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1.
Hepatology ; 77(2): 530-545, 2023 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-36069569

RESUMO

BACKGROUND AND AIMS: Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis. APPROACH AND RESULTS: A targeted enzyme-linked immunosorbent assay-based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL-8, endoglin, periostin, Mac-2-binding protein, MMP-3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin deficiency (A1AT; n = 78), and Alagille syndrome (ALGS; n = 65) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9 years and 9.5 kPa. After adjusting for covariates, there were positive correlations among LSM and endoglin ( p = 0.04) and IL-8 ( p < 0.001) and MMP-7 ( p < 0.001) in participants with BA. The best prediction model for LSM in BA using clinical and lab measurements had an R2 = 0.437; adding IL-8 and MMP-7 improved R2 to 0.523 and 0.526 (both p < 0.0001). In participants with A1AT, CTGF and LSM were negatively correlated ( p = 0.004); adding CTGF to an LSM prediction model improved R2 from 0.524 to 0.577 ( p = 0.0033). Biomarkers did not correlate with LSM in ALGS. A significant number of biomarker/lab correlations were found in participants with BA but not those with A1AT or ALGS. CONCLUSIONS: Endoglin, IL-8, and MMP-7 significantly correlate with increased LSM in children with BA, whereas CTGF inversely correlates with LSM in participants with A1AT; these biomarkers appear to enhance prediction of LSM beyond clinical tests. Future disease-specific investigations of change in these biomarkers over time and as predictors of clinical outcomes will be important.


Assuntos
Síndrome de Alagille , Colestase , Técnicas de Imagem por Elasticidade , Hepatopatias , Humanos , Criança , Fígado/patologia , Metaloproteinase 7 da Matriz , Endoglina , Interleucina-8 , Colestase/patologia , Cirrose Hepática/diagnóstico , Cirrose Hepática/patologia , Hepatopatias/patologia , Biomarcadores , Síndrome de Alagille/patologia
2.
Hepatology ; 76(3): 712-726, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35271743

RESUMO

BACKGROUND AND AIMS: The natural history of gastroesophageal variceal hemorrhage (VH) in biliary atresia (BA) is not well characterized. We analyzed risk factors, incidence, and outcomes of VH in a longitudinal multicenter study. APPROACH AND RESULTS: Participants enrolled in either an incident (Prospective Database of Infants with Cholestasis [PROBE]) or prevalent (Biliary Atresia Study of Infants and Children [BASIC]) cohort of BA were included. Variceal hemorrhage (VH) was defined based on gastrointestinal bleeding in the presence of varices accompanied by endoscopic or nontransplant surgical intervention. Cumulative incidence of VH and transplant-free survival was compared based on features of portal hypertension (e.g., splenomegaly, thrombocytopenia) and clinical parameters at baseline in each cohort (PROBE: 1.5 to 4.5 months after hepatoportoenterostomy [HPE]; BASIC: at enrollment > 3 years of age). Analyses were conducted on 869 children with BA enrolled between June 2004 and December 2020 (521 in PROBE [262 (51%) with a functioning HPE] and 348 in BASIC). The overall incidence of first observed VH at 5 years was 9.4% (95% CI: 7.0-12.4) in PROBE and 8.0% (5.2-11.5) in BASIC. Features of portal hypertension, platelet count, total bilirubin, aspartate aminotransferase (AST), albumin, and AST-to-platelet ratio index at baseline were associated with an increased risk of subsequent VH in both cohorts. Transplant-free survival at 5 years was 45.1% (40.5-49.6) in PROBE and 79.2% (74.1-83.4) in BASIC. Two (2.5%) of 80 participants who had VH died, whereas 10 (12.5%) underwent transplant within 6 weeks of VH. CONCLUSIONS: The low risk of VH and associated mortality in children with BA needs to be considered in decisions related to screening for varices and primary prophylaxis of VH.


