The disparity between patients awaiting transplantation and available organs forced many patients to go overseas to receive a transplant. Few data concerning overseas transplantation in Korea are available and the Korea Society for Transplantation conducted a survey to evaluate the trend and outcome of overseas transplantation. The survey, conducted on June 2006, included 25 hospitals nationwide that followed up patients after receiving kidney transplant (KT) or liver transplant (LT) overseas. The number of KT increased from 6 in 2001 to 206 in 2005 and for LT from 1 to 261. The information about overseas transplant came mostly from other patients (57%). The mean cost for KT was $21,000 and for LT $47,000. Patients were admitted for 18.5 days for KT and 43.4 days for LT. Graft and patient survival was 96.8% and 96.5% for KT (median follow up 23.1 months). Complication occurred in 42.5% including surgical complication (5.3%), acute rejection (9.7%) and infection (21.5%). Patient survival for LT was 91.8% (median follow up 21.2 months). Complication occurred in 44.7% including 19.4% biliary complication. Overseas KT and LT increased rapidly from 2001 to 2005. Survival of patients and grafts was comparable to domestic organ transplantation, but had a high complication rate.
Transplant Tourism; Medical Tourism; Commercial Organ Transplantation; Kidney Transplantation; Liver Transplantation; Outcome
The relationship between patient survival and biopsy-proven acute rejection (BPAR) in liver transplant recipients with hepatitis C remains unclear. The aims of this study were to compare the characteristics of patients with and without BPAR and to identify risk factors for BPAR.
We retrospectively reviewed the records of 169 HCV-RNA-positive patients who underwent LT at three centers.
BPAR occurred in 39 (23.1%) of the HCV-RNA-positive recipients after LT. The 1-, 3-, and 5-year survival rates were 92.1%, 90.3%, and 88.5%, respectively, in patients without BPAR, and 75.7%, 63.4%, and 58.9% in patients with BPAR (P<0.001). Multivariate analyses showed that BPAR was associated with the non-use of basiliximab and tacrolimus and the use of cyclosporin in LT recipients with HCV RNA-positive.
The results of the present study suggest that the immunosuppression status of HCV-RNA-positive LT recipients should be carefully determined in order to prevent BPAR and to improve patient survival.
Hepatitis C virus; Immunosuppression; Rejection; Outcome; Calcineurin antagonists; Tacrolimus
Infectious complications, including bacteria, virus, and fungus, often occur after liver transplantation and are the most frequent causes of in-hospital mortality. The current study prospectively analyze the effect of early enteral feeding in patients after living donor liver transplantation (LDLT)
Between January 2013 and August 2013, 36 patients underwent LDLT. These patients were randomly assigned to receive enteral formula via nasointestinal feeding tubes [enteral feeding (EN) group, n = 17] or maintenance on intravenous fluid until oral diets were initiated (control group, n = 19). All patients completed the study.
The pretransplant and perioperative characteristics of patients did not differ between the 2 groups. The incidence of bacterial infection was significantly lower in the EN group (29.4%) than in the control group (63.2%) (P = 0.043). In addition, the incidence of bile duct complications in the EN group was lower than in the control group (5.9% versus 31.6%, P = 0.041). Multivariate analysis showed that early enteral feeding was closely associated with bacterial infections (odds ratio, 0.178; P = 0.041). There was no statistically significant difference in nutritional status between the 2 groups. There were no cases of in-hospital mortality.
Early enteral feeding after LDLT prevents posttransplant bacterial infection, suggesting the possibility of a reduction of in-hospital mortality as a result of decreased infectious complications.
Pediatric liver transplantation is the standard of care for treatment of liver failure in children. The aim of this study was to identify the characteristics of pediatric liver transplantation in centers located in Korea and determine factors that influence outcomes. This retrospective study was performed using data from between 1988 and 2010 and included all recipients 18 yr old and younger who underwent pediatric liver transplantation in Korea during that period. Our data sources were hospital medical records and the outcome measure was overall patient survival. Univariate and multivariate statistical analyses were undertaken using the Cox proportional hazards model. Five hundred and thirty-four pediatric liver transplantations were performed in 502 children. Median age and average pediatric end-stage liver disease (PELD) score were 20 months and 18 point, respectively. Biliary atresia (57.7%, 308/534) was the most common cause of liver disease. Eighty-two (15.3%) were deceased donor liver transplantations and 454 (84.7%) were living donor liver transplantations. Retransplantation was performed in 32 cases (6%). Overall, 1-, 5-, and 10-yr patient survival rates were 87.8%, 82.2%, and 78.1%, respectively. In multivariate analysis, independent significant predictors of poor patient survival were chronic rejection and retransplantation. This study presents the epidemiologic data for nearly all pediatric liver transplantation in Korea and shows that the independent prognostic factors in patient survival are chronic rejection and retransplantation.
