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1.
J Pediatr Surg ; 2024 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-38413259

RESUMO

BACKGROUND: Currently, graft options for pediatric liver transplantation (PLT) include whole (WL) and partial (P) grafts, in the form of either deceased donor transplantation (DD) or living donor liver transplantation (LD). WL transplants from LD are commonly referred to as domino LT. The objective of this manuscript is to compare the outcomes of PLT performed with each of the available graft options. METHODS: Retrospective cohort study from Jan. 2010 to Dec. 2022. The variables included data on the recipients' preoperative clinical status, intraoperative technical aspects, post-operative complications, and survival studies. There were 4 groups: SPLIT (17), DD-WL (55), LD-WL (824), and LD-P (22). RESULTS: The median age and BW of the recipients was smaller in SPLIT, LD-P, and LD-WL compared to DDT-WL groups. HVOO (HR 15.87, 95% CI 1.89-133.06, P = 0.01), retransplantation (HR 7.94, 95% CI 2.63-24.02, P < 0.01), and malignancies (HR 3.08, 95% CI 1.29-7.37, P = 0.01) were independently associated with decreased patient survival. HAT (HR 27.54, 95% CI 10.44-72.68, P < 0.01) and malignancies (HR 2.42, 95% CI 1.10-5.34, P = 0.03) increased the risk of graft loss. The overall survival in this series was 91.4% (mean follow-up of 74.3 months). Patient and graft survival were not different among groups. CONCLUSION: HAT and malignancies were associated with reduced graft survival. Whole liver from living donors with MSUD presented 100% patient survival at 120 months. Even without statistical differences in survival among the studied groups, LD-P and LD-WL recipients presented a trend towards better outcomes. LEVEL OF EVIDENCE: LEVEL III.

2.
Transplantation ; 107(10): 2087-2097, 2023 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-37750781

RESUMO

BACKGROUND: Over 16 000 children under the age of 15 died worldwide in 2017 because of liver disease. Pediatric liver transplantation (PLT) is currently the standard of care for these patients. The aim of this study is to describe global PLT activity and identify variations between regions. METHODS: A survey was conducted from May 2018 to August 2019 to determine the current state of PLT. Transplant centers were categorized into quintile categories according to the year they performed their first PLT. Countries were classified according to gross national income per capita. RESULTS: One hundred eight programs from 38 countries were included (68% response rate). 10 619 PLTs were performed within the last 5 y. High-income countries performed 4992 (46.4%) PLT, followed by upper-middle- (4704 [44·3%]) and lower-middle (993 [9·4%])-income countries. The most frequently used type of grafts worldwide are living donor grafts. A higher proportion of lower-middle-income countries (68·7%) performed ≥25 living donor liver transplants over the last 5 y compared to high-income countries (36%; P = 0.019). A greater proportion of programs from high-income countries have performed ≥25 whole liver transplants (52.4% versus 6.2%; P = 0.001) and ≥25 split/reduced liver transplants (53.2% versus 6.2%; P < 0.001) compared to lower-middle-income countries. CONCLUSIONS: This study represents, to our knowledge, the most geographically comprehensive report on PLT activity and a first step toward global collaboration and data sharing for the greater good of children with liver disease; it is imperative that these centers share the lead in PLT.


Assuntos
Hepatopatias , Transplante de Fígado , Criança , Humanos , Transplante de Fígado/efeitos adversos , Censos , Doadores Vivos , Morte
3.
Pediatr Transplant ; 27(8): e14555, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37291909

RESUMO

BACKGROUND: The management of complex, intra- and extrahepatic portal vein thrombosis (PVT) after liver transplantation (LT) is challenging. Although most of the patients remain asymptomatic or oligosymptomatic in the chronic setting, some of them may develop severe portal hypertension and related complications, notably gastrointestinal (GI) bleeding. In the emergency scenario, clinical and endoscopic treatments as well as intensive support constitute the bases of conservative management, while more definitive treatment options such as surgical shunting and retransplantation are related to high morbidity rates. Transjugular intrahepatic portosystemic shunt (TIPS) was largely considered of limited role due to technical difficulties arising from extensive PVT. Recently, however, new minimally invasive image-guided techniques emerged, allowing portal vein recanalization and TIPS creation simultaneously (TIPS-PVR), even in complex PVT pretransplant patients. METHODS: Herein, we describe a novel indication for TIPS-PVR in a post-LT adolescent presenting with life-threatening, refractory GI bleeding. RESULTS: The patient presented with complete resolution of the hemorrhagic condition after the procedure, with no deterioration of hepatic function or hepatic encephalopathy. Follow-up Doppler ultrasound after TIPS-PVR showed normal hepatopetal venous flow within the stents, and no evidence of complications, including intraperitoneal or peri splenic bleeding. CONCLUSIONS: This report describes the feasibility of TIPS-PVR in the post-LT scenario complicated by extensive PVT. In this case, a complete resolution of the life-threatening GI bleeding was achieved, with no major complications. Other patients with complex chronic PVT might benefit from the use of the described technique, but further studies are required to determine the correct timing and indications of the procedure, eventually before the occurrence of life-threatening complications.


