RESUMO
BACKGROUND: Pediatric liver transplantation for small recipients presents significant challenges, particularly in securing suitably sized donor organs. This case report illustrates the feasibility of performing an in situ split procurement in an 18.5-kg toddler, the smallest recorded case in the OPTN database to date, for a critically ill 8-week-old infant recipient. CASE PRESENTATION: An 8-week-old infant with severe hepatitis of unknown etiology was urgently listed as Status 1A. An organ offer from a 3.5-year-old donor, requiring a reduction procedure, became available 1939 nautical miles away. Instead of a back-table reduction procedure, we performed an in situ split to reduce cold ischemic time given the distance. The recipient surgery was started ahead of the organ's arrival, and the recipient was ready for graft implantation upon the organ's arrival, resulting in a total of 510 min of cold ischemic time. Post-operatively, the graft did not show signs of significant injury or dysfunction, which expedited recovery from her other medical conditions. CONCLUSIONS: In situ split liver procurement is an invaluable tool for pediatric centers as it effectively provides more graft options for pediatric patients on the waitlist. Additionally, in situ split can offer significant benefits in optimizing recipient surgery, especially when the donor is located at an extreme distance. Despite these benefits, in situ split is not currently widely utilized across transplant centers. Addressing the logistical challenges associated with this technique is crucial for broader implementation and improved patient outcomes.
Assuntos
Transplante de Fígado , Obtenção de Tecidos e Órgãos , Humanos , Transplante de Fígado/métodos , Lactente , Feminino , Obtenção de Tecidos e Órgãos/métodos , Doadores de Tecidos , Pré-Escolar , Isquemia Fria , Tamanho do ÓrgãoRESUMO
The appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon.
Assuntos
Carcinoma Hepatocelular , Hepatoblastoma , Neoplasias Hepáticas , Transplante de Fígado , Criança , Humanos , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Hepatoblastoma/cirurgia , Hepatoblastoma/patologia , Transplante de Fígado/métodos , Resultado do TratamentoRESUMO
BACKGROUND: APSA's Right Child/Right Surgeon Initiative addresses issues concerning patient access to appropriate pediatric surgical care and workforce distribution. The APSA Workforce Committee sought to understand the experiences and motivations of recent graduates of Pediatric Surgery Training Programs entering the workforce. METHODS: Using APSA membership databases, we identified members who completed fellowship training from 2010 to 2019. An online survey was created using Survey Monkey, and invitations to participate were sent via email. RESULTS: 144 of 447 invited participants responded (32% response rate). 91% of respondents participated in dedicated research prior to fellowship, but only 64% perform research during their employment. 23% completed an additional clinical fellowship, but only 54% currently practice within the second field. When asked to identify the top three factors used to choose a position, the most common responses were "location or geography" (71%), "available mentorship" (53%), and "compensation and benefits" (37%). Describing their first position, 77% reported working in an academic institution, 78% reported working in a metropolitan/urban area, and 55% reported working in a free-standing children's hospital. 94% participate in General Surgery resident education, and 49% are faculty within a Pediatric Surgery fellowship. Overall, 92% of respondents were able to find the type of employment position that they had wanted. CONCLUSION: In our survey the overwhelming majority of young pediatric surgeons found the type of job they desired. Most report beginning their practice in more populated, urban areas within academic institutions. Geographic location and work environment played heavily into their employment decisions. These preferences could contribute to continued disparity in access to pediatric surgeons between urban and rural America and to dilution of experience for urban surgeons. Possible solutions include alternative incentive programs for employment in less populated areas or new training models for general surgeons in rural areas to train in fundamentals of Pediatric Surgery.
