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1.
Transplant Proc ; 51(5): 1605-1610, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31155201

RESUMEN

BACKGROUND: Surgery is a key factor in the treatment of hepatoblastoma, but choosing between an aggressive resection and liver transplant may be an extremely difficult task. The aim of this study was to describe the outcomes of patients with advanced hepatoblastoma: pretreatment extent of disease (PRETEXT)/post-treatment extent of disease (POST-TEXT) III and IV undergoing aggressive resections or living donor liver transplant in cases involving the entire liver. Based on this experience, a new protocol for the treatment of these patients was proposed. METHODS: A retrospective study included patients with advanced hepatoblastoma (POST-TEXT III and IV) who were referred for a liver transplant from 2010 to 2017. RESULTS: A total of 24 children were included: 13 (54.2%) were male, with a median age at diagnosis of 42 months (range, 15-120 months), and a history of prematurity was identified in 20.8% of the patients. Ten cases (41.7%) were staged as PRETEXT/POST-TEXT III, and 12 cases (50.0%) were staged as PRETEXT/POST-TEXT IV. Two patients were referred after posthepatectomy recurrence. Five patients underwent a liver transplant, with recurrence and death in 2 patients (40.0%) within a mean period of 6 months. In the extensive hepatectomy group, there was recurrence in 6 patients (31.6%), with disease-free outcomes and overall survival in 63.2% and 94.7% of patients, respectively. CONCLUSION: In cases of advanced hepatoblastoma, an extensive surgical approach is a valuable option. The fact that the team was fully prepared to proceed with living donor liver transplant allowed the surgeon to be more aggressive and to switch to transplantation when resection was not possible.


Asunto(s)
Hepatectomía/métodos , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado/métodos , Niño , Preescolar , Femenino , Hepatectomía/mortalidad , Hepatoblastoma/mortalidad , Humanos , Lactante , Neoplasias Hepáticas/mortalidad , Trasplante de Hígado/mortalidad , Masculino , Recurrencia Local de Neoplasia/epidemiología , Estudios Retrospectivos
2.
Transplant Proc ; 50(10): 3840-3844, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30385044

RESUMEN

OBJECTIVE: Ischemic preconditioning (IPC) was developed to diminish ischemia-reperfusion injury (IRI). There are two main ways of performing it: direct ischemic-preconditioning (DIP) and remote ischemic-preconditioning (RIP). The objectives of this study were to investigate local and systemic effects of DIP and RIP in liver IRI. METHODS: Thirty-two weaning rats (50-70 g body weight; 21 days old) were divided into 4 groups: control (C); ischemia followed by reperfusion (IR); DIP followed by ischemia and reperfusion; and RIP followed by ischemia and reperfusion. In the IR group, the vascular pedicles of medial and left lateral liver lobes were clamped for 60 minutes and then unclamped. In the DIP group, a 10-minute cycle of ischemia followed by a 10-minute reperfusion of the same lobes was performed before 60 minutes of ischemia. In the RIP group, three 5-minute cycles of clamping and unclamping of the femoral vessels were performed before liver ischemia. The animals were euthanized 24 hours after the surgical procedures. RESULTS: The serum levels of liver enzymes were significantly lower in the RIP group compared to the control and IR groups and to the DIP group. The scores of histologic hepatic lesions were significantly lower in RIP animals than those of IR animals (P = .002) and similar to the C group animals. The Bax/BCl-xl relation was lower in the DIP group than that in the RIP group (P = .045) and no differences were observed in histologic analyses of kidney, lung, intestine, and heart. CONCLUSION: In young animals, the beneficial effects of RIP are more evident than those of DIP.


