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1.
Pediatr Transplant ; 26(5): e14279, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35393654

RESUMO

BACKGROUND: Liver biopsy is the gold standard to stage fibrosis in liver disease. Several scoring systems have been studied to predict advanced fibrosis in liver disease. Those scores have not been validated in pediatric liver transplant patients. AIM: Evaluate the performance of three fibrosis scores (FSc) in assessing the presence of advanced fibrosis (AF) in children after orthotopic liver transplantation (OLT). METHODS: Patients < 20 years of age who underwent liver biopsy post-OLT with laboratory values within 1 month of the biopsy were included. Fibrosis was determined by an experienced pathologist (F0-4). We defined AF as F3-4. The following FSc were calculated: AST/ALT ratio, APRI, and FIB-4 index. Receiver operating characteristic curve analysis was done to assess the FSc performance in predicting AF. RESULTS: A total of 232 biopsies were analyzed, of those 42 (18.1%) showed AF (F3-4). FIB-4 was significantly higher in patients with AF compared to those without AF [median value of 1.1 [0.7, 3.0] and 0.6 [0.2, 1.4], respectively (p = .02)]; however, FIB-4 had satisfactory accuracy to diagnose AF with significant overlap and AUC of 0.68 (CI 0.56-0.81). Cutoff points of 0.2 and 3.03 were used to rule in and rule out AF, respectively. AST/ALT and APRI were not significantly different between patients with and without AF. CONCLUSION: Even though FIB-4 had satisfactory accuracy in detecting AF in pediatric transplant patients, noninvasive hepatic FSc developed in adults still performed poorly. Our results highlight the need to develop a reliable pediatric FSc.


Assuntos
Transplante de Fígado , Adulto , Aspartato Aminotransferases , Biomarcadores , Biópsia , Criança , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/patologia , Cirrose Hepática/cirurgia , Contagem de Plaquetas/métodos , Curva ROC , Estudos Retrospectivos , Índice de Gravidade de Doença
2.
Children (Basel) ; 9(3)2022 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-35327746

RESUMO

Background: Pediatric non-alcoholic fatty liver disease (NAFLD) is a major public health concern. Aminotransferase (ALT) is frequently used for screening and monitoring, but few studies have reported typical patterns of ALT elevation in children. Methods: TARGET-NASH is a real-world longitudinal observational cohort of patients with NAFLD receiving care across the United States. Analyses included children enrolled between 1 August 2016, and 12 October 2020, with at least one ALT measurement after enrollment. Peak ALT was based on the first and last available record and categorized into clinical cut points: <70 IU/L, >70−<250 IU/L, and >250 IU/L. A chi-squared test was used to compare differences in proportions, and a Kruskal−Wallis test was used to compare the medians and distributions of continuous responses. Results: Analyses included 660 children with a median age of 13 years. Of the 660, a total of 187 had undergone a biopsy and were more likely to be Hispanic or Latino (67% vs. 57%, p = 0.02) and to have cirrhosis (10% vs. 1%, p < 0.001). The highest ALT scores ranged from 28 U/L to 929 U/L; however, these scores varied across time. The prevalence of cirrhosis or any liver fibrosis stage was most common among children with a peak ALT > 70 U/L. Conclusions: Large variability was seen in ALT among children, including many values > 250 U/L. Higher levels of ALT were associated with increased prevalence of comorbidities and more advanced stages of NAFLD. These findings support an increased need for therapeutics and disease severity assessment in children with peak ALT > 70 U/L.

3.
ACG Case Rep J ; 7(7): e00433, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32766374

RESUMO

Gallbladder adenomyomatosis is a rare condition that is predominantly seen in adults, and only a few cases have been described in the pediatric population. Although it is generally benign, it may present a diagnostic challenge for physicians. Advances in imaging have led to an increase in its detection. Nevertheless, the characteristics and management of this condition in pediatric patients have not been well described. We present a case of a 6-week-old infant boy who was found to have gallbladder adenomyomatosis.

