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2.
Rev. colomb. gastroenterol ; 35(3): 390-393, jul.-set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138799

ABSTRACT

Resumen La enfermedad de Caroli es una rara patología caracterizada por la existencia de dilataciones saculares segmentarias del árbol biliar intrahepático, que, por lo general, afectan a todo el parénquima hepático, aunque es poco frecuente el compromiso biliar extrahepático. Presentamos el caso de un varón que ingresó con un cuadro clínico de 45 días de evolución, consistente en ictericia colestásica, baja ponderal y prurito. La colangiorresonancia informó múltiples dilataciones saculares en las vías biliares intrahepáticas.


Abstract Caroli disease is a condition characterized by cystic dilatation of the intrahepatic biliary tree that usually affects the entire liver parenchyma, although extrahepatic biliary involvement is rare. The following is the case of a male patient who was admitted due to cholestatic jaundice, low weight, and pruritus for 45 days. Magnetic resonance cholangiopancreatography reported multiple cystic dilatations in the intrahepatic bile ducts.


Subject(s)
Humans , Male , Adult , Caroli Disease , Bile Ducts, Intrahepatic , Cholangiopancreatography, Magnetic Resonance , Jaundice
3.
ABCD arq. bras. cir. dig ; 33(1): e1490, 2020. graf
Article in English | LILACS | ID: biblio-1130509

ABSTRACT

ABSTRACT Background: Cholangiocarcinoma is an aggressive neoplasm that usually requires palliative biliary drainage. Photodynamic therapy (PDT) has been described as a successful adjunct treatment to malignant biliary obstruction. Aim: To describe the use of digital cholangioscope to help provide laser light during biliary PDT session using locally developed light source. Method: Patient receives intravenous photosensitizer 24 h before the procedure. It starts with a regular duodenoscopy. After identification of the major papilla and retrograde cannulation, the digital cholangioscope is introduced into the common bile duct. Then, the cholangioscopic examination helps to identify the neoplastic stricture. Under direct visualization lighting catheter is advanced through the cholangioscope. Repositioning is recommended every centimeter to cover all strictured area. At the end of the procedure, a final cholangioscopy assesses the bile duct for the immediate result and adverse events. Result: This procedure was applied in one 82-year-old male due to obstructive jaundice in the last two months. EUS and ERCP revealed a severe dilation of the common bile duct associated with choledocholithiasis. Besides, was revealed dilation of hepatic duct up to a well-circumscribed hypoechoic solid mass measuring 1.8x2 cm compressing the common hepatic duct. The mass was deemed unresectable and the patient was referred for palliative treatment with PDT. He remained asymptomatic for three months. He perished due to complications 15 months after the PDT session. Conclusion: Digital cholangioscopy-guided biliary PDT is feasible and seems safe and effective as an adjunct modality in the palliation of extrahepatic cholangiocarcinoma.


RESUMO Racional: Colangiocarcinoma é neoplasia agressiva que geralmente exige drenagem biliar paliativa. A terapia fotodinâmica (TFD) tem sido descrita como tratamento adjunto bem-sucedido para tratar obstrução biliar maligna. Objetivo: Descrever o emprego do colangioscópio digital para ajudar a fornecer luz de laser durante sessão de TFD biliar usando fonte de luz desenvolvida localmente. Método: Paciente recebe fotossensibilizador intravenoso 24 h antes do procedimento que começa com duodenoscopia regular. Após a identificação da papila principal e da canulação retrógrada, o colangioscópio digital é introduzido no ducto biliar comum. Em seguida, o exame colangioscópico ajuda a identificar a estenose neoplásica. Sob visualização direta, o cateter de iluminação avança através do colangioscópio. Reposicionamento é feito a cada centímetro. Ao final colangioscopia avalia o ducto biliar quanto ao resultado imediato e a eventos adversos. Resultado: Este procedimento foi aplicado em um homem de 82 anos devido à icterícia obstrutiva nos últimos dois meses. EUS e CPRE revelaram dilatação grave do ducto biliar comum associada à coledocolitíase. Além disso, havia dilatação do ducto hepático até massa sólida hipoecóica bem circunscrita, medindo 1,8x2 cm, comprimindo o ducto hepático comum. Ela foi considerada irressecável e paciente encaminhado para tratamento paliativo com TFD que permaneceu assintomático por três meses. Morreu devido a complicações 15 meses após a sessão de TFD. Conclusão: A TFD biliar guiada por colangioscopia digital é viável e parece segura e eficaz como modalidade auxiliar na paliação de colangiocarcinoma extra-hepático.