Liver Transplantation; Children; Survival; Rejection; Retransplantation
Hepatoblastoma is the most common malignant liver tumor in children. The aim of this study was to review our results of hepatoblastoma treatment and to determine the role of surgical treatment in hepatoblastoma.
This is a retrospective clinical study. The medical records of patients with hepatoblastoma, treated between October 1994 and October 2009, were reviewed. The patients were classified according to the pretreatment extent of disease (PRETEXT) grouping system. The main outcome variable was survival. Secondary outcome variables were complete, partial and no response to chemotherapy and surgery, when indicated.
Twenty-seven patients were treated during the observation period. Eighteen were males. Five were PRETEXT group I, 8 group II, 13 group III and 1 group IV. Complete excision was achieved in all patients except in one case that underwent liver transplantation (group IV). Median follow-up and survival rate were 2.3 years and 100%, 6.6 years and 75%, 5.8 years and 92%, 7.7 years and 100%, for groups I to IV, respectively. Twenty patients are currently considered to be in complete response status and three patients are receiving postoperative chemotherapy. Four patients died; the causes of death were cytomegalovirus hepatitis, bone marrow suppression during adjuvant chemotherapy, primarynonfunction after the transplantation for recurrent tumor and metachronous rectal cancer, respectively.
Favorable long-term outcome could be expected for hepatoblastoma with complete tumor excision and adjuvant chemotherapy.
Hepatoblastoma; Surgery; Drug therapy; Transplantation
Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a highly malignant tumor of uncertain histogenesis. Here we report a case of DSRCT involving the stomach, initially misdiagnosed as gastric cancer. A 12-year-old boy presented with upper abdominal pain developed 1 month prior. On gastroscopy, a 7-cm mass was noted involving the esophago-gastric junction to the fundus, and positron emission tomography showed multiple hot uptakes suggesting distant metastasis. Gastroscopic biopsy showed poorly differentiated malignant cells. We diagnosed as stage IV gastric cancer and treated with 6 cycles of chemotherapy. Laparotomy revealed a huge gastric mass along with peritoneal disseminations. Palliative proximal gastrectomy was performed. Pathological examination revealed transmural involvement of DSRCT, and t(11;22)(p12;q12) was demonstrated on fluorescence in situ hybridization test. The chemotherapeutic regimen was changed and the patient underwent 8 additional cycles of post-operative chemotherapy. The patient is now alive and the residual tumor shows no significant changes after chemotherapy.
Stomach; Desmoplastic small round cell tumor; Children
Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (±95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4±10.6% vs. 69.4±9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.
Neuroblastoma; Prognosis; Thorax; Transplantation, Autologous
Anorectal innervation that governs sensation, motor function, and rectal accommodation can be influenced by the type of surgical procedure used to treat children with Hirschsprung disease. At our institution, we began to perform single-stage, laparoscopy-assisted transanal endorectal pull-through (LATEP) with submucosal dissection and anastomosis of the ganglionated bowel at 2 different levels relative to the dentate line.
This retrospective study describes postoperative stool frequency changes in response to this procedure. Forty infants who underwent single-stage LATEP between September 2003 and April 2012 in a single center by the same surgeon were included in our analysis.
The patients were divided in 2 groups: Group A (n = 23) underwent submucosal dissection and anastomosis at 2 mm above the dentate line, and Group B (n = 17) underwent the same procedure with anastomosis 15 mm above the dentate line. Clinical characteristics, clinical findings on the first postoperative visit, and instances of coexisting anomalies did not differ between the 2 groups. Aganglionic segments were found in the rectosigmoid colon in 18 cases (78.2%) in Group A and in 15 cases (88.2%) in Group B. Although the stool frequency was no different at 1, 3, 6, and 12 months after the operation, Group B showed significantly fewer bowel movements than Group A after 2 years (3.77 in Group A vs 2.0 in Group B; P = 0.035) and after 3 years (3.92 vs 1.29; P = 0.009) in patients who had aganglionosis of the rectosigmoid colon. The mean follow-up period was 65.87 ± 28.08 months for Group A and 35.59 ± 18.68 for Group B.