Assuntos
Atresia Biliar , Varizes Esofágicas e Gástricas , Hipertensão Portal , Varizes , Atresia Biliar/complicações , Atresia Biliar/cirurgia , Criança , Varizes Esofágicas e Gástricas/complicações , Hemorragia Gastrointestinal/epidemiologia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/prevenção & controle , Humanos , Hipertensão Portal/etiologia , Lactente , Varizes/complicações
3.
Pediatr Res ; 93(5): 1348-1353, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-35927573

RESUMO

BACKGROUND: More than half of children with pediatric acute liver failure (PALF) experience hepatic encephalopathy (HE), which is related to poor outcomes; however, HE is difficult to diagnose in children. The objective of this study was to evaluate if heart rate variability (HRV), a continuous measure of autonomic nervous system function, was related to the presence and severity of HE as well as clinical outcomes in children with PALF. METHODS: We conducted a retrospective observational cohort study of 38 critically ill children with PALF to examine the association between HRV and HE severity and clinical outcome. HRV was estimated using the integer HRV (HRVi). Categorical variables were compared using the Fisher Exact test and continuous variables were compared using Kruskal-Wallis tests. Associations between grades of HE and minimum and median HRVi were evaluated with Pearson's correlation, with p values <0.05 considered significant. RESULTS: A more negative median and minimum HRVi, indicating poorer autonomic nervous system function, was significantly associated with abnormal EEG findings, presence of HE, and poor outcomes (death or listing for transplant). CONCLUSIONS: Heart rate variability may hold promise to predict outcomes in children with PALF, but these findings should be replicated in a larger sample. IMPACT: The findings of our study suggest that heart rate variability is associated with clinical outcomes in children with acute liver failure, a cohort for which prognostics are challenging, especially in young children and infants. Use of heart rate variability in the clinical setting may facilitate earlier detection of children with pediatric acute liver failure (PALF) at high risk for severe hepatic encephalopathy and poor outcomes. Identification of children with PALF at high risk of decompensation may assist clinicians in making decisions about liver transplantation, an important, but resource-limited, treatment of PALF.


Assuntos
Encefalopatia Hepática , Falência Hepática Aguda , Transplante de Fígado , Lactente , Criança , Humanos , Pré-Escolar , Frequência Cardíaca , Encefalopatia Hepática/diagnóstico , Encefalopatia Hepática/complicações , Estudos Retrospectivos , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/terapia
4.
Curr Gastroenterol Rep ; 25(7): 169-173, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37303027

RESUMO

PURPOSE OF REVIEW: Anastomotic ulceration following intestinal resection is an under- recognized problem in pediatrics. We discuss the relevant literature regarding this condition. RECENT FINDINGS: Anastomotic Ulceration following intestinal resection is a potentially life threatening cause of refractory anemia. Evaluation should include correction of micronutrient deficiencies and endoscopic evaluation by upper and lower endoscopy and small intestinal endoscopy if necessary. Initial treatment by medical therapy may consist of anti-inflammatory agents as well as antibiotics to treat small intestinal bacterial overgrowth. Surgical resection should be considered if refractory to treatment. Anastomotic ulcers in pediatric patients with small bowel resection should be considered as a cause of refractory iron deficiency anemia. Endoscopic evaluation should be undertaken to look for evidence of anastomotic ulcers. Surgical resection should be considered if medical therapy fails.