Assuntos
Varizes Esofágicas e Gástricas , Transplante de Fígado , Derivação Portossistêmica Transjugular Intra-Hepática , Trombose Venosa , Humanos , Adolescente , Veia Porta/cirurgia , Transplante de Fígado/efeitos adversos , Derivação Portossistêmica Transjugular Intra-Hepática/efeitos adversos , Varizes Esofágicas e Gástricas/complicações , Varizes Esofágicas e Gástricas/cirurgia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/cirurgia , Trombose Venosa/cirurgia , Trombose Venosa/complicações , Resultado do Tratamento
4.
Pediatr Transplant ; 27(5): e14518, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36973639

RESUMO

BACKGROUND: The techniques involved in neonatal and infantile transplantation require approaches that can sculpt a left lateral segment (LLS) to the right shape and size and avoid large-for-size syndrome. The aim of this article is to describe the anterior hepatic resection (AHR) of the LLS in pediatric LDLT. METHODS: A retrospective anatomical study of preoperative image studies, description of the technique for AHR, and short-term results. RESULTS: The AHR was performed in eight cases. All donors were male, with average age, BW, and BMI of 28.3 ± 5.9 years, 74.2 ± 9.3 kg, and 24.3 ± 2.6 kg/m2, respectively. Donors were discharged at an average of 3.6 ± 0.8 days. The median recipient age and BW at transplantation were 6.9 (2.7 to 11) months and 5.9 (3.9 to 8) kg, respectively, and the recipient-to-donor body weight ratio (RDBW) was <0.1 in all but one case. The mean percentage reduction in graft weight and in the antero-posterior diameter were 33.2% ± 5.5% and 38.3% ± 12.6%, respectively. The average (SD) GRWR was 4.8% ± 1.7% before all the resections and 3.5% ± 1.0% after the procedures. Seven patients were primarily closed. CONCLUSION: After LLS resection, a nonanatomical anterior resection of the LLS was accomplished without hilar vascular dissection to segments II/III. The final liver graft allowed primary abdominal wall closure in all but one patient, with meaningful adjustments in GRWR. AHR proved to be simple, safe, reproducible, and effective in the presented case series.


Assuntos
Transplante de Fígado , Doadores Vivos , Recém-Nascido , Humanos , Criança , Masculino , Adulto Jovem , Adulto , Feminino , Estudos Retrospectivos , Fígado/cirurgia , Transplante de Fígado/métodos , Hepatectomia/métodos , Resultado do Tratamento
5.
World J Hepatol ; 14(10): 1899-1906, 2022 Oct 27.
Artigo em Inglês | MEDLINE | ID: mdl-36340749

