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1.
Article in English | WPRIM | ID: wpr-914645

ABSTRACT

Background and Objectives@#Mesenchymal stem cells (MSCs) elicit therapeutic effects against liver fibrosis in animal models. Human liver stem cells (HLSCs) are cells isolated from human liver tissue that have mesenchymal morphology and express MSC markers. HLSCs also possess intrahepatic stem cell properties. We introduce a rat model of liver fibrosis and trans-portal transplantation of HLSC to demonstrate alleviation of liver fibrosis. @*Methods@#and Results: Liver fibrosis was induced by intraperitoneal injection of Carbon tetrachloride (CCl 4 ). Sprague Dawley rats underwent simultaneous partial hepatectomy of the left hepatic lobe and HLSC transplantation via the portal vein. Gross appearance of the liver observed following CCl 4 injection showed cholestasis and surface nodularity. Sirius red staining revealed deposition of collagen fibers in the extracellular matrix (ECM). Following HLSC transplantation, human albumin secreting cells were detected by immunohistochemistry in liver specimens. Quantitative measurements of fibrosis area stained by Sirius red were compared between baseline and post-HLSC transplant (1×10 7 cells) following 10 weeks of CCl 4treatment liver specimens. Fibrosis area (p<0.05), serum markers of liver inflammation and fibrosis (AST, ALT levels and APRI, p<0.05) significantly decreased from baseline after HLSC transplantation. RNA expression in liver tissues revealed significant decrease in tissue inhibitor of matrix metalloproteinase 1 (TIMP1), TIMP2 expression and increase in hepatocyte growth factor expression following HLSC transplantation (p<0.05). @*Conclusions@#HLSC transplantation effectively reduced the area of liver fibrosis with increased expression of factors promoting ECM degradation. These findings suggest the potential therapeutic role of HLSCs in various liver diseases presenting with liver fibrosis.

2.
Article in English | WPRIM | ID: wpr-896955

ABSTRACT

Purpose@#The incidence of chronic kidney disease (CKD) has been increasing due to improved survival after liver transplantation (LT). Risk factors of kidney injury after LT, especially perioperative management factors, are potentially modifiable. We investigated the risk factors associated with progressive CKD for 10 years after LT. @*Methods@#This retrospective cohort study included 292 adult patients who underwent LT at a tertiary referral hospital between 2000 and 2008. Renal function was assessed by the e stimated glomerular filtration rate (eGFR) using the Modification of Diet in Renal Disease formula. The area under the curve of serial eGFR (AUCeGFR) was calculated for each patient to assess the trajectory of eGFR over the 10 years. Low AUCeGFR was considered progressive CKD. Linear regression analyses were performed to examine the associations between the variables and AUCeGFR. @*Results@#Multivariable analysis showed that older age (regression coefficient = -0.53, P < 0.001), diabetes mellitus (DM) (regression coefficient = -6.93, P = 0.007), preoperative proteinuria (regression coefficient = -16.11, P < 0.001), preoperative acute kidney injury (AKI) (regression coefficient = -14.35, P < 0.001), postoperative AKI (regression coefficient = -3.86, P = 0.007), and postoperative mean vasopressor score (regression coefficient = -0.45, P = 0.034) were independently associated with progressive CKD. @*Conclusion@#More careful renoprotective management is required in elderly LT patients with DM or preexisting proteinuria. Postoperative AKI and vasopressor dose may be potentially modifiable risk factors for progressive CKD.

