scholarly journals Its Congenital Hepatic Fibrosis; Not Cirrhosis At All

2018 ◽  
Vol 1 (2) ◽  
pp. 124-126
Author(s):  
Ananta Shrestha ◽  
Mamun Al-Mahtab ◽  
Salimur Rahman ◽  
Jahangir Sarkar ◽  
Thupten K Lama
Keyword(s):  
Portal Hypertension ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Rare Condition ◽  
Recessive Trait ◽  
Congenital Hepatic Fibrosis ◽  
Life Threatening ◽  
Hepatic Histology ◽  
Upper Abdomen ◽  
Medical University

Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture inherited as autosomal recessive trait. We report a 19 year-old-female admitted to Bangabandhu Sheikh Mujib Medical University with the complaints of lump in upper abdomen since last 13 years and episodes of fever and abdominal pain for same duration. She was diagnosed with hepatic TB on hepatic histology. Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood. Prognosis of congenital hepatic fibrosis is good. Life threatening events in these patients are related with variceal bleeding and episodes of cholangitis. Owing to relatively good liver function these patients tolerate portosystemic shunt surgeries quite well.Though rare, congenital hepatic fibrosis should be included in the differential diagnosis of portal hypertension in early life.

Congenital hepatic fibrosis of Riedel's lobe causing portal hypertension: Report of a Case

1969 ◽  
Vol 36 (3) ◽  
pp. 143-148 ◽  
Author(s):  
R. G. Farmer ◽  
R. E. Hermann ◽  
B. H. Sullivan
Keyword(s):  
Portal Hypertension ◽  
Hepatic Fibrosis ◽  
Congenital Hepatic Fibrosis

scholarly journals Surgical treatment for secondary aortoesophageal fistula

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Kayo Sugiyama ◽  
Toru Iwahashi ◽  
Nobusato Koizumi ◽  
Toshiya Nishibe ◽  
Toshiki Fujiyoshi ◽  
...  
Keyword(s):  
Open Repair ◽  
Rare Condition ◽  
Aortic Aneurysms ◽  
Total Arch Replacement ◽  
University Hospital ◽  
Aortoesophageal Fistula ◽  
Thoracic Aortic Aneurysms ◽  
Life Threatening ◽  
Tokyo Medical ◽  
Medical University

Abstract Background Aortoesophageal fistula (AEF) is a relatively rare condition that is often life-threatening. Secondary AEF is a complication of previous surgery, which can be more critical and challenging than primary AEF. The number of secondary AEF is increasing due to increase in the number of thoracic endovascular aortic repair (TEVAR). Although TEVAR has become a successful alternative surgical strategy for thoracic aortic aneurysms, secondary AEF after TEVAR might be critical than other secondary AEF because of severe adhesion between the esophagus and residual thoracic aortic wall. Methods This study analyzed six patients with secondary AEF who were treated at Tokyo Medical University Hospital between 2011 and 2016. These participants included four patients who had undergone TEVAR and two who had undergone total arch replacement. Results Although they were subsequently hospitalized for a long period, open surgical repair was completed in two patients who had undergone total arch replacement. TEVAR alone was performed in two patients who had undergone TEVAR and they were discharged without major complications shortly. Combined repair of TEVAR as a bridge to open surgery was planned for two patients who had undergone TEVAR. However, reconstruction of the aorta and esophagus could not be completed in these patients due to severe adhesions, and they died during hospitalization. Conclusions Definitive open repair was successfully performed in patients with secondary AEF after total arch replacement. However, in the patients with secondary AEF after TEVAR, severe adhesion between the aorta and esophagus led to difficulty in performing a successful definitive open repair. The strategy for secondary AEF should, therefore, be decided considering the etiology of secondary AEF. In secondary AEF after TEVAR, definitive open repair is difficult to complete because of catastrophic complication, and palliative treatment using TEVAR without reconstruction of aorta and esophagus can be an alternative.

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 Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Beidi Zhu ◽  
Zunguo Du ◽  
Zhengxin Wang ◽  
Yang Li ◽  
Jiming Zhang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Literature Review ◽  
Hepatic Fibrosis ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Case Series ◽  
Congenital Hepatic Fibrosis ◽  
Multidisciplinary Cooperation ◽  
Central Nervous Systems

Background. Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults. Methods. We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients’ age. Results. A total of eight patients were diagnosed with congenital hepatic fibrosis in the study, including four children and four adults. The onset age of the children, who suffered from severe complications of portal hypertension and needed liver transplantation, ranged from 1 to 15 years old. The disorder developed in adults aged 26 to 60 years old. Three adults complained of recurrent abnormal liver function at the onset of illness, and they mainly received conservative treatments. The literature review included 30 children and 33 adults. In comparison, hepatomegaly was more common in children than in adults (57% vs. 21%, p=0.004). Malformation of kidneys and bile duct abnormalities were common, and multisystem involvement included eyes, other digestive organs, and genital and central nervous systems. Conclusions. Serious complications of portal hypertension developed in children requiring liver transplantation, while adults often had mild-to-moderate liver injuries upon onset. Adults with CHF varied a lot in clinical manifestations. Multiorgan involvement and unusual course are helpful to make a diagnosis. Timely histological assessment by liver biopsy and multidisciplinary cooperation are crucial for definitive diagnosis and early intervention.

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
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