RESUMO

BACKGROUND: Living donor liver transplantation is a safe alternative for patients on a liver transplant list. Donor evaluation goes beyond physical variables to include social, emotional, and ethical aspects. The role of pre-donation sociopsychological evaluation of the donor candidate is as important to the success of the procedure as is the medical assessment. Success implies recovery from the operation and prompt engagement in pre-transplant professional and social activities, without leading to psychological or physical distress. Psychological profiling of potential living liver donors (PLLD) and evaluation of quality of life (QOL) can influence outcomes. AIM: To evaluate the socio-demographics and psychological aspects (QOL, depression, and anxiety) of PLLD for pediatric liver transplantation in a cohort of 250 patients. METHODS: This was a retrospective cohort study of 250 PLLD who underwent psychological pre-donation evaluation between 2015 and 2019. All the recipients were children. The Beck anxiety inventory, Beck depression inventory, and 36-item short-form health survey (SF-36) scores were used to evaluate anxiety (Beck anxiety inventory), depression (Beck depression inventory), and QOL, respectively. RESULTS: A total of 250 PLLD were evaluated. Most of them were women (54.4%), and the mean age was 29.2 ± 7.2 years. A total of 120 (48.8%) PLLD were employed at the time of evaluation for donation; however, most had low income (57% earned < 2 times the minimum wage). A total of 110 patients (44%) did not finish the donation process, and 247 PLLD answered a questionnaire to evaluate depression, anxiety, and QOL (SF-36). Prevalence of depression was of 5.2% and anxiety 3.6%. Although most of the PLLD were optimistic regarding the donation process and never had doubts about becoming a donor, some traces of ambivalence were observed: 46% of the respondents said they would feel relieved if a deceased donor became available. CONCLUSION: PLLD had a low prevalence of anxiety and depression. The foundation for effective and satisfactory results can be found in the pre-transplantation process, during which evaluations must follow rigorous criteria to mitigate potential harm in the future. Pre-donation psychological evaluation plays a predictive role in post-donation emotional responses and mental health issues. The impact of such findings on the donation process and outcomes needs to be further investigated.

6.
Microorganisms ; 10(5)2022 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-35630472

RESUMO

Background: The COVID-19 infection has received the attention of the scientific community due to its respiratory manifestations and association with evolution to severe acute respiratory syndrome (SARS-CoV-2). There are few studies characterizing SARS-CoV-2 in pediatric immunocompromised patients, such as liver transplanted patients. The aim of this study was to analyze the outcomes of the largest cohort of pediatric liver transplant recipients (PLTR) from a single center in Brazil who were infected with COVID-19 during the pandemic. Methods: Cross-sectional study. Primary outcomes: COVID-19 severity. The Cox regression method was used to determine independent predictors associated with the outcomes. Patients were divided into two groups according to the severity of COVID-19 disease: moderate−severe COVID and asymptomatic−mild COVID. Results: Patients categorized as having moderate−severe COVID-19 were younger (12.6 months vs. 82.1 months, p = 0.03), had a higher prevalence of transplantation from a deceased donor (50% vs. 4.3%, p = 0.02), and had a higher prevalence of COVID infection within 6 months after liver transplantation (LT) (75% vs. 5.7%, p = 0.002). The independent predictor of COVID-19 severity identified in the multivariate analysis was COVID-19 infection <6 months after LT (HR = 0.001, 95% CI = 0.001−0.67, p = 0.03). Conclusion: The time interval of less than 6 months between COVID-19 infection and LT was the only predictor of disease severity in pediatric patients who underwent liver transplantation.

7.
Case Rep Surg ; 2022: 1015061, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35223125

RESUMO

METHODS: We present a series of three patients with large hepatocellular adenoma lesions showing a central location, for which the living donor liver transplantation strategy was used as a backup procedure. RESULTS: Hepatocellular adenoma was confirmed by biopsy in all patients. Surgical resection was indicated because of the patients' symptoms and lesion size and growth. All patients had a lesion that was central or in close contact with major vessels. The final decision to proceed with the resection was made intraoperatively. A live donor was prepared for all three patients. Two patients underwent portal vein embolization associated with extended hepatectomy, and a total hepatectomy plus liver transplantation with a living donor was performed in one patient. All patients had good postoperative outcomes. CONCLUSIONS: In the treatment of hepatocellular adenomas for which complex resections are necessary and resectability can only be confirmed intraoperatively, surgical safety can be improved through the use of a living donor backup. Center expertise with living donor liver transplantation is paramount for the success of this approach.

8.
Pediatr Transplant ; 26(3): e14203, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34881487

RESUMO

BACKGROUND: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. METHODS: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. RESULTS: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. CONCLUSION: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.


Assuntos
Hérnia Diafragmática , Transplante de Fígado , Criança , Hérnia Diafragmática/complicações , Hérnia Diafragmática/cirurgia , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
9.
Pediatr Transplant ; 25(8): e14112, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34396644

