Congenital intrahepatic arterioportal fistula is a rare but treatable cause of portal hypertension for which early recognition may lead to successful radiological management. We report an infant presenting with severe failure to thrive, melena and splenomegaly due to a congenital intrahepatic arterioportal fistula, successfully ablated after multiple trials of superselective transarterial embolization. Comprehensive review of congenital cases provides an understanding of the key clinical features defining this syndrome. A classification system is proposed, upon which treatment decisions may be based.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1097/01.mpg.0000221890.13630.ad | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Intrahepatic portosystemic shunt: salvage mechanism for oligohydramnios complicating fetal growth restriction.
Ultrasound Obstet Gynecol
January 2025
Ultrasound Unit, Helen Schneider Hospital for Women, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel.
Objective: Portosystemic shunts in growth-restricted fetuses are more common than previously thought. We aimed to describe fetuses with growth restriction and transient oligohydramnios in which a congenital intrahepatic portosystemic shunt (CIPSS) was noted during follow-up.
Methods: This was a retrospective study of all fetuses diagnosed with growth restriction and transient oligohydramnios during a 5-year period in a large tertiary referral center.
A large type IV-A choledochal cyst mimicking hydatid cyst of the liver: A case report.
Int J Surg Case Rep
January 2025
Department of Surgery, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.
Introduction: Choledochal cysts are rare congenital anomalies of the bile ducts, with adult presentations being uncommon. This case is notable for its atypical presentation in a young adult, mimicking a hydatid cyst in a region where echinococcosis is endemic.
Case Presentation: A 22-year-old female presented with a 3-month history of progressive jaundice, accompanied by 5 months of epigastric and right upper quadrant pain, dark urine, pale stools, pruritus, and significant weight loss.
Retrospective analysis of the incidence and clinicopathological findings associated with ammonium urate urolithiasis in dogs with congenital portosystemic shunts: 363 cases (2010-2023).
J Small Anim Pract
January 2025
Southern Counties Veterinary Specialists, Ringwood, UK.
Objectives: To assess the incidence and clinical findings associated with the presence of ammonium urate urolithiasis in dogs with congenital portosystemic shunts.
Materials And Methods: A retrospective review of dogs diagnosed with extrahepatic portosystemic shunts or intrahepatic portosystemic shunts in 15 referral hospitals between 2010 and 2023. Data including signalment, clinical signs, physical examination findings and clinicopathologic test results at the time of the diagnosis were collected, and the presence of ammonium urate urolithiasis was recorded.
An animal model recapitulates human hepatic diseases associated with mutations.
Proc Natl Acad Sci U S A
January 2025
Key Laboratory of Freshwater Fish Reproduction and Development, Ministry of Education, State Key Laboratory Breeding Base of Eco-Environments and Bio-Resources of the Three Gorges Reservoir Region, School of Life Sciences, Southwest University, Chongqing 400715, China.
Heterozygotic mutations are responsible for various congenital diseases in the heart, pancreas, liver, and other organs in humans. However, there is lack of an animal that can comprehensively model these diseases since GATA6 is essential for early embryogenesis. Here, we report the establishment of a knockout zebrafish which recapitulates most of the symptoms in patients with mutations, including cardiac outflow tract defects, pancreatic hypoplasia/agenesis, gallbladder agenesis, and various liver diseases.
View Article and Find Full Text PDFAldolase B Deficient Mice Are Characterized by Hepatic Nucleotide Sugar Abnormalities.
J Inherit Metab Dis
January 2025
Department of Internal Medicine, Division of Endocrinology and Metabolic Disease, Maastricht University Medical Center+, Maastricht, The Netherlands.
Hereditary fructose intolerance (HFI) is characterized by liver damage and a secondary defect in N-linked glycosylation due to impairment of mannose phosphate isomerase (MPI). Mannose treatment has been shown to be an effective treatment in a primary defect in MPI (i.e.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!