Topi T Luoto1, Mikko P Pakarinen, Timo Jahnukainen, Hannu Jalanko. 1. *Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Central Hospital †Department of Pediatric Nephrology and Transplantation, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Finland.
Abstract
OBJECTIVES: We correlated liver and kidney manifestations in a national cohort of patients with autosomal recessive polycystic kidney disease (ARPKD). METHODS: A total of 27 consecutive patients with ARPKD were included. Hepatobiliary disorders were comparatively evaluated in 2 groups: children in group 1 (n = 10) displayed renal failure as infants and those in group 2 (n = 17) had normal kidney function through the first year of life. RESULTS: Median follow-up time was 10.6 (range, 0.4-40) years. Portal hypertension was diagnosed in 13 patients (48%) at the median age 5.0 (1.5-27.9) years. Esophageal varices developed in 8 patients (30%) at age 8.0 (2.1-11.9) years; 4 patients (15%) had variceal bleeding, and hypersplenism/splenomegaly occurred in 52%, similarly in both groups. Biliary tract dilatation was detected at 2.8 years in group 1 and at 7.9 years in group 2, significantly more frequently in group 1 (60% vs 18%, P = 0.039), causing cholangitis in 2 (20%) versus none in group 2 (P = 0.055). A total of 10 patients (37%) underwent cadaveric liver transplantation (LT) at a median age of 6.6 (1.0-20.0) years. In 1 patient LT was performed because of hepatoblastoma. Nine of these were combined liver-kidney transplantations (CLKT). Patients in group 1 required LT earlier (4.1 years vs 18.2 years, P = 0.017) and more frequently (70% vs 18%, P = 0.01). Overall survival beyond neonatal period was 85%. Two patients died because of infectious complications after CLKT, and 1 patient because of recurrent hepatoblastoma. CONCLUSIONS: Although correlation of renal and liver manifestations was variable, biliary dilatation was associated with early renal failure. CLKT may be a treatment for patients with ARPKD with marked hepatobiliary complications.
OBJECTIVES: We correlated liver and kidney manifestations in a national cohort of patients with autosomal recessive polycystic kidney disease (ARPKD). METHODS: A total of 27 consecutive patients with ARPKD were included. Hepatobiliary disorders were comparatively evaluated in 2 groups: children in group 1 (n = 10) displayed renal failure as infants and those in group 2 (n = 17) had normal kidney function through the first year of life. RESULTS: Median follow-up time was 10.6 (range, 0.4-40) years. Portal hypertension was diagnosed in 13 patients (48%) at the median age 5.0 (1.5-27.9) years. Esophageal varices developed in 8 patients (30%) at age 8.0 (2.1-11.9) years; 4 patients (15%) had variceal bleeding, and hypersplenism/splenomegaly occurred in 52%, similarly in both groups. Biliary tract dilatation was detected at 2.8 years in group 1 and at 7.9 years in group 2, significantly more frequently in group 1 (60% vs 18%, P = 0.039), causing cholangitis in 2 (20%) versus none in group 2 (P = 0.055). A total of 10 patients (37%) underwent cadaveric liver transplantation (LT) at a median age of 6.6 (1.0-20.0) years. In 1 patient LT was performed because of hepatoblastoma. Nine of these were combined liver-kidney transplantations (CLKT). Patients in group 1 required LT earlier (4.1 years vs 18.2 years, P = 0.017) and more frequently (70% vs 18%, P = 0.01). Overall survival beyond neonatal period was 85%. Two patients died because of infectious complications after CLKT, and 1 patient because of recurrent hepatoblastoma. CONCLUSIONS: Although correlation of renal and liver manifestations was variable, biliary dilatation was associated with early renal failure. CLKT may be a treatment for patients with ARPKD with marked hepatobiliary complications.
Authors: Charlotte Gimpel; E Fred Avni; Luc Breysem; Kathrin Burgmaier; Anna Caroli; Metin Cetiner; Dieter Haffner; Erum A Hartung; Doris Franke; Jens König; Max C Liebau; Djalila Mekahli; Albert C M Ong; Lars Pape; Andrea Titieni; Roser Torra; Paul J D Winyard; Franz Schaefer Journal: Radiology Date: 2019-01-01 Impact factor: 11.105
Authors: Nevil Kadakia; Steven J Lobritto; Nadia Ovchinsky; Helen E Remotti; Darrell J Yamashiro; Jean C Emond; Mercedes Martinez Journal: Front Pediatr Date: 2017-06-07 Impact factor: 3.418
Authors: Brian D Friend; Kami Wolfe Schneider; Timothy Garrington; Laurel Truscott; Julian A Martinez-Agosto; Robert S Venick; Eileen Tsai Chambers; Patricia Weng; Douglas G Farmer; Vivian Y Chang; Noah Federman Journal: Mol Genet Genomic Med Date: 2019-06-14 Impact factor: 2.183
Authors: Choong H Lee; Amber K O'Connor; Chaozhe Yang; Joshua M Tate; Trenton R Schoeb; Jeremy J Flint; Stephen J Blackband; Lisa M Guay-Woodford Journal: Physiol Rep Date: 2015-08
Authors: Dorota Wicher; Irena Jankowska; Patryk Lipiński; Paulina Szymańska-Rożek; Jakub Kmiotek; Wojciech Jańczyk; Jacek Rubik; Krystyna Chrzanowska; Piotr Socha Journal: Front Pediatr Date: 2019-01-11 Impact factor: 3.418
Authors: Kathrin Burgmaier; Gema Ariceta; Martin Bald; Anja Katrin Buescher; Mathias Burgmaier; Florian Erger; Michaela Gessner; Ibrahim Gokce; Jens König; Claudia Kowalewska; Laura Massella; Antonio Mastrangelo; Djalila Mekahli; Lars Pape; Ludwig Patzer; Alexandra Potemkina; Gesa Schalk; Raphael Schild; Rukshana Shroff; Maria Szczepanska; Katarzyna Taranta-Janusz; Marcin Tkaczyk; Lutz Thorsten Weber; Elke Wühl; Donald Wurm; Simone Wygoda; Ilona Zagozdzon; Jörg Dötsch; Jun Oh; Franz Schaefer; Max Christoph Liebau Journal: Sci Rep Date: 2020-09-29 Impact factor: 4.379