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.

Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

2021 ◽  
Author(s):  
Patrick Ho Yu Chung ◽  
Edwin Kin Wai Chan ◽  
Fanny Yeung ◽  
Albert Chi Yan Chan ◽  
Jennifer Wai Cheung Mou ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Liver Transplant ◽  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Term Survival ◽  
Native Liver ◽  
The Mean ◽  
Native Liver Survival

Abstract ObjectiveWe present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE).MethodsA retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. The jaundice clearance (JC) rate, native liver survival (NLS) rate and complications were analyzed.ResultsThe mean follow up period was 15.4 +/- 6.2 years. Over 60% of patients remained JC within 2 years after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 years (range: 0.5 to 25 years). The NLS rates at 10, 20 and 30 years were 70.7%, 61.5% and 53.0% respectively with no significant change over the study period. The median bilirubin level among all native liver survivors (n=153) was 24 µmol/L (2 to 162 µmol/L). Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively.ConclusionWith a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long term survival without LT. Although portal hypertension and recurrent cholangitis are common, they do not need to be the indications for LT if managed properly.

Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

2021 ◽  
Author(s):  
Omid Madadi-Sanjani ◽  
David Fortmann ◽  
Udo Rolle ◽  
Burkhard Rodeck ◽  
Ekkehard Sturm ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Data Transfer ◽  
Previous Analysis ◽  
Outcome Data ◽  
Free Survival ◽  
Change In Practice ◽  
Native Liver ◽  
Survival With Native Liver ◽  
Pediatric Liver Transplant

Abstract Introduction The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. Materials and Methods In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. Result Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001–2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001–2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001–2005 [p = 0.5]). Compared with the 2001–2005 analysis, all criteria showed improvement but the differences are statistically not significant. Conclusion Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

Long-term outcome and necessity of liver transplantation in infants with biliary atresia are independent of cytokine milieu in native liver and serum

Cytokine ◽  
2018 ◽  
Vol 111 ◽  
pp. 382-388 ◽  
Author(s):  
Omid Madadi-Sanjani ◽  
Joachim F. Kuebler ◽  
Stephanie Dippel ◽  
Anna Gigina ◽  
Christine S. Falk ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Native Liver ◽  
Cytokine Milieu

scholarly journals Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia Treatment in Russia

Diagnostics ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 686
Author(s):  
Anna Degtyareva ◽  
Alexander Razumovskiy ◽  
Nadezhda Kulikova ◽  
Sergey Ratnikov ◽  
Elena Filippova ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Survival Rate ◽  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Critical Importance ◽  
Long Term Effects ◽  
Age Related

This prospective study enrolled 144 patients after surgical treatment of biliary atresia in early infancy. We analyzed the immediate effectiveness of the surgery and the age-related structure of complications in the up to 16-year follow-up. The immediate 2-year survival rate after the surgery constituted 49.5%. At the time of this writing, 17 of the patients had celebrated their 10th birthdays with good quality of life and no indications for transplantation of the liver. The obtained results underscore the critical importance of surgical correction of biliary atresia by Kasai surgery in the first 60 days of life and subsequent dynamic follow-up of patients for the purpose of the early detection and timely correction of possible complications.

Ninety-one Cases of Tumors of the Anus and Anal Canal.

1967 ◽  
Vol 53 (6) ◽  
pp. 591-600
Author(s):  
Bruno Salvadori ◽  
Giangaspare Valentinis ◽  
Lorenzo Zingo
Keyword(s):  
Survival Rate ◽  
Anal Canal ◽  
National Cancer Institute ◽  
Radical Surgery ◽  
Pelvic Lymphadenectomy ◽  
Surgical Department ◽  
Perineal Resection ◽  
Advanced Tumors

Ninety-one cases of tumors of anus and anal canal, treated in the Surgical Department of the National Cancer Institute of Milan from 1930 to 1965, are discussed. The series consists of 56 females and 35 males; the age of the patients ranges from 31 to 84 years. The distribution of the cases in relation to the age of the patients shows that the highest frequency of tumors was observed in the group of patients of 61–70 years. Twenty-seven patients presented inguinal metastatic nodes; in 18 of them metastases were bilateral. Nineteen patients (20.19 %) presented advanced tumors and could not be radically treated; 33 (36.3 %) underwent radiological treatment; 37 (40.6 %) were amenable to radical surgery: of these, in 26 a perineal resection and in 10 cases an abdomino-perineal resection were performed. In 2 of the last 10 cases a pelvic lymphadenectomy was also performed; in 5 of the patients radically operated a dissection of inguinal nodes was done. A regular follow-up was possible for 29 of the 37 patients radically treated: the 5 years survival rate for the two groups, perineal and abdomino-perineal resection, was of 48 per cent and 50 per cent respectively.