Assuntos
Especialidades Cirúrgicas , Cirurgiões , Escolha da Profissão , Emprego , Bolsas de Estudo , Humanos , Inquéritos e QuestionáriosRESUMO
The field of pediatric solid-organ transplantation has significantly evolved since its beginnings in the early 20th century. As advancements have led to the development of innovative surgical techniques and novel medication regimens, transplantation has now become a routine practice leading to an increase in the rates of organ recipients worldwide. The care of pediatric solid-organ transplant recipients differs from adults in several areas not only due to technically challenging surgeries, but mostly due to the complexity of their immunosuppression management. Although there is large variation of pediatric immunosuppression regimens worldwide, the use of calcineurin inhibitors, either tacrolimus or cyclosporine, still forms the backbone of immunosuppression regimens after solid-organ transplantation. Both medications are relatively well tolerated but are known to have long-term side effects, especially nephrotoxicity and neurotoxicity. The goal of care in long-term pediatric survivors of solid-organ transplant now aims to safely minimize exposure to immunosuppression and to achieve long-term graft tolerance.
Assuntos
Rejeição de Enxerto/prevenção & controle , Imunossupressores/uso terapêutico , Transplante de Órgãos , Criança , Quimioterapia Combinada , Humanos , Imunossupressores/farmacologia , Quimioterapia de Indução/métodos , Quimioterapia de Manutenção/métodosRESUMO
BACKGROUND: Factors influencing survival in children with HCC have not been studied. The objective of this study was to identify prognostic factors in pediatric HCC, and to determine whether regional lymphadenectomy is associated with improved survival. METHODS: We performed a retrospective cohort study using the Surveillance, Epidemiology and End Results (SEER) registry. All patients <20 years old diagnosed with HCC from 1973-2009 were included. Disease-specific survival was compared using Kaplan-Meier statistics and Cox proportional-hazards regression. RESULTS: We identified 238 patients (139 Male: 99 Female). Overall, 112 (47%) received an operation (resection/transplantation). Observed mortality and adjusted hazard of disease-specific death was greater for females (HR=2.07, p=0.013) and older children. Among operative patients, 44% were documented to have a regional lymphadenectomy. Although demographic factors did not differ between lymphadenectomy and non-lymphadenectomy groups, patients who underwent lymphadenectomy had a greater proportion of metastatic disease (24% vs. 15%) and fibrolamellar HCC (53% vs. 31%). Five-year survival for lymphadenectomy patients was superior to non-lymphadenectomy (70% vs. 57%). Adjusted mortality for lymphadenectomy was also improved relative to non-lymphadenectomy (HR=0.26, p=0.013). CONCLUSIONS: HCC in children is associated with poor survival, especially among children older than 4 years and girls. In surgical candidates, regional lymphadenectomy may be associated with improved survival.
Assuntos
Carcinoma Hepatocelular/epidemiologia , Neoplasias Hepáticas/epidemiologia , Excisão de Linfonodo , Adolescente , Distribuição por Idade , Idade de Início , Carcinoma Hepatocelular/secundário , Carcinoma Hepatocelular/cirurgia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Hepatectomia , Humanos , Incidência , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Metástase Linfática , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Programa de SEER , Distribuição por Sexo , Resultado do Tratamento , Estados Unidos/epidemiologia , Adulto JovemRESUMO
Adjusted survival outcomes following hepatic resection and transplantation for pediatric liver tumors have not been compared. To address this question, we conducted a retrospective cohort study using the SEER registry. While SEER lacks certain specifics regarding staging, chemotherapy, comorbidities, and recurrence, important hypothesis-generating data are available and were analyzed using Kaplan-Meier statistics and Cox proportional hazards regression. All SEER patients under the age of 20 yr undergoing surgery for HB (n = 318) or HCC (n = 80) between 1998 and 2009 were included. Of HB patients, 83.3% underwent resection and 16.7% transplantation. Advanced disease, vascular invasion, and satellite lesions were more common among transplant patients. Unadjusted five-yr survival was equivalent, as was the adjusted hazard of death for transplant relative to resection (HR = 0.58, p = 0.63). Of HCC patients, 75.0% underwent resection and 25.0% transplantation. Transplant patients had a higher prevalence of vascular invasion and satellite lesions. Five-yr survival was 53.4% after resection and 85.3% after transplant, and the adjusted hazard of death was significantly lower after transplantation (HR = 0.05, p = 0.045). While transplantation is generally reserved for unresectable tumors, the favorable survival seen in HCC patients suggests that liberalized transplant criteria might improve survival, although further prospective data are needed.
