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1.

Background/ Purpose

Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience.

Methods

A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted.

Results

Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years.

Conclusion

This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.  相似文献   

2.

Introduction

Posttransplant lymphoproliferative disorders (PTLD) arising in solid organ transplant patients show a strong correlation with Epstein-Barr virus (EBV) infection. The aim of our study was to evaluate the frequency of EBV DNA levels before and after transplantation among saliva samples of Iranian kidney transplant patients.

Materials and Methods

In 40 kidney transplant recipients and their healthy donors, we evaluated the EBV DNA levels in saliva samples by quantitative polymerase chain reaction method using samples obtained 1 hour before transplantation surgery. In addition, 3 months after transplantation we evaluated EBV DNA levels in recipients for comparison with preoperative levels. Exclusion criteria included hepatitis B, hepatitis C, and human T-cell leukemia virus infections before transplantation as well as HIV-positive patients under highly active antiretroviral therapy, graft rejection requiring dialysis and active viral infection of oral cavity.

Results

Before renal transplantation, there was no significant difference in saliva EBV DNA levels between kidney donor and recipient patients (P = .8). The levels increased significantly to 67.6% in the recipient group after transplantation (P = .01).

Conclusion

We observed that EBV was more frequently present in the oral mucosa after renal transplantation. Thus, we can follow these patients for EBV infection using saliva examinations as a simple screening method.  相似文献   

3.

Background/Purpose

The emergence of improved outcomes for small bowel (SB) transplantation has raised questions regarding the utility of autologous intestinal lengthening for patients with short bowel syndrome (SBS). Chronic immunosuppression, multiple hospitalizations, and posttransplant lymphoproliferative disease are significant adverse factors. The purpose of this study is to evaluate the 20-year single institution experience with the Bianchi procedure and analyze its role in the management of pediatric SBS.

Methods

Medical records for 19 consecutive patients who underwent the Bianchi procedure from 1984 to 2004 were reviewed. Patients were categorized into 3 groups: less than 5 years, 5 to 9.9 years, and 10 years or more after surgery. Various outcome variables were evaluated. Data are presented in tabular format as the number of patients (%) or average (range).

Results

Nineteen patients were included in the study. Of 16 patients weaned from total parenteral nutrition (TPN), 7 (44%) responded to Bianchi procedure alone and 9 patients (56%) required SB transplant at an average of 4.09 years (range, 0.7-13.64 years) post-Bianchi. Four patients (21%) died, 1 received SB transplant and died of unrelated causes, and 3 were still on TPN and had not received SB transplantation.

Conclusion

The Bianchi procedure facilitated weaning from TPN and eliminated the need for supplemental nutrition in select patients. Although the role of surgery is primarily adjunctive in the treatment of SBS, it offers therapeutic benefit in decreasing parenteral nutrition requirements and promoting intestinal adaptation. In particular, the Bianchi procedure has significant potential to improve the prognosis of pediatric patients with SBS.  相似文献   

4.

Background

Ongoing bleeding after blunt solid organ injury in children may require invasive therapy in the form of either angiographic or operative control. We report our experience in the use of a procoagulant, recombinant activated factor VII (rFVIIa), for controlling persistent bleeding in blunt abdominal trauma in children.

Methods

After institutional review board approval, the records of 8 children with blunt abdominal trauma, persistent bleeding, and managed nonoperatively with rFVIIa were reviewed.

Results

All 8 patients presented to our institution after sustaining blunt abdominal trauma and solid organ injury. All children had evidence of persistent bleeding with a drop in hematocrit and elevation in heart rate. Patients received a single dose of rFVIIa at 75 to 90 μg/kg (1 patient had 24 μg/kg) and had successful control of their bleeding without any further therapeutic intervention. Only 3 patients required a blood transfusion after rFVIIa administration—2 who had subarachnoid hemorrhages and the third during pelvic fixation. There were no cases of thromboembolic events after treatment with rFVIIa.

Conclusions

Recombinant factor VIIa is a useful adjunctive therapy in pediatric patients with evidence of ongoing hemorrhage from blunt abdominal injury and may reduce the need for invasive therapeutic procedures and transfusions.  相似文献   

5.

Objective

To establish a score to predict 30-day mortality and graft loss retrospectively and to validate the score prospectively.