Assuntos
Gastroenteropatias , Úlcera , Humanos , Criança , Úlcera/diagnóstico , Úlcera/etiologia , Úlcera/terapia , Intestinos , Biópsia , Endoscopia Gastrointestinal
5.
J Pediatr ; 245: 117-122, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34560095

RESUMO

OBJECTIVE: To characterize a multi-institutional cohort of pediatric patients who underwent colectomy for familial adenomatous polyposis (FAP). STUDY DESIGN: In this retrospective cohort study, diagnosis and procedure codes were used to identify patients who underwent colectomy for FAP within the Pediatric Health Information System (PHIS). The inclusion criteria were validated at 3 children's hospitals and applied to PHIS to generate a cohort of patients with FAP between 2 and 21 years who had undergone colectomy between 2009 and 2019. Demographics, clinical and surgical characteristics, and endoscopic procedure trends as identified through PHIS are described. Descriptive and comparative statistics were used to analyze data. RESULTS: Within the PHIS, 428 pediatric patients with FAP who underwent colectomy were identified. Median age at colectomy was 14 years (range 2-21 years); 264 patients (62%) received an ileal pouch anal anastomosis and 13 (3%) underwent ileorectal anastomosis. Specific anastomotic surgical procedure codes were not reported for 151 patients (35%). Endoscopic assessment at the surgical institution occurred in 40% of the cohort before colectomy and in 22% of the cohort following colectomy. CONCLUSIONS: In this cohort, colectomy took place at an earlier age than suggested in published guidelines. Ileal pouch anal anastomosis is the predominant procedure for pediatric patients with FAP who underwent colectomy in US pediatric centers. Endoscopic assessment trends before and after surgery suggest that the surgical institution plays a limited role in the care of this population.


Assuntos
Polipose Adenomatosa do Colo , Proctocolectomia Restauradora , Polipose Adenomatosa do Colo/cirurgia , Adolescente , Adulto , Anastomose Cirúrgica , Criança , Pré-Escolar , Colectomia/métodos , Humanos , Íleo/cirurgia , Proctocolectomia Restauradora/métodos , Estudos Retrospectivos , Estados Unidos/epidemiologia , Adulto Jovem
6.
J Pediatr Gastroenterol Nutr ; 73(2): 141-144, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34016882

RESUMO

ABSTRACT: Transient elastography is an imaging technique utilizing shear wave technology to measure liver stiffness. Recent studies have shown success in utilizing this technique in children. Transient elastography is useful in estimating degree of fibrosis in various pediatric liver diseases, including biliary atresia, alpha-1-antitrypsin deficiency, Alagille syndrome, cystic fibrosis-related liver disease, and non-alcoholic steatohepatitis among others. Confounding factors, however, may affect elastography measurements, such as obesity, severe inflammation, nonfasting state, and hepatic congestion, and should be considered whenever interpreting these measurements. Future studies will correlate liver stiffness on transient elastography and severity of disease.


Assuntos
Atresia Biliar , Técnicas de Imagem por Elasticidade , Hepatopatia Gordurosa não Alcoólica , Criança , Previsões , Humanos , Fígado/diagnóstico por imagem , Cirrose Hepática/diagnóstico por imagem , Hepatopatia Gordurosa não Alcoólica/diagnóstico por imagem
7.
J Pediatr Gastroenterol Nutr ; 73(2): 169-177, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34016879

RESUMO

OBJECTIVES: To advance our understanding of monogenic forms of intrahepatic cholestasis. METHODS: Analyses included participants with pathogenic biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 11 (ABCB11) (bile salt export pump; BSEP) or adenosine triphosphatase (ATPase) phospholipid transporting 8B1 (ATP8B1) (familial intrahepatic cholestasis; FIC1), or those with monoallelic or biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 4 (ABCB4) (multidrug resistance; MDR3), prospectively enrolled in the Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis (LOGIC; NCT00571272) between November 2007 and December 2013. Summary statistics were calculated to describe baseline demographics, history, anthropometrics, laboratory values, and mutation data. RESULTS: Ninety-eight participants with FIC1 (n = 26), BSEP (n = 53, including 8 with biallelic truncating mutations [severe] and 10 with p.E297G or p.D482G [mild]), or MDR3 (n = 19, including four monoallelic) deficiency were analyzed. Thirty-five had a surgical interruption of the enterohepatic circulation (sEHC), including 10 who underwent liver transplant (LT) after sEHC. Onset of symptoms occurred by age 2 years in most with FIC1 and BSEP deficiency, but was later and more variable for MDR3. Pruritus was nearly universal in FIC1 and BSEP deficiency. In participants with native liver, failure to thrive was common in FIC1 deficiency, high ALT was common in BSEP deficiency, and thrombocytopenia was common in MDR3 deficiency. sEHC was successful after more than 1 year in 7 of 19 participants with FIC1 and BSEP deficiency. History of LT was most common in BSEP deficiency. Of 102 mutations identified, 43 were not previously reported. CONCLUSIONS: In this cohort, BSEP deficiency appears to be correlated with a more severe disease course. Genotype-phenotype correlations in these diseases are not straightforward and will require the study of larger cohorts.