Assuntos
Carcinoma Hepatocelular/cirurgia , Carcinoma Hepatocelular/terapia , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/terapia , Transplante de Fígado , Adolescente , Criança , Pré-Escolar , Comorbidade , Feminino , Hepatoblastoma/cirurgia , Hepatoblastoma/terapia , Humanos , Lactente , Masculino , Prevalência , Modelos de Riscos Proporcionais , Recidiva , Estudos Retrospectivos , Programa de SEER , Resultado do Tratamento , Estados UnidosRESUMO
BACKGROUND: Intestinal lengthening remains a treatment option in pediatric short bowel syndrome. However, clinical indications and nutritional outcomes from bowel lengthening are not well defined. METHODS: A retrospective review of a prospective database was conducted of patients who underwent bowel lengthening using serial transverse enteroplasty (STEP) at a single center. RESULTS: Sixteen children who underwent 19 STEP procedures were identified. STEP increased median small bowel length from 84 cm (range, 19 to 295 cm) to 103 cm (range, 24 to 375 cm) (P = .0001). Caloric provisions from parenteral nutrition were decreased after STEP (69% vs 0%, P < .01). Of 15 subjects in the nutritional analysis, 12 (80%) had improved enteral tolerance, and 9 (60%) achieved enteral autonomy after STEP. Six STEP procedures were not associated with improvements in enteral nutrition, and 5 (83%) were performed in children with gastroschisis. CONCLUSIONS: A majority of children with short bowel syndrome were weaned off parenteral nutrition after STEP. Gastroschisis may portend a less optimal outcome from the procedure.
Assuntos
Nutrição Enteral/estatística & dados numéricos , Intestino Delgado/cirurgia , Nutrição Parenteral/estatística & dados numéricos , Síndrome do Intestino Curto/cirurgia , Adolescente , Criança , Pré-Escolar , Seguimentos , Humanos , Lactente , Estudos Retrospectivos , Síndrome do Intestino Curto/etiologia , Síndrome do Intestino Curto/terapia , Resultado do TratamentoRESUMO
BACKGROUND: Hepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period. METHODS: We conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing children's hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death. RESULTS: A total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died. CONCLUSIONS: Hepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted.
Assuntos
Carcinoma Hepatocelular/cirurgia , Artéria Hepática/patologia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Complicações Pós-Operatórias/etiologia , Trombose/etiologia , Adolescente , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/patologia , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Hemorragia/epidemiologia , Hemorragia/etiologia , Hemorragia/cirurgia , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Lactente , Hepatopatias/cirurgia , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/fisiopatologia , Masculino , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Trombofilia/etiologia , Washington , Adulto JovemRESUMO
This case presents a complication of attempted separation of parapagus conjoined twins, related to loss of an intact mesenteric-portal venous axis. Despite known lack of a superior mesenteric artery in the right twin, initial evaluation in the operating room suggested that separation was possible. After hepatic division, however, it became apparent that the mesenteric drainage was not separable; and the operation was aborted. Subsequently, significant growth failure and hypoglycemia were noted in the right twin. The situation was corrected by creating a shunt to reinstitute mesenteric flow to the right twin's liver and separating the twin's mesenteric drainage. One year postoperatively, both twins are independently nourishing themselves and have been free from hospitalization with stable glucoses.
Assuntos
Insuficiência de Crescimento/etiologia , Hipoglicemia/etiologia , Veias Mesentéricas/anormalidades , Complicações Pós-Operatórias , Gêmeos Unidos/cirurgia , Anastomose Cirúrgica , Feminino , Humanos , Recém-Nascido , Artérias Mesentéricas/anormalidades , Veias Mesentéricas/cirurgia , Veia Esplênica/cirurgia , Gêmeos Unidos/patologiaRESUMO
OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.