Asunto(s)
Precondicionamiento Isquémico/métodos , Hígado/irrigación sanguínea , Daño por Reperfusión/prevención & control , Animales , Modelos Animales de Enfermedad , Hígado/patología , Masculino , Ratas , Ratas Wistar , Daño por Reperfusión/patología
3.
Transplant Proc ; 47(4): 1033-7, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-26036512

RESUMEN

BACKGROUND: Liver transplantation is an effective treatment for irreversible liver diseases. The incidence of hepatic artery thrombosis remains high. Our objective was to analyze the effect of ligature of the hepatic artery on liver regeneration in a growing animal model. METHODS: Seventy-five male Wistar rats were divided into the following 3 groups: group 1 (sham, G1): incision without intervention; group 2 (G2): 70% hepatectomy; group 3 (G3): 70% hepatectomy and ligation of the hepatic artery. Preceding the 70% hepatectomy, a hepatic artery ligature was performed with resection of a segment of the artery. The liver specimens were stained with hematoxylin-eosin, and immunohistochemical staining for Ki-67 was performed. The expression of the interleukin (IL) 6 gene was studied by means of reverse-transcription polymerase chain reaction. RESULTS: G2 and G3 demonstrated similar tendencies toward an increase in the gain weight ratio over time. The mitotic activity was significantly lower at 72 hours in G3 than in G2. There was no difference between Ki-67 staining between G2 and G3. The expression of the IL-6 gene was present in all of the groups, lower in G1, with no difference between G2 and G3. CONCLUSIONS: The experimental model was feasible and adequate for these investigations. Hepatectomy stimulated hepatocyte proliferation, and the obstruction of the arterial flow did not affect liver regeneration.


Asunto(s)
Hepatectomía , Arteria Hepática/cirugía , Regeneración Hepática/fisiología , Trasplante de Hígado , Hígado/irrigación sanguínea , Procedimientos Quirúrgicos Vasculares/métodos , Animales , Proliferación Celular , Modelos Animales de Enfermedad , Interleucina-6/metabolismo , Ligadura , Hígado/metabolismo , Hígado/patología , Masculino , Ratas , Ratas Wistar
4.
Braz. j. med. biol. res ; 47(10): 850-857, 10/2014. tab, graf
Artículo en Inglés | LILACS | ID: lil-722172

RESUMEN

We previously described a selective bile duct ligation model to elucidate the process of hepatic fibrogenesis in children with biliary atresia or intrahepatic biliary stenosis. Using this model, we identified changes in the expression of alpha smooth muscle actin (α-SMA) both in the obstructed parenchyma and in the hepatic parenchyma adjacent to the obstruction. However, the expression profiles of desmin and TGF-β1, molecules known to be involved in hepatic fibrogenesis, were unchanged when analyzed by semiquantitative polymerase chain reaction (RT-PCR). Thus, the molecular mechanisms involved in the modulation of liver fibrosis in this experimental model are not fully understood. This study aimed to evaluate the molecular changes in an experimental model of selective bile duct ligation and to compare the gene expression changes observed in RT-PCR and in real-time quantitative PCR (qRT‐PCR). Twenty-eight Wistar rats of both sexes and weaning age (21-23 days old) were used. The rats were separated into groups that were assessed 7 or 60 days after selective biliary duct ligation. The expression of desmin, α-SMA and TGF-β1 was examined in tissue from hepatic parenchyma with biliary obstruction (BO) and in hepatic parenchyma without biliary obstruction (WBO), using RT-PCR and qRT‐PCR. The results obtained in this study using these two methods were significantly different. The BO parenchyma had a more severe fibrogenic reaction, with increased α-SMA and TGF-β1 expression after 7 days. The WBO parenchyma presented a later, fibrotic response, with increased desmin expression 7 days after surgery and increased α-SMA 60 days after surgery. The qRT‐PCR technique was more sensitive to expression changes than the semiquantitative method.


Asunto(s)
Animales , Femenino , Masculino , Actinas/metabolismo , Colestasis/complicaciones , Desmina/metabolismo , Cirrosis Hepática/etiología , Hígado/metabolismo , Reacción en Cadena en Tiempo Real de la Polimerasa/métodos , Factor de Crecimiento Transformador beta1/metabolismo , Análisis de Varianza , Actinas/genética , Atresia Biliar , Conductos Biliares/cirugía , Colágeno Tipo I/biosíntesis , Modelos Animales de Enfermedad , Desmina/genética , Expresión Génica , Ligadura , Cirrosis Hepática/metabolismo , Hígado/cirugía , Ratas Wistar , Factor de Crecimiento Transformador beta1/genética
5.
Braz J Med Biol Res ; 47(10): 850-7, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25140817

