Cystic fibrosis and portal hypertension interest of partial splenectomy.

Eur J Pediatr Surg

Hôpital Debrousse, Lyon, France.

Published: February 1993

Extra pulmonary complications can be major in patients suffering of cystic fibrosis who survive long enough without pulmonary problems. Hepatic cirrhosis is often present and is responsible for portal hypertension with splenomegaly, hypersplenism and esophageal varices. In six patients we have performed a partial splenectomy with conservation of the upper pole of the spleen vascularized by gastrosplenic omentum and its vessels. The post-operative complications (3 scar ruptures but no pulmonary decompensation) are not very important considering the benefits: Normal spleen according to clinical, ultrasonic examination and scintigraphy Correction of hypersplenism Diminution of esophageal varices Stability of hepatic functions. With a post-operative follow-up between 2.5 and 7 years, none of the patients had an increased risk of infection. This technique allows a good survival for those patients waiting for pulmonary transplantation.

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-2008-1063501DOI Listing

Publication Analysis

Top Keywords

cystic fibrosis
8
portal hypertension
8
partial splenectomy
8
esophageal varices
8
fibrosis portal
4
hypertension interest
4
interest partial
4
splenectomy extra
4
pulmonary
4
extra pulmonary
4

Similar Publications

Perinatal dysfunction of innate immunity in cystic fibrosis.

Sci Transl Med

January 2025

First Department of Medicine, Cardiology, TUM University Hospital, Technical University of Munich, School of Medicine and Health, Munich 81675, Germany.

In patients with cystic fibrosis (CF), repeated cycles of infection and inflammation eventually lead to fatal lung damage. Although diminished mucus clearance can be restored by highly effective CFTR modulator therapy, inflammation and infection often persist. To elucidate the role of the innate immune system in CF etiology, we investigated a CF pig model and compared these results with those for preschool children with CF.

View Article and Find Full Text PDF

A new era of cystic fibrosis therapy with CFTR modulators.

J Bras Pneumol

January 2025

. Grupo de Pesquisa em Epidemiologia e Genética das Doenças Respiratórias da Infância, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil.

View Article and Find Full Text PDF

The complex (BCC) is a group of Gram-negative bacteria that cause opportunistic infections, most notably in people with cystic fibrosis (CF), and have been associated with outbreaks caused by contaminated medical products. Antimicrobial susceptibility testing (AST) is often used to guide treatment for BCC infections, perhaps most importantly in people with CF who are being considered for lung transplant. However, recent studies have highlighted problems with AST methods.

View Article and Find Full Text PDF

is an understudied, gram-negative, aerobic bacterium that is widespread in the environment and increasingly a cause of opportunistic infections. Treating remains difficult, leading to an increase in disease severity and higher hospitalization rates in people with cystic fibrosis, cancer, and other immunocompromised health conditions. The lack of effective antibiotics has led to renewed interest in phage therapy; however, there remains a great need for well-characterized phages, especially against .

View Article and Find Full Text PDF

Want 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!