Assuntos
Atresia Biliar/patologia , Atresia Biliar/cirurgia , Portoenterostomia Hepática , Atresia Biliar/complicações , Feminino , Humanos , Lactente , Icterícia/etiologia , Masculino , Estudos ProspectivosRESUMO
PURPOSE: Multidisciplinary treatment of pediatric intestinal failure has shown promising results. However, there are limited data as to the optimal time frame for referral of patients to intestinal failure programs. The aim of this study was to explore the relationship of hyperbilirubinemia at referral with patient outcomes in a multidisciplinary program. METHODS: A retrospective analysis was performed of a prospectively collected database from a multidisciplinary intestinal failure program. Multivariable logistic regression adjusted for age at referral was used to model the association between the conjugated bilirubin at referral and risk of mortality. Median values with range are reported. RESULTS: Sixty-two patients were referred from 2005 to 2009. Patients presented at age 6.4 months (0.4-261.4 months) and were followed up for 16.8 (0.3-53.0) months. Nine subjects (14.5%) died, and 12 subjects (19.4%) were listed for combined liver-intestine transplant. A 50% mortality was seen in patients referred with a conjugated bilirubin ≥ 7.2 mg/dL (n = 12), whereas mortality at referral bilirubin levels <7.2 mg/dL was 6%. After adjusting for age at referral, patients with a conjugated bilirubin ≥ 7.2 mg/dL at referral were 15.4 times more likely to die than patients who presented with lower bilirubin levels (P = .001; 95% confidence interval, 2.8-83.4). CONCLUSION: Within a pediatric intestinal failure program, mortality is associated with the degree of hyperbilirubinemia at time of referral. These data strongly suggest that these patients should be referred to a multidisciplinary program early in the evolution of their liver disease.
Assuntos
Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/mortalidade , Enteropatias/mortalidade , Enteropatias/cirurgia , Hepatopatias/diagnóstico , Encaminhamento e Consulta/organização & administração , Centros Médicos Acadêmicos , Pré-Escolar , Estudos de Coortes , Comorbidade , Bases de Dados Factuais , Progressão da Doença , Feminino , Hospitais Pediátricos , Humanos , Hiperbilirrubinemia/cirurgia , Lactente , Recém-Nascido , Enteropatias/diagnóstico , Estimativa de Kaplan-Meier , Hepatopatias/mortalidade , Hepatopatias/cirurgia , Modelos Logísticos , Masculino , Insuficiência de Múltiplos Órgãos/mortalidade , Insuficiência de Múltiplos Órgãos/fisiopatologia , Insuficiência de Múltiplos Órgãos/cirurgia , Análise Multivariada , Transplante de Órgãos/métodos , Transplante de Órgãos/mortalidade , Avaliação de Programas e Projetos de Saúde , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Taxa de Sobrevida , Estados UnidosRESUMO
Survival following pediatric re-transplant is inferior to that following primary transplant. We analyzed UNOS data (1987-2007) to identify factors associated with poor outcomes following re-transplant in both the pre-PELD and PELD eras. There may be a combination of factors associated with a futile pediatric liver re-transplant. Identification of these factors may improve allograft allocation and survival following re-transplantation. Abstract: Survival following pediatric liver re-transplant is distinctly inferior to that following primary transplant. The purpose of this study was to determine factors associated with futile pediatric liver re-transplants before and after introduction of the PELD criteria in February 2002. We analyzed the UNOS database (1987-2008) and identified pediatric patients requiring liver re-transplants before and after PELD criteria. Descriptive characteristics were evaluated and survival analyzed with Cox proportional hazards method. Analysis of 1248 children identified re-transplant survival in the PELD era was significantly better than the pre-PELD era. Multivariable analysis in the pre-PELD era identified number of re-transplants, African American race, ICU pretransplant, recipient weight, creatinine and bilirubin levels, donor age, and cold ischemia time to be significantly associated with poor survival. In the PELD era, ICU hospitalization, weight, and very high bilirubin levels were associated with poor survival. Kaplan-Meier analysis by risk groups demonstrated a significant difference in survival, with the highest risk group experiencing 40-50% one-yr survival. Survival following pediatric liver re-transplantation varies significantly by era and associated risk factors. There may be a combination of factors that predict a futile re-transplant. Pre-operative identification of these factors may improve allograft allocation and recipient survival following re-transplantation.
