical electrical activity as assessed by conventional EEG. In our study, delta band activity was significantly predominant in the frontal regions (Fp1, Fp2, and Fz) both before and after treatment. Moreover, delta band activity remained the predominant frequency in the frontal regions (Fp1, Fp2, and Fz) before and after treatment, suggesting persistent frontal cortical involvement despite treatment.Further studies with larger samples are recommended to clarify whether these EEG changes reflect a transient bilirubin effect or age-related maturation patterns.