4.
Paediatr Drugs ; 20(4): 315-329, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29740791

RESUMO

The prevalence of nonalcoholic fatty liver disease (NAFLD) has increased substantially in the past two decades and NAFLD has now become the most common cause of chronic liver disease in children and adolescents. NAFLD is a broad clinicopathologic spectrum ranging from simple steatosis to varying degrees of necroinflammation called nonalcoholic steatohepatitis (NASH), leading to fibrosis and subsequently to cirrhosis. Despite the increasing prevalence and progressive nature of NAFLD even among children, therapy for NAFLD in both adults and children are limited. Weight loss remains the only consistently effective therapy for NAFLD. Pharmacologic options are even more limited in children than in adults with NAFLD. Vitamin E has been shown to be effective in improving histology in children with NASH. Few pharmacologic options such as metformin, probiotics, omega-3 fatty acids, and cysteamine bitartrate have been studied in children, with limited beneficial effects. However, these studies are limited by small sample size and heterogeneity of outcome assessment after treatment. Recent studies show promising results with bariatric surgery with regards to weight loss and improvement in liver histology in adolescents with NAFLD. In this review article, we discuss epidemiology, pathophysiology, and extrahepatic comorbidities of pediatric NAFLD and review existing therapeutic options for children with NAFLD. We also review novel therapeutic strategies studied in adults that could potentially be studied in children in the future.


Assuntos
Hepatopatia Gordurosa não Alcoólica , Adolescente , Cirurgia Bariátrica , Criança , Dietoterapia , Humanos , Metformina/uso terapêutico , Hepatopatia Gordurosa não Alcoólica/epidemiologia , Hepatopatia Gordurosa não Alcoólica/fisiopatologia , Hepatopatia Gordurosa não Alcoólica/terapia , Prevalência , Vitamina E/uso terapêutico
5.
Biomark Med ; 12(3): 265-273, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29517271

RESUMO

Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the obesity epidemic. Recent studies have clearly shown that the stage of fibrosis in adults with NAFLD is the most important histological feature in long-term outcomes and the development of liver-related complications. Despite the paucity of data regarding the natural history of pediatric NAFLD, its progression to cirrhosis and end-stage liver disease requiring liver transplantation is well documented. Given the high prevalence of NAFLD in children and adults, there is an urgent need to find safe and cost-effective alternatives to biopsy to determine the stage of liver fibrosis. In this review, we provide a concise overview of different noninvasive methods for diagnosing and staging liver fibrosis in children with NAFLD.


Assuntos
Cirrose Hepática/diagnóstico , Hepatopatia Gordurosa não Alcoólica/diagnóstico , Alanina Transaminase/análise , Aspartato Aminotransferases/análise , Biomarcadores/análise , Biomarcadores/sangue , Criança , Técnicas de Imagem por Elasticidade , Humanos , Queratina-18/análise , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico por imagem , Hepatopatia Gordurosa não Alcoólica/complicações
6.
Exp Clin Transplant ; 16(5): 562-567, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-28952917

RESUMO

OBJECTIVES: Several scoring systems have been developed to noninvasively predict the presence of advanced fibrosis in patients with chronic liver disease. Hepatitis C virus and nonalcoholic fatty liver disease are the 2 most common indications for orthotopic liver transplant and are associated with disease recurrence that can lead to fibrosis progression. Here, we evaluated the performance of commonly used fibrosis scores in assessing the presence of advanced fibrosis in patients after orthotopic liver transplant. MATERIALS AND METHODS: Our study consisted of consecutive patients with hepatitis C virus or nonalcoholic fatty liver disease who underwent a liver biopsy after transplant and had laboratory measurements within 1 week of biopsy. Graft fibrosis was determined by an experienced pathologist (stage F0-F4). Advanced fibrosis was defined as stage F3-F4. The following fibrosis scores were calculated for each patient: aspartate aminotransferase/alanine aminotransferase ratio, aspartate aminotransferase/platelet ratio index, and fibrosis-4 index. RESULTS: We analyzed 93 patients with median age of 59 years (25th and 75th percentile of 53 and 64 y) and median body mass index of 31.8 kg/m² (25th and 75th percentile of 27 and 37.6 kg/m²). Of total patients, 41 (44%) were diabetic. Median time to liver biopsy posttransplant was 27.7 months (25th and 75 percentile of 10.8 and 59.9 mo). We found that 54 patients (58%) had no fibrosis, 15 (16.1%) had F1, 8 (8.6%) had F2, 7 (7.5%) had F3, and 9 (9.7%) had F4. Overall, advanced fibrosis (F3-F4) was present in 16 patients. Aspartate aminotransferase/alanine amino-transferase ratio, aspartate aminotransferase/platelet ratio index, and fibrosis-4 index were not significantly different between patients with and without advanced fibrosis (all P > .05). The calculated fibrosis scores had poor diagnostic accuracy for presence of advanced fibrosis posttransplant. CONCLUSIONS: Commonly used liver fibrosis scores are not accurate in predicting the presence of advanced fibrosis in patients after liver transplant.