Subject(s)
Humans , Male , Aged, 80 and over , Photochemotherapy , Bile Duct Neoplasms/drug therapy , Bile Duct Neoplasms/diagnostic imaging , Endoscopy, Digestive System , Cholangiocarcinoma/drug therapy , Cholangiocarcinoma/diagnostic imaging , Bile Ducts, Intrahepatic , Fatal Outcome
4.
Acta Medica Philippina ; : 632-637, 2020.
Article in English | WPRIM | ID: wpr-877361

ABSTRACT

@#We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.


Subject(s)
Butterflies , Bile Ducts, Intrahepatic , Jaundice , Musculoskeletal Abnormalities , Spine
5.
Article in English | WPRIM | ID: wpr-787221

ABSTRACT

Biliary adenofibroma is a rare tumor with a bile duct origin characterized by a complex tubulocystic non-mucin secreting biliary epithelium with abundant fibrous stroma. The MRI features of biliary adenofibroma are not well established. The authors encountered two patients with biliary adenofibroma and reviewed the literature focusing on the MRI findings. A well-circumscribed multicystic tumor with septal enhancement and no intrahepatic bile duct communication may be the characteristic MRI findings of biliary adenofibroma.


Subject(s)
Adenofibroma , Bile Ducts , Bile Ducts, Intrahepatic , Epithelium , Humans , Magnetic Resonance Imaging
6.
Gut and Liver ; : 104-113, 2019.
Article in English | WPRIM | ID: wpr-719361

ABSTRACT

BACKGROUND/AIMS: There have been no nationwide studies to investigate the trends in incidence and 5-year survival rates of intra- and extrahepatic bile duct cancers and gall-bladder cancer. Therefore, our study aimed to describe the incidence and 5-year survival rates of biliary tract cancers by subsites in South Korea. METHODS: A total of 86,134 patients with biliary tract cancers were selected from the National Health Information Database. Age-standardized incidence rates and annual percentage changes were calculated. Life-table methods and log-rank tests were used to determine the differences in survival rates. Cox-proportional hazard models were used to estimate the hazard ratio of the patients with biliary tract cancers. RESULTS: The incidence rate of intra-hepatic bile duct cancer decreased by 1.3% annually from 8.8 per 100,000 in 2006 to 7.8 per 100,000 in 2015. Extrahepatic bile duct cancer also showed a decreasing trend by 2.2% per year from 8.7 per 100,000 in 2006 to 6.7 per 100,000 in 2015. Gallbladder cancer showed the greatest decline, with an annual percentage change of 2.8% from 6.3 per 100,000 to 5.2 per 100,000 during the same period. The 5-year survival rates were 30.0% in gallbladder cancer, 27.8% in extrahepatic bile duct cancer, and 15.9% in intra-hepatic bile duct cancer. CONCLUSIONS: The overall incidence rates of intrahepatic and extrahepatic bile duct cancer and gallbladder cancer decreased from 2006 to 2015. Among biliary tract cancers, intrahepatic bile duct cancers exhibited the highest incidence rate and the worst survival rate.


Subject(s)
Bile Duct Neoplasms , Bile Ducts, Extrahepatic , Bile Ducts, Intrahepatic , Biliary Tract Neoplasms , Biliary Tract , Cholangiocarcinoma , Gallbladder Neoplasms , Humans , Incidence , Korea , Proportional Hazards Models , Survival Rate
7.
Gut and Liver ; : 617-627, 2019.
Article in English | WPRIM | ID: wpr-763888