3.
Article in English | WPRIM | ID: wpr-889251

ABSTRACT

Purpose@#The incidence of chronic kidney disease (CKD) has been increasing due to improved survival after liver transplantation (LT). Risk factors of kidney injury after LT, especially perioperative management factors, are potentially modifiable. We investigated the risk factors associated with progressive CKD for 10 years after LT. @*Methods@#This retrospective cohort study included 292 adult patients who underwent LT at a tertiary referral hospital between 2000 and 2008. Renal function was assessed by the e stimated glomerular filtration rate (eGFR) using the Modification of Diet in Renal Disease formula. The area under the curve of serial eGFR (AUCeGFR) was calculated for each patient to assess the trajectory of eGFR over the 10 years. Low AUCeGFR was considered progressive CKD. Linear regression analyses were performed to examine the associations between the variables and AUCeGFR. @*Results@#Multivariable analysis showed that older age (regression coefficient = -0.53, P < 0.001), diabetes mellitus (DM) (regression coefficient = -6.93, P = 0.007), preoperative proteinuria (regression coefficient = -16.11, P < 0.001), preoperative acute kidney injury (AKI) (regression coefficient = -14.35, P < 0.001), postoperative AKI (regression coefficient = -3.86, P = 0.007), and postoperative mean vasopressor score (regression coefficient = -0.45, P = 0.034) were independently associated with progressive CKD. @*Conclusion@#More careful renoprotective management is required in elderly LT patients with DM or preexisting proteinuria. Postoperative AKI and vasopressor dose may be potentially modifiable risk factors for progressive CKD.

4.
Article in English | WPRIM | ID: wpr-761496

ABSTRACT

Pediatric liver transplantation has evolved into an effective treatment for a variety of liver diseases in the pediatric population. Over the past 25 years, pediatric liver transplantation results in Korea have matched international standards, and Korea has become one of the most important leaders in living donor liver transplantation. This review presents the cumulative outcomes of pediatric liver transplants in Korea and highlights other concerns related to pediatric liver transplantation, particularly pediatric liver allocation policy and split liver transplantation.


Subject(s)
Humans , Korea , Liver Diseases , Liver Transplantation , Liver , Living Donors
5.
Article in English | WPRIM | ID: wpr-717842

ABSTRACT

PURPOSE: This study aimed to report intraoperative abortion of adult living donor liver transplantation (LDLT). METHODS: From June 1997 to December 2016, 1,179 adult LDLT cases were performed. 15 cases (1.3%) of intraoperative abortions in LDLT were described. RESULTS: Among 15 cases, 5 intraoperative abortions were donor-related, and remaining 10 cases were recipient-related. All donor-related abortions were due to unexpected steatohepatitis. Among remaining 10 recipient-related intraoperative abortions, unexpected extension of hepatocellular carcinoma was related in 5 cases. Two cases of intraoperative abortions were related to bowel inflammation, and 2 cases were associated with severe adhesion related to previous treatment. One recipient with severe pulmonary hypertension was also aborted. CONCLUSION: Complete prevention of aborted LDLT is still not feasible. In this regard, further efforts to minimize intraoperative abortion are required.


Subject(s)
Adult , Carcinoma, Hepatocellular , Fatty Liver , Humans , Hypertension, Pulmonary , Inflammation , Liver Transplantation , Liver , Living Donors , Postoperative Care
6.
Article in English | WPRIM | ID: wpr-717796

ABSTRACT

Glycogen storage disease (GSD) IV is a rare autosomal recessive inherited disorder caused by mutations in the gene coding for glycogen branching enzyme leading to progressive liver disease. GSD IV is associated with mutations in GBE1, which encodes the glycogen branching enzyme. We report a case of GSD IV with rare homozygous mutations in the GBE1 gene (c.791G>A (p.Gly264Glu), which was successfully treated by liver transplantation.


Subject(s)
1,4-alpha-Glucan Branching Enzyme , Clinical Coding , Glycogen Storage Disease Type IV , Glycogen Storage Disease , Glycogen , Liver Diseases , Liver Transplantation
7.
Article in English | WPRIM | ID: wpr-715668

ABSTRACT

PURPOSE: Whereas continuous renal replacement therapy (CRRT) has been utilized during liver transplantation (LT), there was a lack of evidence to support this practice. We investigated the adverse events at the perioperative periods in recipients of LT who received preoperative CRRT without intraoperative CRRT. METHODS: We retrospectively reviewed medical records of adult patients (age ≥ 18 years) who received LT between December 2009 and May 2015. Perioperative data were collected from the recipients, who received preoperative CRRT until immediately before LT, because of refractory renal dysfunction. RESULTS: Of 706 recipients, 42 recipients received preoperative CRRT. The mean (standard deviation) Model for end-stage liver disease score were 49.6 (13.4). Twenty-six point two percent (26.2%) of recipients experienced the serum potassium > 4.5 mEq/L before reperfusion and treated with regular insulin. Thirty-eight point one percent (38.1%) of recipients were managed with sodium bicarbonate because of acidosis (base excess 5.5 mEq/L), refractory acidosis, or critical arrhythmias. Mortality was 19% at 30 day and 33.3% at 1 year. CONCLUSION: Although intraoperative CRRT was not used in recipients with severe preoperative renal dysfunction, LT was safely performed. Our experience raises a question about the need for intraoperative CRRT.


