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Vanishing bile duct syndrome in Hodgkin's lymphoma: A case report and literature review

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World Journal of Gastroenterology 2017年第2期23卷 366-372页

作者： Mena Bakhit Thomas R McCarty Sunhee Park Basile Njei Margaret Cho Raffi Karagozian AnnMarie Liapakis Department of Internal medicine Yale University School of Medicine section of digestive diseases Yale University School of Medicine Investigative medicine Program Yale Center of Clinical Investigation Department of Anatomic Pathology Yale University School of Medicine Saint Francis Hospital Gastroenterology and Hepatology

Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.

关键词： Cholestasis Bile ductopenia Vanishing bile duct syndrome Hodgkin’ s lymphoma Liver

Treatment of portal hypertension

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World Journal of Gastroenterology 2012年第11期18卷 1166-1175页

作者： Khurram Bari Guadalupe Garcia-Tsao section of digestive diseases Yale University School of MedicineNew HavenCT and VA-Connecticut Healthcare SystemWest HavenCT 06520United States

Portal hypertension is the main complication of cirrhosis and is defined as an hepatic venous pressure gradient （HVPG） of more than 5 mmHg. Clinically significant portal hypertension is defined as HVPG of 10 mmHg or more. Development of gastroesophageal varices and variceal hemorrhage are the most direct consequence of portal hypertension. Over the last decades significant advancements in the field have led to standard treatment options. These clinical recommendations have evolved mostly as a result of *** controlled trials and consensus conferences among experts where existing evidence has been reviewed and future goals for research and practice guidelines have been pro- posed. Management of varices/variceal hemorrhage is based on the clinical stage of portal hypertension. No specific treatment has shown to prevent the formation of varices. Prevention of first variceal hemorrhage depends on the size/characteristics of varices. In patients with small varices and high risk of bleeding, nonselective β-blockers are recommended, while patients with medium/large varices can be treated with either β-blockers or esophageal band ligation. Standard ofcare for acute variceal hemorrhage consists of vasoacrive drugs, endoscopic band ligation and antibiotics prophylaxis. Transjugular intrahepatic portosystemic shunt （TIPS） is reserved for those who fail standard of care or for patients who are likely to fail （＂early TIPS＂）. Prevention of recurrent variceal hemorrhage consists of the combination of β-blockers and endoscopic band ligation.

关键词： Cirrhosis Portal hypertension Varices Varicealhemorrhage Primary prophylaxis Secondary prophylaxis

Tailoring stepwise treatment for Budd-Chiari syndrome: insights from the Asian Pacific Association for the Study of the Liver (APASL) consensus guidance

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Hepatobiliary Surgery and Nutrition 2023年第1期12卷 118-120页

作者： Lamia Y.Haque Joseph K.Lim section of digestive diseases Department of Internal MedicineYale School of MedicineNew HavenCTUSA

Budd-Chiari syndrome(BCS)is a rare disease consisting of obstruction of the hepatic venous outflow tract,which can occur at any level ranging from small hepatic veins to the inferior vena cava(1-4).BCS can result from intravascular thrombosis or membranous obstruction of the hepatic venous outflow tract,or,less commonly,due to external compression from tumors,nodules,abscesses,cysts,or other intrahepatic lesions(1-3).

关键词： Budd-Chiari syndrome(BCS) hepatic venous outflow obstruction anticoagulation transjugular intrahepatic portosystemic shunts(TIPS) surgery liver transplantation guidelines

Being accompanied to liver discharge clinic: An easy measure to identify potential liver transplant candidates among those previously considered ineligible

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World Journal of Hepatology 2019年第4期11卷 370-378页

作者： Jordan Sack Nilofar Najafian Angela DeLisle Simona Jakab Department of Internal medicine Yale University School of Medicine Northeast Medical Group Yale New Haven Health section of digestive diseases Yale University School of Medicine

BACKGROUND Patients with cirrhosis deemed ineligible for liver transplantation are usually followed in general hepatology or gastroenterology clinics, with the hope of reevaluation once they meet the appropriate criteria. Specific strategies to achieve liver transplant eligibility for these patients have not been *** To assess clinical and sociodemographic factors associated with future liver transplant eligibility among patients initially considered *** This is a retrospective study of patients with cirrhosis considered non-transplant eligible, but without absolute contraindications, who were scheduled in our transitional care liver clinic(TCLC) after discharge from an inpatient liver *** candidacy was assessed 1 year after the first scheduled TCLC *** on clinical and sociodemographic factors were *** Sixty-nine patients were identified and the vast majority were Caucasian men with alcoholic cirrhosis. 46 patients(67%) presented to the first TCLC visit. Seven of 46 patients that showed to the first TCLC visit became transplant candidates,while 0 of 23 patients that no-showed did(15.2% vs 0%, P = 0.08). Six of 7 patients who showed and became transplant eligible were accompanied by family orfriends at the first TCLC appointment, compared to 13 of 39 patients who showed and did not become transplant eligible(85.7% vs 33.3%, P = 0.01).CONCLUSION Patients who attended the first post-discharge TCLC appointment had a trend for higher liver transplant eligibility at 1 year. Being accompanied by family or friends during the first TCLC visit correlated with higher liver transplant eligibility at 1 year(attendance by family or friends was not requested). Patient and family engagement in the immediate post-hospitalization period may predict future liver transplant eligibility for patients previously declined.