Assuntos
Colestase Intra-Hepática , Colestase , Transportadores de Cassetes de Ligação de ATP/genética , Criança , Pré-Escolar , Colestase/genética , Colestase Intra-Hepática/genética , Humanos , Estudos Longitudinais , Mutação
8.
J Pediatr ; 227: 81-86.e4, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32663593

RESUMO

OBJECTIVES: To identify predictors of portal hypertension, liver transplantation, and death in North American youth with alpha-1-antitrypsin (AAT) deficiency, and compare with patients with AAT deficiency elsewhere. STUDY DESIGN: The Childhood Liver Disease Research Network Longitudinal Observational Study of Genetic Causes of Intrahepatic Cholestasis is a prospective, cohort study of pediatric cholestatic liver diseases, including AAT deficiency, enrolling PIZZ and PISZ subjects 0-25 years of age seen since November 2007 at 17 tertiary care centers in the US and Canada. Data from standard-of-care baseline and annual follow-up visits were recorded from medical records, history, physical examination, and laboratory studies. Participants with portal hypertension were identified based on data collected. RESULTS: We enrolled 350 participants (60% male) with a native liver; 278 (79%) entered the cohort without portal hypertension and 18 developed portal hypertension during follow-up. Thirty participants required liver transplantation; 2 patients died during 1077 person-years of follow-up. There was no difference in participants with or without preceding neonatal cholestasis progressing to transplantation or death during the study (12% vs 7%; P = .09), or in experiencing portal hypertension (28% vs 21%; P = .16); the hazard ratio for neonatal cholestasis leading to portal hypertension was P = .04. Development of portal hypertension was associated with a reduced height Z-score. CONCLUSIONS: Portal hypertension in youth with AAT deficiency impacts growth measures. Progression to liver transplantation is slow and death is rare, but the risk of complications and severe liver disease progression persists throughout childhood. A history of neonatal cholestasis is a weak predictor of severe disease.


Assuntos
Colestase Intra-Hepática/complicações , Hipertensão Portal/etiologia , Deficiência de alfa 1-Antitripsina/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Hipertensão Portal/cirurgia , Lactente , Recém-Nascido , Transplante de Fígado , Estudos Longitudinais , Masculino , Adulto Jovem , Deficiência de alfa 1-Antitripsina/sangue
9.
J Pediatr Gastroenterol Nutr ; 70(5): 562-567, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31977949