Assuntos
Falência Hepática/cirurgia , Transplante de Fígado , Futilidade Médica , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Testes de Função Hepática , Masculino , Seleção de Pacientes , Modelos de Riscos Proporcionais , Estudos Prospectivos , Reoperação , Fatores de Risco , Estatísticas não Paramétricas , Taxa de SobrevidaRESUMO
BACKGROUND: The aim of this study was to evaluate the clinical experience of a regional multidisciplinary intestinal failure program for children established in 2005. METHODS: Data were collected from a prospective internal database. Univariate analyses were performed to compare pre- and post-treatment outcomes. Median values are reported. RESULTS: Forty-nine children were referred at an age of 7 months. Remnant small bowel length was 29 cm. With follow-up of 14 months, overall patient survival was 88%. Thirteen bowel-lengthening procedures were performed, thereby increasing small bowel length from 83 to 132 cm (P < .05). Enteral autonomy was achieved in 22 patients (45%), and the caloric requirement for parenteral nutrition was decreased from 100% to 41% (P < .01). Conjugated bilirubin was reduced from 4.1 to 0 mg/dL (P < .05). CONCLUSION: A multidisciplinary approach to pediatric intestinal failure that prioritizes intestinal rehabilitation can achieve successful enteral feeding advancement, improved liver function, and excellent survival in intermediate-range follow-up.
Assuntos
Causas de Morte , Intestino Delgado/cirurgia , Síndrome do Intestino Curto/mortalidade , Síndrome do Intestino Curto/reabilitação , Análise de Variância , Bases de Dados Factuais , Nutrição Enteral/métodos , Feminino , Hospitais Pediátricos , Humanos , Lactente , Intestino Delgado/patologia , Intestino Delgado/transplante , Masculino , Nutrição Parenteral/métodos , Equipe de Assistência ao Paciente/organização & administração , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/reabilitação , Probabilidade , Avaliação de Programas e Projetos de Saúde , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença , Síndrome do Intestino Curto/diagnóstico , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: The aim of this study was to describe patients undergoing the Kasai procedure at children's hospitals, evaluate outcomes, and analyze the association of these outcomes with systemic steroid use. METHODS: Biliary atresia patients (International Classification of Diseases, Ninth Revision, code 751.61) who underwent Kasai procedures at freestanding children's hospitals in the Pediatric Health Information System database from 2003 to 2008 were identified. Descriptive characteristics were examined, and regression analyses were used to determine whether postoperative steroid use was associated with length of stay, mortality, or cholangitis. RESULTS: Of the 516 children identified (40% male, 50% aged < 2 months), 239 (46%) received perioperative steroids. The mean total and postoperative lengths of stay were 14.5 +/- 19.7 and 11.3 +/- 16.3 days, respectively. Postoperative steroid use was significantly associated with a 3.5-day decrease in postoperative length of stay (95% confidence interval, .03-6.97). CONCLUSIONS: Perioperative steroids after the Kasai procedure are associated with shorter postoperative length of stay. Work is needed to ascertain whether this relationship is causal.
