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1.

Background/Purpose

Progressive familial intrahepatic cholestasis syndromes are characterized by impaired bile acid secretion resulting in pruritus, coagulopathy, diarrhea, and malnutrition leading to progressive liver failure and death in childhood. Partial internal or external biliary drainage can relieve symptoms and slow the progression of the disease. Objections to partial external biliary drainage include the need for a permanent biliary stoma with all the inherent complications of a stoma. We propose a novel approach to these diseases—placement of a “button” cholecystostomy tube.

Methods

Under general anesthesia and through a small right subcostal incision, a MIC-KEY button (Kimberly-Clark Worldwide, Inc, Draper, UT) is inserted into the mobilized fundus of the gallbladder and secured with 2 purse-string sutures. Time of drainage is adjusted to relieve pruritus.

Results

Three children with progressive familial intrahepatic cholestasis achieved adequate bile drainage via the cholecystostomy button to relieve pruritus for 1, 2, and 2 ½ years postoperatively, with drainage periods of 12 to 14 hours per day. There were no episodes of cholangitis. Dislodged tubes can be replaced, or stones can be retrieved via the tract that is formed. Patient (parent) acceptance has been excellent.

Conclusion

Button cholecystostomy is simple to perform, relieves pruritus with intermittent (nighttime) drainage, avoids complications of a permanent stoma, avoids an enteric anastomosis, and is accepted by parents.  相似文献   

2.
We described six children with heritable liver disease and hepatocellular carcinoma treated with living-related liver transplantation. Underlying liver diseases were type-1 tyrosinemia (three patients), progressive familial intrahepatic cholestasis type II (two patients), and Wilson's disease (one patient). Two of the tumors were found incidentally during liver transplantation. Number of nodules was 12, 15, 3, 2, and 1 (in two patients). Three patients were treated with chemotherapy before the procedure. Chemotherapy was not given to any patient after liver transplantation. The mean follow-up was 17.7 +/- 6 months (range: 7-24). All patients are tumor recurrence free. Both graft and patient survival rates are 100% at a median of 18.5 months follow-up. Physicians in charge of treating children with heritable liver disease should screen them periodically for the development of hepatocellular carcinoma. Liver transplantation may offer these children better survival rates.  相似文献   

3.
Purpose  We report the long-term outcome of ABO-incompatible living donor liver transplantation (LDLT) performed in our hospital. Methods  We started the LDLT program in 1991 and from that year up to now (2008) 11 patients have received an ABO-incompatible graft. Results  Nine out of the 11 cases have survived from 3.7 years to 13.9 years (mean 7.3 years) and they are in good conditions at present. Seven patients were subjected to preoperative apheresis. Eight patients experienced acute rejection and of them, 6 experienced steroid-resistant rejection that was treated with deoxyspergualin and apheresis. One patient who suffered rapidly progressing rejection died due to liver failure. Three patients who were administered rituximab did not suffer severe rejection nor adverse effects. During the long-term follow up 5 recipients had major complications such as postoperative lymphoproliferative disease, post-transplantation diabetes mellitus, portal vein occlusion and biliary stenosis. But those complications were controlled under stable conditions. Conclusions  We concluded that long-term survival can be expected after ABO-incompatible LDLT provided perioperative complications such as humoral rejection are overcome.  相似文献   