Patients and Methods

Retrospectively in 296 liver transplant recipients, a score was developed that included the peak aspartate aminotransferase concentration within the first week and γ-glutamyltransferase and bilirubin concentrations at day 7 to predict graft loss or patient death within 30 days. The score was then prospectively validated in 86 patients undergoing liver transplantation.

Results

From the retrospective training cohort, cut-off values for prediction of adverse outcomes were determined using receiver operating characteristic curve analysis for peak aspartate aminotransferase (>1870 IU/mL), γ-glutamyltransferase (<214 IU/mL), and bilirubin (>5.75 mg/dL). Sensitivity and specificity of the score to predict an end point from the retrospective cohort were excellently reproduced in the prospective cohort. Overall, fulfilment of at least 2 criteria predicted graft loss or death within 30 days with sensitivity of 0.70 and specificity of 0.78. No patients with values that remained below all 3 thresholds experienced graft loss or death within 30 days.

Conclusions

This simple score calculated from standard laboratory values within the first week after liver transplantation enables prediction of graft loss and patient death within 30 days after transplantation. Early identification of patients at risk may help to improve outcomes by observing these patients more closely and allocating resources for them.  相似文献   

6.

Background and Purpose

In this report, we present the first case of an immunologically impaired child surviving a lytic varicella-zoster virus infection affecting the enteric nervous system. In histological findings, myenteric and submucous enteric ganglia were nearly completely absent owing to virus infection.

Methods

A 3-year-old girl with acute lymphoblastic leukemia and generalized varicella-zoster infection developed an ileus. She underwent multiple laparotomies in which histological sections of the entire small intestine could be obtained.

Results

The histological evaluation of these samples showed a generalized aganglionosis with inflammatory residuals. A more detailed immunohistochemical analysis using neuronal (PGP, enolase), glial (S100), and lymphocytic (LCA) antibodies demonstrated a nearly complete neuronal loss.

Conclusion

To our knowledge, this is the first case of a secondary intestinal aganglionosis after varicella-zoster virus infection.  相似文献   

7.

Background

An inverse association between hospital procedure volume and postoperative mortality has been demonstrated for a variety of pediatric surgical procedures. The objective of our study was to determine whether such an association exists for pediatric liver transplantation.

Methods

We performed a retrospective analysis of pediatric liver transplant procedures included in the Scientific Registry of Transplant Recipients over a 7.5-year time period from July 1, 2000, through December 31, 2007. Pediatric liver transplant centers were divided into three volume categories (high, middle, low) based on absolute annual volume. Mean 1-year patient survival rates and aggregate 1-year observed-to-expected (O:E) patient death ratios were calculated for each hospital volume category and then compared using ordered logistic regression and chi square analyses.

Results

High-volume pediatric liver transplant centers achieved significantly lower aggregate 1-year O:E patient death ratios than low-volume centers. When freestanding children's hospitals (FCH), children's hospitals within adult hospitals (CAH), and other centers (OC) were considered separately, we found that a significant volume-outcomes association existed among OC centers but not among FCH or CAH centers. Low-volume OC centers, which represent 41.6% of all pediatric liver transplant centers and perform 10% of all pediatric liver transplantation, had the least favorable aggregate 1-year O:E patient death ratio of all groups.

Conclusions

We demonstrate that a significant center volume-outcomes relationship exists among OC pediatric liver transplant centers but not among FCH or CAH centers. These findings support the possible institution of minimum annual procedure volume requirements for OC pediatric liver transplant centers.  相似文献   

8.

Background/Purpose

Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome.

Methods

A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994.

Results

Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years.

Conclusions

Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.  相似文献   

9.

Background/Purpose

The success of pediatric orthotopic liver transplantation (OLTxp) has improved greatly since its widespread application in the 1980s. No group has benefited more from this than infants younger than 1 year. The authors reviewed their experience in the management and outcome of children who underwent OLTxp when they were younger than 1 year.

Methods

A retrospective review of the medical records of patients who at the time of OLTxp were younger than 1 year was performed. Patients were stratified according to the period when transplanted.