RESUMO

BACKGROUND: The impact of the COVID pandemic on liver transplant (LT) programs varied among countries. Few data are available about that impact in pediatric liver transplant (PLT) programs. This study aimed at comparing the data of our program in Brazil (2019 vs. 2020). METHODS: Retrospective cohort study. RESULTS: One hundred and seventy-four PLT were performed in the period (93% living donors). Patients were divided into two groups according to the LT date: pre-COVID-19 period (march/2019-February/2020) and COVID-19 period (March/2020-February 2021). In the pre-COVID-19 period, 97 LTs were performed, and 77 LTs were performed in the COVID-19 period. Patients in the COVID-19 period were younger (10.9 months vs. 16 months, p 0.009), had higher PELD scores (15 vs. 14, p 0.04), more ascites (66.2 vs. 51.5%, p 0.03), and more frequently hospitalized before LT (27.3 vs. 17.5%). However, there was no difference in post-LT complications, retransplantation nor survival rates. Six (6.2%) patients from pre-COVID-19 period were COVID positive at a median of 15.5 months (14-17.5), and 6 (7.8%) patients from COVID-19 period were COVID positive at a median of 3 months (20 days-6 months) from LT. There was neither mortality nor complications in those patients. Four (33%) were hospitalized, and one had prolonged intubation. Four (33%) were asymptomatic, 4 (33%) had upper airways symptoms, and the remaining had gastrointestinal symptoms. CONCLUSION: Overall, PLT was not affected during COVID-19 period. Even though patients from COVID-19 period were sicker, there was no significant impact in LT outcomes. All the recipients who tested positive for COVID had a favorable outcome.


Assuntos
COVID-19/epidemiologia , Transplante de Fígado/estatística & dados numéricos , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Hospitais com Alto Volume de Atendimentos , Humanos , Lactente , Masculino , Pandemias , Complicações Pós-Operatórias/epidemiologia , SARS-CoV-2
10.
World J Gastroenterol ; 27(12): 1161-1181, 2021 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-33828392

RESUMO

BACKGROUND: The impact of perioperative blood transfusion on short- and long-term outcomes in pediatric living donor liver transplantation (PLDLT) must still be ascertained, mainly among young children. Clinical and surgical postoperative complications related to perioperative blood transfusion are well described up to three months after adult liver transplantation. AIM: To determine whether transfusion is associated with early and late postoperative complications and mortality in small patients undergoing PLDLT. METHODS: We evaluated the effects of perioperative transfusion on postoperative complications in recipients up to 20 kg of body weight, submitted to PLDLT. A total of 240 patients were retrospectively allocated into two groups according to postoperative complications: Minor complications (n = 109) and major complications (n = 131). Multiple logistic regression analysis identified the volume of perioperative packed red blood cells (RBC) transfusion as the only independent risk factor for major postoperative complications. The receiver operating characteristic curve was drawn to identify the optimal volume of the perioperative RBC transfusion related to the presence of major postoperative complications, defining a cutoff point of 27.5 mL/kg. Subsequently, patients were reallocated to a low-volume transfusion group (LTr; n = 103, RBC ≤ 27.5 mL/kg) and a high-volume transfusion group (HTr; n = 137, RBC > 27.5 mL/kg) so that the outcome could be analyzed. RESULTS: High-volume transfusion was associated with an increased number of major complications and mortality during hospitalization up to a 10-year follow-up period. During a short-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and bleeding complications, with a decrease in rejection complications compared to the LTr. Over a long-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and minor neoplastic complications, with a decrease in rejection complications. Additionally, Cox hazard regression found that high-volume RBC transfusion increased the mortality risk by 3.031-fold compared to low-volume transfusion. The Kaplan-Meier survival curves of the studied groups were compared using log-rank tests and the analysis showed significantly decreased graft survival, but with no impact in patient survival related to major complications. On the other hand, there was a significant decrease in both graft and patient survival, with high-volume RBC transfusion. CONCLUSION: Transfusion of RBC volume higher than 27.5 mL/kg during the perioperative period is associated with a significant increase in short- and long-term postoperative morbidity and mortality after PLDLT.


Assuntos
Transplante de Fígado , Doadores Vivos , Adulto , Transfusão de Sangue , Criança , Pré-Escolar , Transfusão de Eritrócitos/efeitos adversos , Humanos , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos
11.
Pediatr Transplant ; 25(5): e13968, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33590638

RESUMO

BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.