4.
ObjectivesObesity and non-alcoholic fatty liver disease (NAFLD) are rampant in South East Asia. There is paucity of data exploring its’ impact on donor suitability for living donor liver transplantation (LDLT). We aimed to describe and examine the factors related to non-utilization of potential donors in our LDLT programme.MethodsThis is an analysis of prospectively collected data on potential donors for an adult LDLT programme, between January 2017 and December 2019.ResultsFifty-five donors for 33 potential recipients were evaluated. The mean age was 31.6 ± 8.5 years, 52.7% were female and the ethnic divisions were: Chinese (50.9%), Indian (25.5%) and Malay (23.6%). The mean body mass index (BMI) among potential donors was 25.1 ± 4.0 kg/m2; 25.5% of donors had normal BMI, 23.6% were overweight and 50.9% were obese. Using the CAP modality of Fibroscan®, we identified the following grades of hepatic steatosis: 36.6% S0, 19.5% S1, 2.4% S2 and 41.5% S3. The non-utilization rate of our donors was 74.5% (41/55) and the main reasons were significant hepatic steatosis and/or obesity. Compared to suitable donors, unsuitable donors had significantly greater mean BMI, mean CAP scores, higher rates of dyslipidaemia and NAFLD.ConclusionNAFLD and obesity represent major challenges to an emerging LDLT programme in Malaysia.  相似文献   

5.
Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120-200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.  相似文献   

6.

Background/Purpose

The purpose of the study was to describe the initial experience with a novel approach to the surgical treatment of progressive familial intrahepatic cholestasis (PFIC), avoiding the creation of a permanent stoma.

Methods

Two teenaged patients, aged 15 and 17 years, underwent partial internal biliary diversion to treat incontrollable pruritus associated with PFIC. The surgical technique involved the creation of an isolated jejunal conduit, anastomosed proximally in a terminolateral fashion to the gallbladder and distally to the ascending colon. This operation combines the advantages of partially diverting the biliary flow from the enterohepatic cycle, avoiding an external biliary fistula. In one of the patients, this technique was used as a primary procedure, whereas in the other, a previous partial external diversion was converted to an internal diversion.

Results

Both patients had complete resolution of their pruritus and normalization of hepatic laboratory tests. One of the patients developed a mild choleretic diarrhea that can be controlled with eventual use of cholestyramine. No complications were observed related to this operation.

Conclusions

Biliary diversion appears to be a very attractive surgical option for the treatment of PFIC in children with a normal gallbladder. Long-term follow-up is necessary to evaluate late results and eventual complications of this approach.  相似文献   

7.
目的 探讨同种异体原位肝移植术后早期发生严重肝内胆汁淤积的危险因素.方法 回顾性分析2004年8月至2011年2月南方医科大学南方医院收治的225例同种异体原位肝移植患者的临床资料,根据是否发生严重肝内胆汁淤积将患者分为阳性组(60例)和阴性组(165例),对两组患者术前、术中、术后各项指标进行比较分析.计量资料采用t检验,不符合正态分布及方差齐性的计量资料和等级资料采用Wilcoxon秩和检验,计数资料采用x2检验,多因素分析采用Logistic回归模型.结果 术前阳性组患者肝硬化比例、肝性脑病积分、腹腔积液积分、国际标准化比值、PT、TBil、AST较阴性组高,两组比较,差异有统计学意义(x2=6.09,Z=2.22,2.60,2.46,2.84,4.81,3.42,P<0.05);而血清中Alb、Na+、K+、Hb、PLT比阴性组低,两组比较,差异有统计学意义(t=2.10,4.97,Z=2.49,t=3.51,Z=3.66,P<0.05).术中阳性组患者供、受者血型相容的比例、供肝轻度脂肪化比例、冷缺血时间、相对热缺血时间、术中出血量、输注RBC量、输注PLT量、输注冷沉淀量较阴性组高,两组比较,差异有统计学意义(x2 =4.29,13.11,Z=2.45,2.61,3.75,3.20,2.89,3.95,P<0.05).术后阳性组患者急性排斥、肝动脉血栓、肺部感染、血行感染、真菌感染、巨细胞病毒感染发生率比阴性组高,两组比较,差异有统计学意义(x2 =9.87,4.91,8.21,6.29,3.92,9.26,P<0.05).多因素分析发现术前TBil> 51.3 μmol/L、供肝轻度脂肪化、术中输注冷沉淀量、术后急性排斥、肝动脉血栓、术后肺部感染、血行感染、巨细胞病毒感染是发生严重肝内胆汁淤积的独立危险因素(OR=15.82,7.99,2.88,3.03,53.20,3.34,4.11,3.22,P<0.05);而在术前较高的PLT、较长的PT患者术后发生严重肝内胆汁淤积的几率较小(OR=0.33,0.25,P <0.05).术后6个月阳性组和阴性组病死率分别为41.7% (25/60)和19.4% (32/165),两组比较,差异有统计学意义(x2=11.54,P<0.05).结论 纠正受者术前不良的临床因素;术后积极控制感染和抗排斥反应可降低原位肝移植患者术后早期严重肝内胆汁淤积的发生率,并有可能改善早期预后.  相似文献   