The level of submucosal dissection and anastomosis in single-stage LATEP influenced the stool frequency in rectosigmoid aganglionosis.
Hepatic steatosis assessment is of paramount importance for living liver donor selection because significant hepatic steatosis can affect the postoperative outcome of recipients and the safety of the donor. The validity of various noninvasive imaging methods to assess hepatic steatosis remains controversial. The purpose of our study is to investigate the association between noninvasive imaging methods and pathology to detect steatosis in living liver donors and to propose a prediction model for hepatic steatosis.
Liver stiffness measurements (LSMs) and controlled attenuation parameter values in vibration controlled transient elastography, ultrasonography, computed tomography (CT), and magnetic resonance imaging were used as pretransplant screening methods to evaluate living liver donors between 2012 and 2014. Only 1 pathologist assessed tissue sample for hepatic steatosis.
The median age of the 79 living donors (53 men and 26 women) was 32 years (16–68 years). The CT liver–spleen attenuation (L–S) difference and the controlled attenuation parameter values were well correlated with the level of hepatic steatosis on liver pathology. Multivariate analysis showed that liver stiffness measurement (LSM) (β = 0.903; 95% CI, 0.105–1.702; P = 0.027) and the CT L to S attenuation difference (β = −3.322; 95% CI, −0.502 to −0.142; P = 0.001) were closely associated with hepatic steatosis. We generated the following equation to predict total hepatic steatosis: Hepatic steatosis = 0.903 × LSM – 0.322 × CT L to S attenuation difference (AUC = 86.6% and P = 0.001). The values predicted by the equation correlated well with the presence of hepatic steatosis (r = 0.509 and P < 0.001).
The combination of nonenhanced CT L to S attenuation difference and transient elastography using vibration controlled transient elastography provides sufficient information to predict hepatic steatosis in living liver donor candidates.
HCV-related liver disease is the most common indication for liver transplantation (LT) in Western countries, whereas HCV LT is rare in Korea. We conducted a survey of HCV RNA-positive patients who underwent LT and investigated the prognostic factors for patient survival and the effects of immunosuppression.
We retrospectively reviewed the multicenter records of 192 HCV RNA-positive patients who underwent LT.
The 1-, 3-, and 5-year overall survival rates were 78.8%, 75.3%, and 73.1%, respectively. Excluding the cases of hospital mortality (n = 23), 169 patients were evaluated for patient survival. Most patients were genotype 1 (n = 111, 65.7%) or genotype 2 (n = 42, 24.9%). The proportion of living donors for LT (n = 135, 79.9%) was higher than that of deceased donors (deceased donor liver transplantation [DDLT], n = 34, 20.1%). The median donor and recipient ages were 32 years and 56 years, respectively. Twenty-eight patients (16.6%) died during the observation period. Seventy-five patients underwent universal prophylaxis and 15 received preemptive therapy. HCV recurrence was detected in 97 patients. Recipients who were older than 60, received DDLT, used cyclosporine, or suffered acute rejection had lower rates of survival.
Patent survival rates of HCV patients after LT in Korea were comparable with other countries.
Hepatitis C virus; Tacrolimus; Acute rejection; Survival; Antiviral treatments
To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution.
Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group classification used for GCTs in adults to validate the classification guidelines for GCTs in children.
The median patient age was 4.4 years. In 34 patients (51.5%), the primary tumor site was the gonad. Extragonadal GCTs were detected in 32 patients. The 5-year overall survival and event-free survival (EFS) were 92.0%±3.5% and 90.4%±3.7%, respectively. In univariate analysis, tumor histology, metastasis, and elevated alpha-fetoprotein were not prognostic factors in children with extracranial GCTs. However, EFS was poorer in patients with mediastinal disease (n=12, 66.7%±13.6 %) than in those with nonmediastinal disease (n=54, 96.0%±2.8%) (P=0.001). The 5-year EFS was lower in patients older than 10 years, (n=21, 80.0%±8.9%) compared with those younger than 10 years (n=45, 95.2%±3.3%) (P=0.04). Multivariate analysis identified the mediastinal tumor site as the only independent prognostic factor.