Assuntos
Síndrome Hepatopulmonar/cirurgia , Hipóxia/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Hipóxia/diagnóstico , Lactente , Tempo de Internação/estatística & dados numéricos , Cirrose Hepática/fisiopatologia , Masculino , Gravidade do Paciente , Cuidados Pós-Operatórios/estatística & dados numéricos , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
12.
Orphanet J Rare Dis ; 15(1): 309, 2020 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-33131499

RESUMO

BACKGROUND: Maple syrup urine disease (MSUD) is an autosomal recessive inherited metabolic disease caused by deficient activity of the branched-chain α-keto acid dehydrogenase (BCKD) enzymatic complex. BCKD is a mitochondrial complex encoded by BCKDHA, BCKDHB, DBT, and DLD genes. MSUD is predominantly caused by Variants in BCKDHA, BCKDHB, and DBT genes encoding the E1α, E1ß, and E2 subunits of BCKD complex, respectively. The aim of this study was to characterize the genetic basis of MSUD by identifying the point variants in BCKDHA, BCKDHB, and DBT genes in a cohort of Brazilian MSUD patients and to describe their phenotypic heterogeneity. It is a descriptive cross-sectional study with 21 MSUD patients involving molecular genotyping by Sanger sequencing. RESULTS: Eight new variants predicted as pathogenic were found between 30 variants (damaging and non-damaging) identified in the 21 patients analyzed: one in the BCKDHA gene (p.Tyr120Ter); five in the BCKDHB gene (p.Gly131Val, p.Glu146Glnfs * 13, p.Phe149Cysfs * 9, p.Cys207Phe, and p.Lys211Asn); and two in the DBT gene (p.Glu148Ter and p.Glu417Val). Seventeen pathogenic variants were previously described and five variants showed no pathogenicity according to in silico analysis. CONCLUSION: Given that most of the patients received late diagnoses, the study results do not allow us to state that the molecular features of MSUD variant phenotypes are predictive of clinical severity.


Assuntos
Doença da Urina de Xarope de Bordo , 3-Metil-2-Oxobutanoato Desidrogenase (Lipoamida)/genética , Brasil , Estudos Transversais , Humanos , Doença da Urina de Xarope de Bordo/genética , Fenótipo
13.
Transplantation ; 103(3): 536-543, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-29847508

RESUMO

BACKGROUND: Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the LD, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT. METHODS: From September 2012 to September 2017, 11 patients with MSUD underwent LD liver transplantation at our institution, and MSUD livers were used as domino grafts in 11 children. RESULTS: (1) MSUD patients: 10 patients received a left lateral segment. The donor's left hepatic vein (HV) was anastomosed to the confluence of the recipient's 3 HVs. No vascular grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 of 11 patients. (2) MSUD graft recipients: In 8 cases, HV reconstruction was performed between the graft's HV confluence and the recipient's HV confluence, and in 3 cases, a vena cava triangulation was necessary; 6 MSUD grafts required HV venoplasty. No VG were needed for HV reconstruction. VG were used for PV reconstruction in 3 cases due to sclerotic PV. In 2 cases, double arterial anastomoses were performed in the MSUD liver. All patients remain alive and well. CONCLUSIONS: Living donor liver transplantation followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction.


Assuntos
Transplante de Fígado/métodos , Doadores Vivos , Doença da Urina de Xarope de Bordo/cirurgia , Obtenção de Tecidos e Órgãos/métodos , Anastomose Cirúrgica , Índice de Massa Corporal , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Artéria Hepática/cirurgia , Veias Hepáticas/cirurgia , Humanos , Lactente , Fígado/irrigação sanguínea , Fígado/cirurgia , Masculino , Veia Porta/cirurgia , Doadores de Tecidos , Transplantados
14.
Pediatr Transplant ; 22(1)2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29131462

RESUMO

LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD. CVD patients had more abnormal EKG (32.4% vs 14.5%, P < .001), abnormal chest X-ray (11.8% vs 1.4%, P < .001), and altered echocardiography (89.7% vs 15.4%, P < .001) findings compared with the No-CVD group pretransplant. Post-transplant, significant differences between groups were observed related to abnormal EKG (14.7% vs 7.0%, P = .03) and echocardiography (48.5% vs 3.2%, P < .01) findings. Pretransplant ASD spontaneously closed in 22 patients. At 1 and 5 years post-transplant, there was no difference in the survival rate between groups (P = .96). The prevalence of CVD in recipients of LT was high, and its presence was associated with significantly higher cardiac decompensation before and after LT. Minor and moderate cardiovascular disease did not impact the long-term survival.


