The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease

1988 ◽  
Vol 18 (2) ◽  
pp. 123-129 ◽  
Author(s):  
A. Premkumar ◽  
W. E. Berdon ◽  
J. Levy ◽  
J. Amodio ◽  
S. J. Abramson ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Infants And Children ◽  
Polycystic Kidney ◽  
In Infants And Children

scholarly journals Congenital Hepatic Fibrosis and Portal Hypertension in Autosomal Dominant Polycystic Kidney Disease

2012 ◽  
Vol 54 (1) ◽  
pp. 83-89 ◽  
Author(s):  
Kevin O’Brien ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
Joy Bryant ◽  
Katie Piwnica-Worms ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Dominant ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

scholarly journals Occurrence of Portal Hypertension and Its Clinical Course in Patients With Molecularly Confirmed Autosomal Recessive Polycystic Kidney Disease (ARPKD)

2020 ◽  
Vol 8 ◽  
Author(s):  
Dorota Wicher ◽  
Ryszard Grenda ◽  
Mikołaj Teisseyre ◽  
Marek Szymczak ◽  
Paulina Halat-Wolska ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Clinical Course ◽  
Polycystic Kidney

scholarly journals PKHD1 sequence variations in 78 children and adults with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis

2010 ◽  
Vol 99 (2) ◽  
pp. 160-173 ◽  
Author(s):  
Meral Gunay-Aygun ◽  
Maya Tuchman ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
Hailey Edwards ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Sequence Variations

scholarly journals Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

2008 ◽  
Vol 39 (2) ◽  
pp. 100-111 ◽  
Author(s):  
Baris Turkbey ◽  
Iclal Ocak ◽  
Kailash Daryanani ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney

Autosomal-recessive polycystic kidney disease in children

2016 ◽  
Vol 7 (4) ◽  
pp. 45-49 ◽  
Author(s):  
Elvira F Andreeva ◽  
Nadejda D Savenkova ◽  
Mohamed A Tilouche ◽  
Natalya Y Natochina ◽  
Igor V Dug
Keyword(s):  
Portal Hypertension ◽  
Kidney Disease ◽  
Arterial Hypertension ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Varicose Veins ◽  
Clinical Manifestations ◽  
Polycystic Kidney ◽  
Stage Renal Disease ◽  
Neonates And Infants

The aim of the study was to assess the features of development of renal and extrarenal cysts, arterial hypertension, syndrome of portal hypertension in autosomal recessive polycystic kidney disease (ARPKD) in children. Patients and me­thods. With the aim of establishing the type of inheritance of polycystic kidney disease the genealogical analysis of 12 families, clinical ultrasound of the kidneys and abdominal organs, computed tomography. The study included 14 children with ARPKD. Conducted follow-up study of 14 children with ARPKD to determine the age by the detection of cysts based on ultrasound, the features of the initial clinical manifestations and course, complications and outcome.Results: the Age of the children back to the time of detection of the cysts in the kidneys based on ultrasound when ARPKD was 2.3 ± 0.4 month. Identified a high incidence of arterial hypertension in neonates and infants with ARPKD at 92.9%. Extrarenal location of the cysts is set at 71.4%. Syndrome of portal hypertension, bleeding from varicose veins of esophagus and stomach, melanau installed in 5 (35,7%) children. Of the 14 in 5 (35,7%) patients diagnosed ARPKD children with liver fibrosis, which has a favorable prognosis without the formation of renal failure in infants and early childhood, 9 (64,3%) diagnosed with classic ARPKD in neonates and infants that is characterized by progression to end-stage renal disease in the first year of life.

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