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1.
Pediatr Transplant ; 16(8): E352-5, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22574830

RESUMO

Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor-recipient serostatus, especially in relation to the Epstein-Barr virus. The patient was a two-yr-old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor. Nine months after transplantation, one of the adult recipients developed an urothelial tumor in the kidney graft. Imaging tests were repeated monthly in the liver-transplanted child and revealed no abnormalities. However, one yr and two months after the transplantation, the patient developed episodes of fever. At that time, imaging and liver biopsy showed a clear cell tumor of urothelial origin in the graft and the disease was limited to the liver. The patient underwent liver retransplantation, and she is currently free of tumor recurrence. Although rare, the occurrence of tumors in the post-transplant period from cadaver donors, without previously diagnosed tumors, is one of the many problems encountered in the complex world of organ transplantation.


Assuntos
Carcinoma de Células Renais/diagnóstico , Neoplasias Hepáticas/diagnóstico , Transplante de Fígado/efeitos adversos , Adulto , Atresia Biliar/terapia , Cadáver , Carcinoma de Células Renais/etiologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Neoplasias Hepáticas/etiologia , Transplante de Fígado/métodos , Reoperação , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
2.
Pediatr Transplant ; 16(8): E383-7, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22717006

RESUMO

CLN is a frequent histological finding in biopsies after pediatric: LT, and its pathogenesis has not yet been fully clarified and has different causes. Among the vascular causes, VOB is sometimes difficult to diagnose, especially when technical variants such as split-liver, reduced-liver, or living-related LT are utilized. Three liver-transplanted malnourished children (ages 12, 20, and 28 months) developed altered LFTs and post-operative ascites with right pleural effusion (two cases) and jaundice (one case). Doppler ultrasound examinations were normal and liver biopsies showed CLN interpreted as severe ACR. There were no responses to the medical treatment. Additional investigation with CT angiography suggested obstructed hepatic vein drainage, which was confirmed by interventional radiology and angioplasty of the anastomosis between the hepatic vein and the inferior vena cava, with clinical and histological resolution. It is concluded that in malnourished children undergoing LT with technical variations, in which the occurrence of severe ACR is usually less common because of the severity of the patient condition, the finding of CLN should raise the possibility of VOB, so that excessive immunosuppression and its consequences can be avoided.


Assuntos
Veias Hepáticas/patologia , Hepatopatias/diagnóstico , Desnutrição/complicações , Síndrome de Alagille/terapia , Anastomose Cirúrgica , Biópsia , Pré-Escolar , Feminino , Rejeição de Enxerto , Humanos , Lactente , Fígado/patologia , Transplante de Fígado/métodos , Doadores Vivos , Masculino , Necrose , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia Doppler/métodos , Veia Cava Inferior/patologia
3.
Pediatr Transplant ; 15(6): 617-27, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21884347

RESUMO

IRI is closely related to sepsis in ITx setting. Complete understanding of the mechanisms involved in IRI development may improve outcomes. Ortothopic ITx without immunosuppression was performed in order to characterize IRI-associated mucosal damage. Twenty pigs underwent ITx. Two groups were assigned to different CI times: G1: 90 min and, G2: 180 min. Euro-Collins was used as preservation solution. Jejunal fragments were collected at donor laparotomy, 30 min, and 3 days after reperfusion. IRI assessment involved: histopathologic analysis, quantification of MPO-positive cells through immunohistochemical studies, quantification of epithelial apoptotic cells using TUNEL staining, and quantification of IL-6, ET-1, Bak, and Bcl-XL genes expression by RT-PCR. Neutrophilic infiltration increased in a similar fashion in both groups, but lasted longer in G2. Apoptosis detected by TUNEL staining increased and anti-apoptotic gene Bcl-XL expression decreased significantly in G1, 3 days after surgery. Endothelin-1 and IL-6 genes expression increased 30 min after the procedure and returned to baseline 3 days after surgery. In conclusion, IL-6 and ET-1 are involved precociously in the development of intestinal IRI. Apoptosis was more frequently detected in G1 grafts by TUNEL-staining and by RT-PCR.


Assuntos
Apoptose , Endotelinas/metabolismo , Interleucina-6/metabolismo , Intestinos/transplante , Traumatismo por Reperfusão/patologia , Animais , Endotelina-1/biossíntese , Perfilação da Expressão Gênica , Regulação da Expressão Gênica , Imuno-Histoquímica/métodos , Isquemia/patologia , Neutrófilos/patologia , Suínos , Proteína Killer-Antagonista Homóloga a bcl-2/biossíntese , Proteína bcl-X/biossíntese
4.
Pediatr Transplant ; 15(2): 157-60, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21155957

RESUMO

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.