Mid-Term Evaluation of the Unicompartmental Knee Arthroplasty in Patients with BMI of 40 or Greater

2019 ◽  
Author(s):  
Akshay Seth ◽  
Johanna Dobransky ◽  
Waleed Albishi ◽  
Geoffrey F. Dervin
Keyword(s):  
Survival Rate ◽  
Knee Arthroplasty ◽  
Unicompartmental Knee Arthroplasty ◽  
Patient Reported Outcome Measures ◽  
Patient Reported Outcome ◽  
Primary Objective ◽  
Term Survival ◽  
Unicompartmental Knee ◽  
Patient Reported

AbstractLimited evidence is available on mid-term follow-up for patients with body mass index (BMI) ≥ 40 receiving a unicompartmental knee arthroplasty (UKA). The primary objective of this study was to investigate survival of the UKA in patients with BMI ≥ 40. Secondary objectives were to assess functional and patient reported outcome measures (PROMs). Survival analysis with endpoint revision of any component for any reason was conducted using Kaplan–Meier technique on 121 knees (103 patients) that underwent UKA with Oxford Phase 3 implant (Oxford Knee, Biomet, Swindon, United Kingdom) between September 2001 and March 2014 by seven surgeons. Survivorship differences were compared using Log Rank (Mantel-Cox) tests, and Cox Proportional Hazard Model was used to assess predictors of failure. Preoperative PROMs were compared at 2 and 5 years postoperatively using paired t-tests. Mean age of patients was 58 years (43–75), mean BMI 43 kg/m2 (40–51), and mean follow-up 7 years (2 months to 15 years). Survival rate for the whole cohort was 92% at 2 years and 86% at 5 years. Females had a significantly higher revision rate than males (p = 0.043). A total of 19 knees required revision (16 to TKA, 2 polyethylene liner exchanges, and 1 femoral component and liner revision). With respect to PROMs, there was a significant improvement at 2 and 5 years (p < 0.001) on 4 of 5 knee injury and osteoarthritis outcome score subscales. The mid-term survival rate for the Oxford UKA in patients with morbid obesity is similar to that of other nondesigner patient series with BMI ≥ 30, which provides further evidence for the safety of the implant in this patient population with significant improvements on PROMs at short and mid-term follow-up.

Trapeziometacarpal total joint arthroplasty for osteoarthritis: 199 patients with a minimum of 10 years follow-up

2019 ◽  
Vol 45 (5) ◽  
pp. 443-451 ◽  
Author(s):  
Miguel Martin-Ferrero ◽  
Clarisa Simón-Pérez ◽  
Maria B. Coco-Martín ◽  
Aureliio Vega-Castrillo ◽  
Héctor Aguado-Hernández ◽  
...  
Keyword(s):  
Survival Rate ◽  
Total Joint Arthroplasty ◽  
Hand Function ◽  
Joint Arthroplasty ◽  
Implant Survival ◽  
Level Of Evidence ◽  
Term Survival ◽  
Cumulative Survival

We report outcomes of 228 consecutive patients with total joint arthroplasty using the Arpe® prosthesis, among which 216 trapeziometacarpal joints in 199 patients had a minimum of 10 years follow-up. The cumulative survival rate of the 216 implants at 10 years using the Kaplan–Meyer method was 93%. Two hundred joints were functional and painless. We found good integration and positioning of the components in 184 (93%) of the joints. Sixteen prostheses failed. We conclude that this implant has acceptable long-term survival rate and restores good hand function. We also report our methods to improve implant survival and to decrease the risk of component malpositioning, and failure rate. Level of evidence: II

Outcomes of cardiovascular surgery for chronic dialysis patients in current Japan

2019 ◽  
Vol 27 (6) ◽  
pp. 464-470
Author(s):  
Hiroshi Kurazumi ◽  
Masaya Takahashi ◽  
Shigeru Ikenaga
Keyword(s):  
Survival Rate ◽  
Cardiovascular Surgery ◽  
Hazard Ratio ◽  
Dialysis Patients ◽  
Term Survival ◽  
Long Term Follow Up ◽  
History Of ◽  
Atherosclerosis Obliterans