关键词： Cirrhosis Transitional clinic Transplant listing Support Family

Pseudocirrhosis in a pancreatic cancer patient with liver metastases: A case report of complete resolution of pseudocirrhosis with an early recognition and management

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World Journal of Gastroenterology 2008年第10期14卷 1622-1624页

作者： Soonmo Peter Kang Tamar Taddei Bruce McLennan Jill Lacy yale Cancer Center Yale University School of Medicine New Haven Connecticut 06520 United States yale university school of medicine Department of Medicine Section of Digestive Diseases New Haven Connecticut 06520 United States yale university school of medicine Department of Diagnostic Radiology New Haven Connecticut 06520 United States

We report a case of pseudocirrhosis arising in the setting of regression of liver metastases from pancreatic cancer. A 55-year-old asymptomatic woman presented to our clinic with newly diagnosed metastatic pancreatic cancer with extensive liver metastases. She underwent systemic chemotherapy with gemcitabine and oxaliplatin (GEMOX). After 8 cycles of therapy, she had a remarkable response to the therapy evidenced by decline of carcinoembryonic antigen (CEA) and CA19 by > 50% and nearly complete resolution of hepatic metastases in computed tomography (CT) scan. Shortly after, she developed increasing bilateral ankle edema and ascites, associated with dyspnea, progressive weight gain, and declining performance status. Gemcitabine and oxaliplatin were discontinued as other causes of her symptoms such as congestive heart disease or venous thrombosis were ruled out. CT scan 6 mo after the initiation of GEMOX revealed worsening ascites with a stable pancreatic mass. However, it also revealed a lobular hepatic contour, segmental atrophy, and capsular retraction mimicking the appearance of cirrhosis. She was managed with aggressive diuresis and albumin infusions which eventually resulted in a resolution of the above- mentioned symptoms as well as complete resolution of pseudocirrhotic appearance of the liver and ascites in CT scan. This case demonstrates that pancreatic cancer patients can develop pseudocirrhosis. Clinicians and radiologist should be well aware of this entity asearly recognition and management can lead to a near complete recovery of liver function and much improved quality of life as illustrated in this case.

关键词： Pseudocirrhosis Pancreatic cancer Nodular regenerative hyperplasia Chemotherapy induced liver toxicity

Emerging concepts in the care of patients with cirrhosis and septic shock

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World Journal of Hepatology 2023年第4期15卷 497-514页

作者： Jose Victor Jimenez Guadalupe Garcia-Tsao Saad Saffo section of digestive diseases Yale School of MedicineNew HavenCT 06520United States

Septic shock impacts approximately 6% of hospitalized patients with cirrhosis and is associated with high rates of morbidity and mortality. Although a number of landmark clinical trials have paved the way for incremental improvements in the diagnosis and management of septic shock in the general population, patients with cirrhosis have largely been excluded from these studies and critical knowledge gaps continue to impact the care of these individuals. In this review,we discuss nuances in the care of patients with cirrhosis and septic shock using a pathophysiology-based approach. We illustrate that septic shock may be challenging to diagnose in this population in the context of factors such as chronic hypotension, impaired lactate metabolism, and concomitant hepatic encephalopathy. Furthermore, we demonstrate that the application of routine interventions such as intravenous fluids, vasopressors, antibiotics, and steroids should be carefully considered among those with decompensated cirrhosis in light of hemodynamic, metabolic, hormonal, and immunologic disturbances. We propose that future research should include and characterize patients with cirrhosis in a systematic manner, and clinical practice guidelines may need to be refined accordingly.

关键词： Cirrhosis Septic shock Intravenous fluids Vasopressors Antibiotics Steroids

Treatment of Hepatitis C in Patients Undergoing Immunosuppressive Drug Therapy

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Journal of Clinical and Translational Hepatology 2016年第3期4卷 206-227页

作者： Kohtaro Ooka Joseph K.Lim Department of Internal medicine Yale University School of Medicine New Haven CT USA yale Liver Center Section of Digestive Diseases Yale University School of Medicine New Haven CT USA