Assuntos
Calcineurina/efeitos adversos , Imunossupressores/efeitos adversos , Transplante de Fígado/efeitos adversos , Imageamento por Ressonância Magnética/métodos , Síndrome da Leucoencefalopatia Posterior/induzido quimicamente , Adolescente , Síndrome de Alagille , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Inibidores de Calcineurina , Criança , Ciclosporinas/efeitos adversos , Ciclosporinas/uso terapêutico , Feminino , Seguimentos , Humanos , Encefalopatia Hipertensiva/induzido quimicamente , Encefalopatia Hipertensiva/diagnóstico , Imunossupressores/uso terapêutico , Transplante de Fígado/métodos , Doadores Vivos , Masculino , Monitorização Fisiológica/métodos , Síndrome da Leucoencefalopatia Posterior/diagnóstico , Doenças Raras , Medição de Risco , Índice de Gravidade de Doença , Imunologia de Transplantes/fisiologia
5.
Pediatr Transplant ; 13(1): 81-7, 2009 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-18452497

RESUMO

BA is the most important disease requiring liver transplantation in children. Common BDL in rats is a classic experimental model to study biliary obstruction. The response of the neonatal animal to BDL has yet to be completely understood and few reports have focused on the behavioral differences of the liver between neonatal and adult animals. Ninety newborn Wistar rats aged six days, weighing 8.0-13.9 g, and 90 adult Wistar rats weighing 199.7-357.0 g, were submitted to BDL. After surgery, they were randomly divided and killed on the 3rd, 5th, 7th, 14th, 21st and 28th day post-BDL. Hepatic biopsies were obtained and the following were measured: (i) semiquantification of the bile ductule proliferation and inflammatory infiltrate by HE stain, (ii) quantification of portal and periportal fibrosis with the Sirius-red stain. Although the initial response of ductule proliferation and inflammatory infiltrate were less intense in the newborn animal, the portal and periportal fibrosis were higher when compared with adult animals (p < 0.0491). These findings may contribute to the understanding of the pathophysiology of BA.


Assuntos
Atresia Biliar/fisiopatologia , Modelos Animais de Doenças , Animais , Animais Recém-Nascidos , Ductos Biliares/cirurgia , Ducto Colédoco , Dilatação Patológica , Feminino , Fibrose , Ligadura , Masculino , Ratos , Ratos Wistar
6.
J Vasc Interv Radiol ; 19(10): 1439-48, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18760627

RESUMO

PURPOSE: To evaluate retrospectively the midterm and long-term results of percutaneous endovascular treatment of venous outflow obstruction after pediatric liver transplantation. MATERIALS AND METHODS: During a 9-year period, 18 children with obstruction of a hepatic vein (HV) or inferior vena cava (IVC) anastomosis underwent percutaneous transluminal angioplasty (PTA) with balloon dilation or stent placement in case of PTA failure after liver transplantation. Patients' body weights ranged from 7.7 kg to 42.6 kg (mean, 18.8 kg +/- 9). Potential predictors of patency were compared between balloon dilation and stent placement groups. RESULTS: Forty-two procedures were performed (range, 1-11 per patient; mean, 2). Technical and initial clinical success were achieved in all cases. Major complications included one case of pulmonary artery stent embolization and one case of hemothorax. Three children (25%) with HV obstruction were treated with PTA and nine (75%) were treated with stent placement. Three children with IVC obstruction (75%) were treated with PTA and one (25%) was treated with a stent. There were two children with simultaneous obstruction at the HV and IVC; one was treated with PTA and the other with a stent. Cases of isolated HV stenosis have a higher probability of patency with balloon-expandable stent treatment compared with balloon dilation (P < .05). Follow-up time ranged from 7 days to 9 years (mean, 42 months +/- 31), and the primary assisted patency rate was 100% when stent placement was performed among the first three procedures. CONCLUSIONS: In cases of venous outflow obstruction resulting from HV and/or IVC lesions after pediatric liver transplantation, percutaneous endovascular treatment with balloon dilation or stent placement is a safe and effective alternative treatment that results in long-term patency.