The prognosis of children with extracranial GCTs was favorable. However, nongerminomatous mediastinal tumors were associated with poor survival in children. Further research is needed to improve the prognosis of children with malignant mediastinal GCTs.
Germ cell and embryonal neoplasms; Child; Prognosis; Mediastinum; Survival
Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy.
This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group).
Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077).
Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.
Alagille syndrome; Kasai operation; Cholestasis; Liver transplantation
Diaphragmatic hernias (DH) occurring after pediatric liver transplantation (LT) are rare. However, such complications have been previously reported in the literature and treatment has always been surgical repair via laparotomy. We report our experience of minimally invasive thoracoscopic approach for repair of DH occurring after LT in pediatric recipients.
From April 2010 to December 2014, 7 cases of DH were identified in pediatric LT recipient in Samsung Medical Center. Thoracoscopic repair was attempted in 3 patients. Patients’ medical records were retrospectively reviewed.
Case 1 was a 12-month-old boy, having received deceased donor LT for biliary atresia (BA) 5 months ago. He presented with dyspnea and left-sided DH was detected. Thoracoscopic repair was successfully done and the boy was discharged at postoperative day 7. Case 2 was a 13-month-old boy, having received deceased donor LT for BA 2 months ago. He presented with vomiting and right-sided DH was detected. Thoracoscopic repair was done along with primary repair of herniated small bowel that was perforated while attempting reduction into the peritoneal cavity. The boy recovered from the surgery without complications and was discharged on the 10th postoperative day. Case 3 was a 43-month-old girl, having received deceased donor LT for Alagille syndrome 28 months ago. She was diagnosed with right-sided DH during steroid pulse therapy for acute rejection. Thoracoscopic repair was attempted but a segment of necrotic bowel was noticed along with bile colored pleural effusion and severe adhesion in the thoracic cavity. She received DH repair with small bowel resection and anastomosis via laparotomy.
Thoracoscopic repair was attempted in 3 cases of DH occurring after LT in pediatric recipients. With experience and expertise in pediatric minimally invasive surgery, thoracoscopic approach is feasible in this rare population of patients.
Cytomegalovirus (CMV) infections in liver transplant recipients are common and result in significant morbidity and mortality. Intravenous ganciclovir or oral valganciclovir are the standard treatment for CMV infection. The present study investigates the efficacy of oral valganciclovir in CMV infection as a preemptive treatment after liver transplantation.
Between 2012 and 2013, 161 patients underwent liver transplantation at Samsung Medical Center. All patients received tacrolimus, steroids, and mycophenolate mofetil. Patients with CMV infection were administered oral valganciclovir (VGCV) 900mg/day daily or intravenous ganciclovir (GCV) 5mg/kg twice daily as preemptive treatment. Stable liver transplant recipients received VGCV.
Eighty-three patients (51.6%) received antiviral therapy as a preemptive treatment because of CMV infection. The model for end-stage liver disease (MELD) score and the proportions of Child-Pugh class C, hepatorenal syndrome, and deceased donor liver transplantation in the CMV infection group were higher than in the no CMV infection group. Sixty-one patients received GCV and 22 patients received VGCV. The MELD scores in the GCV group were higher than in the VGCV group, but there were no statistical differences in the pretransplant variables between the two groups. AST, ALT, and total bilirubin levels in the GCV group were higher than in the VGCV group when CMV infection occurred. The incidences of recurrent CMV infection in the GCV and VGCV groups were 14.8% and 4.5%, respectively (P=0.277).
Oral valganciclovir is feasible as a preemptive treatment for CMV infection in liver transplant recipients with stable graft function.