Subject(s)
Acidosis , Adult , Arrhythmias, Cardiac , Humans , Hyperkalemia , Insulin , Liver Diseases , Liver Transplantation , Liver , Medical Records , Mortality , Perioperative Period , Potassium , Renal Replacement Therapy , Reperfusion , Retrospective Studies , Sodium Bicarbonate , Transplant Recipients
8.
Article in English | WPRIM | ID: wpr-719202

ABSTRACT

PURPOSE: The purpose of this study was to describe the long-term effects of stenting in patients with hepatic venous outflow obstruction (HVOO), who underwent living donor liver transplantation (LDLT). METHODS: Between January 2000 and December 2009, 622 adult patients underwent LDLT at our hospital, and of these patients, 21 (3.3%) were diagnosed with HVOO; among these patients, 17 underwent stenting. The patients were divided into early or late groups according to the time of their HVOO diagnoses (cutoff: 60 days after liver transplantation). RESULTS: The median follow-up period was 54.2 months (range, 0.5–192.4 months). Stent insertion was successful in 8 of 10 patients in the early group and 6 of 7 in the late group. The 5-year primary patency rates were 46% and 20%, respectively. In both groups, patients with recurrent HVOO at the beginning showed kinking confirmed by venography. Patients who carried their stents for more than 3 years maintained long-term patency. There was no significant difference in spleen size between groups; however, when the groups were compared according to whether they maintained patency, spleens tended to be smaller in the patency-maintained group. CONCLUSION: Unlike stenosis, if kinking is confirmed on venography, stenting is not feasible in the long term for patients with LDLT.


Subject(s)
Adult , Budd-Chiari Syndrome , Constriction, Pathologic , Diagnosis , Follow-Up Studies , Hepatic Veins , Humans , Liver Transplantation , Liver , Living Donors , Phlebography , Spleen , Stents
9.
Article in English | WPRIM | ID: wpr-713268

ABSTRACT

PURPOSE: Infection remains the main cause of morbidity and mortality in liver transplantation (LT) recipients; however infection is notoriously difficult to diagnose because its usual signs and symptoms of infection may be masked or absent. This study comprises an analysis of bacterial infections in the early period after LT. METHODS: This is a study of 129 adults who underwent LT from January 2013 to December 2013, and it includes patients who were followed daily from the day of transplantation to 1-week posttransplantation using bacteriological cultures of blood, urine, sputum, and drained ascites. RESULTS: The following factors were significantly different between the positive and negative culture groups: living donor LT vs. deceased donor LT (odds ratio [OR], 3.269; P = 0.003), model for end-stage liver disease score (OR, 4.364; P < 0.001), and Child-Pugh classification (P = 0.007). Neither positive culture nor negative culture was associated with infection within 4 weeks of surgery (P = 0.03), and most events were due to surgical complications (75%). CONCLUSION: Since the full effect of immunosuppression is not yet present during the first month after LT, we suggest that the number of bacterial culture test could be reduced such that they are performed every other day depending on patient's situation.


Subject(s)
Adult , Ascites , Bacterial Infections , Classification , Culture Techniques , Humans , Immunosuppression Therapy , Liver Diseases , Liver Transplantation , Liver , Living Donors , Masks , Mortality , Sputum , Tissue Donors
10.
Article in English | WPRIM | ID: wpr-226732

ABSTRACT

Encapsulating peritoneal sclerosis (EPS) is a rare cause of intestinal obstruction by a thick fibrous membrane wrapping around the small intestine. It is a possible complication after liver transplantation (LT) that can be fatal. This report describes 2 cases of EPS after LT that were successfully treated with surgery, corticosteroids, tamoxifen, and mammalian target of rapamycin inhibitor. After treatment in both cases, the patients were able to start oral feeding and have been symptom free for more than 1 year. These cases suggests that for the management of EPS, surgical treatment is mandatory when the patients present with symptoms of intestinal obstruction or if there are findings suggestive of decreased mural perfusion. Surgery should be accompanied with medical treatment to prevent the relapse of EPS.