Assuntos
Atresia Biliar/tratamento farmacológico , Atresia Biliar/cirurgia , Portoenterostomia Hepática/métodos , Esteroides/uso terapêutico , Atresia Biliar/diagnóstico , Atresia Biliar/mortalidade , Estudos de Coortes , Intervalos de Confiança , Bases de Dados Factuais , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Seguimentos , Mortalidade Hospitalar/tendências , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Portoenterostomia Hepática/efeitos adversos , Cuidados Pós-Operatórios/métodos , Probabilidade , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
PURPOSE: The purpose of this study was to describe the population of pediatric patients waiting for intestinal transplant and to evaluate the risk of death or transplant by specific disease states. METHODS: We studied the United Network for Organ Sharing (UNOS) database (Jan 1,1991 to 5/16/08) for patients 21 years old or younger at first listing for intestinal transplant and examined their age, sex, weight, and diagnoses. Time to list removal was summarized with cumulative incidence curves. Multinomial logistic regression was used to compare relative risk ratios for removal from the list for transplant, death, or other reasons. RESULTS: We identified 1712 children listed for intestinal transplant (57% male, 51% <1 year, weight 8.1 kg [IQR, 6.1-14.1] at listing). Median age and weight at transplant (n = 852) were 1 year (IQR, 1-5) and 10 kg (IQR, 6.5-16.3). Regression analysis demonstrated significant differences in outcomes among disease conditions (P < .001). Compared to the gastroschisis group, the relative risk ratio for death versus transplant was higher in the necrotizing enterocolitis group (P = .015), lower in the short gut syndrome group (P = .001), and not different in the volvulus group (P = .94) after adjustment for weight and sex. CONCLUSIONS: We conclude that the relative risk of transplant vs death varies significantly by the disease condition of the patient.
Assuntos
Enterocolite Necrosante/cirurgia , Gastrosquise/cirurgia , Volvo Intestinal/cirurgia , Intestinos/transplante , Seleção de Pacientes , Síndrome do Intestino Curto/cirurgia , Obtenção de Tecidos e Órgãos/estatística & dados numéricos , Transplante/estatística & dados numéricos , Listas de Espera , Fatores Etários , Causas de Morte , Pré-Escolar , Bases de Dados Factuais/estatística & dados numéricos , Enterocolite Necrosante/epidemiologia , Enterocolite Necrosante/mortalidade , Feminino , Gastrosquise/epidemiologia , Gastrosquise/mortalidade , Alocação de Recursos para a Atenção à Saúde , Humanos , Incidência , Lactente , Volvo Intestinal/epidemiologia , Volvo Intestinal/mortalidade , Modelos Logísticos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Risco , Fatores Sexuais , Síndrome do Intestino Curto/epidemiologia , Síndrome do Intestino Curto/mortalidade , Estados Unidos/epidemiologiaRESUMO
Mesenchymal hamartoma of the liver is the second most common benign liver tumor of childhood. Ultrasonography demonstrates a cystic lesion that may be very large. Computed tomographic scan will further establish the location and its relationship to vascular anatomy. Most will be amenable to gross total resection with clear margins. To illustrate the radiological and gross operative features, we present a 9-month-old male infant with a mesenchymal hamartoma with classic features.
Assuntos
Hamartoma , Hepatopatias , Hamartoma/diagnóstico , Hamartoma/cirurgia , Humanos , Lactente , Hepatopatias/diagnóstico , Hepatopatias/cirurgia , MasculinoRESUMO
BACKGROUND: Dementia is a common and growing global public health problem. It leads to a high burden of suffering for society with an annual cost of $100 billion in the US and $10 billion in the UK. New strategies for both treatment and prevention of dementia are currently being developed. Implementation of these strategies will depend on the presence of a viable community or primary care based dementia screening and diagnosis program and patient acceptance of such a program. OBJECTIVE: To compare the acceptance, perceived harms and perceived benefits of dementia screening among older adults receiving their care in two different primary health care systems in two countries. DESIGN: A Cross-sectional study. SETTING: Primary care clinics in Indianapolis, USA and Kent, UK. PARTICIPANTS: A convenience sample of 245 older adults (Indianapolis, n = 125; Kent, n = 120). OUTCOMES: Acceptance of dementia screening and its perceived harms and benefits as determined by a 52-item questionnaire (PRISM-PC questionnaire). RESULTS: Four of the five domains were significantly different across the two samples. The UK sample had significantly higher dementia screening acceptance scores (p < 0.05); higher perceived stigma scores (p < 0.05); higher perceived loss of independence scores (p < 0.01); and higher perceived suffering scores (p < 0.01) than the US sample. Both groups perceived dementia screening as beneficial (p = 0.218). After controlling for prior experience with dementia, acceptance and stigma were marginalized. CONCLUSIONS: Older adults attending primary care clinics across the Atlantic value dementia screening but have significant concerns about dementia screening although these concerns differed between the two countries. Low acceptance rates and high rates of perceived harms might be a significant barrier for the introduction of treatment or preventive methods for dementia in the future.