Background The number of dialysis patients in Japan is rising, with an increasing number requiring cardiovascular surgery. Methods We investigated the short- and long-term outcomes in 70 dialysis patients among a total of 1124 who underwent cardiovascular surgery in our hospital between 2004 and 2016. We investigated outcomes following open surgery and identified factors that affected the prognosis. We also compared the long-term survival rate with the survival rate of the Japanese dialysis population. Results The long-term survival rate was 70.6%, 51.1%, and 19.2% after 3, 5, and 10 years, respectively. The causes of long-term death were heart disease in 8 patients, cerebrovascular disease in 7, cachexia in 3, infection in 2, and other causes in 3. The freedom from cardiac death was 88.7%, 77.9%, and 54.9% after 3, 5, and 10 years, respectively. Multivariate analysis using Cox’s proportional hazard model showed that a history of atherosclerosis obliterans (hazard ratio 5.4, p = 0.05) and mediastinitis (hazard ratio 10.2, p = 0.03) were risk factors for death in long-term follow-up, and a history of atherosclerosis obliterans was an independent risk factor for cardiac death in long-term follow-up (hazard ratio 5.3, p = 0.01). Five-year survival of the study subjects was comparable to that of the Japanese dialysis population. Conclusions The prognosis for dialysis patients after open surgery was equivalent to that of Japanese dialysis patients in general. A high proportion of late postoperative deaths were due to heart disease. Patients with atherosclerosis obliterans had a poor prognosis.

scholarly journals How to evaluate diagnosis and management of biliary atresia in the era of liver Transplantation in China

2018 ◽  
Vol 1 (1) ◽  
pp. e000002
Author(s):  
Jianghua Zhan ◽  
Yajun Chen ◽  
Kenneth K Y Wong
Keyword(s):  
Liver Transplantation ◽  
Survival Rate ◽  
Early Diagnosis ◽  
Biliary Atresia ◽  
Further Education ◽  
Monitoring Methods ◽  
Kasai Operation ◽  
First Choice ◽  
Choice Of Treatment ◽  
Hepatobiliary Diseases

BackgroundBiliary atresia (BA) is one of the most challenging hepatobiliary diseases in children. Notwithstanding the reasonable outcome of liver transplantation (LT), portoenteral anastomosis (Kasai operation) is still the first choice of treatment for patients with BA. How to improve the survival rate of the patients with BA in the era of LT is a new challenge.Data sourcesBased on recent original publications and the experience with the BA in China, we review many factors that influence BA survival situation, including early diagnosis and screen plan, defects of early treatment, Kasai operation, and LT and indicate present questions about BA diagnosis and treatment in China.ResultsBA diagnosis may also be delayed due to insufficient understanding of BA and lack of jaundice monitoring methods at different levels of the hospitals in China. Further education of the physicians at smaller city hospitals about BA, neonatal jaundice and cholestasis would be helpful in improving early diagnosis of BA. Early surgical intervention is still the only guarantee to improve the survival rate of BA with native liver.ConclusionsIn the era of LT, especially in China, Kasai operation can provide waiting time for living donor LT and improve the success rate of LT and minimize the rapid deterioration of liver function of the children with BA and decrease the mortality in patients with BA.

scholarly journals Liver Transplantation for Hepatic Epithelioid Hemangioendothelioma: The Canadian Multicentre Experience

2008 ◽  
Vol 22 (10) ◽  
pp. 821-824 ◽  
Author(s):  
Carmine G Nudo ◽  
Eric M Yoshida ◽  
Vincent G Bain ◽  
Denis Marleau ◽  
Phil Wong ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Survival Rate ◽  
Liver Lesion ◽  
Epithelioid Hemangioendothelioma ◽  
Rare Entity ◽  
Rare Tumour ◽  
Time To Recurrence ◽  
The Mean ◽  
Mean Time

INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare entity. At the present time, there is no standardized effective therapy. Liver transplantation (LT) has emerged as a treatment for this rare tumour.OBJECTIVE: To evaluate the outcome of liver transplantation for HEHE at eight centres across Canada.METHODS: The charts of patients who were transplanted for HEHE at eight centres across Canada were reviewed.RESULTS: A total of 11 individuals (eight women and three men) received a LT for HEHE. All LTs were performed between 1991 and 2005. The mean (± SD) age at LT was 38.7±13 years. One patient had one large liver lesion (17 cm × 14 cm × 13 cm), one had three lesions, one had four lesions and eight had extensive (five or more) liver lesions. One patient had spleen involvement and two had involved lymph nodes at the time of transplantation. The mean duration of follow-up was 78±63 months (median 81 months). Four patients (36.4%) developed recurrence of HEHE with a mean time to recurrence of 25±25 months (median 15.6 months) following LT. The calculated survival rate following LT for HEHE was 82% at five years.CONCLUSIONS: The results of LT for HEHE are encouraging, with a recurrence rate of 36.4% and a five-year survival rate of 82%. Further studies are needed to help identify patients who would benefit most from LT for this rare tumour.

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