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1.
Curr Gastroenterol Rep ; 25(7): 169-173, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37303027

RESUMEN

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.


Asunto(s)
Enfermedades Gastrointestinales , Úlcera , Humanos , Niño , Úlcera/diagnóstico , Úlcera/etiología , Úlcera/terapia , Intestinos , Biopsia , Endoscopía Gastrointestinal
2.
Gastrointest Endosc Clin N Am ; 33(2): 401-421, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36948753

RESUMEN

Upper gastrointestinal bleeding (UGIB) in children has many causes, with its prevalence varying by age. Often presenting as hematemesis or melena, the initial treatment is stabilization of the patient, including protection of the airway, fluid resuscitation, and a transfusion hemoglobin threshold of 7 g/L. Endoscopy should be performed with the goal of using combinations of therapies to treat a bleeding lesion, generally involving epinephrine injection along with either cautery, hemoclips, or hemospray. This review discusses the diagnosis and treatment of variceal and non-variceal gastrointestinal bleeding in children with a focus on current advances in the treatment of severe UGIB.


Asunto(s)
Várices Esofágicas y Gástricas , Humanos , Niño , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/terapia , Endoscopía Gastrointestinal/efectos adversos , Prevalencia
3.
Hepatology ; 77(2): 530-545, 2023 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-36069569

RESUMEN

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.


Asunto(s)
Síndrome de Alagille , Colestasis , Diagnóstico por Imagen de Elasticidad , Hepatopatías , Humanos , Niño , Hígado/patología , Metaloproteinasa 7 de la Matriz , Endoglina , Interleucina-8 , Colestasis/patología , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/patología , Hepatopatías/patología , Biomarcadores , Síndrome de Alagille/patología
4.
Inflamm Bowel Dis ; 28(10): 1537-1542, 2022 10 03.
Artículo en Inglés | MEDLINE | ID: mdl-34964861

RESUMEN

BACKGROUND: Endoscopic mucosal healing is the gold standard for evaluating Crohn's disease (CD) treatment efficacy. Standard endoscopic indices are not routinely used in clinical practice, limiting the quality of retrospective research. A method for retrospectively quantifying mucosal activity from documentation is needed. We evaluated the simplified endoscopic mucosal assessment for CD (SEMA-CD) to determine if it can accurately quantify mucosal severity recorded in colonoscopy reports. METHODS: Pediatric patients with CD underwent colonoscopy that was video recorded and evaluated via Simple Endoscopic Score for CD (SES-CD) and SEMA-CD by central readers. Corresponding colonoscopy reports were de-identified. Central readers blinded to clinical history and video scoring were randomly assigned colonoscopy reports with and without images. The SEMA-CD was scored for each report. Correlation with video SES-CD and SEMA-CD were assessed with Spearman rho, inter-rater, and intrarater reliability with kappa statistics. RESULTS: Fifty-seven colonoscopy reports were read a total of 347 times. The simplified endoscopic mucosal assessment for CD without images correlated with both SES-CD and SEMA-CD from videos (rho = 0.82, P < .0001 for each). The addition of images provided similar correlation. Inter-rater and intrarater reliability were 0.93 and 0.92, respectively. CONCLUSIONS: The SEMA-CD applied to retrospective evaluation of colonoscopy reports accurately and reproducibly correlates with SES-CD and SEMA-CD of colonoscopy videos. The SEMA-CD for evaluating colonoscopy reports will enable quantifying mucosal healing in retrospective research. Having objective outcome data will enable higher-quality research to be conducted across multicenter collaboratives and in clinical registries. External validation is needed.


Asunto(s)
Enfermedad de Crohn , Niño , Colonoscopía , Enfermedad de Crohn/diagnóstico por imagen , Enfermedad de Crohn/tratamiento farmacológico , Humanos , Reproducibilidad de los Resultados , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
6.
J Pediatr ; 245: 117-122, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-34560095

RESUMEN

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.