Assuntos
Ensaios Enzimáticos Clínicos , Cirrose Hepática/diagnóstico , Cirrose Hepática/cirurgia , Transplante de Fígado , Contagem de Plaquetas , Idoso , Alanina Transaminase/sangue , Aspartato Aminotransferases/sangue , Biomarcadores/sangue , Biópsia , Feminino , Hepatite C/sangue , Hepatite C/complicações , Hepatite C/diagnóstico , Humanos , Cirrose Hepática/sangue , Cirrose Hepática/etiologia , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Hepatopatia Gordurosa não Alcoólica/sangue , Hepatopatia Gordurosa não Alcoólica/complicações , Hepatopatia Gordurosa não Alcoólica/diagnóstico , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
7.
J Adolesc Young Adult Oncol ; 6(2): 286-293, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27996360

RESUMO

PURPOSE: Orthotopic liver transplantation (OLT) is curative for hepatocellular carcinoma (HCC). HCC is typically a disease of older adults (OAs); therefore, characteristics and outcomes of OLT for young adults (YAs) (ages 18-40) are not described. The objective of this study was to assess the characteristics and outcomes of YAs with HCC receiving OLT and compare these to OAs (ages >40 years). METHODS: YAs with HCC who had OLT from the United Network for Organ Sharing (UNOS) database were included in this study. As a comparison group, OAs with HCC were matched 4:1 to the YA group. Descriptive statistics of demographics, comorbidities, and outcomes were generated. Kaplan-Meier product limit estimates were used to assess patient and graft survival. Conditional logistic regression and Cox proportional hazards frailty models were used to compare the groups. RESULTS: A total of 464 YAs received OLT for HCC. The most common underlying liver diseases were hepatitis C virus (21.3%), hepatitis B virus (HBV, 15.5%), and autoimmune/cholestatic disease (12.3%). An increased number of YAs received OLT for HCC after implementation of model for end-stage liver disease scoring. One thousand two hundred eighty OAs served as the comparison group. Post-transplant 5-year survival was 73.1% in YAs with a retransplantation rate of 7.8%. In OAs, survival and retransplantation rates were lower (68.6% p = 0.093; 4.3% p = 0.001). CONCLUSION: Four hundred sixty-four YAs with HCC received OLT in the UNOS database. Compared to the older population, survival and retransplantation rates were higher. HBV, which is vaccine preventable, is a frequent contributor to HCC in YAs.


Assuntos
Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Adolescente , Adulto , Fatores Etários , Carcinoma Hepatocelular/etiologia , Estudos de Casos e Controles , Colangite Esclerosante/complicações , Colestase/complicações , Bases de Dados Factuais , Doença Hepática Terminal , Feminino , Sobrevivência de Enxerto , Hepatite B Crônica/complicações , Hepatite C Crônica/complicações , Hepatite Autoimune/complicações , Humanos , Estimativa de Kaplan-Meier , Cirrose Hepática Biliar/complicações , Neoplasias Hepáticas/etiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Reoperação , Índice de Gravidade de Doença , Estados Unidos , Adulto Jovem
8.
Dig Liver Dis ; 48(8): 909-13, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27198736

RESUMO

BACKGROUND: Within the spectrum of nonalcoholic fatty liver disease (NAFLD), recent evidence suggests that adult patients with nonalcoholic steatohepatitis (NASH) have significantly lower blood lysosomal acid lipase (LAL) activity than those with steatosis. This has not been studied in pediatric patients with NAFLD. AIM: Investigate blood LAL activity in pediatric patients with NAFLD and assess its correlation with histological severity. METHODS: We collected data on consecutive children with biopsy-proven NAFLD including demographics, anthropometrics, and routine laboratory tests. The histological features were graded according to the NAFLD activity scoring proposed by Kleiner et al. Blood LAL activity was measured prospectively using Lalistat 2. RESULTS: A total of 168 children were included for analysis. Mean age was 12.6±8.5 years, 60.1% were males and 52.4% had NASH. Children with significant fibrosis (stage 2-3, n=64) had a significantly lower LAL activity compared to those with mild fibrosis (stage 0-1, n=104). There was no significant difference in LAL activity between children with NASH compared to those without NASH. CONCLUSION: Reduced blood LAL activity correlates with severity of liver fibrosis in children with NAFLD indicating a potential role of reduced LAL activity in the pathogenesis of NAFLD-induced fibrosis.


Assuntos
Cirrose Hepática/sangue , Hepatopatia Gordurosa não Alcoólica/sangue , Esterol Esterase/sangue , Adolescente , Adulto , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Itália , Fígado/diagnóstico por imagem , Fígado/patologia , Cirrose Hepática/patologia , Masculino , Hepatopatia Gordurosa não Alcoólica/patologia , Índice de Gravidade de Doença , Ultrassonografia , Adulto Jovem
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