Subject(s)
Adrenal Cortex Hormones , Humans , Intestinal Obstruction , Intestine, Small , Liver Transplantation , Liver , Membranes , Perfusion , Peritoneal Fibrosis , Recurrence , Sirolimus , Tamoxifen , Transplant Recipients
11.
Article in English | WPRIM | ID: wpr-125183

ABSTRACT

PURPOSE: Hepatocellular carcinoma (HCC) is a rarely occurring disease in the pediatric population. We report our center's experience of management of HCC in children and adolescents. METHODS: From 1996 to 2012, 16 patients aged 18 or younger were diagnosed with HCC at our center. The medical records of these 16 patients were retrospectively reviewed. RESULTS: There were 9 boys and 7 girls. Median age at diagnosis of HCC was 14.5 years. All patient had pathologically confirmed diagnosis of HCC. Three patients had distant metastasis at the time of HCC diagnosis. Eight patients were surgically managed, including 4 liver resections, 3 liver transplantations, and 1 intraoperative radiofrequency ablation. The remaining 8 patients received systemic chemotherapy. Overall, 6 patients are alive at median 63.6 months after diagnosis of HCC. All survivors were surgically managed patients. CONCLUSION: HCC is a rare disease occurring in childhood. Patients with systemic disease have poor outcome. Liver transplantation may be a good option for treatment of pediatric HCC.


Subject(s)
Adolescent , Carcinoma, Hepatocellular , Catheter Ablation , Child , Diagnosis , Drug Therapy , Female , Humans , Liver , Liver Transplantation , Medical Records , Neoplasm Metastasis , Pediatrics , Rare Diseases , Retrospective Studies , Survivors
12.
Article in English | WPRIM | ID: wpr-134101

ABSTRACT

PURPOSE: There is no consensus regarding the difference in outcomes of HCV in patients who receive living donor liver transplantation (LDLT) or compared to deceased donor liver transplantation (DDLT). The aims of this study were to compare characteristics between LDLT and DDLT groups and to identify risk factors affecting patient survival. METHODS: We retrospectively reviewed the multicenter records of 192 HCV RNA-positive patients who underwent liver transplantation. RESULTS: Thirty-five patients underwent DDLT, and 146 underwent LDLT. The 1-, 3-, and 5-year patient survival rates were 66.7%, 63.0%, and 63.0% in the DDLT group and 86.1%, 82.3%, and 79.5% in the LDLT group (P = 0.024), respectively. After propensity matching, the patient survival curve of the LDLT group was higher than that of the DDLT group. However, there was no statistically significant difference in patient survival between the 2 groups (P = 0.061). Recipient age ≥ 60 years, LDLT, and use of tacrolimus were positively associated with patient survival in multivariate analyses. CONCLUSION: LDLT appears to be suitable for HCV-infected patients if appropriate living donor is available.


Subject(s)
Case-Control Studies , Consensus , Hepacivirus , Hepatitis C , Hepatitis , Humans , Immunosuppression Therapy , Liver Transplantation , Liver , Living Donors , Multivariate Analysis , Propensity Score , Retrospective Studies , Risk Factors , Survival Rate , Tacrolimus , Tissue Donors
13.
Article in English | WPRIM | ID: wpr-134100

ABSTRACT

PURPOSE: There is no consensus regarding the difference in outcomes of HCV in patients who receive living donor liver transplantation (LDLT) or compared to deceased donor liver transplantation (DDLT). The aims of this study were to compare characteristics between LDLT and DDLT groups and to identify risk factors affecting patient survival. METHODS: We retrospectively reviewed the multicenter records of 192 HCV RNA-positive patients who underwent liver transplantation. RESULTS: Thirty-five patients underwent DDLT, and 146 underwent LDLT. The 1-, 3-, and 5-year patient survival rates were 66.7%, 63.0%, and 63.0% in the DDLT group and 86.1%, 82.3%, and 79.5% in the LDLT group (P = 0.024), respectively. After propensity matching, the patient survival curve of the LDLT group was higher than that of the DDLT group. However, there was no statistically significant difference in patient survival between the 2 groups (P = 0.061). Recipient age ≥ 60 years, LDLT, and use of tacrolimus were positively associated with patient survival in multivariate analyses. CONCLUSION: LDLT appears to be suitable for HCV-infected patients if appropriate living donor is available.