Assuntos
Angioplastia com Balão/métodos , Transplante de Fígado/efeitos adversos , Insuficiência Venosa/etiologia , Insuficiência Venosa/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Resultado do Tratamento
7.
Clinics (Sao Paulo) ; 73: e289, 2018 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-29995099

RESUMO

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.


Assuntos
Atresia Biliar/cirurgia , Portoenterostomia Hepática/métodos , Fatores Etários , Atresia Biliar/mortalidade , Atresia Biliar/patologia , Brasil/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Icterícia Neonatal/patologia , Icterícia Neonatal/cirurgia , Estimativa de Kaplan-Meier , Fígado/patologia , Fígado/cirurgia , Transplante de Fígado/métodos , Transplante de Fígado/mortalidade , Masculino , Portoenterostomia Hepática/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
8.
J Pediatr Surg ; 49(4): 525-7, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24726105

RESUMO

BACKGROUND/PURPOSE: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation. METHODS: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013. RESULTS: Over the last 14 years, 185 liver transplants were performed in pediatric recipients of grafts from living donors. Among the donors, 166 left lateral segments (89.7%), 18 left lobes without the caudate lobe (9.7%) and 1 right lobe (0.5%) were harvested. The donor age ranged from 16 to 53 years, and the weight ranged from 47 to 106 kg. In 10 donors, an additional graft of the donor inferior mesenteric vein was harvested to substitute for a hypoplastic recipient portal vein. The transfusion of blood products was required in 15 donors (8.1%). The mean hospital stay was 5 days. No deaths occurred, but complications were identified in 23 patients (12.4%): 9 patients experienced abdominal pain and severe gastrointestinal symptoms and 3 patients required reoperations. Eight donors presented with minor bile leaks that were treated conservatively, and 3 patients developed extra-peritoneal infections (1 wound collection, 1 phlebitis and 1 pneumonia). Eight grafts (4.3%) showed primary dysfunction resulting in recipient death (3 cases of fulminant hepatitis, 1 patient with metabolic disease, 1 patient with Alagille syndrome and 3 cases of biliary atresia in infants under 1 year old). There was no relation between donor complications and primary graft dysfunction (P=0.6). CONCLUSIONS: Living donor transplantation is safe for the donor and presents a low morbidity. The donor surgery may be performed by a team of trained pediatric surgeons.


Assuntos
Doença Hepática Terminal/cirurgia , Hepatectomia/métodos , Transplante de Fígado , Doadores Vivos , Pediatria , Cirurgiões , Coleta de Tecidos e Órgãos/métodos , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
9.
Clinics ; 73: e289, 2018. tab, graf
Artigo em Inglês | LILACS | ID: biblio-952809

RESUMO

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Atresia Biliar/cirurgia , Portoenterostomia Hepática/métodos , Fatores de Tempo , Atresia Biliar/mortalidade , Atresia Biliar/patologia , Brasil/epidemiologia , Portoenterostomia Hepática/mortalidade , Taxa de Sobrevida , Estudos Retrospectivos , Fatores Etários , Transplante de Fígado/métodos , Transplante de Fígado/mortalidade , Resultado do Tratamento , Estimativa de Kaplan-Meier , Icterícia Neonatal/cirurgia , Icterícia Neonatal/patologia , Fígado/cirurgia , Fígado/patologia
10.
J Pediatr Surg ; 46(1): 214-6, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21238670

RESUMO

BACKGROUND: Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients. METHODS: We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post-umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT. RESULTS: It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post-umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now. CONCLUSIONS: The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post-umbilical catheterization EHPVT is yet to be clearly evaluated.


Assuntos
Hipertensão Portal/cirurgia , Veia Porta/cirurgia , Derivação Portossistêmica Cirúrgica/métodos , Trombose Venosa/cirurgia , Adolescente , Cateterismo/efeitos adversos , Criança , Pré-Escolar , Feminino , Hemorragia Gastrointestinal/prevenção & controle , Hemorragia Gastrointestinal/cirurgia , Humanos , Hipertensão Portal/etiologia , Lactente , Veias Jugulares/transplante , Masculino , Veias Mesentéricas/cirurgia , Transplante Autólogo/métodos , Resultado do Tratamento , Trombose Venosa/complicações
11.
J Pediatr Surg ; 46(7): 1379-84, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21763838

