Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease

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Abstract

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3years, range 1-22.3years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5years (range 3.1-33.6years). One and 5years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n=3), sclerotherapy (n=2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n=1). Of the nine survivors (mean age 12.8years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.

Original languageEnglish (US)
Pages (from-to)360-365
Number of pages6
JournalAmerican Journal of Transplantation
Volume2
Issue number4
DOIs
StatePublished - Apr 1 2002

Fingerprint

Autosomal Recessive Polycystic Kidney
Kidney Transplantation
Morbidity
Kidney
Transplants
Portal Hypertension
Dilatation
Bile Ducts
Survival Rate
Hypersplenism
Surgical Portasystemic Shunt
Transjugular Intrahepatic Portasystemic Shunt
Sclerotherapy
Cholangitis
Esophageal and Gastric Varices
Mortality
Congenital Hepatic Fibrosis
Liver
Splenomegaly
Liver Failure

Keywords

  • Autosomal recessive polycystic kidney disease
  • Congenital hepatic fibrosis

Cite this

@article{4999136fafd340f3b96224af4ee76a8e,
title = "Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease",
abstract = "Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3years, range 1-22.3years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5years (range 3.1-33.6years). One and 5years patient survival rates were 93{\%} and 86{\%}, respectively. Overall five patients (36{\%}) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87{\%} and 70{\%}, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n=3), sclerotherapy (n=2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n=1). Of the nine survivors (mean age 12.8years) 78{\%} have functioning grafts (one liver-kidney transplant), 63{\%} have portal hypertension and 22{\%} have asymptomatic biliary dilatation. Complications of CHF developed in 79{\%} of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29{\%} and accounted for 80{\%} (4/5) of the deaths.",
keywords = "Autosomal recessive polycystic kidney disease, Congenital hepatic fibrosis",
author = "Khalid Khan and Schwarzenberg, {Sara Jane} and Sharp, {Harvey L.} and Matas, {Arthur J.} and Chavers, {Blanche M.}",
year = "2002",
month = "4",
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doi = "10.1034/j.1600-6143.2002.20412.x",
language = "English (US)",
volume = "2",
pages = "360--365",
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TY - JOUR

T1 - Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease

AU - Khan, Khalid

AU - Schwarzenberg, Sara Jane

AU - Sharp, Harvey L.

AU - Matas, Arthur J.

AU - Chavers, Blanche M.

PY - 2002/4/1

Y1 - 2002/4/1

N2 - Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3years, range 1-22.3years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5years (range 3.1-33.6years). One and 5years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n=3), sclerotherapy (n=2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n=1). Of the nine survivors (mean age 12.8years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.

AB - Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3years, range 1-22.3years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5years (range 3.1-33.6years). One and 5years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n=3), sclerotherapy (n=2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n=1). Of the nine survivors (mean age 12.8years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.

KW - Autosomal recessive polycystic kidney disease

KW - Congenital hepatic fibrosis

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