Portal Hypertension Due to Extensive Hepatic Cysts in Autosomal Dominant Polycystic Kidney Disease

Misra, Anurag; Loyalka, Pranav; Alva, Felix

doi:10.1097/00007611-199906000-00015

Cited by 15 publications

(10 citation statements)

References 7 publications

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“…Although cirrhosis and heart failure represent potentially confounding disorders in the current study of cisterna chyli, patients with these disorders were excluded from the current study. It is, therefore, feasible that the dilated cisternae chyli observed in ADPKD patients in this study may reflect increased lymphatic fluid production from transudation by kidney and liver cysts, and by the inflammation that characterizes this disorder .…”

Section: Discussionmentioning

confidence: 87%

Cisterna chyli in autosomal dominant polycystic kidney disease

Thimmappa

Blumenfeld

Cerilles

et al. 2014

Magnetic Resonance Imaging

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Section: Discussionmentioning

confidence: 87%

Cisterna chyli in autosomal dominant polycystic kidney disease

Thimmappa

Blumenfeld

Cerilles

et al. 2014

Magnetic Resonance Imaging

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“…Rarely, patients may develop massive polycystic liver disease that requires surgical resection. There have been case reports of ADPKD patients with liver disease characterized by ductal plate malformation as seen in ARPKD 9–11 as well as patients who develop portal hypertension secondary to compression effect of the portal vein from extensive liver cysts 12 …”

Section: Clinical Featuresmentioning

confidence: 99%

Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Wen

2011

Clinical Translational Sci

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Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD-associated liver disease and will highlight areas of ongoing research into its pathogenesis and potential treatment.

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“…9 However, the incidence of clinically relevant PVO and ICVS is probably rare as the literature is limited to a few case reports. 10,[14][15][16][17] Interestingly, both presentations may be caused by a single, strategically located (very) large cyst. 10,18…”

Section: Incidence Of Portal Hypertension In Pldmentioning

confidence: 99%

“…In addition, the cohort study documented that liver biopsy findings were compatible with abnormal portal spaces in 67% of patients and portal vein dilation was present in 7% of patients . However, the incidence of clinically relevant PVO and ICVS is probably rare as the literature is limited to a few case reports . Interestingly, both presentations may be caused by a single, strategically located (very) large cyst …”

Section: Introductionmentioning

confidence: 99%

Management of portal hypertension and ascites in polycystic liver disease

Bernts

Drenth

Tjwa

2019

Liver International

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Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication. Diagnosis is made with imaging such as ultrasound, computed tomography or magnetic resonance imaging. Therapy includes conventional therapy with diuretics and paracentesis, and medical therapy using somatostatin analogues. Based on disease phenotype various (non‐)surgical liver‐volume reducing therapies, hepatic or portal venous stenting, transjugular intrahepatic portosystemic shunts and liver transplantation may be considered. Because of complicated anatomy, use of high‐risk interventions and lack of empirical evidence, patients should be treated in expert centres.

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Portal Hypertension Due to Extensive Hepatic Cysts in Autosomal Dominant Polycystic Kidney Disease

Cited by 15 publications

References 7 publications

Cisterna chyli in autosomal dominant polycystic kidney disease

Cisterna chyli in autosomal dominant polycystic kidney disease

Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Management of portal hypertension and ascites in polycystic liver disease

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