RESUMO

BACKGROUND/PURPOSE: The introduction of the piggyback technique for reconstruction of the liver outflow in reduced-size liver transplants for pediatric patients has increased the incidence of hepatic venous outflow block (HVOB). Here, we proposed a new technique for hepatic venous reconstruction in pediatric living-donor liver transplantation. METHODS: Three techniques were used: direct anastomosis of the orifice of the donor hepatic veins and the orifice of the recipient hepatic veins (group 1); triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins (group 2); and a new technique, which is a wide longitudinal anastomosis performed at the anterior wall of the inferior vena cava (group 3). RESULTS: In groups 1 and 2, the incidences of HVOB were 27.7% and 5.7%, respectively. In group 3, no patient presented HVOB (P = .001). No difference was noted between groups 2 and 3. CONCLUSIONS: Hepatic venous reconstruction in pediatric living-donor liver transplantation must be preferentially performed by using a wide longitudinal incision at the anterior wall of the recipient inferior vena cava. As an alternative technique, triangulation of the recipient inferior vena cava, including the orifices of the 3 hepatic veins, may be used.


Assuntos
Veias Hepáticas/cirurgia , Hepatopatia Veno-Oclusiva/prevenção & controle , Transplante de Fígado/métodos , Doadores Vivos , Complicações Pós-Operatórias/prevenção & controle , Veia Cava Inferior/cirurgia , Adolescente , Adulto , Anastomose Cirúrgica/métodos , Criança , Pré-Escolar , Feminino , Hepatectomia/métodos , Hepatopatia Veno-Oclusiva/epidemiologia , Hepatopatia Veno-Oclusiva/etiologia , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Stents , Resultado do Tratamento , Adulto Jovem
12.
Cardiovasc Intervent Radiol ; 33(1): 90-6, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-19543942

RESUMO

The purpose of this study was to evaluate the mid- and long-term results of percutaneous transhepatic cholangiography (PTC) and biliary drainage in children with isolated bilioenteric anastomotic stenosis (BAS) after pediatric liver transplantation. Sixty-four children underwent PTC from March 1993 to May 2008. Nineteen cholangiograms were normal; 10 showed intrahepatic biliary stenosis and BAS, and 35 showed isolated BAS. Cadaveric grafts were used in 19 and living donor grafts in 16 patients. Four patients received a whole liver, and 31 patients received a left lobe or left lateral segment. Roux-en-Y hepaticojejunostomy was performed in all patients. Indication for PTC was based on clinical, laboratory, and histopathologic findings. In patients with isolated BAS, dilation and biliary catheter placement, with changes every 2 months, were performed. Patients were separated into 4 groups according to number of treatment sessions required. The drainage catheter was removed if cholangiogram showed no significant residual stenosis and normal biliary emptying time after a minimum of 6 months. The relationship between risk factors (recipient's weight <10 kg, previous exposure to Cytomegalovirus, donor-recipient sex and weight relations, autoimmune disease as indication for transplantion, previous Kasai's surgery, use of reduced liver grafts, chronic or acute rejection occurrence) and treatment was evaluated. Before PTC, fever was observed in 46%, biliary dilation in 23%, increased bilirubin in 57%, and increased gamma-glutamyltransferase (GGT) in 100% of patients. In the group with BAS, 24 of 35 (69%) patients had histopathologic findings of cholestasis as did 9 of 19 (47%) patients in the group with normal PTC. Of the 35 patients, 23 (65.7%) needed 1 (group I), 7 needed 2 (group II), 4 needed 3 (group III), and 1 needed 4 treatment sessions (group IV). The best results were observed after 1 treatment session, and the mean duration of catheter placement and replacement was 10 months. The primary patency rate was 61.2%, and the recurrence rate was 34.3% (group I). Seven patients (7 of 35; 20%) had their stricture treated with a second treatment session (group II). The average drainage time in group II was 24 months. During a period >20 months, 4 patients (4 of 35; 11.4%) required 1 additional treatment session (group III), and 1 patient (1 of 35; 2.9%) had a catheter placed at the end of the study period (group IV). Drainage time in group I was significantly shorter than those in groups II, III, and IV (p < 0.05). There was no statistically significant relation between therapeutic response and the selected risk factors (p > 0.05). The majority of complications, such as catheter displacement and leakage, were classified as minor; however, 2 patients (5.7%) with hemobilia were noted. Complications increased according to the need for reintervention. In conclusion, balloon dilation and percutaneous drainage placement is safe and effective, and it has long-term patency for children with BAS after liver transplantation. Because of prolonged treatment time, reintervention may be necessary, thereby increasing the complication rate. Balloon dilation and percutaneous drainage placement should be considered as the first treatment option because of its minimally invasive nature.