RESUMEN

We previously described a selective bile duct ligation model to elucidate the process of hepatic fibrogenesis in children with biliary atresia or intrahepatic biliary stenosis. Using this model, we identified changes in the expression of alpha smooth muscle actin (α-SMA) both in the obstructed parenchyma and in the hepatic parenchyma adjacent to the obstruction. However, the expression profiles of desmin and TGF-ß1, molecules known to be involved in hepatic fibrogenesis, were unchanged when analyzed by semiquantitative polymerase chain reaction (RT-PCR). Thus, the molecular mechanisms involved in the modulation of liver fibrosis in this experimental model are not fully understood. This study aimed to evaluate the molecular changes in an experimental model of selective bile duct ligation and to compare the gene expression changes observed in RT-PCR and in real-time quantitative PCR (qRT-PCR). Twenty-eight Wistar rats of both sexes and weaning age (21-23 days old) were used. The rats were separated into groups that were assessed 7 or 60 days after selective biliary duct ligation. The expression of desmin, α-SMA and TGF-ß1 was examined in tissue from hepatic parenchyma with biliary obstruction (BO) and in hepatic parenchyma without biliary obstruction (WBO), using RT-PCR and qRT-PCR. The results obtained in this study using these two methods were significantly different. The BO parenchyma had a more severe fibrogenic reaction, with increased α-SMA and TGF-ß1 expression after 7 days. The WBO parenchyma presented a later, fibrotic response, with increased desmin expression 7 days after surgery and increased α-SMA 60 days after surgery. The qRT-PCR technique was more sensitive to expression changes than the semiquantitative method.


Asunto(s)
Actinas/metabolismo , Colestasis/complicaciones , Desmina/metabolismo , Cirrosis Hepática/etiología , Hígado/metabolismo , Reacción en Cadena en Tiempo Real de la Polimerasa/métodos , Factor de Crecimiento Transformador beta1/metabolismo , Actinas/genética , Análisis de Varianza , Animales , Conductos Biliares/cirugía , Atresia Biliar , Colágeno Tipo I/biosíntesis , Desmina/genética , Modelos Animales de Enfermedad , Femenino , Expresión Génica , Ligadura , Hígado/cirugía , Cirrosis Hepática/metabolismo , Masculino , Ratas Wistar , Factor de Crecimiento Transformador beta1/genética
6.
Ultrasound Obstet Gynecol ; 42(1): 70-6, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23349059

RESUMEN

OBJECTIVE: To evaluate the effect of early fetoscopic tracheal occlusion (FETO) (22-24 weeks' gestation) on pulmonary response and neonatal survival in cases of extremely severe isolated congenital diaphragmatic hernia (CDH). METHODS: This was a multicenter study involving fetuses with extremely severe CDH (lung-to-head ratio < 0.70, liver herniation into the thoracic cavity and no other detectable anomalies). Between August 2010 and December 2011, eight fetuses underwent early FETO. Data were compared with nine fetuses that underwent standard FETO and 10 without fetoscopic procedure from January 2006 to July 2010. FETO was performed under maternal epidural anesthesia, supplemented with fetal intramuscular anesthesia. Fetal lung size and vascularity were evaluated by ultrasound before and every 2 weeks after FETO. Postnatal therapy was equivalent for both treated fetuses and controls. Primary outcome was infant survival to 180 days and secondary outcome was fetal pulmonary response. RESULTS: Maternal and fetal demographic characteristics and obstetric complications were similar in the three groups (P > 0.05). Infant survival rate was significantly higher in the early FETO group (62.5%) compared with the standard group (11.1%) and with controls (0%) (P < 0.01). Early FETO resulted in a significant improvement in fetal lung size and pulmonary vascularity when compared with standard FETO (P < 0.01). CONCLUSIONS: Early FETO may improve infant survival by further increases of lung size and pulmonary vascularity in cases with extremely severe pulmonary hypoplasia in isolated CDH. This study supports formal testing of the hypothesis with a randomized controlled trial.