Assuntos
Atitude Frente a Saúde/etnologia , Comparação Transcultural , Demência/diagnóstico , Programas de Rastreamento/psicologia , Inquéritos e Questionários , Negro ou Afro-Americano , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Demência/psicologia , Feminino , Humanos , Masculino , Aceitação pelo Paciente de Cuidados de Saúde , Atenção Primária à Saúde/organização & administração , Reino Unido/epidemiologia , Estados Unidos/epidemiologia , População BrancaRESUMO
BACKGROUND: Primary Epstein-Barr virus (EBV) infection is the most important risk factor for development of posttransplant lymphoproliferative disorder (PTLD). Pediatric patients are often EBV seronegative pretransplant placing them at high risk. In the immune-competent population, primary herpesvirus infection is associated with higher morbidity with increasing age. METHODS: We performed a retrospective cohort study to describe the outcome of pediatric renal transplant recipients with primary EBV infection. All patients received 3 months of ganciclovir prophylaxis. Real-time quantitative polymerase chain reaction was used to determine the EBV viral load. Primary EBV infection was categorized as PTLD, symptomatic infection, or subclinical infection. RESULTS: There were a total of 46 patients with primary EBV infection: 11 developed PTLD, 12 had symptomatic infection, and 23 had subclinical infection. Adolescents were significantly more likely to develop PTLD than younger transplant recipients (P=0.05, chi-square). Multivariate analysis using logistic regression found that older age was the only significant risk factor for PTLD (odds ratio 1.24, 95% confidence interval 1.04-1.47; P=0.03). Among the 11 cases of PTLD, there were two deaths and two graft failures which all occurred in adolescent recipients (P=0.002). CONCLUSIONS: Among pediatric renal transplant recipients with primary EBV infection, adolescents are at significantly higher risk to develop PTLD and have poorer outcomes compared to younger recipients.
Assuntos
Fatores Etários , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/etiologia , Transplante de Rim/efeitos adversos , Transtornos Linfoproliferativos/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Infecções por Vírus Epstein-Barr/virologia , Feminino , Rejeição de Enxerto/etiologia , Humanos , Modelos Logísticos , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/mortalidade , Masculino , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco , Carga ViralRESUMO
PURPOSE: Traditional treatment of giant omphaloceles with silo closure has been associated with respiratory insufficiency, hemodynamic compromise, dehiscence, and inability to close the abdomen with subsequent death. To minimize such complications, initial nonoperative management with delayed closure of the defect has been used. METHODS: Between January 1981 and December 2002, 111 patients with omphaloceles were treated. Twenty-two patients with giant omphaloceles (19 containing liver) underwent initial nonoperative management consisting of silver sulfadiazine dressing changes. After pulmonary and other comorbidities stabilized, the contents were gradually reduced with a loose elastic bandage, and delayed closure was planned at 6 to 12 months. The medical records of these 22 patients were retrospectively reviewed to determine the efficacy and safety of this technique in the setting of severe associated anomalies. Those 15 patients (n = 15) from the latter 10 years were further reviewed to determine additional end points (length of hospital stay, length of intensive care unit stay, duration of mechanical ventilation, time to feed, time to closure, and type of closure). RESULTS: Of the 15 patients treated during the latter 10 years, mean gestational age and birth weight were 38 +/- 1.4 weeks and 3.1 +/- 0.57 kg, respectively. Median length of stay after birth was 20 days (range, 5-239 days). Median time to full diet was 8 days (range, 4-80 days). Four patients were discharged on oral feedings only, 7 with combination oral/gavage, and 4 with tube