8.
9.
10.
A clear understanding of the mechanisms in steatotic livers that trigger cholestasis or hyperbilirubinemia after living donor liver transplantation (LDLT) remains elusive. We hypothesized that microarchitectural disturbance might occur within regenerating steatotic livers without impairment of hepatic proliferative activity. Liver biopsy specimens from 67 LDLT recipients taken at the 10th postoperative day were scored for the numbers of portal tracts per area (nPT/A) of liver tissue and for intrahepatic cholestasis, and immunostained by proliferating cell nuclear antigen (PCNA) and Ki-67. The preoperative degree of macrovesicular steatosis (MaS) was independently associated with cholestasis after LDLT (P < 0.001). Serum total bilirubin results on the 1st, 3rd, and 7th days post-LDLT in MaS+ (5-30% of MaS; n = 37) patients were significantly higher than those in MaS- (<5% of MaS; n = 30) patients (P = 0.030, 0.042, and 0.019, respectively). Mean numbers of positively stained hepatocytes were 53.1 +/- 12.0 in patients with MaS and 48.0 +/- 17.1 in those without MaS by PCNA (P = 0.390), and 24.4 +/- 10.5 and 24.0 +/- 14.0 by Ki-67 (P = 0.940). However, a significant negative correlation was found between the degree of MaS and nPT/A (P = 0.013), and nPT/A was correlated with the grade of histological cholestasis (r = 0.350, P = 0.039). Intrahepatic cholestasis and hyperbilirubinemia after LDLT could be caused by scanty morphologic change of portal tract during steatotic liver regeneration.  相似文献   

11.
The current liver allocation system requires reevaluation because of the advancements in peri‐transplantation care and surgical techniques. And, the role of living donor liver transplantation (LDLT) in an emergency has not been determined yet. Retrospective review of all patients undergoing emergency liver transplantation (LT) from January 2000 to June 2010 was conducted, and clinical data were analyzed. Of the total 505 LTs, 69 patients (13.7%) underwent an emergency LT. Of these, 54 patients (78.3%) underwent LDLT using a right liver, and 15 patients (21.7%) underwent deceased donor liver transplantation (DDLT). The overall hospital mortality was 21.7% (15/69). The leading cause of death after transplantation was sepsis (60.0%). Multivariate analysis demonstrated that a model for end‐stage liver disease (MELD) >33 [hazard ratio (HR), 16.6; 95% confidence interval (CI), 1.443–191.632; p = 0.024] and existence of pre‐transplantation intubation (HR, 18.2; 95% CI, 1.463–225.483; p = 0.024) were independent factors associated with poor survival after emergency LT. LDLT group and DDLT group showed no difference in hospital mortality (p = 0.854) and graft survival (p = 0.861). Thus, MELD score and respiratory insufficiency could be parameters predicting post‐transplant survival. And, LDLT using the right liver could be an appropriate alternative to DDLT in an emergency.  相似文献   

