scholarly journals Congenital Cholestatic Syndromes: What Happens When Children Grow Up?

2007 ◽  
Vol 21 (11) ◽  
pp. 743-751 ◽  
Author(s):  
Simon C Ling
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Alagille Syndrome ◽  
Nutritional Deficiencies ◽  
Published Data ◽  
Caroli Disease ◽  
Progressive Encephalopathy ◽  
Biliary Sepsis ◽  
Affected Parent

Although advances in the management of children with congenital cholestasis have enabled many to survive into adulthood with their native livers, even the most common of these conditions remains rare in adult hepatology practice. Among four congenital cholestatic syndromes (biliary atresia, Alagille syndrome, Caroli disease and congenital hepatic fibrosis, and progressive familial intrahepatic cholestasis), the published data on outcomes of the syndromes into adulthood suggest that a spectrum of severity of liver disease can be expected, from cirrhosis (almost universal in adults with biliary atresia who have not required liver transplantation) to mild and subclinical (eg, in the previously undiagnosed affected parent of an infant with Alagille syndrome). Complications associated with portal hypertension and nutritional deficiencies are common, and other associated features of the cholestatic syndrome may require appropriate attention, such as congenital heart disease in Alagille syndrome. Indications for liver transplantation include synthetic failure, progressive encephalopathy, intractable pruritus, recurrent biliary sepsis and recurrent complications of portal hypertension. Improved understanding of biliary physiology will hopefully translate into improved therapy for children and adults with cholestasis.

scholarly journals Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

PLoS ONE ◽  
2016 ◽  
Vol 11 (2) ◽  
pp. e0149681 ◽  
Author(s):  
Sook Min Hwang ◽  
Tae Yeon Jeon ◽  
So-Young Yoo ◽  
Ji Hye Kim ◽  
Ben Kang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Alagille Syndrome ◽  
Preoperative Ct ◽  
End Stage

Variceal Bleeds in Patients with Biliary Atresia

2017 ◽  
Vol 28 (05) ◽  
pp. 439-444 ◽  
Author(s):  
Mauri Witt ◽  
Niels Bax ◽  
Henkjan Verkade ◽  
René Scheenstra ◽  
Ruben de Kleine ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Variceal Bleeding ◽  
Laboratory Tests ◽  
Subsequent Development ◽  
Kasai Portoenterostomy ◽  
Receive Treatment ◽  
Related Mortality

Introduction Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds. Materials and Methods We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed. Results In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4–111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred. Conclusion One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.

scholarly journals A young adult with biliary atresia and liver decompensation

2019 ◽  
Vol 2 (1) ◽  
pp. 38-40
Author(s):  
Panyavee Pitisuttithum ◽  
Piyawat Komolmit
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Lymph Node ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Liver Decompensation ◽  
Native Liver ◽  
Peribiliary Cysts ◽  
Collateral Vessels ◽  
Paraesophageal Lymph Node

Biliary atresia is a progressive fibro-obliterative disease of the bile duct, commonly diagnosed within a few months of life. After hepatoportoenterostomy was successfully performed, about one fourth of biliary atresia patients have grown up to the age of twenty with their native liver. However, progression to liver cirrhosis, development of portal hypertension, or cholangitis are the major problems of adult with biliary atresia. Monitoring of these complications and listing for liver transplantation whenever indicated are the key when caring adult with biliary atresia.   Figure 1  แสดง CT abdomen พบว่ามีตับโต และมีลักษณะของตับแข็งและ ภาวะความดันของระบบหลอดเลือด portal สูงขึ้น (collateral vessels, splenomegaly, ascites) พบการขยายตัวของท่อน้ำดีในตับ (multifocal dilated ducts and peribiliary cysts along bilateral intrahepatic ducts) และต่อมน้ำเหลืองโตในช่องท้อง (mesenteric, gastrohepatic, hepatoduodenal, aortocaval, para aortic, right anterior diaphragmatic, lower paraesophageal lymph node ขนาดสูงสุด 1.8 ซม.)

scholarly journals Caroli Disease (Clinical Observation)

2019 ◽  
Vol 28 (6) ◽  
pp. 77-83
Author(s):  
I. G. Nikitin ◽  
A. A. Karabinenko ◽  
A. E. Nikitin ◽  
E. I. Dedov ◽  
D. G. Zhukova ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Bile Ducts ◽  
Abdominal Cavity ◽  
Unknown Origin ◽  
Symptomatic Therapy ◽  
Biliary Cirrhosis ◽  
Caroli Disease ◽  
Intrahepatic Bile Ducts ◽  
The Moment