Asunto(s)
Anomalías Múltiples/cirugía , Oclusión con Balón , Fetoscopía , Hernias Diafragmáticas Congénitas , Enfermedades Pulmonares/cirugía , Pulmón/anomalías , Anomalías Múltiples/metabolismo , Anomalías Múltiples/mortalidad , Anomalías Múltiples/fisiopatología , Oclusión con Balón/métodos , Femenino , Fetoscopía/métodos , Fetoscopía/mortalidad , Edad Gestacional , Hernia Diafragmática/metabolismo , Hernia Diafragmática/mortalidad , Hernia Diafragmática/fisiopatología , Hernia Diafragmática/cirugía , Humanos , Pulmón/metabolismo , Pulmón/fisiopatología , Pulmón/cirugía , Enfermedades Pulmonares/metabolismo , Enfermedades Pulmonares/mortalidad , Enfermedades Pulmonares/fisiopatología , Procedimientos Quirúrgicos Mínimamente Invasivos , Embarazo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Tráquea/embriología , Tráquea/fisiopatología , Resultado del Tratamiento , Ultrasonografía Doppler en Color , Ultrasonografía Prenatal/métodos
8.
Dis Esophagus ; 26(5): 475-8, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22816994

RESUMEN

Esophagocoloplasty is a commonly performed procedure for esophageal replacement in children. Traditionally, mechanical bowel preparation (MBP) is performed before this operation. However, this practice has been questioned, initially in adults and now in children. The aim of this study was to evaluate the influence of MBP on esophagocoloplasty in a series of children. Data collected from 164 patients who underwent esophagocoloplasty in the Pediatric Surgery Division, University of São Paulo Medical School, from February 1978 to July 2011 were reviewed for postoperative complications. In 134 patients, at least one kind of MBP was performed before the surgery (PREP group). MBP was omitted in 30 patients (NO-PREP group). There was no statistical difference between the groups in the rates of evisceration, colocolic, or cologastric anastomotic dehiscence and death. However, in the NO-PREP group, the incidence of cervical leakage (6.6%) was significantly decreased in comparison with the classical PREP group (25.3%) (P= 0.03). The results of this study suggest that the omission of MBP has a positive impact on the incidence of postoperative complications in esophagocoloplasty.


Asunto(s)
Fuga Anastomótica/etiología , Colon/cirugía , Colon/trasplante , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Esófago/cirugía , Estómago/cirugía , Adulto , Anciano , Anastomosis Quirúrgica/efectos adversos , Niño , Procedimientos Quirúrgicos del Sistema Digestivo/efectos adversos , Atresia Esofágica/cirugía , Estenosis Esofágica/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
10.
Ultrasound Obstet Gynecol ; 39(1): 20-7, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-22170862

RESUMEN

OBJECTIVE: Severe pulmonary hypoplasia and pulmonary arterial hypertension are associated with reduced survival in congenital diaphragmatic hernia (CDH). We aimed to determine whether fetal endoscopic tracheal occlusion (FETO) improves survival in cases of severe isolated CDH. METHODS: Between May 2008 and July 2010, patients whose fetuses had severe isolated CDH (lung-to-head ratio < 1.0, liver herniation into the thoracic cavity and no other detectable anomalies) were assigned randomly to FETO or to no fetal intervention (controls). FETO was performed under maternal epidural anesthesia supplemented with fetal intramuscular anesthesia. Tracheal balloon placement was achieved with ultrasound guidance and fetoscopy between 26 and 30 weeks of gestation. All cases that underwent FETO were delivered by the EXIT procedure. Postnatal therapy was the same for both treated fetuses and controls. The primary outcome was survival to 6 months of age. Other maternal and neonatal outcomes were also evaluated. RESULTS: Twenty patients were enrolled randomly to FETO and 21 patients to standard postnatal management. The mean gestational age at randomization was similar in both groups (P = 0.83). Delivery occurred at 35.6 ± 2.4 weeks in the FETO group and at 37.4 ± 1.9 weeks in the controls (P < 0.01). In the intention-to-treat analysis, 10/20 (50.0%) infants in the FETO group survived, while 1/21 (4.8%) controls survived (relative risk (RR), 10.5 (95% CI, 1.5-74.7), P < 0.01). In the received-treatment analysis, 10/19 (52.6%) infants in the FETO group and 1/19 (5.3%) controls survived (RR, 10.0 (95% CI, 1.4-70.6) P < 0.01). CONCLUSION: FETO improves neonatal survival in cases with isolated severe CDH.