12.
The efficacy of auxiliary partial orthotopic liver transplantation (APOLT) to overcome the problems associated with a markedly steatotic graft in a living donor has not been fully explored. We have recently performed APOLT in a patient with alcoholic liver disease, where the only potential candidate donor was affected by 50% macrovesicular steatosis and 30% microvesicular steatosis. The recipient's left liver was resected and the donor's left liver, corresponding to a 0.46% graft-to-recipient weight ratio, was orthotopically transplanted. The postoperative course of this patient was uneventful, except for a transient large amount of ascites. Native liver volume in the recipient serially decreased, and the volume of the graft serially increased after transplantation. Four months after transplantation, the donor and recipient are doing well with a normal liver function. In conclusion, APOLT may be a feasible solution for a markedly steatotic living donor graft in patients with alcoholic liver disease.  相似文献   

13.
BackgroundTo evaluate the impact of steatosis and/or idiopathic portal inflammation (IPI) in living donor livers on recipients’ clinical outcomes.MethodsWe assessed 305 qualified donor liver samples from June 2013 to December 2018. Donors and recipients’ clinical characteristics, including follow-up data were retrieved. The graft and overall survival with/without steatosis or portal inflammation were compared by Kaplan-Meier analysis.ResultsFor living donors, the medium age of was 31.2 (28, 35.8) years old; liver histopathology showed macrovesicular steatosis: 0–5% 264/305 (86.6%) and 5–30% 41/305 (13.4%), IPI: no 220/305 (72.1%) and mild 85/305 (27.9%). For recipients, the medium age was 1.0 (0.6, 1.5) years old; the median pediatric-end-stage-liver-disease score was 16 (5.0, 26.0) and medium follow-up time was 32.8 (24.8, 52.0) months. Biliary atresia (69.5%) was the main indication for liver transplantation (LT).ConclusionsThe presence of steatosis and portal inflammation of the donor liver did not impact the clinical outcomes including transaminase or bilirubin normalization, short-/long-term complications and recipients’ survival. However, recipients with high pediatric-end-stage-liver-disease score (>16) receiving donor liver with portal inflammation, but not steatosis, had trend negative effect on recipients’ survival. In conclusion, donor livers with mild steatosis and portal inflammation were qualified for pediatric living donor LT. However, donor liver with mild portal inflammation would better not be allocated to recipients with high pediatric-end-stage-liver-disease score. This study provided new evidence in pediatric living donor liver allocation.  相似文献   

14.
Heparin is widely used to reduce the incidence of vascular thrombosis after liver transplantation. Appropriate use of heparin based on changes in coagulation and fibrinolytic profiles, however, has not yet been discussed in detail. We performed living-donor liver transplantation for 128 adult patients. In this series, dalteparin (25 IU/kg/d) was administered until post-operative day (POD) 2. On POD 3, the anticoagulant drug was changed to heparin (unfractionated heparin sodium, 5000 U/d), the dose of which was changed according to the level of activated clotting time (ACT) targeted between 130 and 160 s. The plasma level of plasmin-alpha2 plasmin inhibitor complex, thrombin-antithrombin III complex (TAT), and fibrin degradation product D-dimer (FDP-DD) were monitored in the 21 patients. Predictors for heparin doses were analyzed among clinical parameters (n = 128). Four patients (3%) were complicated with thrombosis despite the above-mentioned anticoagulation protocol. Transfusion and/or relaparotomy for hemostasis were necessary for bleeding in 19 patients (15%). The TAT level markedly elevated until POD 3 and FDP-DD peaked later. The required heparin dose to maintain adequate ACT levels increased linearly until POD 8, and kept constant thereafter, which correlated with the weight of the liver graft (p = 0.01). Thus, frequent monitoring of the heparin dosage is necessary to keep the ACT level in the target range in the first post-operative week. High hemorrhage complications in our series indicate that the lower target ACT range may be preferable in the second post-operative week.  相似文献   