ABSTRACT

Intraductal papillary neoplasms of the bile duct (IPNBs) are known to show various pathologic features and biological behaviors. Recently, two categories of IPNBs have been proposed based on their histologic similarities to pancreatic intraductal papillary mucinous neoplasms (IPMNs): type 1 IPNBs, which share many features with IPMNs; and type 2 IPNBs, which are variably different from IPMNs. The four IPNB subtypes were re-evaluated with respect to these two categories. Intestinal IPNBs showing a predominantly villous growth may correspond to type 1, while those showing papillay-tubular or papillay-villous growth correspond to type 2. Regarding gastric IPNB, those with regular foveolar structures with varying numbers of pyloric glands may correspond to type 1, while those with papillary-foveolar structures with gastric immunophenotypes and complicated structures may correspond to type 2. Pancreatobiliary IPNBs that show fine ramifying branching may be categorized as type 1, while others containing many complicated structures may be categorized as type 2. Oncocytic type, which displays solid growth or irregular papillary structures, may correspond to type 2, while papillary configurations with pseudostratified oncocytic lining cells correspond to type 1. Generally, type 1 IPNBs of any subtype develop in the intrahepatic bile ducts, while type 2 IPNBs develop in the extrahepatic bile duct. These findings suggest that IPNBs arising in the intrahepatic ducts are biliary counterparts of IPMNs, while those arising in the extrahepatic ducts display differences from prototypical IPMNs. The recognition of these two categories of IPNBs with reference to IPMNs and their anatomical location along the biliary tree may deepen our understanding of IPNBs.


Subject(s)
Bile Ducts , Bile Ducts, Extrahepatic , Bile Ducts, Intrahepatic , Bile , Biliary Tract , Cholangiocarcinoma , Gastric Mucosa , Mucins
8.
Article in English | WPRIM | ID: wpr-741331

ABSTRACT

Hepatic duct diverticulum is a rare form of choledochal cyst that does not fit into the most widely used Todani classification system. Because of its rarity, it may be difficult for clinicians to diagnose and treat it. Here, we present a case of left hepatic diverticulum in a 57-year-old woman with epigastric pain. At presentation, there were mild elevations in the liver function tests. Computed tomography and magnetic resonance cholangiopancreatography showed diverticulum-like cystic lesion with sludge ball near the confluence portion of both intrahepatic bile duct, but the origin of the lesion could not be identified. The clinical impression was type II choledochal cyst. Surgical excision was planned due to recurrent abdominal pain. The operative findings revealed diverticulum arising from left hepatic duct. Histopathology confirmed the lesion to be diverticulum lined by biliary epithelium. The patient had no postoperative complication and no further symptoms since the operation.


Subject(s)
Abdominal Pain , Bile Ducts , Bile Ducts, Intrahepatic , Cholangiopancreatography, Magnetic Resonance , Choledochal Cyst , Classification , Diverticulum , Epithelium , Female , Hepatic Duct, Common , Humans , Liver Function Tests , Middle Aged , Postoperative Complications , Sewage
9.
Article in Korean | WPRIM | ID: wpr-741330

ABSTRACT

Bronchobiliary fistula (BBF) is a rare complication after transcatheter arterial chemoembolization (TACE) for hepatocellular carcinoma. We present a case of BBF that developed 5 months after 11 TACE procedures in a 78-year-old male patient with a 3-month history of a persistent cough and yellowish sputum. BBF was found between the bronchus and the right intrahepatic bile duct (B7) by magnetic resonance cholangiopancreatography. On the initial endoscopic retrograde cholangiopancreatography (ERCP), we failed to approach to the BBF due to severe hilar obstruction. Percutaneous transhepatic biliary drainage (PTBD) was performed, and 30-50 mL of bile was drained daily. But the BBF was not resolved until 2 months after PTBD. The patient was treated by placement of a plastic stent to the BBF site during the second ERCP. BBF was resolved 7 days after ERCP on the PTBD tubogram. The patient remained asymptomatic after the stent placement, and there was no recurrence at the 2-month follow-up ERCP.