Asunto(s)
Oclusión con Balón/métodos , Fetoscopía/métodos , Hernias Diafragmáticas Congénitas , Tráquea/patología , Adolescente , Adulto , Brasil/epidemiología , Femenino , Edad Gestacional , Hernia Diafragmática/embriología , Hernia Diafragmática/mortalidad , Hernia Diafragmática/fisiopatología , Hernia Diafragmática/terapia , Humanos , Lactante , Masculino , Oportunidad Relativa , Embarazo , Tráquea/embriología , Tráquea/fisiopatología , Resultado del Tratamiento , Adulto Joven
12.
Transplant Proc ; 43(1): 161-4, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21335177

RESUMEN

OBJECTIVE: The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors. METHODS: We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010. RESULTS: Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis). CONCLUSIONS: Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio.


Asunto(s)
Familia , Trasplante de Hígado , Donadores Vivos , Adolescente , Adulto , Niño , Femenino , Humanos , Inmunosupresores/administración & dosificación , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
13.
Transplant Proc ; 43(1): 181-3, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21335182

RESUMEN

INTRODUCTION: Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures. OBJECTIVE: The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes. METHODS: We performed a retrospective analysis of patient charts and analysis of complications and survival. RESULTS: BA was the most common indication for OLT (207/409; 50.6%). The median age of subjects was 26 months (range, 7-192). Their median weight was 11 kg (range, 5-63) with 110 children (53.1%) weighing ≤10 kg. We performed 126 transplantations from cadaveric donors (60.8%) and 81 from living-related donors (LRD) (39.2%). Retransplantation was required for 31 recipients (14.9%), primarily due to hepatic artery thrombosis (HAT; 64.5%). Other complications included the following: portal vein thrombosis (PVT; 13.0%), biliary stenosis and/or fistula (22.2%), bowel perforation (7.0%), and posttransplantation lymphoproliferative disorder (PTLD; 5.3%). Among the cases of OLT for other causes, the median age of recipients was 81 months (range, 11-17 years), which was higher than that for children with BA. Retransplantation was required in 3.5% of these patients (P < .05), mostly due to HAT. The incidences of PVT, bowel perforation, and PTLD were significantly lower (P < .05). There was no significant difference between biliary complications in the 2 groups. The overall survival rates at 1 versus 5 years were 79.7% versus 68.1% for BA, and 81.2% versus 75.7% for other causes, respectively. CONCLUSIONS: Children who undergo OLT for BA are younger than those engrafted for other causes, displaying a higher risk of complications and retransplantations.


Asunto(s)
Atresia Biliar/cirugía , Trasplante de Hígado , Adolescente , Niño , Humanos , Estudios Retrospectivos
14.
Transplant Proc ; 43(1): 194-5, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21335186

RESUMEN

BACKGROUND/PURPOSE: Posttransplantation portal vein thrombosis (PVT) can have severe health consequences, and portal hypertension and other consequences of the long-term privation of portal inflow to the graft may be hazardous, especially in young children. The Rex shunt has been used successfully to treat PVT patients since 1998. In 2007, we started to perform this surgery in patients with idiopathic PVT and late posttransplantation PVT. Herein we have reported our experience with this technique in acute posttransplantation PVT. METHODS: Three patients of ages 12, 15, and 18 months underwent cadaveric (n = 1) or living donor (n = 2) orthotopic liver transplantation (OLT). All patients had biliary atresia with portal vein hypoplasia; they developed acute PVT on the first postoperative day. They underwent a mesenteric-portal surgical shunt (Rex shunt) using a left internal jugular vein autograft (n = 2) or cadaveric iliac vein graft (n = 1) on the first postoperative day. RESULTS: The 8-month follow-up has confirmed shunt patency by postoperative Doppler ultrasound. There have been no biliary complications to date. CONCLUSIONS: The mesenteric-portal shunt (Rex shunt) using an autograft of the left internal jugular or a cadaveric vein graft should be considered for children with acute PVT after OLT. These children usually have small portal veins; reanastomosis is often unsuccessful. In addition, this technique has the advantage to avoid manipulation of the hepatic hilum and biliary anastomosis. Although this study was based on a limited experience, we concluded that this technique is feasible, with great benefits to and low risks for these patients.