Assuntos
Doenças Biliares/etiologia , Doenças Biliares/terapia , Colangiografia/métodos , Drenagem/métodos , Transplante de Fígado/efeitos adversos , Adolescente , Anastomose Cirúrgica/efeitos adversos , Ductos Biliares Intra-Hepáticos , Doenças Biliares/diagnóstico , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Criança , Pré-Escolar , Constrição Patológica/etiologia , Constrição Patológica/terapia , Humanos , Lactente , Recém-Nascido , Radiologia Intervencionista
13.
J Pediatr Surg ; 44(10): 1877-83, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19853741

RESUMO

BACKGROUND: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up. METHOD: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction. RESULTS: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count. CONCLUSION: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence.


Assuntos
Endoscopia/métodos , Escleroterapia/métodos , Trombose Venosa/terapia , Adolescente , Criança , Varizes Esofágicas e Gástricas/cirurgia , Varizes Esofágicas e Gástricas/terapia , Esofagoscopia , Feminino , Seguimentos , Hemorragia Gastrointestinal/cirurgia , Humanos , Hiperesplenismo , Hipertensão Portal/terapia , Hepatopatias , Estudos Longitudinais , Veia Porta , Derivação Portossistêmica Cirúrgica , Recidiva , Estudos Retrospectivos , Derivação Esplenorrenal Cirúrgica , Resultado do Tratamento , Trombose Venosa/cirurgia
14.
J Pediatr Surg ; 44(11): 2083-7, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19944212

RESUMO

PURPOSE: Hepatectomy remains a complex operation even in experienced hands. The objective of the present study was to describe our experience in liver resections, in the light of liver transplantation, emphasizing the indications for surgery, surgical techniques, complications, and results. METHODS: The medical records of 53 children who underwent liver resection for primary or metastatic hepatic tumors were reviewed. Ultrasonography, computed tomographic (CT) scan, and needle biopsy were the initial methods used to diagnose malignant tumors. After neoadjuvant chemotherapy, tumor resectability was evaluated by another CT scan. Surgery was performed by surgeons competent in liver transplantation. As in liver living donor operation, vascular anomalies were investigated. The main arterial anomalies found were the right hepatic artery emerging from the superior mesenteric artery and left hepatic artery from left gastric artery. Hilar structures were dissected very close to liver parenchyma. The hepatic artery and portal vein were dissected and ligated near their entrance to the liver parenchyma to avoid damaging the hilar vessels of the other lobe. During dissection of the suprahepatic veins, the venous infusion was decreased to reduce central venous pressure and potential bleeding from hepatic veins and the vena cava. RESULTS: Fifty-three children with hepatic tumors underwent surgical treatment, 47 patients underwent liver resections, and in 6 cases, liver transplantation was performed because the tumor was considered unresectable. There were 31 cases of hepatoblastoma, with a 9.6% mortality rate. Ten children presented with other malignant tumors-3 undifferentiated sarcomas, 2 hepatocellular carcinomas, 2 fibrolamellar hepatocellular carcinomas, a rhabdomyosarcoma, an immature ovarian teratoma, and a single neuroblastoma. These cases had a 50% mortality rate. Six children had benign tumors-4 mesenchymal hamartoma, 1 focal nodular hyperplasia, and a mucinous cystadenoma. All of these children had a favorable outcome. Hepatic resections included 22 right lobectomies, 9 right trisegmentectomies, 8 left lobectomies, 5 left trisegmentectomies, 2 left segmentectomies, and 1 case of monosegment (segment IV) resection. The overall mortality rate was 14.9%, and all deaths were related to recurrence of malignant disease. The mortality rate of hepatoblastoma patients was less than other malignant tumors (P = .04). CONCLUSION: The resection of hepatic tumors in children requires expertise in pediatric surgical practice, and many lessons learned from liver transplantation can be applied to hepatectomies. The present series showed no mortality directly related to the surgery and a low complication rate.