The indication for hepatocellular carcinoma (HCC) is expanding in living donor liver transplantation (LDLT). Early detection and effective management of recurrence has become an important issue in LDLT for HCC. This study aimed to find an optimal screening protocol in terms of screening interval and screening tools by analyzing recurrence pattern after LDLT for HCC. A total of 205 LDLT patients in two centers from February 1999 to October 2010 was reviewed. Recurrence appeared in 55 cases. Six risk factors for recurrence were identified: preoperative alpha-fetoprotein >400, Edmonson grade 3 or 4, tumor size >7 cm, tumor number ≥7, minimal tumor necrosis in the transarterial chemoembolization group and positive micro-vascular invasion. Four groups with different ranges of index scores showed different recurrence-free survival and median time to recurrence. Group I showed low and late recurrence. Groups II and III showed linearly increased rate of recurrence until 18 months. Group IV showed very early recurrence within 6 months. Across the groups, extra-hepatic recurrence developed in more than 40% of cases and multi-organ recurrence rate was 20%. The screening interval should be different based on the risk of recurrence. Screening should include work-up for extra-hepatic recurrence as well as intra-hepatic recurrence.
Carcinoma; Hepatocellular; Living Donors; Liver Transplantation; Recurrence; Screening
Rapid fluid warming has been a cardinal measure to maintain normothermia during fluid resuscitation of hypovolemic patients. A previous laboratory simulation study with different fluid infusion rates showed that a fluid warmer using magnetic induction is superior to a warmer using countercurrent heat exchange. We tested whether the simulation-based result is translated into the clinical liver transplantation.
Two hundred twenty recipients who underwent living donor liver transplantation between April 2009 and October 2011 were initially screened. Seventeen recipients given a magnetic induction warmer (FMS2000) were matched 1 : 1 with those given a countercurrent heat exchange warmer (Level-1 H-1000) based on propensity score. Matched variables included age, gender, body mass index, model for end-stage liver disease score, graft size and time under anesthesia. Core temperatures were taken at predetermined time points.
Level-1 and FMS groups had comparable core temperature throughout the surgery from skin incision, the beginning/end of the anhepatic phase to skin closure. (P = 0.165, repeated measures ANOVA). The degree of core temperature changes within the dissection, anhepatic and postreperfusion phase were also comparable between the two groups. The minimum intraoperative core temperature was also comparable (Level 1, 35.6℃ vs. FMS, 35.4℃, P = 0.122).
A countercurrent heat exchange warmer and magnetic induction warmer displayed comparable function regarding the maintenance of core temperature and prevention of hypothermia during living donor liver transplantation. The applicability of the two devices in liver transplantation needs to be evaluated in various populations and clinical settings.
Body temperature change; Hypothermia; Living donors; Liver transplantation; Rewarming
Biliary strictures and bile leaks account for the majority of biliary complications after living donor liver transplantation (LDLT). The aim of this study was to examine differences in biliary complications after adult LDLTs were performed by an experienced senior surgeon and an inexperienced junior surgeon. Surgeries included bile duct reconstruction after adult LDLT using a right liver graft, and risk factors for biliary stricture were identified.
We retrospectively reviewed the medical records of 136 patients who underwent LDLT in order to identify patients who developed biliary complications.
The senior surgeon performed 102 surgeries and the junior surgeon performed 34 surgeries. The proportion of patients with biliary stricture was similar between the senior and the junior surgeons (27.5% vs 26.5%; P = 0.911). However, the incidence of biliary leakage was higher in patients of the junior surgeon than in those of the senior surgeon (23.5% vs 2.9%; P = 0.001). The frequency of percutaneous drainage was also higher for the junior surgeon than the senior surgeon because of the junior surgeon’s high leakage rate of the drainage. When the junior surgeon performed bile duct anastomosis, biliary leakage occurred in 7 patients between the 11th and 20th cases. However, biliary leakage occurred in only 1 case thereafter.
Bile duct reconstruction performed by beginner surgeons in LDLT using right lobe grafts should be cautiously monitored and observed by a senior surgeon until an inexperienced junior surgeon has performed at least 20 cases, because of the high incidence of biliary leakage related to surgeon’s inexperience in bile duct reconstructions in LDLT.
There have been intermittent reports of peroneal neuropathy (PN) occurring after liver transplantation. Although PN may not be viewed as a serious complication by liver transplant (LT) recipients who require the transplant for survival, PN can significantly reduce quality of life. The incidence of PN appears to have increased after the use of gel pads was introduced. These gel pads, which are placed under patients’ knees during surgery, are used to reduce lower back strain and prevent contact between the peroneal nerve at the fibular head and the hard surface of the operating table. The aim of the present study was to investigate the association, if any, between the use of gel pads and the incidence of PN.
