regenerating nodule
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2015 ◽  
Vol 5 (4) ◽  
Author(s):  
Sunil Pawar ◽  
Vinay Zanwar ◽  
Ashok Mohite ◽  
Ravindra Surude ◽  
Pravin Rathi ◽  
...  

Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis. This constellation of diagnosis belongs to the ciliopathy group of disorders. The spectrum of ciliopathy disorders has been evolving, and it varies from mild to severe manifestations.


2010 ◽  
Vol 40 (10) ◽  
pp. 1696-1698 ◽  
Author(s):  
Sylvia C. Wetli ◽  
Eva S. Gralla ◽  
Susanne Schibli ◽  
Enno Stranzinger

1991 ◽  
Vol 15 (3) ◽  
pp. 495-497 ◽  
Author(s):  
Hajime Fujimoto ◽  
Guio Uchiyama ◽  
Tsutomu Araki ◽  
Kenji Kachi ◽  
Masahito Karikomi ◽  
...  
Keyword(s):  

1988 ◽  
Vol 75 (11) ◽  
pp. 1124-1128 ◽  
Author(s):  
N. Nagasue ◽  
H. Yukaya ◽  
Y.-C. Chang ◽  
N. Kimura ◽  
N. Ota ◽  
...  

1974 ◽  
Vol 121 (2) ◽  
pp. 401-411 ◽  
Author(s):  
JACK G. RABINOWITZ ◽  
MAHESH KINKABWALA ◽  
SIDNEY ULREICH

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