Subject(s)
Case-Control Studies , Consensus , Hepacivirus , Hepatitis C , Hepatitis , Humans , Immunosuppression Therapy , Liver Transplantation , Liver , Living Donors , Multivariate Analysis , Propensity Score , Retrospective Studies , Risk Factors , Survival Rate , Tacrolimus , Tissue Donors
14.
Medical Principles and Practice. 2017; 26 (3): 221-228
in English | IMEMR | ID: emr-188527

ABSTRACT

Objectives: In this study, peripheral blood lymphocytes were compared between a brand-name and a generic tacrolimus group in stable liver transplant recipients


Subjects and Methods: Sixteen patients who underwent ABO-compatible living donor liver transplants between 2012 and 2013 and had stable graft function were included in this study. Ten patients received brand-name tacrolimus and 6 patients re-ceived generic tacrolimus. CDS, CD47 CDS, yd, CD4+FoxP3+, and CD3-CD56+ T cells were analyzed in peripheral blood obtained preoperatively and 4,8,12, and 24 weeks after liver transplantation. Categorical variables were compared using a x2 test or Fisher exact test, and continuous variables were compared using the Mann-Whitney U test


Results: Regarding the baseline and perioperative characteristics, there were no statistically significant differences between the 2 groups. Immunosuppression also was not different. Subtype analysis of T-cell populations carried out in parallel showed similar levels of CD3, CD4, CDS, and ydJ cells with brand-name tacrolimus and generic tacrolimus in stable liver transplant recipients. However, the levels of CD4+Foxp3+ and CD3-CD56+ T cells were higher in the brand-name tacrolimus group than in the generic tacrolimus group 8 weeks after transplantation [p < 0.05]


Conclusions: The level of CD4+Foxp3+ T cells was higher in the brand-name tacrolimus group than in the generic tacrolimus group after transplantation. This finding showed that brand-name tacrolimus could have more potential immunosuppressive activity than generic tacrolimus regarding the contribution of CD4+Foxp3+ T cells to graft tolerance in liver transplant recipients


Subject(s)
Humans , Female , Male , Adult , Middle Aged , Aged , Liver Transplantation , Drugs, Generic/therapeutic use , Lymphocytes/drug effects , Living Donors , ABO Blood-Group System , Statistics, Nonparametric
15.
Article in English | WPRIM | ID: wpr-188163

ABSTRACT

BACKGROUND/AIMS: 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. METHODS: We retrospectively reviewed the records of 169 HCV-RNA-positive patients who underwent LT at three centers. RESULTS: 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. CONCLUSION: 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.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Biopsy , Cyclosporine/therapeutic use , Drug Therapy, Combination , Genotype , Graft Rejection/mortality , Hepacivirus/genetics , Hepatitis C/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Liver Transplantation/adverse effects , Polymerase Chain Reaction , RNA, Viral/blood , Recombinant Fusion Proteins/therapeutic use , Recurrence , Retrospective Studies , Survival Rate , Tacrolimus/therapeutic use
16.
Article in English | WPRIM | ID: wpr-207931

ABSTRACT

We report a case of recurrent cardiac arrest during a nontransplant operation in a liver transplant recipient with prior cardiac arrest during liver transplantation. A 45-year-old man who experienced cardiac arrest for 17 minutes during the preanhepatic phase of liver transplantation–which was performed 34 months ago–did not survive the recurrent cardiac arrest during portal venoplasty. Variant angina was not suspected for the first cardiac arrest; however, myocardial infarction by coronary vasospasm was revealed to be the cause of the second cardiac arrest.


Subject(s)
Coronary Vasospasm , Heart Arrest , Humans , Liver Transplantation , Liver , Middle Aged , Myocardial Infarction , Transplant Recipients
17.
Article in English | WPRIM | ID: wpr-59525

ABSTRACT

PURPOSE: 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. METHODS: We retrospectively reviewed the multicenter records of 192 HCV RNA-positive patients who underwent LT. RESULTS: 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. CONCLUSION: Patent survival rates of HCV patients after LT in Korea were comparable with other countries.