Asunto(s)
Poliposis Adenomatosa del Colon , Proctocolectomía Restauradora , Poliposis Adenomatosa del Colon/cirugía , Adolescente , Adulto , Anastomosis Quirúrgica , Niño , Preescolar , Colectomía/métodos , Humanos , Íleon/cirugía , Proctocolectomía Restauradora/métodos , Estudios Retrospectivos , Estados Unidos/epidemiología , Adulto Joven
8.
J Pediatr Gastroenterol Nutr ; 73(2): 169-177, 2021 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-34016879

RESUMEN

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.


Asunto(s)
Colestasis Intrahepática , Colestasis , Transportadoras de Casetes de Unión a ATP/genética , Niño , Preescolar , Colestasis/genética , Colestasis Intrahepática/genética , Humanos , Estudios Longitudinales , Mutación
9.
J Pediatr Gastroenterol Nutr ; 73(2): 141-144, 2021 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-34016882

RESUMEN

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.


Asunto(s)
Atresia Biliar , Diagnóstico por Imagen de Elasticidad , Enfermedad del Hígado Graso no Alcohólico , Niño , Predicción , Humanos , Hígado/diagnóstico por imagen , Cirrosis Hepática/diagnóstico por imagen , Enfermedad del Hígado Graso no Alcohólico/diagnóstico por imagen
10.
Inflamm Bowel Dis ; 27(10): 1585-1592, 2021 10 18.
Artículo en Inglés | MEDLINE | ID: mdl-33382069

RESUMEN

OBJECTIVES: Endoscopic mucosal improvement is the gold standard for assessing treatment efficacy in clinical trials of Crohn's disease. Current endoscopic indices are not routinely used in clinical practice. The lack of endoscopic information in large clinical registries limits their use for research. A quick, easy, and accurate method is needed for assessing mucosal improvement for clinicians in real-world practice. We developed and tested a novel simplified endoscopic mucosal assessment for Crohn's disease (SEMA-CD). METHODS: We developed a 5-point scale for ranking endoscopic severity of ileum and colon based on Simple Endoscopic Score for Crohn's disease (SES-CD). Central readers were trained to perform SES-CD and SEMA-CD. Pediatric patients with Crohn's disease undergoing colonoscopy were enrolled. Video recordings of colonoscopies were de-identified and randomly assigned to blinded central readers. The SES-CD and SEMA-CD were scored for each video. The SES-CD was considered the validated standard for comparison. Correlation was assessed with Spearman rho, inter- and intrarater reliability with kappa statistics. RESULTS: Fifty-seven colonoscopies were read a total of 212 times. Correlation between SEMA-CD and SES-CD was strong (rho = 0.98, P < 0.0001). Inter-rater reliability for SEMA-CD was 0.80, and intrarater reliability was 0.83. Central readers rated SEMA-CD as easier than SES-CD. CONCLUSION: The SEMA-CD accurately and reproducibly correlates with the standard SES-CD. Central readers viewed SEMA-CD as easier than SES-CD. Use of SEMA-CD in practice should enable collecting mucosal improvement information in large populations of patients. This will improve the quality of research that can be conducted in clinical registries. External validation is needed.


Asunto(s)
Enfermedad de Crohn , Niño , Colon/fisiopatología , Colonoscopía/métodos , Enfermedad de Crohn/diagnóstico , Humanos , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad
11.
Cancer Prev Res (Phila) ; 14(2): 215-222, 2021 02.
Artículo en Inglés | MEDLINE | ID: mdl-33097490

