Idiopathic non-cirrhotic portal hypertension in a patient with Talaromyces marneffei infection: a case report

The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patient...

Full description

Saved in:
Bibliographic Details
Published in:BMC infectious diseases 2023-03, Vol.23 (1), p.125-125, Article 125
Main Authors: Ye, Xiuling, Quan, Xin, Guo, Xu, Wang, Zhidong, Wu, Hao
Format: Article
Language:English
Subjects:
Abdomen
Autoimmune diseases
Bacterial infections
Biopsy
Birth defects
Case Report
Case reports
Case studies
Causes of
Colorectal diseases
Complications and side effects
Congenital defects
Disorders
Endoscopy
Esophageal and Gastric Varices
Gastrointestinal diseases
Gastrointestinal Hemorrhage
Genetic disorders
Health aspects
Hematological diseases
Hemoglobin
HIV
HIV patients
Human immunodeficiency virus
Humans
Hyper-IgE syndrome
Hypertension
Idiopathic non-cirrhotic portal hypertension
Idiopathic Noncirrhotic Portal Hypertension
Immune system
Immunodeficiency
Immunoglobulin E
Immunology
Infection
Infections
Infectious diseases
Intestinal infections
Intestine
Intraabdominal Infections
Job's syndrome
Liver cirrhosis
Liver diseases
Lymphocytes
Mutation
Mycoses
Pathogenesis
Patients
Portal hypertension
Primary immunodeficiencies
Talaromyces marneffei
Tomography
Veins & arteries
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patients with HIV, or various haematological disorders or autoimmune diseases. We aim to discuss the possible etiopathogenesis of INCPH. We reported that a patient with intestinal infection of T. Marneffei and hyper-IgE syndrome, a group of rare primary immunodeficiency disorders, was finally diagnosed with INCPH for gastroesophageal variceal bleeding. The diagnosis was mainly based on histopathological features. Transjugular intrahepatic portosystemic shunt was performed and there was no recurrence of melena during the six-month follow-up. In the context of immunodeficiency, INCPH may associated with intestinal infections. Thus, screening for enterogenic infection and immunological disorders in patients with unexplained portal hypertension is necessary.
ISSN:1471-2334
1471-2334
DOI:10.1186/s12879-023-08090-6