Subject(s)
Cyclosporine , Genotype , Hepacivirus , Hospital Mortality , Humans , Immunosuppression Therapy , Korea , Liver Diseases , Liver Transplantation , Liver , Living Donors , Recurrence , Retrospective Studies , Survival Rate , Tacrolimus , Tissue Donors
18.
Article in Korean | WPRIM | ID: wpr-27977

ABSTRACT

PURPOSE: The congenital H-type rectovestibular fistula, a fistula between the anorectum and genital tract besides a normal anus is a rare variant of anorectal deformities. This disease needs proper treatment but there are no standard of diagnosis and treatment. The purpose of this report is to review a 13-year experience of approach and management for H-type rectovestibular fistula at a single institution. METHODS: From February 2002 to August 2015, we cared for 11 patients who had congenital H-type rectovestibular fistula and reviewed their clinical presentation, accompanied anomalies, diagnostic modalities, operative technique, and postoperative progress. RESULTS: Most patients with H-type rectovestibular fistula presented with symptoms including vestibular defecation and major labial abscess. We could find the fistula tract in most of patients by fistulography using contrast dye. All of the patients had been operated. There were 2 recurrences after surgical treatment who had inflammation and infection associated with the fistula. All other patients recovered without complications. CONCLUSION: We think the operation including fistulectomy and repair of perineal body through a transanal approach can be a feasible option to the congenital H-type rectovestibular fistula. Also, combined inflammation and infection should be treated prior to surgery to reduce postoperative complications.


Subject(s)
Abscess , Anal Canal , Congenital Abnormalities , Defecation , Diagnosis , Fistula , Humans , Inflammation , Postoperative Complications , Recurrence
19.
Article in English | WPRIM | ID: wpr-160916

ABSTRACT

PURPOSE: To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution. METHODS: 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. RESULTS: 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. CONCLUSION: 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.


Subject(s)
Adult , alpha-Fetoproteins , Child , Classification , Disease-Free Survival , Drug Therapy , Germ Cells , Gonads , Humans , Mediastinal Diseases , Mediastinum , Multivariate Analysis , Neoplasm Metastasis , Neoplasms, Germ Cell and Embryonal , Prognosis , Survival Rate
20.
Article in Korean | WPRIM | ID: wpr-87050

ABSTRACT

PURPOSE: Cervical lymphangiomas are rare lymphovascular malformations arising in the neck, which form huge fluid-containing cysts. Treatment of the malformation consists of surgery and sclerotherapy. However, the optimal approach is still controversial. Here, we describe a series of cervical lymphangiomas which have been treated with surgical approaches. METHODS: We retrospectively investigated the medical records of 82 patients who had been diagnosed with cervicofacial lymphangioma from 2001 to 2012 in our center. A closed suction drainage with negative pressure was placed on the operative lesion following excision to prevent reaccumulation of lymphatic fluid and the drainage tube was removed after injecting OK-432 through the tube. RESULTS: Twelve patients underwent surgical excision of cervical lymphangioma. The median patient age was 3 months at the time of the operation. The patients have been followed-up over a period of 34 months. When lesions were located near vital organs such as the trachea or carotid artery or did not respond to repetitive OK-432 injections, surgical treatment might bring good outcomes. However, swallowing difficulty, lip palsy, or dyslalia due to adjacent nerve damage temporarily appeared as postoperative complications. Five children had tracheostomy due to tracheal or subglottic stenosis and 2 patients had gastrostomy due to aspiration while they eat after surgery. CONCLUSION: Surgery for cervicofacial lymphangioma should be conducted carefully in selective cases. A well thought-out surgical plan with a multidisciplinary surgical team approach and placement of closed suction drainage tube after surgery and adjuvant OK-432 sclerotherapy through drainage tube seem to be helpful for good outcome.


Subject(s)
Carotid Arteries , Child , Constriction, Pathologic , Deglutition , Drainage , Gastrostomy , Humans , Lip , Lymphangioma , Medical Records , Neck , Paralysis , Picibanil , Postoperative Complications , Retrospective Studies , Sclerotherapy , Speech Disorders , Suction , Trachea , Tracheostomy
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