RESUMO
Hepatic abscesses in premature infants are rare with less than 100 case reports documented in literature. We report a case of a premature infant diagnosed with hepatic microabscesses secondary to eosinophilic colitis. A 33 4/7-week preterm female neonate presented with bilious emesis, abdominal distention, and severe hematochezia. Eosinophilic enterocolitis was suspected. Hypoechoic regions in the anterior liver identified on computed tomography were considered liver microabscesses. This unique case exemplifies how prematurity increases the risk of mucosal damage in the presence of eosinophilic colitis causing enteric bacteria to seed into the liver through the portal vein, resulting in hepatic microabscesses.
RESUMO
OBJECTIVE: To identify changes in demographics, outcomes, and risk factors for patient and graft loss in patients with biliary atresia undergoing liver transplantation since Pediatric End-Stage Liver Disease implementation (2002). STUDY DESIGN: Demographics and outcomes were compared between patients enrolled in the Society of Pediatric Liver Transplantation registry before (n = 547) and after (n = 1477) 2002. Kruskal-and χ2 Wallis tests identified significant differences between eras. Risk factors for patient and graft loss after 2002 were determined by Cox regression model analysis of time to event data. RESULTS: Significant patient differences after 2002 support increasing disease severity including more status 1 patients and those with a derived Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease score of greater than 30 awaiting transplant. Both patient and graft survival improved after 2002 from 90% to 97% and 81% to 90%, respectively (primary transplant; P < .0001). Significant differences in complications within 30 days included reduced relisting for transplant, rejection, culture-positive infection, repeat operation, hepatic artery thrombosis, portal vein thrombosis, and death/transplant before discharge. Multivariable analysis identified deceased technical variant vs whole graft and retransplantation predictive for patient death, hazard ratios of 4.041 and 8.308, respectively. Deceased technical variant vs whole graft (hazard ratio, 1.963) and donor age 0-5 months vs 1-17 years (hazard ratio, 5.525) were risk factors for graft loss. CONCLUSIONS: The overall outcomes of patients receiving liver transplantation for patients with biliary atresia have improved since 2002 despite evidence of increased disease severity at the time of transplant. Risk factors impacting post-transplant morbidity and mortality in patients with biliary atresia are now mainly surgical including donor variables.
Assuntos
Atresia Biliar/classificação , Transplante de Fígado/mortalidade , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Adolescente , Atresia Biliar/cirurgia , Criança , Pré-Escolar , Doença Hepática Terminal/classificação , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Recém-Nascido , Transplante de Fígado/efeitos adversos , Estudos Longitudinais , Masculino , Sistema de Registros , Reoperação/estatística & dados numéricos , Fatores de Risco , Índice de Gravidade de DoençaRESUMO
OBJECTIVE: To investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis. STUDY DESIGN: We retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year. We defined "normalization" as a GGT level <50 IU/L without experiencing portal hypertensive or dominant stricture events, liver transplantation, or death during the first year. RESULTS: We identified 263 patients, median age 12.1 years at diagnosis, treated with UDCA at a median dose of 15 mg/kg/d. Normalization occurred in 46%. Patients with normalization had a lower prevalence of Crohn's disease, lower total bilirubin level, lower aspartate aminotransferase to platelet ratio index, greater platelet count, and greater serum albumin level at diagnosis. The 5-year survival with native liver was 99% in those patients who achieved normalization vs 77% in those who did not. CONCLUSIONS: Less than one-half of the patients treated with UDCA have a complete GGT normalization in the first year after diagnosis, but this subset of patients has a favorable 5-year outcome. Normalization is less likely in patients with a Crohn's disease phenotype or a laboratory profile suggestive of more advanced hepatobiliary fibrosis. Patients who do not achieve normalization could reasonably stop UDCA, as they are likely not receiving clinical benefit. Alternative treatments with improved efficacy are needed, particularly for patients with already-advanced disease.
