Chronic renal failure and portal hypertension - is portosystemic shunt indicated?

Tsimaratos, Michel; Cloarec, Sylvie; Roquelaure, Bertrand; Retornaz, K.; Picon, G; Chabrol, B.; Guys, Jean-Michel; Sarles, J.; Nivet, Hubert

doi:10.1007/s004679900268

Cited by 22 publications

(14 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This evolving renal insufficiency has the potential to complicate efforts to use portosystemic shunting as a treatment option for refractory recurrent variceal hemorrhage or other complications of portal hypertension. The existing literature reveals a few cases in which this is an issue (8–10), although, in general, this may be more theoretical than real in most circumstances. Surgical or interventional portosystemic shunting is not necessarily contraindicated in CHF/ARPKD, but it needs to be carefully considered in the context of the existing state and pace of the renal disease.…”

Section: Discussionmentioning

confidence: 99%

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

View full text Add to dashboard Cite

Objectives The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD). Methods A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation. Results Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0–38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33–75 years). There was a relative paucity of data on combined liver-kidney transplantation. Conclusions Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.

show abstract

Section: Discussionmentioning

confidence: 99%

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

View full text Add to dashboard Cite

show abstract

“…This compensatory mechanism is limited with significant renal dysfunction. Placement of a shunt in a child with ESKD can increase the risk of hepatic encephalopathy from hyperammonemia [37]. Portosystemic shunt may be considered after successful renal transplantation.…”

Section: Portosystemic Shuntingmentioning

confidence: 99%

Transplantation in autosomal recessive polycystic kidney disease: liver and/or kidney?

et al. 2014

View full text Add to dashboard Cite

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by enlarged kidneys with dilated collecting ducts and congenital hepatic fibrosis. There is a variable rate of progression of kidney and liver disease. Portal hypertension and Caroli's disease occur from liver involvement that contributes to morbidity and mortality. Approximately 40 % of patients have a severe disease phenotype leading to rapid onset of end-stage kidney disease (ESKD) and signs of portal hypertension and the rest may have predominant involvement of either the kidney or liver. It is important for the physician to establish the extent of organ involvement before deciding on the ultimate plan of management, especially when transplantation is required. Isolated renal transplantation can be considered when liver involvement is minimal. If hepatobiliary disease is prominent, and kidney function is preserved, management options are based on individual characteristics. In the presence of significant liver disease and ESKD, consideration should be given to combined liver kidney transplantation, which can be beneficial in eliminating the consequences of both kidney and liver disease. However, this is a complex surgical procedure that needs to be performed at experienced transplant centers. Improvement in surgical techniques has considerably improved short-term graft survival with the added advantage of the liver offering immunologic protection to the kidney allograft.

show abstract

“…Portal decompressive surgical shunts are uncommon in pediatric ARPKD patients and are only implanted at specialized transplant centers [20]. Because normal kidney function plays a pivotal role in ammonia disposal, porto-systemic shunting can be especially contraindicated in patients with impaired kidney function, unless they have successfully undergone kidney transplantation [12,21].…”

Section: Hepatobiliary Diseasementioning

confidence: 99%

“…In the largest reported series of ARPKD patients with advanced CHF, the most common procedure was a splenorenal shunt [12]. However, owing to consecutive hyperammonemia and an increase in renal ammonia disposal in response to decreased hepatic detoxification, this intervention has recently been associated with a high risk of hepatic encephalopathy in patients who in the short term progressed to ESRD [21]. Because normal kidney function is central to ammonia disposal, portosystemic shunting cannot be recommended for anephric patients or those with ESRD unless they have already successfully undergone kidney transplantation [12,21].…”

Section: Treatment Options For Kidney-related and Non-kidney-related mentioning

confidence: 99%

Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes

Büscher

Weber

et al. 2013

Pediatr Nephrol

View full text Add to dashboard Cite

Autosomal recessive polycystic kidney disease (ARPKD), although less frequent than the dominant form, is a common, inherited ciliopathy of childhood that is caused by mutations in the PKHD1-gene on chromosome 6. The characteristic dilatation of the renal collecting ducts starts in utero and can present at any stage from infancy to adulthood. Renal insufficiency may already begin in utero and may lead to early abortion or oligohydramnios and lung hypoplasia in the newborn. However, there are also affected children who have no evidence of renal dysfunction in utero and who are born with normal renal function. Up to 30 % of patients die in the perinatal period, and those surviving the neonatal period reach end stage renal disease (ESRD) in infancy, early childhood or adolescence. In contrast, some affected patients have been diagnosed as adults with renal function ranging from normal to moderate renal insufficiency to ESRD. The clinical spectrum of ARPKD is broader than previously recognized. While bilateral renal enlargement with microcystic dilatation is the predominant clinical feature, arterial hypertension, intrahepatic biliary dysgenesis remain important manifestations that affect approximately 45 % of infants. All patients with ARPKD develop clinical findings of congenital hepatic fibrosis (CHF); however, non-obstructive dilation of the intrahepatic bile ducts in the liver (Caroli's disease) is seen at the histological level in only a subset of patients. Cholangitis and variceal bleeding, sequelae of portal hypertension, are life-threatening complications that may occur more often in advanced cases of liver disease. In this review we focus on common and uncommon kidney-related and non-kidney-related phenotypes. Clinical management of ARPKD patients should include consideration of potential problems related to these manifestations.

show abstract

Chronic renal failure and portal hypertension - is portosystemic shunt indicated?

Cited by 22 publications

References 7 publications

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Transplantation in autosomal recessive polycystic kidney disease: liver and/or kidney?

Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes

Contact Info

Product

Resources

About