The medical records of 261 adult LT recipients were retrospectively reviewed. The recipients were divided into gel pad (n = 167) and non-gel pad (n = 94) groups. The incidence and possible risk factors of PN were compared between the two groups.
The overall incidence of PN was 8.0% (21/261). The occurrence of PN was significantly higher in the gel pad group than in the non-gel pad group (10.8% vs. 3.2%; p < 0.05). Other possible risk factors were comparable between the two patient groups.
As the use of gel pads may increase the incidence of PN, we recommend against the use of gel pads under the knees of LT recipients.
liver transplantation; patient positioning; peroneal neuropathy
Fibrous hamartoma (FH) of infancy is a distinctive fibrous growth that most frequently occurs at birth and during the postnatal period. It is important for clinicians and pathologists to recognize this entity to avoid an aggressive approach.
We herein describe the clinicopathologic features of 9 FHs diagnosed at a single institution between 1997 and 2010.
There were 7 boys and 2 girls, and the mean age of presentation was 14.7 months. The common locations were the lower back and gluteal region (n = 3) and scrotum (n = 2). They were solitary lesions, and measured 1.0 to 7.0 cm in maximum diameter (mean, 4.9 cm). The excised masses tended to be poorly circumscribed, and consisted of an intimate mixture of firm, gray-white tissue with fat. Histologically, these lesions were composed of 3 components forming a vague, irregular, organoid pattern: well-defined intersecting trabeculae of fibrocollagenous tissue; loosely textured areas of small, rounded, primitive mesenchymal cells; and mature fat. Over a median follow-up of 72 months, no patient showed recurrence.
FH should be distinguished from other forms of fibromatosis and malignant tumors because it is benign and usually cured by local excision.
Hamartoma; Infant; Soft tissue neoplasms; Differential diagnosis; Fibromatosis
Graft-versus-host disease (GVHD) is a rare complication after kidney transplantation. We describe a 62-year-old female with end-stage renal disease due to hypertension. She received a kidney with 4 mismatched human leukocyte antigen (HLA) out of 6 HLA - A, B, DR from a deceased donor. After the procedure, the patient showed watery diarrhea on postoperative day (POD) 45. An endoscopic biopsy of the colon revealed some apoptotic cells consistent with GVHD. Thrombocytopenia was gradually developed on POD 54. She received steroid pulse therapy, and thrombocytopenia did not progress. However, pneumonia, renal failure, and cardiac failure occurred. She died due to multiple organ failure. We must consider GVHD in renal transplant recipients without homozygous or identical HLA, who had only watery diarrhea without other typical GVHD symptoms such as skin rash and fever, although GVHD is rare in renal transplant recipients.
Graft-versus-host disease; Human leukocyte antigen; Kidney donation; Kidney transplantation
Pneumatosis intestinalis is an uncommon disorder characterized by an accumulation of gas in the bowel wall. We described three cases undertaking liver transplantation. The patients developed diarrhea in three cases and high fever in two. An abdominal X-ray and computed tomography scan demonstrated extensive pneumatosis intestinalis in the colon with pneumoperitoneum mimicking hollow organ perforation. However, the patients had no abdominal symptoms and there was no evidence of peritonitis. The infection work-up was negative except one case with cytomegalovirus antigenemia. After one week of conservative management including bowel rest and antibiotic therapy, their pneumoperitoneum resolved spontaneously without any complication. Pneumatosis intestinalis should be considered as a differential diagnosis after adult liver transplantation with patients suffering from watery diarrhea and fever. Pneumoperitoneum, air-density in mesentery and retroperitoneum in patients with pneumatosis intestinalis without signs of peritonitis improved with conservative management, which included bowel rest and antibiotic therapy.
Liver transplantation; Pneumatosis intestinalis; Watery diarrhea
A 50-year-old male, renal transplant recipient, was admitted with fever and chest discomfort. At admission, chest radiologic finding was negative and echocardiography showed minimal pericardial effusion. After 2 days of admission, chest pain worsened and blood pressure fell to 60/40 mmHg. Emergency echocardiography showed a large amount of pericardial effusion compressing the entire heart. Pericardiocentesis was performed immediately. Mycobacterium tuberculosis was isolated from pericardial fluid. Tuberculosis pericarditis should be considered as the cause of cardiac tamponade in renal transplant recipients, even with the absence of pericardial effusion in the initial study or suggestive history.