Subject(s)
Aged , Bile , Bile Ducts, Intrahepatic , Bronchi , Carcinoma, Hepatocellular , Cholangiopancreatography, Endoscopic Retrograde , Cholangiopancreatography, Magnetic Resonance , Cough , Drainage , Fistula , Follow-Up Studies , Humans , Male , Plastics , Recurrence , Sputum , Stents
10.
Article in Chinese | WPRIM | ID: wpr-813019

ABSTRACT

The malignant degree of cholangiocarcinoma is high, and the early diagnosis is difficult. The vast majority of patients are unresectable when they are diagnosed. The patients have low quality of life and short survival cycle. Traditional radiotherapy and chemotherapy have poor efficacy and lead to side effects, and thus lack effective control measures for cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography (ERCP) is an important method for diagnosing and treating biliary tract diseases. Photodynamic therapy (PDT) is a new local treatment for cholangiocarcinoma. In recent years, ERCP-mediated PDT treatment of cholangiocarcinoma has gradually emerged. ERCP-mediated PDT can effectively relieve the symptoms of patients with cholangiocarcinoma, improve the patients' quality of life, prolong the survival cycle, and is expected to become a new treatment for cholangiocarcinoma.


Subject(s)
Bile Duct Neoplasms , Diagnosis , Bile Ducts, Intrahepatic , Cholangiocarcinoma , Diagnosis , Cholangiopancreatography, Endoscopic Retrograde , Humans , Photochemotherapy , Quality of Life
11.
Clinical Endoscopy ; : 596-599, 2018.
Article in English | WPRIM | ID: wpr-717965

ABSTRACT

An 89-year-old man was referred to our hospital for treatment of hepatolithiasis causing recurrent cholangitis. He had undergone a prior Whipple procedure. Computed tomography demonstrated left-sided hepatolithiasis. First, we conducted peroral direct cholangioscopy (PDCS) using an ultraslim endoscope. Although PDCS was successfully conducted, it was unsuccessful in removing all the stones. The stones located in the B2 segment were difficult to remove because the endoscope could not be inserted deeply into this segment due to the small size of the intrahepatic bile duct. Next, we substituted the endoscope with an upper gastrointestinal endoscope. After positioning the endoscope, the SpyGlass digital system (SPY-DS) was successfully inserted deep into the B2 segment. Upon visualizing the residual stones, we conducted SPY-DS-guided electrohydraulic lithotripsy. The stones were disintegrated and completely removed. In cases of PDCS failure, a treatment strategy using the SPY-DS can be considered for patients with hepatolithiasis after a Whipple procedure.


Subject(s)
Aged, 80 and over , Bile Ducts, Intrahepatic , Cholangitis , Endoscopes , Endoscopes, Gastrointestinal , Humans , Lithotripsy
12.
Article in English | WPRIM | ID: wpr-715714

ABSTRACT

PURPOSE: The aim of this study was to describe our treatment experiences with patients who had acute abdomen (AA) with common bile duct (CBD) dilatation. METHODS: The treatment outcomes in children with AA and CBD dilatation were retrospectively reviewed. According to the shape of the intrahepatic bile ducts on ultrasonography (US), the origin of the pain was estimated as choledochal cyst (CC) complication or choledocholithiasis in normal CBD. Patients with complicated CC underwent surgery, and patients with choledocholithiasis in a normal appearing CBD underwent symptomatic treatment initially. RESULTS: Of the 34 patients, 30 (88.2%) were female. The mean age of the patients was 6.4±4.9 (range, 0.8–17) years. Seventeen (50.0%) patients had CBD stones and 17 (50.0%) did not. Surgical treatment was performed in 20 (58.8%) patients, 2 of whom underwent preoperative stone removal with endoscopic retrograde cholangiopancreatography and an operation. Conservative treatment was applied in 12 (35.3%) patients (8 with and 4 without stones), 1 of whom developed symptom relapse and underwent an operation. Among the 8 patients with CBD stones, 4 (4/17, 23.5%) had complete resolution of the stones and recovery of the CBD diameter after conservative treatment. US findings of patients with stone showed a fusiform or cylindrical shape of the CBD in 14 (82.4%) patients. CONCLUSION: The presence of stones in the distal CBD and the US features of CBD dilatation may be helpful to diagnose and treat the causes of biliary dilatation. Conservative treatment can be considered as initial therapy in patients with uncomplicated CBD dilatation with stone.