RESUMEN

Juvenile polyposis syndrome (JPS) is a clinically diagnosed hamartomatous polyposis syndrome that increases the risk of gastrointestinal cancer. Approximately 40%-50% of JPS is caused by a germline disease-causing variant (DCV) in the SMAD4 or BMPR1A genes. The aim of this study was to characterize the phenotype of DCV-negative JPS and compare it with DCV-positive JPS. Herein, we analyzed a cohort of 145 individuals with JPS from nine institutions, including both pediatric and adult centers. Data analyzed included age at diagnosis, family history, cancer history, need for colectomy/gastrectomy, and polyp number and location. Compared with DCV-positive JPS, DCV-negative JPS was associated with younger age at diagnosis (P < 0.001), lower likelihood of having a family history of JPS (P < 0.001), and a lower risk of colectomy (P = 0.032). None of the DCV-negative individuals had gastric or duodenal polyps, and polyp burden decreased after the first decade compared with DCV-positive JPS. Subgroup analysis between SMAD4 and BMPR1A carriers showed that SMAD4 carriers were more likely to have a family history of JPS and required gastrectomy. Taken together, these data provide the largest phenotypic characterization of individuals with DCV-negative JPS to date, showing that this group has distinct differences compared with JPS due to a SMAD4 or BMPR1A variant. Better understanding of phenotype and cancer risk associated with JPS both with and without a DCV may ultimately allow for individualized management of polyposis and cancer risk.Prevention Relevance: Juvenile Polyposis Syndrome (JPS) is a gastrointestinal cancer predisposition syndrome requiring lifelong surveillance, however there is limited data comparing individuals with and without a germline disease-causing variant in SMAD4 or BMPR1A Herein we show that individuals with JPS without an underlying disease-causing variant have distinct phenotypic differences including lack of upper gastrointestinal polyps and lower rates of a family history of JPS, suggesting that a different approach to management may be appropriate in this population.


Asunto(s)
Receptores de Proteínas Morfogenéticas Óseas de Tipo 1/genética , Colectomía/estadística & datos numéricos , Poliposis Intestinal/congénito , Síndromes Neoplásicos Hereditarios/genética , Proteína Smad4/genética , Espera Vigilante/estadística & datos numéricos , Adolescente , Adulto , Factores de Edad , Anciano , Niño , Preescolar , Colectomía/normas , Colonoscopía/normas , Colonoscopía/estadística & datos numéricos , Femenino , Estudios de Seguimiento , Mutación de Línea Germinal , Humanos , Poliposis Intestinal/diagnóstico , Poliposis Intestinal/genética , Poliposis Intestinal/terapia , Masculino , Anamnesis/estadística & datos numéricos , Persona de Mediana Edad , Síndromes Neoplásicos Hereditarios/diagnóstico , Síndromes Neoplásicos Hereditarios/terapia , Guías de Práctica Clínica como Asunto , Medicina de Precisión/métodos , Medicina de Precisión/estadística & datos numéricos , Espera Vigilante/normas , Adulto Joven
12.
JPGN Rep ; 2(3): e095, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37205964

RESUMEN

ARPC1B is important in the maintenance and assembly of the ARP2/3 complex. Loss of this complex due to ARPC1B mutation results in impairment of actin polymerization and subsequent defects in chemotaxis, cell migration, and DNA repair. Individuals with this rare mutation present in infancy and have abnormal innate and adaptive immune responses. They develop immune-mediated inflammatory disease with associated platelet defects, eosinophilia, rashes, and bowel disease. Recurrent gastrointestinal hemorrhage has been described in known cases. Here, we report a case with endoscopic and histologic findings in a patient with this rare mutation.

14.
J Pediatr Gastroenterol Nutr ; 70(5): 562-567, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-31977949

RESUMEN

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.


Asunto(s)
Esófago de Barrett , Reflujo Gastroesofágico , Adolescente , Biopsia , Niño , Unión Esofagogástrica , Femenino , Reflujo Gastroesofágico/etiología , Humanos , Masculino , Metaplasia , Estudios Retrospectivos
15.
Curr Opin Pediatr ; 30(5): 653-659, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30188872

RESUMEN

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.


Asunto(s)
Quemaduras Químicas/diagnóstico por imagen , Quemaduras Químicas/etiología , Suministros de Energía Eléctrica/efectos adversos , Endoscopía del Sistema Digestivo , Cuerpos Extraños/diagnóstico por imagen , Imanes/efectos adversos , Radiografía , Preescolar , Ingestión de Alimentos , Esófago/diagnóstico por imagen , Esófago/lesiones , Cuerpos Extraños/complicaciones , Cuerpos Extraños/cirugía , Tracto Gastrointestinal/diagnóstico por imagen , Tracto Gastrointestinal/lesiones , Humanos , Juego e Implementos de Juego , Guías de Práctica Clínica como Asunto
16.
J Pediatr Gastroenterol Nutr ; 67(4): 441-445, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30028827

RESUMEN

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.


