scholarly journals BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

2014 ◽  
Vol 51 (1) ◽  
pp. 53-58 ◽  
Author(s):  
Thais Costa Nascentes QUEIROZ ◽  
Alexandre Rodrigues FERREIRA ◽  
Eleonora Druve Tavares FAGUNDES ◽  
Mariza Leitão Valadares ROQUETE ◽  
Francisco José PENNA
Keyword(s):  
Biliary Atresia ◽  
Waiting Time ◽  
Neonatal Period ◽  
Biliary Tree ◽  
Probability Of Survival ◽  
Age At Surgery ◽  
Pediatric Service ◽  
Extrahepatic Biliary Tree ◽  
Observation Period

Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

Biliary Atresia Revisited

2004 ◽  
Vol 7 (2) ◽  
pp. 109-124 ◽  
Author(s):  
Ellen Kahn
Keyword(s):  
Biliary Atresia ◽  
Congenital Anomalies ◽  
Cell Transformation ◽  
Biliary Tree ◽  
Bile Acid Metabolism ◽  
Biliary Cirrhosis ◽  
Intrahepatic Bile Ducts ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Extrahepatic Biliary Tree

Extrahepatic biliary atresia (EHBA) is an inflammatory fibrosing process affecting the extrahepatic and intrahepatic biliary tree resulting in fibrous obliteration of the extrahepatic biliary tract, ductopenia of intrahepatic bile ducts, and biliary cirrhosis. EHBA is divided into a correctable and a noncorrectable type with focal patency of the otherwise atretic biliary tree in the former and no patency of the biliary tree in the noncorrectable type. EHBA is divided in a fetal, prenatal or embryonic, and a more common, perinatal, acquired form. The symptoms of the former start shortly after birth and there is frequently an association with a variety of congenital anomalies. Children with the perinatal form become jaundiced several weeks after birth; no associated congenital anomalies are present. Morphologically, an inflammatory and fibrosing process of the extrahepatic biliary tree leads to complete lumenal obliteration. The liver is characterized by a nonspecific giant cell transformation, and portal expansion by fibrous connective tissue with marked ductular proliferation. With time, ductopenia and biliary cirrhosis develop. The diffential diagnosis with other conditions with similar microscopic patterns such as alpha-1 antitrypsin deficiency, total parental nutrition, obstruction by a choledochal cyst, arteriohepatic dysplasia, familial progressive intra-hepatic cholestasis, and alteration of the bile acid metabolism is discussed. In the fetal group, abnormalities in different genes seem to play a role; ductal plate malformation is another possibility. Different etiologies have been postulated in the perinatal form of EHBA: genetic susceptilibility, vascular factors, toxins, and infections mainly by rotavirus and reovirus. The pathogenesis is complex. EHBA is a heterogenous disease, resulting from a combination of genetic factors, insults, and activation of different genetic and immunologic pathways. The treatment of EHBA is surgical, with anastomosis between the biliary tree and the intestine in the correctable type and a hepatic portoenterostomy (HPE) for the noncorrectable group. HPE is a temporizing treatment allowing the infant to develop and grow, followed in the majority of the patients by liver transplantation.

scholarly journals Radiation Stricture of the Biliary Ducts: An Emerging New Entity?

HPB Surgery ◽  
1992 ◽  
Vol 5 (4) ◽  
pp. 267-270 ◽  
Author(s):  
A. Halevy ◽  
A. Adam ◽  
G. Stamp ◽  
I. S. Benjamin ◽  
L. H. Blumgart
Keyword(s):  
Radiation Therapy ◽  
Obstructive Jaundice ◽  
Clinical Picture ◽  
Biliary Tree ◽  
Recurrent Tumour ◽  
Radiation Stricture ◽  
Biliary Ducts ◽  
Extrahepatic Biliary Tree ◽  
Malignant Condition

Two patients with stricture of the extrahepatic biliary tree are described. Both patients presented with a clinical picture of obstructive jaundice one to two years following radiotherapy for a malignant condition. As no recurrent tumour was detected in either of the patients the strictures were considered to be the result of radiation therapy. Bilio-enteric decompression was performed in both patients who are well at follow up one and ten years after the procedure.

scholarly journals Recent advances in understanding biliary atresia

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 218 ◽  
Author(s):  
Andrew Wehrman ◽  
Orith Waisbourd-Zinman ◽  
Rebecca G Wells
Keyword(s):  
Biliary Atresia ◽  
Extrahepatic Bile Duct ◽  
Biliary Tree ◽  
Liver Parenchyma ◽  
Early Diagnostics ◽  
The Past ◽  
Recent Advances ◽  
Neonatal Liver ◽  
Extrahepatic Biliary Tree ◽  
Extrahepatic Bile Duct Obstruction

Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

scholarly journals Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation

Medicina ◽  
2020 ◽  
Vol 57 (1) ◽  
pp. 16
Author(s):  
Raluca-Cristina Apostu ◽  
Vlad Fagarasan ◽  
Catalin C. Ciuce ◽  
Radu Drasovean ◽  
Dan Gheban ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Survival Rate ◽  
Biliary Atresia ◽  
Smooth Muscle Actin ◽  
Term Survival ◽  
Surgical Department ◽  
Screening Programs ◽  
Age At Surgery ◽  
Native Liver

Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1–4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice’s clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection.

scholarly journals Management of Biliary Atresia: A Review

2016 ◽  
Vol 39 (1) ◽  
pp. 38-45
Author(s):  
Khan Lamia Nahid ◽  
Md Rokonuzzaman ◽  
ASM Bazlul Karim ◽  
Kaniz Fatema ◽  
Azmeri Sultana
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Biliary Tree ◽  
Unknown Etiology ◽  
Biliary Cirrhosis ◽  
Dark Urine ◽  
Kasai Operation ◽  
Age At Surgery ◽  
Life Threatening ◽  
Optimum Management

Biliary atresia (BA) is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. It is the most common cause of neonatal cholestasis and most common indication for liver transplantation in children. Biliary Atresia clinically presents with jaundice, pale stool, dark urine and hepatomegaly. The treatment of biliary atresia is surgical and is recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (Kasai operation) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/ or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated. Age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. So early detection of biliary atresia is necessary for optimum management and overall survival of the patient.Bangladesh J Child Health 2015; VOL 39 (1) :38-45

scholarly journals Exome Sequencing in Individuals with Isolated Biliary Atresia

2019 ◽  
Author(s):  
Ramakrishnan Rajagopalan ◽  
Ellen A. Tsai ◽  
Christopher M. Grochowski ◽  
Susan M. Kelly ◽  
Kathleen M. Loomes ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Exome Sequencing ◽  
Stress Responses ◽  
Rare Variants ◽  
De Novo ◽  
Genetic Model ◽  
Single Gene ◽  
Biliary Tree ◽  
Causal Genes ◽  
Extrahepatic Biliary Tree

AbstractBiliary atresia (BA) is a severe pediatric liver disease resulting in necroinflammatory obliteration of the extrahepatic biliary tree. BA presents within the first few months of life as either an isolated finding or with additional syndromic features. The etiology of isolated BA is unknown, with evidence for infectious, environmental, and genetic risk factors described. However, to date, there are no definitive causal genes identified for isolated BA in humans, and the question of whether single gene defects play a major role remains open. We performed exome-sequencing in 100 North American patients of European descent with isolated BA (including 30 parent-child trios) and considered several experimental designs to identify potentially deleterious protein-altering variants that may be involved in the disease. In a case-only analysis, we did not identify genes with variants shared among more than two probands, and burden tests of rare variants using a case-case control design did not yield significant results. In the trio analysis of 30 simplex families (patient and parent trios), we identified 66 de novo variants in 66 genes including a nonsense variant, p.(Cys30Ter), in the gene STIP1. STIP1 is a co-chaperone for the heat-shock protein, HSP90AA1, and has been shown to have diverse functions in yeast, flies and mammals, including stress-response.ConclusionOur results do not support the hypothesis that a simple genetic model is responsible for the majority of cases of isolated BA. Our finding of a de novo mutation in a candidate gene for BA (STIP1) linked to evolutionarily conserved stress responses suggests further exploration of how genetic susceptibility and environmental exposure interact to cause BA is warranted.

HRA Model for Hypercholesterolemia Based on a Longitudinal Health Database

1998 ◽  
Vol 37 (02) ◽  
pp. 130-133
Author(s):  
T. Kishimoto ◽  
Y. Iida ◽  
K. Yoshida ◽  
M. Miyakawa ◽  
H. Sugimori ◽  
...  
Keyword(s):  
Total Cholesterol ◽  
Proportional Hazards ◽  
Proportional Hazards Model ◽  
Model Analysis ◽  
Serum Total Cholesterol ◽  
Hazards Model ◽  
The Mean ◽  
Health Database ◽  
Observation Period

AbstractTo evaluate the risk factors for hypercholesterolemia, we examined 4,371 subjects (3,207 males and 1,164 females) who received medical checkups more than twice at an AMHTS in Tokyo during the period from 1976 through 1991; and whose serum total cholesterol was under 250 mg/dl. The mean follow-up duration was 6.6 years. A self-registering questionnaire was administered at the time of the health checkup. The endpoint of this study was the onset of hypercholesterolemia when the level of serum total cholesterol was 250 mg/dl and over. We compared two prognosis groups (normal and hypercholesterol) in terms of age, examination findings and lifestyle. After assessing each variable, we employed Cox's proportional hazards model analysis to determine the factors related to the occurrence of hypercholesterolemia. According to proportional hazards model analysis, total cholesterol, triglyceride and smoking at the beginning, and hypertension during the observation period were selected in males; and total cholesterol at the beginning and age were selected in females to determine the factors related to the occurrence of hypercholesterolemia.

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