Assuntos
Doenças Cardiovasculares/complicações , Doença Hepática Terminal/cirurgia , Transplante de Fígado , Adolescente , Doenças Cardiovasculares/diagnóstico por imagem , Doenças Cardiovasculares/mortalidade , Criança , Pré-Escolar , Ecocardiografia , Doença Hepática Terminal/complicações , Doença Hepática Terminal/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Transplante de Fígado/mortalidade , Masculino , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
15.
Radiographics ; 37(6): 1612-1631, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29019744

RESUMO

Liver transplant is considered to be the last-resort treatment approach for pediatric patients with end-stage liver disease. Despite the remarkable advance in survival rates, liver transplant remains an intricate surgery with significant morbidity and mortality. Early diagnosis of complications is crucial for patient survival but is challenging given the lack of specificity in clinical presentation. Knowledge of the liver and vascular anatomy of the donor and the recipient or recipients before surgery is also important to avoid complications. In this framework, radiologists play a pivotal role on the multidisciplinary team in both pre- and postoperative scenarios by providing a road map to guide the surgery and by assisting in diagnosis of complications. The most common complications after liver transplant are (a) vascular, including the hepatic artery, portal vein, hepatic veins, and inferior vena cava; (b) biliary; (c) parenchymal; (d) perihepatic; and (e) neoplastic. The authors review surgical techniques, the role of each imaging modality, normal posttransplant imaging features, types of complications after liver transplant, and information required in the radiology report that is critical to patient care. They present an algorithm for an imaging approach for pediatric patients after liver transplant and describe key points that should be included in radiologic reports in the pre- and postoperative settings. Online supplemental material is available for this article. ©RSNA, 2017.


Assuntos
Diagnóstico por Imagem , Transplante de Fígado/métodos , Complicações Pós-Operatórias/diagnóstico por imagem , Criança , Humanos
16.
Pediatr Transplant ; 21(7)2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28736976

RESUMO

Although rare, ALF caused by disseminated HSV infection is associated with high mortality in the neonatal population. This condition is often diagnosed relatively late due to the absence of specific signs. We present a case involving a neonate with ALF submitted to living donor liver transplantation without a prior diagnosis. The patient had no skin or mucosal lesions, and IgM serology was negative for HSV-1 and HSV-2. Immunohistochemical staining of the liver explant was positive for herpes virus infection, and the patient subsequently received antiviral drug treatment, with a good outcome. Due to organ shortages and the rarity of the aforementioned condition, LT has seldom been reported for the treatment of ALF caused by herpes virus infection; however, LT may be the only option for neonates with fulminant hepatitis. The use of living donors in an urgent scenario is well established in Eastern countries and safely applicable for pediatric patients with ALF.


Assuntos
Hepatite Viral Humana/cirurgia , Herpes Simples/cirurgia , Falência Hepática Aguda/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Feminino , Hepatite Viral Humana/complicações , Herpes Simples/complicações , Humanos , Recém-Nascido , Falência Hepática Aguda/virologia
17.
Pediatr Transplant ; 20(7): 994-999, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27412691

RESUMO

The association between LT and gastrectomy is not common. Only two studies reported the gastrectomy/LT association in children. Here, we report three children who underwent LT who required a concomitant or sequential gastrectomy for different reasons. Patient 1, a 16-yr-old boy, during the LT, underwent a partial gastrectomy due to extensive injury to the duodenum. He had a previous and unusual portoenterostomy performed in the duodenum. Bowel reconstruction was performed using an intestinal loop that was first used for the bilio-enteric anastomosis and then connected to the gastric stump. Patient 2, a 22-month-old female child, underwent a partial gastrectomy with a Roux-en-Y reconstruction during a retransplantation. She had a large perforated gastric ulcer blocked by the allograft liver. Patient 3, a 26-month-old male child, five yr after living donor LT, was submitted to a partial gastrectomy because of gastric outlet obstruction. The histopathology was compatible with eosinophilic gastritis. The association between LT and gastrectomy in the pediatric population is extremely rare. Appropriate knowledge of the previous transplantation technique is very important. Further studies are required to assess the outcomes of the different types of gastric reconstruction in pediatric recipients.