Assuntos
Colangite Esclerosante/sangue , Colangite Esclerosante/tratamento farmacológico , Ácido Ursodesoxicólico/uso terapêutico , gama-Glutamiltransferase/sangue , Adolescente , Análise de Variância , Biomarcadores/sangue , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Testes de Função Hepática , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Falha de Tratamento , Resultado do TratamentoRESUMO
OBJECTIVES: Biliary atresia (BA) is a progressive neonatal fibroinflammatory cholangiopathy. We hypothesized that intravenous immunoglobulin (IVIg) would be safe, feasible, acceptable, and efficacious for the treatment of BA. The primary objective of this study was to establish the feasibility, acceptability, and safety profile of IVIg administration after hepatoportoenterostomy (HPE) in BA. The secondary objective was to determine the treatment efficacy of IVIg based on good bile drainage and survival with the native liver. METHODS: A multicenter, prospective, open-labeled, phase I/IIA trial of IVIg was conducted, with 1âg/kg/dose of IVIg infused at 3-5, 30, and 60 days post-HPE, and subjects followed for 360 days post-HPE. Twenty-nine participants completed the study. RESULTS: Administration of IVIg infusions was feasible and acceptable in 79%. None of the serious adverse events (SAEs) were directly related to IVIg infusions; however, 90% of participants had an SAE. Compared with a historical placebo-arm group, there was no significant increase in the proportion of IVIg participants with a serum total bilirubin <1.5âmg/dL at 90, 180, or 360 days post-HPE. Survival with the native liver in the IVIg participants showed no significant benefit over the historical placebo arm, with a difference at 360 days of -11.9% (IVIg: 58.6%, placebo: 70.5%; 90% UCB: 2.1%; Pâ>â0.05). CONCLUSIONS: Although IVIg infusions in infants with BA post-HPE were feasible, acceptable and safe, there was no trend to lower bilirubin levels or improved 360-day survival with the native liver. CLINICAL TRIAL: Safety Study of Intravenous Immunoglobulin Post-Portoenterostomy in Biliary Atresia; #NCT01854827.
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Atresia Biliar/cirurgia , Imunoglobulinas Intravenosas/uso terapêutico , Atresia Biliar/mortalidade , Pré-Escolar , Drenagem , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Lactente , Recém-Nascido , Transplante de Fígado , Masculino , Portoenterostomia Hepática , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/cirurgia , Estudos Prospectivos , Resultado do TratamentoRESUMO
OBJECTIVE: To investigate the impact of corticosteroid therapy on the growth of participants in the Steroids in Biliary Atresia Randomized Trial (START) conducted through the Childhood Liver Disease Research Network. The primary analysis in START indicated that steroids did not have a beneficial effect on drainage in a cohort of infants with biliary atresia. We hypothesized that steroids would have a detrimental effect on growth in these infants. STUDY DESIGN: A total of 140 infants were enrolled in START, with 70 randomized to each treatment arm: steroid and placebo. Length, weight, and head circumference were obtained at baseline and follow-up visits to 24 months of age. RESULTS: Patients treated with steroids had significantly lower length and head circumference z scores during the first 3 months post-hepatoportoenterostomy (HPE), and significantly lower weight until 12 months. Growth trajectories in the steroid and placebo arms differed significantly for length (P < .0001), weight (P = .009), and head circumference (P < .0001) with the largest impact noted for those with successful HPE. Growth trajectory for head circumference was significantly lower in patients treated with steroids irrespective of HPE status, but recovered during the second 6 months of life. CONCLUSIONS: Steroid therapy following HPE in patients with biliary atresia is associated with impaired length, weight, and head circumference growth trajectories for at least 6 months post-HPE, especially impacting infants with successful bile drainage. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00294684.