Subject(s)
Abdomen, Acute , Bile Ducts, Intrahepatic , Child , Cholangiopancreatography, Endoscopic Retrograde , Choledochal Cyst , Choledocholithiasis , Common Bile Duct , Dilatation , Female , Humans , Recurrence , Retrospective Studies , Ultrasonography
13.
Article in Korean | WPRIM | ID: wpr-714525

ABSTRACT

Intrahepatic duct (IHD) stone is the presence of calculi within the intrahepatic bile duct specifically located proximal to the confluence of the left and right hepatic ducts. This stone is characterized by its intractable nature and frequent recurrence, requiring multiple therapeutic interventions. Without proper treatment, biliary strictures and retained stones can lead to repeated episodes of cholangitis, liver abscesses, secondary biliary cirrhosis, portal hypertension, and death from sepsis or hepatic failure. The ultimate treatment goals for IHD stones are complete removal of the stone, the correction of the associated strictures, and the prevention of recurrent cholangitis. A surgical resection can satisfy the goal of treatment for hepatolithiasis, i.e., complete removal of the IHD stones, stricture, and the risk of cholangiocarcinogenesis. On the other hand, in some cases, such as bilateral IHD stones, surgery alone cannot achieve these goals. Therefore, the optimal treatments require a multidisciplinary approach, including endoscopic and radiologic interventional procedures before and/or after surgery. Percutaneous transhepatic cholangioscopic lithotomy (PTCS-L) is particularly suited for patients at poor surgical risk or who refuse surgery and those with previous biliary surgery or stones distributed in multiple segments. PTCS-L is relatively safe and effective for the treatment of IHD stones, and complete stone clearance is mandatory to reduce the sequelae of IHD stones. An IHD stricture is the main factor contributing to incomplete clearance and stone recurrence. Long-term follow-up is required because of the overall high recurrence rate of IHD stones and the association with cholangiocarcinoma.


Subject(s)
Bile Ducts, Intrahepatic , Calculi , Cholangiocarcinoma , Cholangitis , Constriction, Pathologic , Follow-Up Studies , Hand , Hepatectomy , Hepatic Duct, Common , Humans , Hypertension, Portal , Liver Abscess , Liver Cirrhosis, Biliary , Liver Failure , Recurrence , Sepsis
14.
Article in English | WPRIM | ID: wpr-717606

ABSTRACT

BACKGROUND: Intraductal papillary neoplasm of the bile duct (IPNB) is a recently defined entity and its clinical characteristics and classifications have yet to be established. We aimed to clarify the clinical features of IPNB and determine the optimal morphological classification criteria. METHODS: From 2003 to 2016, 112 patients with IPNB who underwent surgery were included in the analysis. After pathologic reexamination by a specialized biliary-pancreas pathologist, previously suggested morphological and anatomical classifications were compared using the clinicopathologic characteristics of IPNB. RESULTS: In terms of histologic subtypes, most patients had the intestinal type (n = 53; 48.6%) or pancreatobiliary type (n = 33; 30.3%). The simple “modified anatomical classification” showed that extrahepatic IPNB comprised more of the intestinal type and tended to be removed by bile duct resection or pancreatoduodenectomy. Intrahepatic IPNB had an equally high proportion of intestinal and pancreatobiliary types and tended to be removed by hepatobiliary resection. Morphologic classifications and histologic subtypes had no effect on survival, whereas a positive resection margin (75.9% vs. 25.7%; P = 0.004) and lymph node metastasis (75.3% vs. 30.0%; P = 0.091) were associated with a poor five-year overall survival rate. In the multivariate analysis, a positive resection margin and perineural invasion were important risk factors for survival. CONCLUSION: IPNB showed better long-term outcomes after optimal surgical resection. The “modified anatomical classification” is simple and intuitive and can help to select a treatment strategy and establish the proper scope of the operation.


Subject(s)
Bile Duct Neoplasms , Bile Ducts , Bile Ducts, Extrahepatic , Bile Ducts, Intrahepatic , Bile , Cholangiocarcinoma , Classification , Humans , Lymph Nodes , Multivariate Analysis , Neoplasm Metastasis , Pancreaticoduodenectomy , Risk Factors , Survival Rate
15.
Article in English | WPRIM | ID: wpr-739758

ABSTRACT

A 75-year-old male with recurrent pancreatic adenocarcinoma after a previous Whipple's procedure presented with jaundice. The local advancement of the tumor caused obstructions of the common bile duct, intrahepatic bile duct hilum, and small bowel. Endoscopic stent insertion was precluded by the Roux-en-Y reconstruction. A successful transhepatic percutaneous single-access stenting of the whole biliary tree and intestine was achieved by H-configured triple stenting by combining T-configured dual stent placement in the biliary system with a duodenal stent insertion across the bottom of the anastomosis after looping a wire in the afferent limb. The ‘H-configured’ stents remained patent for 10 months without major or minor complications. This technique adds a new minimal-invasive and effective palliative option for patients with obstruction of a bilio-enteric anastomosis inaccessible to endoscopy.