Results

Eighty-one infants younger than 1 year underwent OLTxp. End-stage liver disease secondary to biliary atresia was the most common indication for transplantation. The overall survival was 77%. One-year patient and graft survival improved from 58% and 50% in the period 1986-1989, respectively, to 88% and 81% in the period 2000-2003, respectively. Technical complications such as hepatic artery thrombosis (n = 5) and portal vein thrombosis (n = 8) occurred, and although 4 patients required retransplantation, all but one survived. Complications associated with immunosuppression, sepsis/multisystem organ failure (MSOF) (n = 11), and posttransplant lymphoproliferative disease (PTLD) (n = 1) were the most common cause of poor outcome.

Conclusions

Successful OLTxp in infants is possible with improved posttransplant survival during the study period. Technical complications (hepatic artery thrombosis/portal vein thrombosis) may require retransplantation but were uncommon causes of patient loss. Multisystem organ failure was the most significant adverse complication. The consequences of immunosuppression (MSOF/PTLD) were the most common cause of patient loss. Further improvement in overall survival will require better immunosuppressive strategies.  相似文献   

10.

Objective

The purpose of this study was to identify the risk of respiratory disease for developing inguinal hernia (IH).

Study design

A retrospective case-control study reviewed medical records of 100 outborn infant males, gestational age (GA) less than 35 weeks, admitted between July 1999 and November 2002 to our level III neonatal intensive care unit. Prospectively selected variables were reviewed from 50 cases of IH and 50 controls.

Results

Premature infants expressing IH were more likely to have had lower GA and birth weight, longer duration of mechanical ventilation, use of high-frequency oscillating ventilator (HFOV), higher peak mean airway pressures during mechanical ventilation, and postnatal dexamethasone exposure. Adjusted results showed dexamethasone, HFOV in infants less than 28 weeks GA, and length of mechanical ventilation remained significant. Multivariate analysis revealed dexamethasone as an independent risk for the expression of IH.

Conclusions

The association of IH expression with mechanical support and dexamethasone administration was substantial. These data provide further caution when using either HFOV or dexamethasone in preterm infants with respiratory insufficiency.  相似文献   

11.

Introduction

Posttransplantation lymphoproliferative disorder (PTLD) remains an uncommon complication of solid organ transplantation, with a high mortality rate reported after conventional therapies. Epstein-Barr virus (EBV) may cause PTLD, but most EBV infections after transplantation are clinically silent reactivations, so the detection of PTLD is often delayed. Recently we experienced the rare case of intrarenal graft PTLD found by macrohematuria in a simultaneous pancreas and kidney transplant recipient. The grafts were saved by treatments with rituximab, cyclophosphamide, hydroxydaunorubicin, and prednisone-based chemotherapy (R-CHOP) after reduction of immunosuppression (IR).

Methods

This 37-year-old man with insulin-dependent diabetes underwent simultaneous pancreas and kidney transplantation (SPK) with enteric drainage. Six months after transplantation, he displayed macrohematuria, which we investigated by blood tests, computer tomography (CT) scan, positron emission tomography (PET)-CT, and magnetic resonance imaging, recognizing a tumor in the transplanted renal graft. An open biopsy showed a CD20-positive PTLD. We started treatments with IR, rituximab (375 mg/m2, weekly for 2 cycles) and R-CHOP therapy: rituximab (375 mg/m2) plus CHOP every 3 weeks for 6 cycles.

Results

IR and R-CHOP therapy achieved a complete remission (CR). CR has continued for 14 months at the time of writing. The maximum level of EBV DNA was 259 copies/μg DNA, but 2 months after these therapies, the level had decreased to normal. The patient had no impairment of pancreas and kidney graft functions.

Conclusions

The outcome of intragraft PTLD in the kidney of an SPK recipient suggested that the negative impact of IR on graft function may be compensated by the immunosuppressive effects of rituximab, allowing reduced immunosuppression during chemotherapy.  相似文献   

12.
13.

Background

Congenital diaphragmatic hernia (CDH) is a condition with significant mortality and respiratory morbidity. Long-term neurodevelopmental outcome is not yet well documented.

Methods

The authors examined all cases of CDH at their institution over a 12-month period and performed magnetic resonance imaging (MRI) of the brain postoperatively.

