scholarly journals Magnetic resonance elastography to quantify liver disease severity in autosomal recessive polycystic kidney disease

2020 ◽  
Author(s):  
Erum A. Hartung ◽  
Juan S. Calle-Toro ◽  
Carolina Maya Lopera ◽  
Jessica Wen ◽  
Robert H. Carson ◽  
...  
Keyword(s):  
Liver Disease ◽  
Magnetic Resonance ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Disease Severity ◽  
Autosomal Recessive ◽  
Magnetic Resonance Elastography ◽  
Polycystic Kidney

scholarly journals Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease

2019 ◽  
Vol 209 ◽  
pp. 107-115.e5 ◽  
Author(s):  
Erum A. Hartung ◽  
Jessica Wen ◽  
Laura Poznick ◽  
Susan L. Furth ◽  
Kassa Darge
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Disease Severity ◽  
Autosomal Recessive ◽  
Ultrasound Elastography ◽  
Polycystic Kidney

scholarly journals Imaging manifestations of Caroli disease with autosomal recessive polycystic kidney disease: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiuzhen Yao ◽  
Weiqun Ao ◽  
Jianhua Fang ◽  
Guoqun Mao ◽  
Chuanghua Chen ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Magnetic Resonance ◽  
Kidney Disease ◽  
Portal Vein ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney ◽  
Left Liver Lobe ◽  
Caroli Disease ◽  
T1 And T2

Abstract Background Both Caroli disease (CD) and autosomal recessive polycystic kidney disease (ARPKD) are autosomal recessive disorders, which are more commonly found in infants and children, for whom surviving to adulthood is rare. Early diagnosis and intervention can improve the survival rate to some extent. This study adopted the case of a 26-year-old pregnant woman to explore the clinical and imaging manifestations and progress of CD concomitant with ARPKD to enable a better understanding of the disease. Case presentation A 26-year-old pregnant woman was admitted to our hospital for more than 2 months following the discovery of pancytopenia and increased creatinine. Ultrasonography detected an enlarged left liver lobe, widened hepatic portal vein, splenomegaly, and dilated splenic vein. In addition, both kidneys were obviously enlarged and sonolucent areas of varying sizes were visible, but color Doppler flow imaging revealed no abnormal blood flow signals. The gestational age was approximately 25 weeks, which was consistent with the actual fetal age. Polyhydramnios was detected but no other abnormalities were identified. Magnetic resonance imaging revealed that the liver was plump, and polycystic liver disease was observed near the top of the diaphragm. The T1 and T2 weighted images were the low and high signals, respectively. The bile duct was slightly dilated; the portal vein was widened; and the spleen volume was enlarged. Moreover, the volume of both kidneys had increased to an abnormal shape, with multiple, long, roundish T1 and T2 abnormal signals being observed. Magnetic resonance cholangiopancreatography revealed that intrahepatic cystic lesions were connected with intrahepatic bile ducts. The patient underwent a genetic testing, the result showed she carried two heterozygous mutations in PKHD1. The patient was finally diagnosed with CD with concomitant ARPKD. The baby underwent a genetic test three months after birth, the result showed that the patient carried one heterozygous mutations in PKHD1, which indicated the baby was a PKHD1 carrier. Conclusions This case demonstrates that imaging examinations are of great significance for the diagnosis and evaluation of CD with concomitant ARPKD.

Liver Disease in Autosomal Recessive Polycystic Kidney Disease

2014 ◽  
Vol 59 (2) ◽  
pp. 190-196 ◽  
Author(s):  
Topi T. Luoto ◽  
Mikko P. Pakarinen ◽  
Timo Jahnukainen ◽  
Hannu Jalanko
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney

Appearance of autosomal recessive polycystic kidney disease in magnetic resonance imaging and RARE-MR-urography

2000 ◽  
Vol 30 (3) ◽  
pp. 156-160 ◽  
Author(s):  
S. Kern ◽  
L.-B. Zimmerhackl ◽  
F. Hildebrandt ◽  
B. Ermisch-Omran ◽  
M. Uhl
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Resonance Imaging ◽  
Polycystic Kidney ◽  
Mr Urography

scholarly journals PPAR-γ agonist ameliorates kidney and liver disease in an orthologous rat model of human autosomal recessive polycystic kidney disease

2011 ◽  
Vol 300 (2) ◽  
pp. F465-F474 ◽  
Author(s):  
Daisuke Yoshihara ◽  
Hiroki Kurahashi ◽  
Miwa Morita ◽  
Masanori Kugita ◽  
Yoshiyuki Hiki ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
Body Weight ◽  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Interstitial Fibrosis ◽  
Polycystic Kidney ◽  
Ppar Γ ◽  
Cystic Area

In autosomal recessive polycystic kidney disease (ARPKD), progressive enlargement of fluid-filled cysts is due to aberrant proliferation of tubule epithelial cells and transepithelial fluid secretion leading to extensive nephron loss and interstitial fibrosis. Congenital hepatic fibrosis associated with biliary cysts/dilatations is the most common extrarenal manifestation in ARPKD and can lead to massive liver enlargement. Peroxisome proliferator-activated receptor γ (PPAR-γ), a member of the ligand-dependent nuclear receptor superfamily, is expressed in a variety of tissues, including the kidneys and liver, and plays important roles in cell proliferation, fibrosis, and inflammation. In the current study, we determined that pioglitazone (PIO), a PPAR-γ agonist, decreases polycystic kidney and liver disease progression in the polycystic kidney rat, an orthologous model of human ARPKD. Daily treatment with 10 mg/kg PIO for 16 wk decreased kidney weight (% of body weight), renal cystic area, serum urea nitrogen, and the number of Ki67-, pERK1/2-, and pS6-positive cells in the kidney. There was also a decrease in liver weight (% of body weight), liver cystic area, fibrotic index, and the number of Ki67-, pERK1/2-, pERK5-, and TGF-β-positive cells in the liver. Taken together, these data suggest that PIO inhibits the progression of polycystic kidney and liver disease in a model of human ARPKD by inhibiting cell proliferation and fibrosis. These findings suggest that PPAR-γ agonists may have therapeutic value in the treatment of the renal and hepatic manifestations of ARPKD.

Liver disease in autosomal recessive polycystic kidney disease

2005 ◽  
Vol 9 (5) ◽  
pp. 634-639 ◽  
Author(s):  
Benjamin L. Shneider ◽  
Margret S. Magid
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney
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