Subject(s)
Adenocarcinoma , Aged , Bile Ducts, Intrahepatic , Biliary Tract , Cholestasis , Common Bile Duct , Endoscopy , Extremities , Gastric Outlet Obstruction , Humans , Intestines , Jaundice , Male , Stents
16.
Article in Spanish | LILACS, BNUY, UY-BNMED | ID: biblio-1253741

ABSTRACT

Se describe el caso clínico de una mujer de 62 años en la que se diagnostica una estenosis del colédoco en el contexto de hipertensión portal prehepática (cavernomatosis portal).


We report the case of a 62 years old woman with a choledochal stricture and chronic obstruction of the portal vein (portal cavernomatosis).


Relatamos o caso de uma mulher de 62 anos com diagnóstico de estenose do colédoco no contexto da hipertensão portal pré-hepática (cavernomatose portal).


Subject(s)
Humans , Female , Middle Aged , Cholestasis, Intrahepatic/etiology , Cholestasis, Intrahepatic/diagnostic imaging , Hypertension, Portal/complications , Hypertension, Portal/diagnostic imaging , Bile Ducts, Intrahepatic/pathology , Abdominal Pain/etiology , Common Bile Duct/pathology , Constriction, Pathologic , Gallbladder/pathology
17.
Article in English | WPRIM | ID: wpr-219268

ABSTRACT

Alagille syndrome (AGS) is a complex multisystem disorder that involves mainly the liver, heart, eyes, face, and skeleton. The main associated clinical features are chronic cholestasis due to a paucity of intrahepatic bile ducts, congenital heart disease primarily affecting pulmonary arteries, vertebral abnormalities, ocular embryotoxon, and peculiar facies. The manifestations generally become evident at a pediatric age. AGS is caused by defects in the Notch signaling pathway due to mutations in JAG1 or NOTCH2. It is inherited in an autosomal dominant pattern with a high degree of penetrance, but variable expressivity results in a wide range of clinical features. Here we report on a 31-year-old male patient who presented with elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase, and was diagnosed with AGS associated with the JAG1 mutation after a comprehensive workup.


Subject(s)
Adult , Alagille Syndrome , Alkaline Phosphatase , Bile Ducts, Intrahepatic , Cholestasis , Facies , gamma-Glutamyltransferase , Heart , Heart Defects, Congenital , Humans , Liver , Male , Penetrance , Pulmonary Artery , Skeleton
18.
Clinical Endoscopy ; : 400-403, 2017.
Article in English | WPRIM | ID: wpr-195022

ABSTRACT

Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5–20 years, and symptoms may seldom occur throughout the patient’s life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones. CBD stones were treated successfully with endoscopic retrograde cholangiopancreatography (ERCP).


Subject(s)
Adult , Bile , Bile Ducts, Intrahepatic , Caroli Disease , Cholangiopancreatography, Endoscopic Retrograde , Cholangitis , Choledocholithiasis , Common Bile Duct , Dilatation , Humans , Liver , Liver Abscess , Liver Cirrhosis, Biliary
19.
Gut and Liver ; : 771-780, 2017.
Article in English | WPRIM | ID: wpr-82311

ABSTRACT

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren’s syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition. Due to the high prevalence of extrahepatic diseases in patients with PBC, it is important to complete a thorough medical history at the time of diagnosis. Prompt recognition of extrahepatic disease can lead to improved patient outcomes and quality of life. The following review summarizes the most common extrahepatic conditions associated with PBC.


Subject(s)
Autoimmune Diseases , Bile Ducts, Intrahepatic , Cholangitis , Cholestasis , Diagnosis , Humans , Liver Diseases , Prevalence , Quality of Life , Scleroderma, Systemic , Thyroid Gland
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