15.
Pediatric recipients of living-donor liver transplants (LDLT) can often discontinue immunosuppression (IS). We examined factors affecting development of operational tolerance (OT), defined as off IS for >1 year, in this population. A historic cohort analysis was conducted in 134 pediatric primary semi-allogeneic LDLT. Multivariate logistic regression analysis was used. The frequency of peripheral regulatory T cells (Tregs) was determined at >10 years post-Tx by FACS analysis. IS was successfully discontinued in 84 tolerant patients (Gr-tol), but not in 50 intolerant patients (Gr-intol). The Gr-intol consisted of 24 patients with rejection (Gr-rej) and 26 with fibrosis of grafts (Gr-fib). The absence of early rejection [odds ratio (OR) 2.79, 95% CI 1.11-7.02, P = 0.03], was a positive independent predictor, whereas HLA-A mismatch (0.18, 0.03-0.91, P = 0.04) was a negative predictor. HLA-DR mismatches did not affect OT. The Treg frequency was significantly decreased in Gr-intol (4.9%) compared with Gr-tol (7.6%) (P = 0.003). There were increased levels of tacrolimus in the first week in Gr-Tol (P = 0.02). Although HLA-B mismatch (8.73, 1.09-70.0, P = 0.04) was a positive independent predictor of OT, its clinical significance remains doubtful. In this large cohort of pediatric LDLT recipients, absence of early rejection, HLA-A match and the later predominance of Tregs are factors associated with OT.  相似文献   

16.
Anonymous nondirected living liver donation (ANLLD), sometimes referred to as “altruistic” donation, occurs when a biologically unrelated person comes forward to donate a portion of his/her liver to a transplant candidate who is unknown to the donor. Here, we explore the current status of ANLLD with special consideration of published reports; US experience; impact on donor psychosocial outcomes; barriers to donation; and current global trends with respect to ethical considerations. Between 1998 and 2019, 105 anonymous nondirected living liver donor (ND-LLD) transplants have been documented in the US Scientific Registry of Transplant Recipients. Sixteen donors (15%) were reported to experience a postoperative complication. Currently, 89 donors remain alive (85%), 16 (15%) have unknown status, and none are confirmed deceased. Although there are only a handful of case series, these data suggest that ANLLD is a feasible option. While there are no liver-specific data, studies involving anonymous nondirected kidney donors suggest that anonymous donation does not adversely impact psychosocial outcomes in donors or recipients. There are substantial financial burdens and ethical considerations related to ANLLD. Further studies are required to assess donor demographics, psychosocial motivations, long-term health-related quality of life, and financial impact of ANLLD.  相似文献   

17.
Bruns H, Hillebrand N, Schneider T, Hinz U, Fischer L, Schmidt J, Goldschmidt AJW, Schemmer P. LabMELD‐based organ allocation increases total costs of liver transplantation: a single‐center experience.
Clin Transplant 2011: 25: E558–E565. © 2011 John Wiley & Sons A/S. Abstract: Introduction: In 2006, model for end‐stage liver disease (MELD)‐based allocation was implemented in the Eurotransplant (ET) region. Sick patients, who in general require more resources, are prioritized. In this analysis, the effect of MELD on costs for liver transplantation (LTx) was assessed. Methods: Total costs for LTx before and after implementation of MELD were identified in 256 patients from January 2005–December 2007. Forty‐nine patients (Re‐LTx, HU listings, and 30‐d mortality) were excluded from further analysis. The costs of LTx in 207 patients have been correlated with their corresponding labMELD; 84 and 123 LTx before and after implementation of MELD were compared, and patient survival was monitored. Results: A positive correlation exists between labMELD and costs (r2 = 0.28; p < 0.05). Only nominal correlation existed between the Child–Pugh classification and costs. The labMELD scores can be stratified into four groups (I: 6–10, II: 11–18, III: 19–24, and IV: >24), with an increase of €15.672 ± 2.233 between each group (p < 0.05). Recipients’ labMELD at the time of LTx increased significantly in the MELD‐based allocation system. Costs increased by €11.650/patient (p < 0.05), while median survival decreased from 1219 to 869 d (p < 0.05). Conclusion: LabMELD‐based allocation increased total costs of LTx. In accordance with other studies, the sickest patients need the most resources.  相似文献   

18.