Assuntos
Neoplasias Hepáticas/cirurgia , Transplante de Fígado/métodos , Fatores Etários , Perda Sanguínea Cirúrgica , Carcinoma Hepatocelular/mortalidade , Carcinoma Hepatocelular/cirurgia , Seguimentos , Hepatectomia/métodos , Hepatoblastoma/mortalidade , Hepatoblastoma/cirurgia , Humanos , Lactente , Neoplasias Hepáticas/mortalidade , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/cirurgia , Complicações Pós-Operatórias/etiologia , Taxa de Sobrevida , Tomografia Computadorizada por Raios X , Resultado do Tratamento
15.
Clinics (Sao Paulo) ; 63(5): 689-94, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18925331

RESUMO

BACKGROUND AND AIMS: Although there is much known about liver diseases, some aspects remain unclear, such as the nature of the differences between the diseases observed in newborn infants and those in adults. For example, how do newborns respond to duct epithelial cell injury? Do the stellate cells in newborns respond similarly to those in adults during biliary obstruction? METHODS: Ninety newborn Wistar rats aged six days, weighing 8.0 - 13.9 g each, and 90 adult rats weighing 199.7 - 357.0 g each, were submitted to bile duct ligation. After surgery, they were randomly divided and sacrificed on the 3rd, 5th, 7th, 14th, 21st or 28th day post-bile duct ligation. Hepatic biopsies were obtained and immunohistochemical semi-quantification of desmin and alpha-SMA expression was performed in hepatic stellate cells and in myofibroblasts in the portal space, and between the portal space and the liver lobule. RESULTS: Desmin expression in the myofibroblast cells post-bile duct ligation was higher in young rats, reaching its peak level in a shorter time when compared to the adult animals. The differences between the groups for alpha-SMA expression were less significant than for desmin. CONCLUSIONS: These findings indicate that there is an increase in the number of collagen-producing myofibroblast cells in young animals, suggesting that there is more intense fibrosis in this population. This finding may explain why young animals with bile duct obstruction experience more intense portal fibrosis that is similar to the pathology observed in the livers of newborns with biliary atresia.


Assuntos
Atresia Biliar/patologia , Colestase/patologia , Fibroblastos/patologia , Células Estreladas do Fígado/patologia , Sistema Porta/patologia , Actinas/análise , Fatores Etários , Animais , Animais Recém-Nascidos , Biomarcadores/análise , Desmina/análise , Modelos Animais de Doenças , Proteínas da Matriz Extracelular/análise , Feminino , Ligadura , Masculino , Ratos , Ratos Wistar
16.
Cardiovasc Intervent Radiol ; 31(6): 1244-8, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18756372

RESUMO

Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Derivação Portossistêmica Transjugular Intra-Hepática/métodos , Stents , Síndrome de Budd-Chiari/diagnóstico por imagem , Criança , Feminino , Humanos , Politetrafluoretileno , Portografia , Radiografia Intervencionista
17.
Pediatr Radiol ; 38(4): 403-8, 2008 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-18214459

RESUMO

BACKGROUND: Accurate diagnosis of portal vein (PV) stenosis by real-time and color Doppler US (CD-US) after segmental liver transplantation in children can decrease morbidity by avoiding unnecessary biopsy, PV hypertension, thrombosis and loss of the graft. OBJECTIVE: To evaluate CD-US parameters for the prediction of PV stenosis after segmental liver transplantation in children. MATERIALS AND METHODS: We retrospectively reviewed 61 CD-US examinations measuring the diameter at the PV anastomosis, velocities at the anastomosis (PV1) and in the segment proximal to the anastomosis (PV2), and the PV1/PV2 velocity ratio. The study group comprised patients with stenosis confirmed by angiography and the control group comprised patients with a good clinical outcome. RESULTS: PV stenosis was seen in 12 CD-US examinations. The mean PV diameter was smaller in the study group (2.6 mm versus 5.7 mm) and a PV diameter of <3.5 mm was highly predictive of stenosis (sensitivity 100%, specificity 91.8%). CONCLUSION: A PV diameter of <3.5 mm is a highly predictive CD-US parameter for the detection of hemodynamically significant stenosis on angiography.