Pericardiac tamponade; Renal transplantation; Tuberculosis; Tuberculosis pericarditis
This study was undertaken to evaluate the long-term treatment of esophageal strictures in children with corrosive esophagitis and to determine the effect of self-bougienage on recurrent strictures.
Materials and Methods
We reviewed the medical records of nine children that were treated for corrosive esophageal strictures from May 2000 to May 2008. Six males and three females were included and their average age was 30 months. Six patients had ingested acids, two patents had ingested alkali, and one ingested an unknown agent.
The interval between caustic ingestion and esophageal stricture ranged from one to eight weeks. The average length of the esophageal strictures was 3.8 cm (range, 1 to 9.2 cm). Four patients had a long segment stricture (longer than 5 cm) and one patient had multiple strictures. The most common site of involvement was the upper third followed by the mid third of the esophagus. Eight patients received repeated dilatation using a balloon catheter or bougie dilator. Among the eight patients, two patients had complete resolution of symptoms and six patients required surgery. Among five patients that developed restenosis of the esophageal anastomosis site, three patients had improved symptoms after self-bougienage and two patients had improved symptoms with repeated balloon dilatation or endoscopic bougienage. There were no complications in these patients.
Although a small number of patients were studied, self-bougienage was safe, less invasive, and effective for the management of esophageal restenosis in patients who required frequent dilation after surgery.
Corrosive esophageal stricture; restenosis; bougie dilatation; balloon dilatation; self-bougienage
We identified pediatric liver transplant recipients with successful withdrawal of immunosuppression who developed tolerance in Korea.
Materials and Methods
Among 105 pediatric patients who received liver transplantation and were treated with tacrolimus-based immunosuppressive regimens, we selected five (4.8%) patients who had very low tacrolimus trough levels. Four of them were noncompliant with their medication and one was weaned off of immunosuppression due to life threatening posttransplant lymphoproliferative disorder. We reviewed the medical records with regard to the relationship of the donor-recipients, patient characteristics and prognosis, including liver histology, and compared our data with previous reports.
Four patients received the liver transplantation from a parent donor and one patient from a cadaver donor. A trial of withdrawal of the immunosuppressant was started a median of 45 months after transplantation (range, 14 months to 60 months), and the period of follow up after weaning from the immunosuppressant was a median of 32 months (range, 14 months to 82 months). None of the five patients had rejection episodes after withdrawal of the immunosuppression; they maintained normal graft function for longer than 3 years (median, 38 months; range, 4 to 53 months). The histological findings of two grafts 64 and 32 months after weaning-off of the medication showed no evidence of chronic rejection.
The favorable markers for successful withdrawal of immunosuppression were 1) long-term (> 3 years) stable graft function, 2) no rejection for longer than 1 year after withdrawal of immunosuppression, 3) non-immune mediated liver diseases, and 4) pediatric patients.
Pediatric liver transplantation; withdrawal of immunosuppression; tacrolimus
High-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) was applied to improve the prognosis of patients with high-risk stage 3 neuroblastoma. From January 1997 to December 2006, 28 patients were newly diagnosed as stage 3 neuroblastoma. Nine of 11 patients with N-myc amplification and 5 of 17 patients without N-myc amplification (poor response in 2 patients, persistent residual tumor in 2 and relapse in 1) underwent single or tandem HDCT/ASCR. Patients without high-risk features received conventional treatment modalities only. While 8 of 9 patients underwent single HDCT/ASCR and the remaining one patient underwent tandem HDCT/ASCR during the early study period, all 5 patients underwent tandem HDCT/ASCR during the late period. Toxicities associated with HDCT/ASCR were tolerable and there was no treatment-related mortality. While the tumor relapsed in two of eight patients in single HDCT/ASCR group, all six patients in tandem HDCT/ASCR group remained relapse free. The 5-yr event-free survival (EFS) from diagnosis, in patients with N-myc amplification, was 71.6±14.0%. In addition, 12 of 14 patients who underwent HDCT/ASCR remained event free resulting in an 85.1±9.7% 5-yr EFS after the first HDCT/ASCR. The present study demonstrates that HDCT/ASCR may improve the survival of patients with high-risk stage 3 neuroblastoma.
Neuroblastoma; High-dose Chemotherapy; Autologous Stem Cell Rescue; Prognosis; N-myc