Results

Eight of 10 patients survived to have an MR brain scan. All 8 patients had cerebral injury detected on MRI, with a predominance of white matter and deep nuclear gray matter injury.

Conclusions

Cerebral injury detected with MRI is common in infants with congenital diaphragmatic hernia. The long-term significance of these MRI abnormalities is unknown, although the need for good long-term neurodevelopmental follow-up of infants with CDH is highlighted.  相似文献   

14.

Background/Purpose

The aim of this study was to determine whether a novel laparoscopic technique for pediatric umibilical and epigastric hernia repair is safe, quick, effective, durable, and cosmetically acceptable.

Methods

Forty-one consecutive umbilical and 13 epigastric hernias were repaired laparoscopically. Six patients had concomitant epigastric and umbilical hernias repaired during one procedure. Two 3-mm ports were used via a lateral approach. Mean age was 4.2 years for those with an umbilical hernia and 2.0 years for those with an epigastric hernia.

Results

All procedures were completed laparoscopically. The mean operative time was 27 minutes for umbilical hernia repair and 16 minutes for epigastric hernia repair. There were no intraoperative or postoperative complications. Follow-up was 100% and ranged from 6 to 35 months. There have been no recurrent hernias, and patient/parent satisfaction was subjectively excellent.

Conclusion

This case series demonstrated that this novel technique of laparoscopic anterior abdominal wall hernia repair is safe, quick, effective, durable, and cosmetically acceptable. It requires few instruments and minimal advanced laparoscopic techniques.  相似文献   

15.

Purpose

Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy.

Methods

All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, α-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels.

Results

Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 ± 48 months). Patients were transplanted a mean of 4 ± 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival.

Conclusions

Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.  相似文献   

16.

Introduction

Malignancies are a serious long-term complication among liver transplant recipients, with an overall incidence of 4.5%-15%. Posttransplantation lymphoproliferative disease (PTLD) is one of the leading causes of late death. Its development is related to complex interactions between immunosuppressive drugs and environmental agents. The aim of this study was to analyze risk factors for PTLD and survival after orthotopic liver transplantation (OLT) compared with solid tumors.

Patients and Methods

We undertook a retrospective review of the clinical histories of adult patients who underwent OLT between July 1986 and February 2001, and who had been followed until 2005. This study comprised 528 adult recipients who survived more than 2 months after OLT. We excluded pediatric, partial-organ, and multiorgan recipients.

Results

No differences were observed concerning gender, viral etiology of hepatitis, calcineurin inhibitor regimen, or steroid maintenance period. Treated acute rejection episodes accounted for 53.3% of patients who developed PTLD compared with 47.3% in the control group (P = .787). Patients with solid tumors were older at the time of diagnosis than those with PTLD (57.5 ± 8.13 years vs 48.8 ± 13.9; P = .002). The overall mortality rate for PTLD was 55.5%, which did not differ significantly from solid tumors.

Conclusions

PTLD develops in younger patients after OLT. Various immunosuppressive regimens do not seem to influence the incidence of PTLD or other solid tumors.  相似文献   

17.

Aim

To assess the consequences of graft steatosis on postoperative liver function as compared with normal liver grafts.

Patients and methods

From January 2005 to December 2007, liver transplant patients were prospectively included, excluding those who experienced arterial or biliary complications or presented acute rejection. All patients had a surgical biopsy after reperfusion. Patients were compared according to the rate of macrovacuolar steatosis: namely above or below 20%.

Results

Fifty-three patients were included: 10 in the steatosis group and 43 in the control group. No significant difference was observed in terms of morbidity, mortality, and primary non- or poor function. Nevertheless, biological changes after the procedure were significantly different during the first postoperative week. Prothrombin time, serum bilirubin, and transaminases were significantly increased among the steatosis group compared with the control group (P < .05).

Conclusion

This case-controlled study including a small number of patients, described postoperative biological changes among liver transplantations with steatosis in the graft.  相似文献   

18.

Background

To our knowledge, the impact of venous tumour thrombus (VTT) consistency in patients affected by renal cell carcinoma (RCC) has never been addressed.

Objective

To analyse the effect of VTT consistency on cancer-specific survival (CSS).

Design, setting, and participants

We retrospectively analysed 174 consecutive patients with RCC and renal vein or inferior vena cava (IVC) VTT who underwent surgical treatment between 1989 and 2007 at our institute.

