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Overlap syndromes between autoimmune hepatitis and both primary biliary cirrhosis and primary sclerosing cholangitis are well described, but overlap between primary biliary cirrhosis and primary sclerosing cholangitis is not recognized. We present two cases of an unusual autoimmune liver disease, both of which included an overlap between primary biliary cirrhosis and primary sclerosing cholangitis, while one had features of all three conditions. The diagnoses were based on clinical, biochemical, serological, histological and cholangiographic findings. The two cases were identified from a consecutive cohort of 261 patients with autoimmune liver disease followed prospectively in secondary care over a 20-year period, which gives perspective to this uncommon overlap syndrome. 相似文献
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Lyons JM Karkar A Correa-Gallego CC D'Angelica MI DeMatteo RP Fong Y Kingham TP Jarnagin WR Brennan MF Allen PJ 《HPB : the official journal of the International Hepato Pancreato Biliary Association》2012,14(7):469-475
Background
The optimal surgical management of patients found to have unresectable pancreatic cancer at open exploration remains unknown.Methods
Records of patients who underwent non-therapeutic laparotomy for pancreatic cancer during 2000–2009 and were followed until death at Memorial Sloan–Kettering Cancer Center, New York, were reviewed.Results
Over the 10-year study period, 157 patients underwent non-therapeutic laparotomy. Laparotomy alone was performed in 21% of patients; duodenal bypass, biliary bypass and double bypass were performed in 11%, 30% and 38% of patients, respectively. Complications occurred in 44 (28%) patients. Three (2%) patients died perioperatively. Postoperative interventions were required in 72 (46%) patients following exploration. The median number of inpatient days prior to death was 16 (interquartile range: 8–32 days). Proportions of patients requiring interventions were similar regardless of the procedure performed at the initial operation, as were the total number of inpatient days prior to death. Patients undergoing gastrojejunostomy required fewer postoperative duodenal stents and those undergoing operative biliary drainage required fewer postoperative biliary stents.Conclusions
In this study, duodenal, biliary and double bypasses in unresectable patients were not associated with fewer invasive procedures following non-therapeutic laparotomy and did not appear to reduce the total number of inpatient hospital days prior to death. Continued effort to identify unresectability prior to operation is justified. 相似文献5.
John B. Ammori MD Nancy E. Kemeny MD Yuman Fong MD Andrea Cercek MD Ronald P. Dematteo MD Peter J. Allen MD T. Peter Kingham MD Mithat Gonen PhD Philip B. Paty MD William R. Jarnagin MD Michael I. D’Angelica MD 《Annals of surgical oncology》2013,20(9):2901-2907
Background
When feasible, surgical treatment of colorectal liver metastases (CRLM) is the treatment of choice. Regional hepatic artery infusional (HAI) chemotherapy effectively treats CRLM. The combination of HAI and systemic chemotherapy may downsize tumors and allow for complete resection and/or ablation (R/A). This study analyzes the combination of HAI and systemic chemotherapy for treating unresectable CRLM, focusing on conversion to complete R/A.Methods
All patients with unresectable CRLM treated with HAI and systemic chemotherapy from 2000 to 2009 were included. Patients who responded sufficiently to undergo complete R/A were compared to those who did not convert. Survival was compared using a landmark analysis to account for bias.Results
A total of 373 patients were included; 93 patients (25 %) subsequently underwent complete R/A. The percentage of patients submitted to complete R/A increased from 16 % during 2000–2003 to 30 % during 2004–2009. Factors associated with conversion on multivariate analysis were more recent treatment (2004–2009), no prior chemotherapy, clinical risk score <3, treatment on clinical protocol, and younger age. Median and predicted 5-year survival from the time of HAI pump placement was 59 months and 47 %, respectively, in the patients who converted to complete R/A, compared with 16 months and 6 %, respectively in those who did not (p < 0.001).Conclusions
Despite extensive disease, 25 % of patients with unresectable CRLM responded sufficiently to undergo complete R/A following HAI plus systemic chemotherapy. Combination HAI and systemic chemotherapy is an effective strategy to convert patients to complete resection with an associated excellent long-term survival. 相似文献6.