Asunto(s)
Atresia Biliar/cirugía , Trasplante de Hígado , Vena Porta/cirugía , Derivación Portosistémica Quirúrgica , Trombosis/cirugía , Enfermedad Aguda , Humanos , Lactante , Vena Porta/patología
15.
Dis Esophagus ; 24(1): 25-9, 2011 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-20545969

RESUMEN

Esophagocoloplasty and gastric transposition are two major methods for esophageal substitution in children with esophageal atresia, and there is broad agreement that these operations should not be performed before the children start walking. However, there are some reported advantages of performing such operations in the first months of life or in the neonatal period. In this study, we compared our experience with esophageal substitution procedures performed in walking children with esophageal atresia, with the outcomes of children who had the operation before the third month of life reported in the literature. The purpose of this study was to establish if we have to wait until the children start walking before indicating the esophageal replacement procedure. From February 1978 to October 2009, 129 children with esophageal atresia underwent esophageal replacement in our hospital (99 colonic interpositions and 30 gastric transpositions). The records of these patients were reviewed for data regarding demographics, complications (leaks, graft failures, strictures, and graft torsion), and mortality and compared with those reported in the two main articles on esophageal replacement in the neonatal period or in patients less than 3 months of age. The main complication of our casuistic was cervical anastomosis leakage, which sealed spontaneously in all except in four patients. One patient of the esophagocoloplasty group developed graft necrosis and three patients in the gastric transposition group had gastric outlet obstruction, secondary to axial torsion of the stomach placed in the retrosternal space. The long-term outcome of the patients in both groups was considered good to excellent in terms of normal weight gain, absence of dysphagia, and other gastrointestinal symptoms. The comparisons of the main complications and mortality rates in walking children with esophageal substitutions performed in the first months of life showed that the incidences of cervical anastomotic leaks and graft failures were similar, but mortality rate in the first few months of life was significantly greater than that observed in our group of patients (P= 0.001). Based on the comparison of our results with those of published series, we conclude that the recommendation of performing esophagocoloplasty or total gastric transposition in children with esophageal atresia after they start walking is still valid.


Asunto(s)
Fuga Anastomótica/sangre , Colon/trasplante , Atresia Esofágica/cirugía , Esofagoplastia/efectos adversos , Esofagoplastia/métodos , Esófago/cirugía , Estómago/cirugía , Trasplante Heterotópico/métodos , Factores de Edad , Anastomosis Quirúrgica , Fuga Anastomótica/etiología , Distribución de Chi-Cuadrado , Estenosis Esofágica/etiología , Esofagoplastia/mortalidad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reoperación , Estudios Retrospectivos , Anomalía Torsional/etiología , Caminata
16.
Transplant Proc ; 42(5): 1763-8, 2010 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-20620519

RESUMEN

Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication following solid organ transplantation that has been linked to Epstein-Barr virus (EBV) infection. The aim of this article was to describe a single-center experience with the multiplicity of clinical presentations of PTLD. Among 350 liver transplantations performed in 303 children, 13 survivor children displayed a histological diagnosis of PTLD (13/242 survivors; 5.4%). The age at diagnosis ranged from 12 to 258 months (median, 47), and the time from transplantation ranged from 1 to 84 months (median, 13). Ten of these children (76.9%) were EBV-naïve prior to transplantation. Fever was present in all cases. The clinical signs at presentation were anemia (92.3%), diarrhea and vomiting (69.2%), recurrent upper airway infections (38.4%), Waldeyer ring lymphoid tissue hypertrophy (23.0%), abdominal mass lesions (30.7%), massive cervical and mediastinal adenopathy (15.3%), or gastrointestinal and respiratory symptoms (30.7%). One child developed fulminant hepatic allograft failure secondary to graft involvement by PTLD. Polymorphic PTLD was diagnosed in 6 patients; 7 had the diagnosis of lymphoma. Treatment consisted of stopping immunosuppression as well as starting intravenous gancyclovir and anti-CD20 monoclonal antibody therapy. The mortality rate was 53.8%. The clinical presentation of PTLD varied from fever of unknown origin to fulminant hepatic failure. The other symptoms that may be linked to the diagnosis of PTLD are pancytopenia, tonsil and adenoid hypertrophy, cervical or mediastinal lymph node enlargement, as well as abdominal masses. Despite numerous advances, the optimal treatment approach for PTLD is not completely known and the mortality rate is still high.