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Corticosteroides/efeitos adversos , Atresia Biliar/tratamento farmacológico , Atresia Biliar/cirurgia , Insuficiência de Crescimento/induzido quimicamente , Sarcopenia/induzido quimicamente , Corticosteroides/uso terapêutico , Atresia Biliar/mortalidade , Peso Corporal/efeitos dos fármacos , Cefalometria/métodos , Desenvolvimento Infantil/efeitos dos fármacos , Desenvolvimento Infantil/fisiologia , Pré-Escolar , Método Duplo-Cego , Insuficiência de Crescimento/epidemiologia , Insuficiência de Crescimento/fisiopatologia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Monitorização Fisiológica/métodos , Portoenterostomia Hepática/métodos , Portoenterostomia Hepática/mortalidade , Cuidados Pós-Operatórios/métodos , Estudos Prospectivos , Valores de Referência , Medição de Risco , Sarcopenia/epidemiologia , Sarcopenia/fisiopatologia , Fatores de Tempo , Resultado do TratamentoAssuntos
Fibrose Cística/complicações , Hepatopatias/complicações , Pneumopatias/complicações , Pâncreas/patologia , Cisto Pancreático/complicações , Dor Abdominal , Adolescente , Biópsia , Colagogos e Coleréticos/uso terapêutico , Colangiopancreatografia por Ressonância Magnética , Colangite Esclerosante/complicações , Constipação Intestinal , Anticoncepcionais Orais/uso terapêutico , Feminino , Humanos , Icterícia/complicações , Fígado/patologia , Cistos Ovarianos/tratamento farmacológico , Pâncreas/enzimologia , Prurido/complicações , Radiografia Abdominal , Tomografia Computadorizada por Raios X , Ácido Ursodesoxicólico/uso terapêutico , Redução de PesoRESUMO
OBJECTIVE: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool. STUDY DESIGN: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage. Test scores were translated to age- and sex-dependent z scores, generating a maximum frailty score of 10. RESULTS: The median frailty score of the cohort was 4 (IQR 3, 5). Subjects with ESLD had significantly higher frailty scores (median 5; IQR 4, 7) than subjects with CCLD (median 3; IQR 2, 4); (P < .0001). Area under the curve receiver operating characteristic for frailty scores to discriminate between ESLD and CCLD was 0.83 (95% CI 0.73, 0.93). Forty-six percent of children with ESLD were frail and there was no correlation between pediatric frailty scores and physician's global assessments (r = -0.24, 95% CI -0.53, 0.10). CONCLUSIONS: A novel frailty tool assessed additional dimensions of health, not captured by standard laboratory measures and identified the sickest individuals among a cohort of children with chronic liver disease. This tool may have applicability to other children with chronic disease.
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Fragilidade/diagnóstico , Hepatopatias/complicações , Adolescente , Composição Corporal , Criança , Pré-Escolar , Doença Crônica , Estudos Transversais , Feminino , Fragilidade/etiologia , Marcha , Força da Mão , Humanos , Hepatopatias/fisiopatologia , Masculino , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
There is little detailed clinical information on recurrent primary sclerosing cholangitis (rPSC) after liver transplantation in children. Our purpose was to describe the characteristics of children who had experienced rPSC after liver transplantation so that we could identify potential risk factors for recurrence. Clinical information for pediatric patients undergoing transplantation for primary sclerosing cholangitis (PSC) was retrospectively reviewed, and variables related to the pretransplant diagnosis of PSC and posttransplant variables were abstracted. The studied variables included the following: cytomegalovirus/Epstein-Barr virus status, early/late rejection, induction regimen, immunosuppression in the first year, steroid-resistant rejection, diagnosis of inflammatory bowel disease, and human leukocyte antigen markers commonly associated with PSC. A diagnosis of rPSC was made on the basis of radiographic features, histology, or both. Twelve patients underwent liver transplantation for PSC between 1993 and 2012. Patients received tacrolimus for maintenance immunosuppression after induction with steroids (n = 6) or thymoglobulin (n = 6). Three patients were diagnosed with rPSC 44, 60, and 62 months after transplantation. A fourth patient underwent retransplantation for graft failure with features of both hepatic artery stenosis and rPSC. This patient had distinct histological features of rPSC in the second graft. Three of the 4 patients were 7 years old or younger at the diagnosis of PSC. The patient and graft survival rates were similar for the steroid and thymoglobulin groups. All 4 children with rPSC received steroid-free thymoglobulin induction. In conclusion, our observation of an association between thymoglobulin, and age less than 10 years at the diagnosis of PSC, and rPSC adds to the existing suggestion of a link between the immune environment and the pathogenesis of rPSC. Defining the natural history of rPSC and searching for the etiology and risk factors of rPSC are important for the long-term outcomes of pediatric patients.