The incidence of Caroli disease is estimated to be 1 case per 1 million people; as a result, there are very few available clinical observations. At the moment, the treatment of Caroli disease is limited to symptomatic therapy and the prevention of complications, as well as to corrective surgery and liver transplantation.Aim. The aim of this observation is to describe one case of a rare congenital liver disease – Caroli disease – characterised by a segmented non-obstructive fibrocystic dilation of the intrahepatic bile ducts.Key findings. A 21-year-old woman was hospitalised with the signs of a systemic inflammatory reaction, hepatosplenomegaly, jaundice, portal hypertension, hepatocellular insufficiency, as well as with the manifestations of cytolytic and cholestatic syndromes. At the age of 8, she was diagnosed with a cyst of the bile ducts, which was treated with cystoenteroanastomosis. At the age of 20, in connection with high portal hypertension, portocaval shunting was performed and a mesenteric-caval anastomosis was applied. During the present hospitalisation, an expansion of the intrahepatic bile ducts was revealed by ultrasound and MSCT of the abdominal cavity with contrast, which made it possible to diagnose Caroli disease.Conclusion. A case of Caroli disease is described, which resulted in continuously recurrent cholangitis and biliary cirrhosis. This state required liver transplantation. Caroli disease should be included in differential diagnosis in patients suffering from the fever of unknown origin and cholestasis syndrome. An early diagnosis of Caroli disease is important for preventing complications, improving the quality of life and increasing the life expectancy of patients suffering from this rare disease.

Indication of liver transplantation for jaundice-free biliary atresia with portal hypertension

2011 ◽  
Vol 16 (4) ◽  
pp. 7-11 ◽  
Author(s):  
Yukihiro Sanada ◽  
Koichi Mizuta ◽  
Taizen Urahashi ◽  
Yoshiyuki Ihara ◽  
Taiichi Wakiya ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia

scholarly journals Extrahepatic Biliary Atresia: The Importance of Early Referral

JMS SKIMS ◽  
2014 ◽  
Vol 17 (1) ◽  
pp. 39-40
Author(s):  
Wani Sajad ◽  
Bhat Nisar ◽  
Aejaz Baba ◽  
Gowhar Mufti ◽  
Khursheed Ahmad Sheikh
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Bile Ducts ◽  
Early Referral ◽  
Diffusion Of Information ◽  
Extrahepatic Biliary Atresia ◽  
Extrahepatic Bile Ducts ◽  
And Diffusion

Extrahepatic biliary atresia (EHBA), characterized by obliteration or discontinuity of extrahepatic bile ducts, is still the major cause for liver transplantation among children nowadays [1]. All untreated children eventually die due to complications resulting from portal hypertension and liver cirrhosis. The exchange and diffusion of information that can make the diagnosis of EHBA easier is of utmost importance, since prognosis is improved when patients are surgically treated by portoenterostomy in the first 2 months of life. JMS 2014;17(1):39-40

The Need for Consensus About Liver Transplantation For Patients With Neuropsychiatric Wilson’s Disease

2021 ◽  
pp. 152692482110028
Author(s):  
Alberto Ferrarese ◽  
Patrizia Burra
Keyword(s):  
Liver Transplantation ◽  
Patient Outcomes ◽  
Wilson’S Disease ◽  
Therapeutic Option ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Published Data ◽  
Neurological Improvement ◽  
Effective Therapeutic Option

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.

scholarly journals De novo gastric cancer developing after liver transplantation from deceased donor for biliary atresia: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Naruki Higashidate ◽  
Suguru Fukahori ◽  
Shinji Ishii ◽  
Nobuyuki Saikusa ◽  
Naoki Hashizume ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
De Novo ◽  
Epigastric Pain ◽  
Gastroesophageal Junction ◽  
Deceased Donor ◽  
Lymphoproliferative Disease ◽  
Endoscopic Examination ◽  
De Novo Malignancy

Abstract Background Apart from Kasai’s procedure, liver transplantation (LTx) has dramatically improved the outcome of children with biliary atresia (BA). However, de novo malignancy has been reported to be one of the major causes of late mortality after LTx among adults. We report a rare case of de novo gastric cancer developing after LTx for BA received during childhood. Case presentation A 21-year-old male patient who had undergone LTx for BA at age 2 years occasionally visited our outpatient clinic due to symptoms of epigastric pain and dysphagia. Endoscopic examination and computed tomography revealed advanced gastric cancer at the gastroesophageal junction with multiple liver metastases. Despite systemic chemotherapy, the disease progressed, resulting in patient’s death 2 years after the diagnosis. Conclusions De novo malignancy in the absence of post-transplant lymphoproliferative disease is rare in pediatric patients who received LTx. To the best of our knowledge, no report has been available on the development of gastric cancer after LTx for BA during childhood. Primary physicians should therefore establish a follow-up plan for patients receiving LTx for BA considering the potential for the development of de novo malignancy, including gastric cancer, despite its rarity.

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