Asunto(s)
Trasplante de Hígado/efectos adversos , Trastornos Linfoproliferativos/diagnóstico , Complicaciones Posoperatorias/patología , Atresia Biliar/cirugía , Niño , Preescolar , Neoplasias del Colon/patología , Ciclosporina/uso terapéutico , Quimioterapia Combinada , Infecciones por Virus de Epstein-Barr/epidemiología , Femenino , Herpesvirus Humano 4/aislamiento & purificación , Humanos , Inmunosupresores/uso terapéutico , Lactante , Trasplante de Hígado/inmunología , Ganglios Linfáticos/patología , Linfoma de Células B/patología , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/patología , Masculino , Prednisona/uso terapéutico , Estudios Retrospectivos , Sobrevivientes , Tacrolimus/uso terapéutico
17.
Transplant Proc ; 41(3): 901-3, 2009 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-19376384

RESUMEN

BACKGROUND AND AIMS: Liver transplantation (OLT) in children has seen significant improvements in recent years. Long-term immunosuppressive strategies have focused on avoiding the risks of long-term immunosuppression, particularly nephrotoxicity, de novo malignancy and late infections. Since its introduction in renal transplantation in 1999, sirolimus (SRL) has been used by an increasing number of liver transplant centers. The aim of this study was to review the experience using SRL in pediatric liver transplant recipients at a single center. METHODS: Between 1989 and 2006, 318 children underwent OLT including 13 who were converted to SRL therapy because of tacrolimus-related side effects. The indications were posttransplant lymphoproliferative disease (PTLD; n = 11), nephrotoxicity (n = 1), and de novo autoimmune hepatitis (n = 1). One patient with PTLD previously concurrently displayed chronic rejection. SRL dosages ranged between 0.4 and 5 mg/d. The median duration of follow-up was 18 months. RESULTS: PTLD recurred in 1 patient. There were no episodes of acute rejection. One child developed hyperlipidemia that resolved with diet and medication. CONCLUSIONS: Conversion from tacrolimus to SRL in selected pediatric liver transplant recipients is safe. Children with PTLD may benefit from immunosuppression with SRL after liver transplantation.


Asunto(s)
Trasplante de Hígado/inmunología , Sirolimus/uso terapéutico , Adolescente , Cadáver , Niño , Preescolar , Femenino , Humanos , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Lactante , Fallo Hepático/cirugía , Trasplante de Hígado/efectos adversos , Donadores Vivos , Trastornos Linfoproliferativos/etiología , Masculino , Complicaciones Posoperatorias/inmunología , Estudios Retrospectivos , Tacrolimus/efectos adversos , Donantes de Tejidos
18.
Transplant Proc ; 41(3): 955-6, 2009 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-19376396

RESUMEN

BACKGROUND AND PURPOSE: Late portal vein thrombosis (PVT) can be extremely well tolerated, although portal hypertension and other consequences of the long-term deprivation of portal inflow to the graft may be hazardous, especially in young children. Recently, the "Rex shunt" has been used successfully to treat these patients. We now report the initial experience with this novel technique. METHODS: A 3-year-old girl with PVT at 7 months after whole organ cadaveric liver transplant displayed portal hypertension with an episode of gastrointestinal bleeding, requiring a mesenteric-portal surgical shunt ("Rex shunt") using a left internal jugular vein autograft. RESULTS: Upon current follow-up of 6 months, postoperative Doppler ultrasound confirmed shunt patency. Endoscopic status was significantly improved after surgery with resolution of portal hypertension. There was no recurrence of bleeding. CONCLUSIONS: The mesenteric-portal shunt ("Rex shunt"), using a left internal jugular vein autograft, should be considered for children with late PVT after liver transplantation. Although this is an initial experience, we may conclude that this technique is feasible, with great potential benefits and low risks for these patients.