RESUMO

OBJECTIVES: Barrett esophagus (BE) and intestinal metaplasia of gastroesophageal junction (IMGEJ) are rare in the pediatric population. This multi-institutional retrospective study evaluated the clinicopathologic characteristics and natural history of BE and IMGEJ in children. METHODS: Data from 20 BE patients (70% boys, mean age: 14.9 years) and 17 IMGEJ patients (71% boys, mean age: 14 years) were retrospectively obtained from chart review. Endoscopic and pathologic findings from index and follow-up endoscopies were analyzed. RESULTS: Most patients (70% BE and 59% IMGEJ) had underlying conditions which put them at risk for gastroesophageal reflux disease. Increased body mass index (BMI) was observed in patients without underlying conditions (BE: 30.1 ±â€Š9.8; IMGEJ: 23.9 ±â€Š6.3) compared with those with underlying conditions (BE: 19.6 ±â€Š7.8; IMGEJ: 16.4 ±â€Š2.1) (BE, P = 0.02; IMGEJ, P = 0.01). Incomplete intestinal metaplasia (IM) was the predominant histology seen in BE (80%) and IMGEJ patients (75%). Dysplasia and malignancy were not identified in the initial and follow-up biopsies. Concurrent gastric biopsies showed various findings (79% BE and 40% IMGEJ were normal), with 1 IMGEJ patient showing coexisting gastric IM (7%). Follow-up in 12 BE patients (mean follow-up time 51.6 months) showed 100% persistent endoscopic disease and 58% persistent IM histologically. Three of 6 IMGEJ patients (mean follow-up time 24 months) demonstrated endoscopic and histologic features consistent with BE on subsequent procedures. Moreover, a subset of BE (57%) and IMGEJ patients (67%) who underwent endoscopy before initial diagnosis showed nongoblet columnar mucosa above the anatomic gastroesophageal junction. CONCLUSIONS: Increased BMI may be a risk factor for BE and IMGEJ in pediatric patients without underlying conditions. Nongoblet columnar metaplasia and IMGEJ might represent incomplete forms of BE. Our data suggest that these patients should be closely monitored.


Assuntos
Esôfago de Barrett , Refluxo Gastroesofágico , Adolescente , Biópsia , Criança , Junção Esofagogástrica , Feminino , Refluxo Gastroesofágico/etiologia , Humanos , Masculino , Metaplasia , Estudos Retrospectivos
10.
J Pediatr Gastroenterol Nutr ; 68(6): 763-767, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30908382

RESUMO

Portal hypertension (PHT) is a major cause of morbidity and mortality in pediatric liver diseases. Thus, research into causes and disease modifiers in PHT in these conditions is vitally important. PHT is rarely directly or indirectly measured in the assessment of children with chronic liver disease. A straightforward, reproducible definition of PHT could be invaluable for consistently identifying patients with PHT and for grouping these patients according to their risk of complications from their disease. We propose the term Clinically Evident Portal Hypertension (CEPH) to denote clinical findings that demonstrate evidence of elevated portal pressure. When CEPH criteria are met, PHT is highly likely to be present, although it is likely that PHT exists for variable periods of time before meeting CEPH criteria. Use of this research definition of CEPH will allow for consistent identification of these patients by clinicians in nearly any clinical setting and serve as a clinical milepost that may dictate future prognosis in pediatric patients with cirrhosis.


Assuntos
Gastroenterologia/normas , Hipertensão Portal/diagnóstico , Pediatria/normas , Avaliação de Sintomas/normas , Terminologia como Assunto , Criança , Feminino , Gastroenterologia/métodos , Humanos , Hipertensão Portal/classificação , Fígado/irrigação sanguínea , Masculino , Pediatria/métodos
11.
Hepatology ; 65(5): 1645-1654, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28027587

RESUMO

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs. >24 months postoperatively, respectively; P < 0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. CONCLUSION: This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).


Assuntos
Colestase Intra-Hepática/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/estatística & dados numéricos , Circulação Êntero-Hepática , Adolescente , Criança , Pré-Escolar , Colestase Intra-Hepática/sangue , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
12.
Curr Opin Pediatr ; 30(5): 653-659, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30188872