Assuntos
Gastrectomia/métodos , Falência Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Transplante de Fígado/métodos , Adolescente , Anastomose em-Y de Roux/métodos , Anastomose Cirúrgica , Pré-Escolar , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Masculino , Reoperação , Estudos Retrospectivos
18.
Transplantation ; 100(9): 1944-54, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27362317

RESUMO

BACKGROUND: Biliary complications (BCs) remain an important cause of morbidity after pediatric liver transplantation. Technical factors have already been implicated in the development of BCs. Previous reports have associated the use of partial grafts, particularly living donor grafts, with a higher incidence of BCs. Our aim is to study the factors associated with the development of BCs in a large cohort of pediatric liver transplant recipients. METHODS: Retrospective cohort study of 670 children (<18 years of age) who underwent a primary liver transplant between March 2000 and January 2015. Patients who did and did not develop BCs were compared with identify associated factors. Univariate and multivariate analyses were performed. RESULTS: A total of 115 patients (17.2%) developed BCs (83 strictures and 44 leaks). Of the study participants, 594 had living donor liver transplants. Multiple arterial anastomoses was a protective factor for BCs, and a ductoplasty was a risk factor. Living donor grafts and multiple biliary anastomoses were more frequently associated with leaks. Patients with BCs had a higher reoperation rate and longer hospital stays. There was no difference in patient or graft survival. CONCLUSIONS: Technical factors play a major role in the development of BCs, particularly leaks. Strictures are more frequently associated with an inadequate arterial supply to the bile duct, and multiple arterial anastomoses may protect children from this complication. The use of partial grafts was not an independent factor for BCs in high-volume centers that are experienced with this technique.


Assuntos
Fístula Anastomótica/etiologia , Colestase/etiologia , Transplante de Fígado/efeitos adversos , Fatores Etários , Fístula Anastomótica/diagnóstico , Fístula Anastomótica/cirurgia , Procedimentos Cirúrgicos do Sistema Biliar/efeitos adversos , Distribuição de Qui-Quadrado , Pré-Escolar , Colestase/diagnóstico , Colestase/cirurgia , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Tempo de Internação , Transplante de Fígado/métodos , Doadores Vivos , Masculino , Análise Multivariada , Razão de Chances , Modelos de Riscos Proporcionais , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos
19.
Pediatr Transplant ; 20(6): 813-8, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27329427

RESUMO

Re-LT is the only recourse for patients with liver graft failure. However, survival rates after re-LT are lower than those of primary transplants. Few reports are available regarding re-LT with LDs in children. The objective of this study was to describe our cohort of patients retransplanted with LD and emphasize the technical aspects of a re-LT with LD. This is a retrospective report of a series of 18 children (<18 yr old) submitted to 20 re-LT from January 1997 to December 2013 at Hospital Sirio-Libanes and Hospital AC Camargo Cancer Center. The one- and five-yr survival for patients retransplanted with LD was 70.6% and 58.6%. Little technical modifications from a regular LD primary transplant were needed in patients retransplanted with LD. Seven (38.8%) patients presented vascular complications following re-LT and three presented biliary complications (16.6%). In conclusion, a re-LT with LD is an acceptable alternative for children who experience liver graft failure and it does not compromise the donor pool. Further experience with re-LT with LD may support this therapy.


Assuntos
Transplante de Fígado , Doadores Vivos , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Transplante de Fígado/métodos , Transplante de Fígado/mortalidade , Masculino , Avaliação de Resultados em Cuidados de Saúde , Reoperação , Estudos Retrospectivos
20.
Transplantation ; 100(6): 1161-4, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27203583

RESUMO

We reviewed the history, volume, outcomes, uniqueness, and challenges of living donor liver transplantation (LDLT) in Latin America. We used the data from the Latin American and Caribbean Transplant Society, local transplant societies, and opinions from local transplant experts. There are more than 160 active liver transplant teams in Latin America, but only 30 centers have used LDLT in the past 2 years. In 2014, 226 LDLTs were done in the region (8.5% of liver transplant activities). Living donor liver transplantation is mainly restricted to pediatric patients. Adult-to-adult LDLT activities decreased after the implementation of the model for end-stage liver disease score and a concomitant increase on the rate of deceased donors per million population. Posttransplant outcome analysis is not mandatory, transparent or regulated in most countries. More experienced teams have outcomes comparable to international expert centers, but donor and recipient morbidity might be underreported. Latin America lags behind in terms of the number of adult LDLT and the rate of living donor utilization in comparison with other continents with similar donation rates. Local alliances and collaborations with major transplant centers in the developed world will contribute to the development of LDLT in Latin America.


Assuntos
Doença Hepática Terminal/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Brasil , Doença Hepática Terminal/etnologia , Humanos , Relações Interinstitucionais , Cooperação Internacional , América Latina , Índice de Gravidade de Doença , Obtenção de Tecidos e Órgãos , Resultado do Tratamento
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