Asunto(s)
Hipertensión Portal/cirugía , Trasplante de Hígado/efectos adversos , Trombosis de la Vena/cirugía , Cadáver , Preescolar , Várices Esofágicas y Gástricas/etiología , Femenino , Estudios de Seguimiento , Humanos , Hipertensión Portal/etiología , Venas Yugulares/cirugía , Esplenomegalia/cirugía , Donantes de Tejidos , Trasplante Autólogo , Trombosis de la Vena/etiología
19.
Dis Esophagus ; 21(8): 746-50, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18847453

RESUMEN

Fundoplication has been commonly performed in neurologically impaired and normal children with complicated gastroesophageal reflux disease. The relationship between gastroesophageal reflux disease and respiratory diseases is still unclear. We aimed to compare results of open and laparoscopic procedures, as well as the impact of fundoplication over digestive and respiratory symptoms. From January 2000 to June 2007, 151 children underwent Nissen fundoplication. Data were prospectively collected regarding age at surgery, presence of neurologic handicap, symptoms related to reflux (digestive or respiratory, including recurrent lung infections and reactive airways disease), surgical approach, concomitant procedures, complications, and results. Mean age was 6 years and 9 months. Eighty-two children (54.3%) had neurological handicaps. The surgical approach was laparoscopy in 118 cases and laparotomy in 33. Dysphagia occurred in 23 patients submitted to laparoscopic and none to open procedure (P = 0.01). A total of 86.6% of patients with digestive symptoms had complete resolution or significant improvement of the problems after the surgery. A total of 62.2% of children with recurrent lung infections showed any reduction in the frequency of pneumonias. Only 45.2% of patients with reactive airway disease had any relief from bronchospasm episodes after fundoplication. The comparisons demonstrated that Nissen fundoplication was more effective for the resolution of digestive symptoms than to respiratory manifestations (P = 0.04). Open or laparoscopic fundoplication are safe procedures with acceptable complication indices and the results of the surgery are better for digestive than for respiratory symptoms.


Asunto(s)
Enfermedades del Sistema Digestivo/prevención & control , Fundoplicación , Reflujo Gastroesofágico/complicaciones , Reflujo Gastroesofágico/cirugía , Laparoscopía , Enfermedades Respiratorias/prevención & control , Adolescente , Niño , Preescolar , Enfermedades del Sistema Digestivo/etiología , Estudios de Seguimiento , Fundoplicación/efectos adversos , Fundoplicación/métodos , Humanos , Lactante , Laparoscopía/efectos adversos , Laparotomía/efectos adversos , Estudios Prospectivos , Enfermedades Respiratorias/etiología , Resultado del Tratamiento
20.
Dis Esophagus ; 21(1): 73-7, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18197943

RESUMEN

Whenever the surgeon uses the stomach as an esophageal substitute, either one of two techniques is generally performed: total gastric transposition or gastric tube esophagoplasty. No existing reports compare the complications associated with these two surgical procedures. The purpose of this study is to review the authors' experience with total gastric transposition and verify whether this technique is superior to gastric tube esophagoplasty in children by comparing the main complications with those reported in the publications of gastric tubes esophagoplasties in the English language literature published in the last 38 years. A total of 35 children underwent total gastric transposition according to the classical technique. Most of these patients (27, or 77.1%) had long gap esophageal atresia. The most frequently observed complications were compared to those reported in nine studies of gastric tube esophagoplasty comprising 184 patients. Mortality and graft failure rates were also compared. Seven patients (20.0%) presented with leaks, all of which closed spontaneously. Six children were reoperated, three experienced gastric outlet obstruction secondary to axial torsion of the stomach placed in the retrosternal space and the other three experienced delayed gastric emptying that required revision of the piloroplasty. There were two deaths (5.7%) and no graft failure. Strictures were observed in five patients (14.2%) and all of these were resolved with endoscopic dilatations. Six patients had diarrhea that spontaneously resolved. In the late follow-up period, all patients were on full feed and thriving well. The comparisons with gastric tube patients demonstrated that the total gastric transposition group presented with significantly less leaks and strictures (P = 0.0001 and 0.001, respectively). The incidence of death and graft failure was not statistically different. In conclusion, gastric transposition is as a simple technical procedure for esophageal replacement in children with satisfactory results, and is superior to gastric tube esophagoplasty.


Asunto(s)
Enfermedades del Esófago/cirugía , Esofagoplastia , Estómago/trasplante , Adolescente , Niño , Preescolar , Dilatación , Estenosis Esofágica/etiología , Vaciamiento Gástrico , Obstrucción de la Salida Gástrica/etiología , Humanos , Lactante , Complicaciones Intraoperatorias , Neumotórax/etiología , Complicaciones Posoperatorias , Reoperación , Estudios Retrospectivos
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