Camilo Correa-Gallego MD Richard Do MD Jennifer LaFemina MD Mithat Gonen PhD Michael I. D’Angelica MD Ronald P. DeMatteo MD Yuman Fong MD T. Peter Kingham MD Murray F. Brennan MD William R. Jarnagin MD Peter J. Allen MD 《Annals of surgical oncology》2013,20(13):4348-4355
Background
Clinical decision making for patients with intraductal papillary mucinous neoplasms (IPMN) of the pancreas is challenging. Even with strict criteria for resection, most resected lesions lack high-grade dysplasia (HGD) or invasive carcinoma.Methods
We evaluated patients who underwent resection of histologically confirmed IPMN and had preoperative imaging available for review. A hepatobiliary radiologist blinded to histopathologic subtype reviewed preoperative imaging and recorded cyst characteristics. Patients with mixed-type IPMN were grouped with main-duct lesions for this analysis. Based on an ordinal logistic regression model, we devised two independent nomograms to predict the findings of adenoma, high-grade dysplasia (HGD–CIS), and invasive carcinoma, separately in both main and branch-duct IPMN. Bootstrap validation was used to evaluate the performance of these models, and a concordance index was derived from this internal validation.Results
There were 219 patients who met criteria for this study. Branch-duct IPMN (bdIPMN) comprised 56 % of the resected lesions. The proportion of HGD–CIS was 15 % for bdIPMN and 33 % for main-duct lesions (mdIPMN); P = 0.003. Invasive carcinoma was identified in 15 % of bdIPMN and 41 % of main-duct lesions (P < 0.001). On multivariate regression, patient gender, history of prior malignancy, presence of solid component, and weight loss were found to be significantly associated with the ordinal outcome for patients with mdIPMN and built into the nomogram (concordance index 0.74). For patients with bdIPMN weight loss, solid component, and lesion diameter were associated with the outcome; (concordance index 0.74).Conclusion
Based on the analysis of patients selected for resection, two nomograms were created that predict a patient’s individual likelihood of harboring HGD or invasive malignancy in radiologically diagnosed IPMN. External validation is ongoing. 相似文献7.
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Ioannis T. Konstantinidis Pedro Mastrodomenico Constantinos T. Sofocleous Karen T. Brown George I. Getrajdman Mithat Gönen Peter J. Allen T. Peter Kingham Ronald P. DeMatteo Yuman Fong William R. Jarnagin Michael I. D’Angelica 《Journal of gastrointestinal surgery》2016,20(4):748-756
Background
Improvements in liver surgery have led to decreased mortality rates. Symptomatic perihepatic collections (SPHCs) requiring percutaneous drainage remain a significant source of morbidity.Study Design
A single institution’s prospectively maintained hepatic resection database was reviewed to identify patients who underwent hepatectomy between January 2004 and February 2012.Results
Data from 2173 hepatectomies performed in 2040 patients were reviewed. Overall, 200 (9 %) patients developed an SPHC, the majority non-bilious (75.5 %) and infected (54 %). Major hepatic resections, larger than median blood loss (≥360 ml), use of surgical drains, and simultaneous performance of a colorectal procedure were associated with an SPHC on multivariate analysis. Non-bilious, non-infected (NBNI) collections were associated with lower white blood cell (WBC) counts, absence of a bilio-enteric anastomosis, use of hepatic arterial infusion pump (HAIP), and presence of metastatic disease, and resolved more frequently with a single interventional radiology (IR) procedure (85 vs 46.5 %, p?<?0.001) more quickly (15 vs 30 days, p?=?0.001).Conclusions
SPHCs developed in 9 % of patients in a modern series of hepatic resections, and in one third were non-bilious and non-infected. In the era of modern interventional radiology, the need for re-operation for SPHC is exceedingly rare. A significant proportion of minimally symptomatic SPHC patients may not require drainage, and strategies to avoid unnecessary drainage are warranted.9.
Hari Nathan Matthew J. Weiss Gerald A. Soff Michelle Stempel Ronald P. DeMatteo Peter J. Allen T. Peter Kingham Yuman Fong William R. Jarnagin Michael I. D’Angelica 《Journal of gastrointestinal surgery》2014,18(2):295-303
Introduction
Pharmacologic prophylaxis (PP) is recommended for patients undergoing general surgical procedures with at least moderate risk of venous thromboembolism (VTE). The role of PP in patients undergoing hepatectomy is controversial, however, due to concerns regarding postoperative liver dysfunction and bleeding.Methods
We conducted a retrospective analysis of a prospectively maintained institutional database in order to clarify the relationship between PP, postoperative INR, and risk of VTE.Results
Postoperative VTE occurred in 55 of 2,147 patients (2.6 %) and was independently associated with advanced age, higher BMI, longer procedure time, and development of a major complication, as well as higher postoperative INR (≥1.5 versus <1.5: OR 2.50, P?=?0.006). Patients undergoing more extensive liver resection with higher postoperative INR were less likely to receive PP, but receipt of PP demonstrated no relationship with either VTE incidence or hemorrhagic complications.Conclusions
In this large single-institution study, incidence of VTE was not associated with PP but was associated with higher postoperative INR, contrary to the notion that postoperative liver dysfunction is protective against VTE. The interplay between prothrombotic and antithrombotic mechanisms in posthepatectomy patients must be more completely characterized before broad recommendations can be made regarding PP use in these patients. 相似文献10.
Brian R. Untch Keisha P. Bonner Kevin K. Roggin Diane Reidy-Lagunes David S. Klimstra Mark A. Schattner Yuman Fong Peter J. Allen Michael I. D’Angelica Ronald P. DeMatteo William R. Jarnagin T. Peter Kingham Laura H. Tang 《Journal of gastrointestinal surgery》2014,18(3):457-463