Intervention

All patients underwent radical nephrectomy and thrombectomy.

Measurements

Pathologic specimens were reviewed by a single uropathologist. In addition to traditional pathologic features, the morphologic aspect of the tumour thrombus was evaluated to distinguish solid from friable patterns. The prognostic role of thrombus consistency (solid vs friable) on CSS was assessed by means of Cox regression models.

Results and limitations

The VTT was solid in 107 patients (61.5%) and friable in 67 patients (38.5%). The presence of a friable VTT increased the risk of having synchronous nodal or distant metastases, higher tumour grade, higher pathologic stage, and simultaneous perinephric fat invasion (all p < 0.05). The median follow-up was 24 mo. The median CSS was 33 mo; the median CSS was 8 mo in patients with a friable VTT and 55 mo in patients with a solid VTT (p < 0.001). On multivariable analyses, the presence of a friable VTT was an independent predictor of CSS (p = 0.02). The power of our conclusion may be somewhat limited by the relatively small study population and the retrospective nature of the study.

Conclusions

In patients with RCC and VTT, the presence of a friable thrombus is an independent predictor of CSS. If our finding is confirmed by further studies, the consistency of the tumour thrombus should be introduced into routine pathologic reports to provide better patient risk stratification.  相似文献   

19.

Introduction

The most common neoplasias among transplant patients are skin cancers and lymphoproliferative disorders.

Objective

To characterize lung transplanted recipients who developed malignancies.

Methods

A retrospective analysis of clinical records of our patients.

Results

Seven patients developed malignancies: skin cancer (n = 5; 71%), and adenocarcinomas of prostatic, gastric, and lung (n = 1 each). One patient developed two hematologic malignancies: T-cell lymphoma and multiple myeloma. Among five patients who died (71%), 3 were due to advanced neoplasia. The mean presentation time was 4.3 years. Skin cancers were resected. The patient with lung adenocarcinoma developed pleural involvement and died. The patient with T-cell lymphoma was treated, but succumbed afterward due to multiple myeloma. The patient with gastric adenocarcinoma died at 3 months after the diagnosis, and the patient with prostate cancer underwent surgery without disease recurrence.

Conclusion

Malignancies are a late complication of transplant recipients that require a prompt diagnosis and treatment to improve outcomes.  相似文献   

20.

Objective

Programs for immunized transplant recipients are essential to achieve graft survivals comparable to those of non-immunized recipients. The threshold in Poland is a PRA by the complement-dependent cytotoxicity (CDC) method greater than 50%, which includes approximately 3.8% of the patients. At the same time the United Network for Organ Sharing there recipients represent approximately 16% of the waiting list in the United Network for Organ Sharing (UNOS). The underestimation of the immunized group in Poland may be due to differences in laboratory techniques to assess alloantibodies.

Materials and methods

This study investigated 55 potential recipients with a PRA by CDC > 50%. We used the following algorithm to assess their immunization: Luminex screening test for an HLA antibody; specificity assessed with Luminex Single Antigen, vPRA (evaluation of immunization of the patient); and analysis of acceptable HLA incompatibilities (HLAMatchmaker).

Results

All recipients were positive class I anti-HLA antibodies and 94.5% were positive for class II. For the groups of subjects with PRA-CDC from 50% to 79% versus those greater than 80%, the average values of PRA-CDC were 62.2% and 89.5%, respectively. The virtual PRA results for these groups were 95.7% and 97.2%, respectively. In addition, anti-HLA-Cw, anti-DQ and anti-DP antibodies were detected in 77%, 84%, and 51% of recipients, respectively. Immunized recipients reported to the next transplant were characterized by the antibodies against mismatch only in 68%. For all potential recipients, additional acceptable non-compliance was determined with HLAMatchmaker: 152 specificity for locus A and 252 for locus B.

Conclusions

Evaluation of immunization status of recipient candidates should be routinely performed using tests to assess class and specificity as well as level of alloantibodies to enable determination of a safe potential donor. As a routine test, PRA-CDC underestimates the number of highly immunized patients. Exclusion from the list of patients with repeated non-compliance is a simplification, which reduces their chance for transplantation.  相似文献   

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