Background

The purpose of the study was to determine the distribution of excreted bile during cholestatic periods and in remission in patients with progressive familial intrahepatic cholestasis (PFIC) after surgery with partial external biliary diversion (PEBD), using hepatobiliary scintigraphy.

Methods

Using intravenously administered technetium Tc 99m-labeled mebrofenin, the distribution of bile during periods of biochemical cholestasis and in remission was investigated in patients with PFIC operated with PEBD. Stomal bile, urine, and feces from the patients were collected during 24 hours after administration of technetium Tc 99m-labeled mebrofenin; and the fractions of remaining radioactivity in the 3 compartments and the remaining isotopic activity in the body were quantified using scintigraphy.

Results

Nine patients (4 boys and 5 girls) were studied. The median age was 13 (range, 5-24) years, and they had been operated with PEBD at a median time of 10 (range, 4-14) years before entering the study. Thirteen scintigraphic examinations were analyzed: 8 during noncholestatic remission (n = 7 patients) and 5 during cholestasis (n = 3 patients). The patients studied during remission discharged a significantly larger fraction of isotopic activity through the stoma (median, 90% vs 22%; P < .05) and a significantly lower fraction through the urine (median, 2.5% vs 15%; P < .05) compared with the patients studied during cholestasis.

Conclusion

Hepatobiliary scintigraphy could detect substantial differences in the output of bile. Further studies are needed to determine whether these differences may explain the mechanism of the PEBD operation or merely are secondary to the degree of cholestasis.  相似文献   

19.
Abstract: Background: Macrovesicular steatosis of the liver is associated with early dysfunction or poor function of the graft after transplantation; however, it can be quantified accurately only through a liver biopsy that sometimes may not be available and whose result is anyway known when the recipient has already been selected. It would, therefore, be helpful to be able to predict the degree of steatosis, on the basis of non‐invasive readily available variables. Methods: Data from 374 deceased liver donors from whom a liver biopsy had been taken were analyzed with the receiver operating characteristic area [area under the curve (AUC)] to identify variables that could predict the degree of macrovesicular steatosis classified as: absent to mild (0–30%) and moderate to severe (>30%). Results: Steatosis was associated significantly with donor age, increased body mass index (BMI), presence of type II diabetes, ultrasonographic features, heavy alcohol consumption, transaminases [aspartate‐aminotransferase and alanine‐aminotransferase (ALT)], gamma‐glutamyl‐transpeptidase, and glucose blood levels. The combination of BMI, elevation of ALT, presence of type II diabetes, history of heavy alcohol consumption, and ultrasonography signs of steatosis could identify steatosis >30% accurately with an AUC of 0.86 (95% CI = 0.81–0.91). Conclusion: Fatty infiltration in liver donors can be estimated based on clinical and biochemical parameters.  相似文献   

20.
A nationwide survey of living donor liver transplantation (LDLT) for hepatitis C virus (HCV)‐positive recipients was performed in Japan. A total of 514 recipients are reported and included in the study. The cumulative patient survival rate at 5 and 10 years was 72% and 63%, respectively. Of the 514 recipients, 142 patients (28%) died until the end of the observation, among which the leading cause was recurrent hepatitis C (42 cases). According to Cox regression multivariate analysis, donor age (>40), non‐right liver graft, acute rejection episode, and absence of a sustained virologic response were independent prognostic factors. Of the 514 recipients, 361 underwent antiviral treatment mainly with pegylated‐interferon and ribavirin (preemptive treatment in 150 patients and treatment for confirmed recurrent hepatitis in 211). The dose reduction rate and discontinuation rate were 40% and 42%, respectively, with a sustained virologic response rate of 43%. In conclusion, patient survival of HCV‐positive recipients after LDLT was good, with a 10‐year survival of 63%. Right liver graft might be preferable for HCV‐positive recipients in an LDLT setting.  相似文献   

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