Asunto(s)
Biopsia/efectos adversos , Procedimientos Quirúrgicos del Sistema Digestivo/efectos adversos , Fallo Hepático Agudo/diagnóstico , Complicaciones Posoperatorias/epidemiología , Adolescente , Biopsia/métodos , Niño , Preescolar , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Relación Normalizada Internacional , Hígado/patología , Hígado/cirugía , Fallo Hepático Agudo/etiología , Masculino , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Factores de Riesgo
17.
Hepatology ; 65(5): 1645-1654, 2017 05.
Artículo en Inglés | MEDLINE | ID: mdl-28027587

RESUMEN

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).


Asunto(s)
Colestasis Intrahepática/cirugía , Procedimientos Quirúrgicos del Sistema Digestivo/estadística & datos numéricos , Circulación Enterohepática , Adolescente , Niño , Preescolar , Colestasis Intrahepática/sangre , Procedimientos Quirúrgicos del Sistema Digestivo/efectos adversos , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
18.
J Pediatr Surg ; 52(2): 268-272, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27916445

RESUMEN

BACKGROUND/PURPOSE: PFIC is a family of bile acid (BA) transport disorders that may result in serious liver disease requiring transplantation. We reviewed our experience with PEBD as a method to improve liver function and avoid transplantation. METHODS: All patients with PFIC were reviewed. Outcomes included changes in serum BA, conversion to ileal bypass (IB), and survival without transplantation. Statistics were obtained using paired t-test and Wilcoxon test. RESULTS: Thirty-five patients with PFIC were identified. Data were available in 24. Twenty-four children (12 males) underwent PEBD: 10 PFIC-1, 13 PFIC-2, and one PFIC-3. BA levels decreased in PFIC-1 patients (1724±3215 to 11±6µmol/L, P=0.03) and in the single PFIC-3 patient (821 to 11.2µmol/L), but not significantly in PFIC-2 patients (193±99 to 141±118µmol/L, P=0.15). Seven patients were converted to IB. There were no significant changes in BA levels following conversion. Five-year transplant-free survival was 100% in PFIC-1 and PFIC-3, but only 38% (5/13) in PFIC-2 (P=0.004). CONCLUSION: PEBD is an effective procedure to reduce total BA levels and improve symptoms in PFIC patients. However, it appears to be less efficacious in the PFIC-2 group. The higher BA levels could contribute to ongoing liver damage, and thus a higher transplant rate in PFIC-2 patients. LEVEL OF EVIDENCE: Level IV.


Asunto(s)
Subfamilia B de Transportador de Casetes de Unión a ATP/deficiencia , Procedimientos Quirúrgicos del Sistema Biliar/métodos , Colestasis Intrahepática/cirugía , Anastomosis Quirúrgica , Niño , Preescolar , Femenino , Vesícula Biliar/cirugía , Humanos , Lactante , Recién Nacido , Yeyuno/cirugía , Masculino , Estudios Retrospectivos , Estomas Quirúrgicos , Resultado del Tratamiento
19.
Liver Int ; 36(5): 755-60, 2016 05.
Artículo en Inglés | MEDLINE | ID: mdl-26201540

RESUMEN

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.


Asunto(s)
Síndrome de Alagille/patología , Síndrome de Alagille/fisiopatología , Bilirrubina/sangre , Biomarcadores/sangre , Biopsia , Preescolar , Colestasis/fisiopatología , Colesterol/sangre , Europa (Continente) , Femenino , Humanos , Lactante , Cooperación Internacional , Cirrosis Hepática/fisiopatología , Modelos Logísticos , Masculino , Análisis Multivariante , América del Norte , Curva ROC , Estudios Retrospectivos
20.
J Pediatr Gastroenterol Nutr ; 61(2): 215-9, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25806676

RESUMEN

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.


Asunto(s)
Endoscopía Capsular , Enfermedades Intestinales/patología , Síndrome del Intestino Corto/complicaciones , Úlcera/patología , Adolescente , Anemia/etiología , Endoscopía Capsular/métodos , Niño , Preescolar , Femenino , Hemorragia Gastrointestinal/etiología , Humanos , Masculino , Estudios Retrospectivos , Úlcera/complicaciones , Úlcera/cirugía
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