RESUMO

PURPOSE OF REVIEW: Pediatric foreign body ingestion is a common occurrence that presents a challenge both to pediatric gastroenterologists and primary care providers. Increasing prevalence of smaller, more technologically advanced toys in the household has resulted in an increased exposure to higher voltage batteries and powerful magnets that carry a high incidence of morbidity and mortality. This review highlights the latest findings regarding the patients at risk for button battery and magnet ingestions, the symptoms of presentation, and complications of these objects in contributing to long-standing gastrointestinal injury. RECENT FINDINGS: Button batteries may lead to esophageal injury within a few hours. Batteries retained in the esophagus are larger in diameter on average and size is associated with esophageal impaction as well as higher grade esophageal injury. Magnet ingestions, when multiple or with another metallic object, are often initially asymptomatic but may have acute worsening, and therefore warrant close monitoring. SUMMARY: Button battery and magnet ingestions have increased in incidence over the past two decades. Recent literature demonstrates that higher voltage, larger lithium button batteries, and prevalence of high-powered magnets can lead to significant morbidity. High suspicion, early referral, and removal may lead to improved outcomes.


Assuntos
Queimaduras Químicas/diagnóstico por imagem , Queimaduras Químicas/etiologia , Fontes de Energia Elétrica/efeitos adversos , Endoscopia do Sistema Digestório , Corpos Estranhos/diagnóstico por imagem , Imãs/efeitos adversos , Radiografia , Pré-Escolar , Ingestão de Alimentos , Esôfago/diagnóstico por imagem , Esôfago/lesões , Corpos Estranhos/complicações , Corpos Estranhos/cirurgia , Trato Gastrointestinal/diagnóstico por imagem , Trato Gastrointestinal/lesões , Humanos , Jogos e Brinquedos , Guias de Prática Clínica como Assunto
13.
J Pediatr Gastroenterol Nutr ; 67(4): 441-445, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30028827

RESUMO

OBJECTIVES: Liver biopsy can be a valuable tool to help determine the etiology of pediatric acute liver failure (PALF), but is often not performed due to safety concerns. The primary aim was to describe the incidence of major complications after liver biopsy performed in the setting of PALF. METHODS: Medical records from 2006 to 2016 were reviewed. Patients age 0 to 17 years, who met criteria for PALF, and had a liver biopsy performed while their international normalized ratio (INR) was ≥1.5 were included. RESULTS: A total of 26 cases of liver biopsy in the setting of PALF were identified. The majority (n = 22, 85%) of patients had primary liver disease. Most biopsies (n = 17, 65%) were performed by the transjugular route, with 5 (19%) performed percutaneously under ultrasound guidance and 4 (15%) during a surgical procedure. Median INR before biopsy was 2.1 (IQR = 1.73-2.9). Blood products were given before or during the procedure in 23 (88%) cases. One patient (3.8%) had a major complication of biopsy-associated bleeding requiring a blood transfusion. An additional 3 patients had a hemoglobin decrease of 2.1 to 2.9 g/dL post-biopsy that was attributed to the procedure but no interventions were necessary. Biopsy results contributed to establishing a diagnosis in 62% (n = 16) of cases, and influenced treatment decisions in 9 of those cases. CONCLUSIONS: Liver biopsy is safe in the majority of patients with PALF and associated with infrequent major complications. Clinicians should consider performing liver biopsy in this setting, especially when the transjugular approach is feasible, since findings may guide diagnosis and therapy.


Assuntos
Biópsia/efeitos adversos , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Falência Hepática Aguda/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Adolescente , Biópsia/métodos , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Coeficiente Internacional Normatizado , Fígado/patologia , Fígado/cirurgia , Falência Hepática Aguda/etiologia , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco
14.
Pediatr Transplant ; 21(1)2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27762481

RESUMO

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%-10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7-16 years) with a median Hgb of 2.8 g/dL (range 1.8-6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid-jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant.


