Universität zu Köln

Wicher, Dorota, Grenda, Ryszard, Teisseyre, Mikolaj, Szymczak, Marek, Halat-Wolska, Paulina, Jurkiewicz, Dorota, Liebau, Max Christoph, Ciara, Elztbieta, Rydzanicz, Malgorzata, Kosinska, Joanna, Chrzanowska, Krystyna and Jankowska, Irena (2020). Occurrence of Portal Hypertension and Its Clinical Course in Patients With Molecularly Confirmed Autosomal Recessive Polycystic Kidney Disease (ARPKD). Front. Pediatr., 8. LAUSANNE: FRONTIERS MEDIA SA. ISSN 2296-2360

Full text not available from this repository.

Abstract

Purpose: Liver involvement in autosomal recessive polycystic kidney disease (ARPKD) leads to the development of portal hypertension and its complications. The aim of this study was to analyze the occurrence of the portal hypertension and its clinical course and the dynamics in patients with molecularly confirmed ARPKD in a large Polish center. Moreover, the available options in diagnostics, prevention and management of portal hypertension in ARPKD will be discussed. Materials and Methods: The study group consisted of 17 patients aged 2.5-42 years. All patients had ARPKD diagnosis confirmed by molecular tests. Retrospective analysis included laboratory tests, ultrasound and endoscopic examinations, transient elastography and clinical evaluation. Results: Any symptom of portal hypertension was established in 71% of patients. Hypersplenism, splenomegaly, decreased portal flow and esophageal varices were found in 47, 59, 56, and 92% of patients, respectively. Gastrointestinal bleeding occurred in four of 17 patients. Endoscopic variceal ligation (EVL) was performed at least once in nine patients with esophageal varices. Conclusions: Portal hypertension and its complications are present in a significant percentage of ARPKD patients. They should be under the care of multidisciplinary nephrology-gastroenterology/hepatology team. Complications of portal hypertension may occur early in life. Endoscopic methods of preventing gastroesophageal bleeding, such as endoscopic variceal ligation, are effective and surgical techniques, including liver transplantation, are required rarely.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Wicher, Dorota UNSPECIFIED UNSPECIFIED UNSPECIFIED Grenda, Ryszard UNSPECIFIED UNSPECIFIED UNSPECIFIED Teisseyre, Mikolaj UNSPECIFIED UNSPECIFIED UNSPECIFIED Szymczak, Marek UNSPECIFIED UNSPECIFIED UNSPECIFIED Halat-Wolska, Paulina UNSPECIFIED UNSPECIFIED UNSPECIFIED Jurkiewicz, Dorota UNSPECIFIED UNSPECIFIED UNSPECIFIED Liebau, Max Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Ciara, Elztbieta UNSPECIFIED UNSPECIFIED UNSPECIFIED Rydzanicz, Malgorzata UNSPECIFIED UNSPECIFIED UNSPECIFIED Kosinska, Joanna UNSPECIFIED UNSPECIFIED UNSPECIFIED Chrzanowska, Krystyna UNSPECIFIED UNSPECIFIED UNSPECIFIED Jankowska, Irena UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-311523
DOI:	10.3389/fped.2020.591379
Journal or Publication Title:	Front. Pediatr.
Volume:	8
Date:	2020
Publisher:	FRONTIERS MEDIA SA
Place of Publication:	LAUSANNE
ISSN:	2296-2360
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language PRIMARY PROPHYLAXIS; CHILDREN; MANAGEMENT; TRANSPLANTATION; MANIFESTATIONS; DIAGNOSIS; LIGATION; VARICES Multiple languages Pediatrics Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/31152