With 185 million people chronically infected globally,hepatitis C is a leading bloodborne ***-oral regimens of direct acting agents have superior efficacy compared to the historical interferon-based regimens and are significantly more ***,trials of both types of regimens have often excluded patients on immunosuppressive medications for reasons other than organ ***,these patients-most often suffering from malignancy or autoimmune diseases-could stand to benefit from these *** this study,we systematically review the literature on the treatment of hepatitis C in these neglected *** on patients with organ transplants is more robust and this literature is reviewed here *** systematic review produced 2273 unique works,of which 56 met our inclusion criteria and were used in our *** quality of data was low;only 3 of the 56 studies were randomized controlled *** virologic response was reported ***-containing regimens achieved this end-point at rates comparable to that in immunocompetent *** adverse effects and death were *** on all-oral regimens were sparse,but in the most robust study,rates of sustained virologic response were again comparable to immunocompetent individuals (40/41).Efficacy and safety of interferoncontaining regimens and all-oral regimens were similar to rates in immunocompetent individuals;however,there were few interventional *** large number of case reports and case series makes conclusions vulnerable to publication *** firm conclusions are challenging,given the dearth of high-quality studies,our results demonstrate that antiviral therapy can be safe and *** advent of all-oral regimens offers patients and clinicians greatly increased chances of cure and fewer side *** data reveal that these regimens may confer such benefits in immunosuppressed individuals as *** pr

关键词： MeSH termsHepatitis C/dt[drug therapy] Interferons Direct acting antivirals Immunosuppressive agents Chemotherapy

Calcium signaling in cholangiocytes

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World Journal of Gastroenterology 2006年第22期12卷 3466-3470页

作者： Noritaka Minagawa Barbara E Ehrlich Michael H Nathanson Departments of medicine and Pharmacology yale university school of medicine New Haven CTUnited States section of digestive diseases yale university school of medicine 1 Gilbert StreetRoom TAC S241DNew HavenCT 06519United States

Cytosolic Ca^2＋ is an important second messenger in virtually every type of cell. Moreover, Ca^2＋ generally regulates multiple activities within individual cells. This article reviews the cellular machinery that is responsible for Ca^2＋ signaling in cholangiocytes. In addition, two Ca^2＋-mediated events in cholangiocytes are discussed： bicarbonate secretion and apoptosis. Finally, emerging evidence is reviewed that Ca^2＋ signaling is involved in the pathogenesis of diseases affecting the biliary tree and that Ca^2＋ signaling pathways can be manipulated to therapeutic advantage in the treatment of cholestatic disorders.

关键词： Ca^2+ Cholangiocyte Inositol trisphosphate Bile secretion Cholestasis Apoptosis

Resection of pancreatic cystic neoplasms in recurrent acute pancreatitis prevents recurrent pancreatitis but does not identify more malignancies

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World Journal of Gastroenterology 2021年第15期27卷 1630-1642页

作者： Thiruvengadam Muniraj Harry R Aslanian Loren Laine Priya A Jamidar James F Farrell Kisha A Mitchell Ronald R Salem section of digestive diseases Department of MedicineYale University School of MedicineNew HavenCT 06510United States Department of Pathology Yale University School of MedicineNew HavenCT 06510United States Department of Surgery Yale University School of MedicineNew HavenCT 06510United States

BACKGROUND Recurrent acute pancreatitis(RAP)may be a presenting feature of and an indication for resection of pancreatic cysts,including intra-ductal papillary mucinous neoplasm(IPMN).Few data are available regarding the prevalence of malignancy and post-operative RAP in this *** To study the role of resection to help prevent RAP and analyze if presentation as RAP would be a predictor for *** This retrospective study assessed 172 patients who underwent surgical resection of pancreatic cystic neoplasms at a university hospital between 2002 and *** prevalence of preoperative high-risk cyst features,and of neoplasia was compared between patients with and without *** identify the cause of pancreatitis,all the patients had a detailed history of alcohol,smoking,medications obtained,and had cross-sectional imaging(contrast-enhanced computed tomography/magnetic resonance imaging)and endoscopic ultrasound to look for gallstone etiology and other structural causes for *** incidence of RAP post-resection was the primary *** IPMN accounted for 101 cases(58.7%){[branch duct(BD)59(34.3%),main duct(MD)42](24.4%)}.Twenty-nine(16.9%)presented with RAP(mean 2.2 episodes):15 had BD-IPMN,8 MD-IPMN,5 mucinous cystic neoplasm and 1 serous cystic *** was similar among those with vs without RAP for all patients[6/29(20.7%)vs 24/143(16.8%)]and IPMN patients[6/23(26.1%)vs 23/78(29.5%)],although tended to be higher with RAP in BD-IPMN,[5/15(33.3%)vs 3/44(6.8%),P=0.04].At mean follow-up of 7.2 years,1(3.4%)RAP patient had post-resection *** mean episodes of acute pancreatitis before vs after surgery were 3.4 vs 0.02(P<0.0001).CONCLUSION Malignancy was not increased in patients with pancreatic cystic neoplasms who have RAP compared to those without *** addition,specific cyst characteristics were not clearly associated with *** incidence of RAP was markedly decreased in almost all patients following cyst resect

关键词： Pancreatic cyst Pancreatic neoplasm Pancreatitis Malignancies