Assuntos
Endoscopia por Cápsula , Varizes Esofágicas e Gástricas/diagnóstico , Hipertensão Portal/diagnóstico , Jejuno/irrigação sanguínea , Transplante de Fígado/efeitos adversos , Adolescente , Criança , Varizes Esofágicas e Gástricas/complicações , Feminino , Hemorragia Gastrointestinal/prevenção & controle , Hemoglobinas/análise , Humanos , Hipertensão Portal/complicações , Masculino , Complicações Pós-Operatórias
15.
J Pediatr ; 170: 211-7.e1-2, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26725209

RESUMO

OBJECTIVES: To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life. STUDY DESIGN: Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 µM) at any time in the first 3 months (TB <2.0, all others TB ≥ 2) after HPE was examined as a biomarker, using Kaplan-Meier survival and logistic regression. RESULTS: Fifty percent (68/137) of infants had TB < 2.0 in the first 3 months after HPE. Transplant-free survival at 2 years was significantly higher in the TB < 2.0 group vs TB ≥ 2 (86% vs 20%, P < .0001). Infants with TB ≥ 2 had diminished weight gain (P < .0001), greater probability of developing ascites (OR 6.4, 95% CI 2.9-14.1, P < .0001), hypoalbuminemia (OR 7.6, 95% CI 3.2-17.7, P < .0001), coagulopathy (OR 10.8, 95% CI 3.1-38.2, P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P < .0001), or LT or death (OR 16.8, 95% CI 7.2-39.2, P < .0001). CONCLUSIONS: Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB <2.0 mg/dL within 3 months of HPE may enhance early outcomes. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00061828 and NCT00294684.


Assuntos
Atresia Biliar/cirurgia , Bilirrubina/sangue , Progressão da Doença , Portoenterostomia Hepática , Ascite/epidemiologia , Atresia Biliar/epidemiologia , Biomarcadores/sangue , Canadá/epidemiologia , Pré-Escolar , Bases de Dados Factuais , Coagulação Intravascular Disseminada/epidemiologia , Seguimentos , Transtornos do Crescimento/epidemiologia , Humanos , Hipoalbuminemia/epidemiologia , Lactente , Recém-Nascido , Transplante de Fígado/estatística & dados numéricos , Modelos Logísticos , Prognóstico , Estudos Prospectivos , Estados Unidos/epidemiologia
16.
Liver Int ; 36(5): 755-60, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-26201540

RESUMO

BACKGROUND & AIMS: Liver disease in Alagille syndrome is highly variable. Many of the patients presenting with severe cholestasis early in life improve spontaneously; 10-20%, however, have progressive disease. It is currently not possible to predict long-term hepatic outcomes in Alagille syndrome. This international, multicentre study was aimed at identifying early life predictors of liver disease outcome. METHODS: Retrospective clinical, laboratory and radiographic data from a cohort of 144 Alagille syndrome patients, whose long-term hepatic outcomes had been determined a priori based on previously published criteria, were collected. RESULTS: Sixty-seven patients had mild and 77 had severe hepatic outcome. Univariate analysis demonstrated that cholestasis and fibrosis on biopsy, as well as the presence of xanthomata were significantly different between the groups (P < 0.05 for all). Mixed model analysis revealed that total serum bilirubin and serum cholesterol were also associated with outcome (P = 0.001 and P = 0.002, respectively). Graphical representation of the data revealed a change in total bilirubin levels between 12 and 24 months of age in the mild group. Recursive partitioning identified a threshold for total bilirubin of 3.8 mg/dl (65 mmol/L) in that age-frame that differentiated between outcomes. A multivariable logistic regression model was developed using fibrosis, xanthomata and the total bilirubin cut-off of 3.8 mg/dl (65 mmol/L), which generated an area under the ROC curve of 0.792. CONCLUSIONS: The long-term hepatic outcomes of patients with Alagille syndrome can be predicted based on serum total bilirubin between the ages of 12-24 months combined with fibrosis on liver biopsy and the presence of xanthomata on physical examination.