feedings. Pulmonary hypoplasia or pulmonary hypertension was present in 11 (50%) of 22 patients. There were 11 patients with major cardiac anomalies, 14 with a patent ductus arteriosus, and 8 with a patent foramen ovale. Three early complications (2 ruptured sacs and 1 bleeding sac) and 1 late complication (gastric necrosis) occurred in the initial nonoperative period. In addition, 4 patients were treated for line sepsis, 1 patient for acute renal insufficiency, and 1 for aspiration pneumonia. Three patients required tracheostomy and were discharged with home ventilators. There were no complications associated with the use of silver sulfadiazine. Of the 22 patients, 16 have undergone delayed repair, 2 did not require repair, 1 is awaiting repair, 2 died before closure, and 1 was lost to follow-up. Delayed closure was achieved at a median age of 14 months (range, 2-28 months) and mean weight of 8.8 +/- 3.3 kg. Four patients required implantation of mesh for definitive closure. Median postoperative length of stay was 4 days (range, 2-21 days). Postoperative complications included prolonged ileus, recurrent ventral hernia, and prolonged intubation. Overall mortality rate was 9.1%. One death occurred after diaphragmatic hernia repair, and 1 death was from overwhelming sepsis in the patient with a late gastric perforation. CONCLUSION: The use of silver sulfadiazine dressing changes for initial nonoperative management of giant omphaloceles is a safe and effective bridge to delayed closure. We recommend this method as initial nonoperative management given the high incidence of associated cardiopulmonary malformations because it may facilitate enteral feeding, minimize respiratory compromise, and reduce morbidity and mortality.
Assuntos
Anti-Infecciosos Locais/administração & dosagem , Bandagens , Fasciotomia , Hérnia Umbilical/terapia , Sulfadiazina de Prata/administração & dosagem , Parede Abdominal/cirurgia , Administração Tópica , Feminino , Hérnia Umbilical/cirurgia , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Tempo , Cicatrização/efeitos dos fármacosRESUMO
BACKGROUND: Ramstedt pyloromyotomy through a right upper quadrant (RUQ) transverse incision has been the traditional treatment for hypertrophic pyloric stenosis. Recently, laparoscopic (LAP) and circumumbilical (UMB) approaches have been introduced as alternative methods to improve cosmesis, but concerns about greater operative times, costs, and complications remain. This study compares the three operative techniques and examines their advantages and complication rates. STUDY DESIGN: We performed a retrospective review of patients undergoing pyloromyotomy at a children's hospital between January 1997 and June 2003. RESULTS: Two hundred ninety patients underwent pyloromyotomy by LAP (n = 51), RUQ (n = 190), or UMB (n = 49). Complication rate, time to ad libitum feeding, incidence of emesis, and postoperative length of stay did not differ considerably among groups. Two LAP patients were converted to RUQ. Mucosal perforation occurred in three patients each in the RUQ and UMB groups, but none in the LAP group. Operative times were considerably less for LAP (25 +/- 9 minutes) than for RUQ (32 +/- 9 minutes) and UMB (42 +/- 12 minutes) (p < 0.05, ANOVA, Bonferroni). Charges related to operations and anesthesia were considerably greater for UMB (operation: US 1,574 dollars +/- US 433 dollars; anesthesia: US 731 dollars +/- US 190 dollars) compared with the other two groups (p < 0.05, ANOVA, Bonferroni), but did not differ between LAP (operation: US 1,299 dollars +/- US 311 dollars; anesthesia: US 586 dollars +/- US 137 dollars) and RUQ (operation: US 1,237 dollars +/- US 411 dollars; anesthesia: US 578 dollars +/- US 167 dollars). Data are presented as mean +/- SD. CONCLUSIONS: Advantages of LAP include a shorter mean operative time without higher complications or costs. UMB is associated with the greatest mean operative time and costs. Laparoscopic pyloromyotomy is a safe and effective approach to the treatment of hypertrophic pyloric stenosis.