Assuntos
Hepatopatia Veno-Oclusiva/diagnóstico por imagem , Transplante de Fígado , Veia Porta/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico por imagem , Ultrassonografia Doppler em Cores , Adolescente , Anastomose Cirúrgica , Angiografia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade
18.
Pediatr Radiol ; 38(4): 409-14, 2008 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-18214460

RESUMO

BACKGROUND: Accurate diagnosis of hepatic vein (HV) stenosis by real-time and color Doppler US (CD-US) after segmental liver transplantation in children can decrease morbidity because it allows unnecessary biopsy, obstruction or thrombosis and loss of the graft to be avoided. OBJECTIVE: To evaluate CD-US parameters to predict HV stenosis after segmental liver transplantation in children. MATERIALS AND METHODS: Retrospective review of 79 CD-US examinations measuring velocity at the HV anastomosis (HV1) and the main trunk 1-2 cm proximal to the HV/IVC anastomosis (HV2), the HV1/HV2 ratio and the spectral waveform of HV2. The study group comprised patients with stenosis confirmed by angiography. The control group comprised patients with a good clinical outcome. RESULTS: HV stenosis was seen in 12 CD-US examinations. The mean HV1/HV2 ratio was higher in the study group (6.0 versus 4.0). An HV1/HV2 ratio of >4.1 was predictive of HV stenosis (sensitivity 83%, specificity 76%). CONCLUSION: An HV1/HV2 ratio of >4.1 is a highly predictive CD-US parameter for the detection of hemodynamically significant HV stenosis on angiography.


Assuntos
Veias Hepáticas/diagnóstico por imagem , Hepatopatia Veno-Oclusiva/diagnóstico por imagem , Transplante de Fígado , Complicações Pós-Operatórias/diagnóstico por imagem , Ultrassonografia Doppler em Cores , Adolescente , Angiografia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade
19.
Pediatr Transplant ; 12(1): 91-4, 2008 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-18186894

RESUMO

HAT is the main cause of graft loss in pediatric living-related LTx. Revascularization of the graft by thrombectomy and re-anastomosis has been reported to be effective for graft salvage in cases of HAT and should be attempted when potential donors are not available for emergency re-transplantation. Immediate complications secondary to revascularization attempts in cases of HAT are not described. Late complications are mainly related to biliary tree ischemia. We report a case of child who experienced intimal hepatic artery dissection, which extended into intra-hepatic branches of the artery after a thrombectomy with a Fogarty balloon catheter in an attempt to restore arterial flow after HAT. This complication led to acute deterioration of the graft and the need for emergency re-transplantation.


Assuntos
Sobrevivência de Enxerto , Artéria Hepática/patologia , Transplante de Fígado/efeitos adversos , Trombose/cirurgia , Túnica Íntima/patologia , Atresia Biliar , Cateterismo , Criança , Humanos , Circulação Hepática , Transplante de Fígado/métodos , Doadores Vivos , Reoperação , Terapia de Salvação , Trombectomia , Trombose/etiologia
20.
Pediatr Transplant ; 11(1): 82-6, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17239128

RESUMO

Few studies have evaluated the long-term use of MMF in liver transplanted children with renal dysfunction. The aim of this study is to report the experience of a pediatric transplantation center on the efficacy and security of long-term use of a MMF immunosuppressant protocol with reduced doses of CNIs in stable liver transplanted children with renal dysfunction secondary to prolonged use of CsA or Tac. Between 1988 and 2003, 191 children underwent OLT and 11 patients developed renal dysfunction secondary to CNIs toxicity as evaluated by biochemical renal function parameters. The interval between liver transplantation and the introduction of the protocol varied from one to 12 yr. Renal function was evaluated by biochemical parameters in five phases: immediately prior to MMF administration; 3, 6, 12 and 24 months after the introduction of MMF. Among the patients, nine of them (82%) showed improvement of renal function parameters in comparison with the pretreatment values. The two patients that did not show any improvement were patients in whom the interval of time between OLT and the introduction of MMF was longer. All parameters of liver function remained unchanged. No episodes of acute or chronic rejection or increases in infection rates during the period were detected. Two patients developed transitory diarrhea and leukopenia that were reverted with reduction of MMF dosage. In conclusion, in liver transplanted pediatric patients with CNI-induced chronic renal dysfunction, the administration of MMF in addition to reduced doses of CNIs promotes long-term improvement in renal function parameters with no additional risks.


Assuntos
Nefropatias/tratamento farmacológico , Falência Hepática/cirurgia , Transplante de Fígado/imunologia , Ácido Micofenólico/análogos & derivados , Complicações Pós-Operatórias/tratamento farmacológico , Adolescente , Nitrogênio da Ureia Sanguínea , Criança , Pré-Escolar , Creatinina/sangue , Creatinina/metabolismo , Ciclosporina/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Testes de Função Renal , Masculino , Ácido Micofenólico/uso terapêutico , Estudos Retrospectivos , Tacrolimo/uso terapêutico , Ácido Úrico/sangue , Vacinação
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