Assuntos
Síndrome de Alagille/patologia , Síndrome de Alagille/fisiopatologia , Bilirrubina/sangue , Biomarcadores/sangue , Biópsia , Pré-Escolar , Colestase/fisiopatologia , Colesterol/sangue , Europa (Continente) , Feminino , Humanos , Lactente , Cooperação Internacional , Cirrose Hepática/fisiopatologia , Modelos Logísticos , Masculino , Análise Multivariada , América do Norte , Curva ROC , Estudos Retrospectivos
18.
J Pediatr ; 167(3): 706-10.e1, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26163084

RESUMO

OBJECTIVES: To determine abdominal radiograph use and frequency of digital rectal examinations in children presenting to the emergency department (ED) with abdominal pain and suspected constipation and to determine the impact of an educational module on their use. STUDY DESIGN: Retrospective chart review of patients evaluated at a pediatric ED because of the complaint of abdominal pain who had the discharge diagnosis of constipation over two 2-month periods, one before and one after an educational module. RESULTS: Comparing pre- and posteducational module periods, there was a significant decrease in abdominal radiograph utilization (69.5% vs 26.4%, respectively, P ≤ .001) and significant increase in performance of digital rectal examination (22.9% vs 47.3%, respectively, P ≤ .001). We demonstrated a 33.6% reduction in abdominal radiograph in children who had a digital rectal examination as part of their examination. Overall, we demonstrated a 43.1% decrease in patients receiving an abdominal radiograph. When time and costs of an abdominal radiograph are considered, this results in significant cost savings. CONCLUSIONS: An educational module reviewing the established criteria for the diagnosis of constipation and presented to ED providers results in increased use of digital rectal examination and decreased use of abdominal radiograph in patients evaluated for abdominal pain and ultimately diagnosed with constipation. The change also was associated with reduction in cost and time and radiation exposure in the ED for these patients.


Assuntos
Constipação Intestinal/diagnóstico , Exame Retal Digital/estatística & dados numéricos , Serviço Hospitalar de Emergência , Capacitação em Serviço , Radiografia Abdominal/estatística & dados numéricos , Dor Abdominal/etiologia , Adolescente , Chicago , Criança , Pré-Escolar , Redução de Custos , Feminino , Humanos , Masculino , Corpo Clínico/educação , Profissionais de Enfermagem/educação , Estudos Retrospectivos
20.
J Pediatr Gastroenterol Nutr ; 61(2): 215-9, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25806676

RESUMO

OBJECTIVES: Anastomotic ulcers are a known cause of anemia in children with a history of intestinal resection. Upper endoscopy and colonoscopy can be used to diagnose these ulcers; however, the area of involvement may be difficult to visualize with standard endoscopic techniques. Capsule endoscopy (CE) offers an alternative method for visualizing the small bowel in these patients. We describe a cohort of patients with short bowel syndrome (SBS) and anemia who had anastomotic ulcers detected by CE. METHODS: Retrospective chart review of patients with SBS at our institution who underwent CE for chronic gastrointestinal (GI) blood loss. RESULTS: Four patients who underwent a total of 6 CE procedures were identified. The underlying diagnoses included necrotizing enterocolitis (n = 2), gastroschisis (n = 1), and jejunal atresia (n = 1). All of the patients had their ileocecal valves resected during previous surgeries and had received blood transfusions within the previous several months. The median age at the time of CE was 5.5 years (range 4-14 years). Enterocolonic anastomotic ulcers were noted in the studies as wide, flat circumferential lesions with a white base. The CE results guided a change in medical management in all of the 4 patients, including surgical revision of their anastomosis. CONCLUSIONS: CE may be a helpful adjunctive tool for detecting anastomotic ulcers in patients with SBS and chronic GI blood loss.


Assuntos
Endoscopia por Cápsula , Enteropatias/patologia , Síndrome do Intestino Curto/complicações , Úlcera/patologia , Adolescente , Anemia/etiologia , Endoscopia por Cápsula/métodos , Criança , Pré-Escolar , Feminino , Hemorragia Gastrointestinal/etiologia , Humanos , Masculino , Estudos Retrospectivos , Úlcera/complicações , Úlcera/cirurgia
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