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Previous studies have demonstrated the safety of performing endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population; however, few have addressed the outcomes of children undergoing ERCP during acute pancreatitis (AP). We hypothesize that ERCP performed in the setting of AP can be executed with similar technical success and adverse event profiles to those in pediatric patients without pancreatitis. Using the Pediatric ERCP Database Initiative, a multi-national and multi-institutional prospectively collected dataset, we analyzed 1124 ERCPs. One hundred and ninety-four (17%) of these procedures were performed in the setting of AP. There were no difference in the procedure success rate, procedure time, cannulation time, fluoroscopy time, or American Society of Anesthesiology class despite patients with AP having higher American Society of Gastrointestinal Endoscopy grading difficulty scores. This study suggests that ERCP can be safely and efficiently performed in pediatric patients with AP when appropriately indicated.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatite , Criança , Humanos , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Pancreatite/diagnóstico por imagem , Pancreatite/cirurgia , Pancreatite/epidemiologia , Doença Aguda , Estudos Retrospectivos , FluoroscopiaRESUMO
This study examines the role of trainee involvement with pediatric endoscopic retrograde cholangiopancreatography (ERCP) and whether it affects the procedure's success, post-procedural adverse outcomes, and duration. A secondary analysis of the Pediatric ERCP Database Initiative, an international database, was performed. Consecutive ERCPs on children <19 years of age from 18 centers were entered prospectively into the database. In total 1124 ERCPs were entered into the database, of which 320 (28%) were performed by trainees. The results showed that the presence of trainees did not impact technical success ( P = 0.65) or adverse events rates ( P = 0.43). Rates of post-ERCP pancreatitis, pain, and bleeding were similar between groups ( P > 0.05). Fewer cases involving trainees were in the top quartile (>58 minutes) of procedural time (19% vs 26%; P = 0.02). Overall, our findings indicate trainee involvement in pediatric ERCP is safe.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatite , Criança , Humanos , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Pancreatite/epidemiologia , Pancreatite/etiologia , Estudos RetrospectivosRESUMO
OBJECTIVES: Chronic pancreatitis (CP) is rare in childhood but impactful because of its high disease burden. There is limited literature regarding the management of CP in children, specifically about the various surgical approaches. Herein, we summarize the current pediatric and adult literature and provide recommendations for the surgical management of CP in children. METHODS: The literature review was performed to include the scope of the problem, indications for operation, conventional surgical options as well as total pancreatectomy with islet autotransplantation, and outcomes following operations for CP. RESULTS: Surgery is indicated for children with debilitating CP who have failed maximal medical and endoscopic interventions. Surgical management must be tailored to the patient's unique needs, considering the anatomy and morphology of their disease. A conventional surgical approach (eg, drainage operation, partial resection, combination drainage-resection) may be considered in the presence of significant and uniform pancreatic duct dilation or an inflammatory head mass. Total pancreatectomy with islet autotransplantation is the best surgical option in patients with small duct disease. The presence of genetic risk factors often portends a suboptimal outcome following a conventional operation. CONCLUSIONS: The morphology of disease and the presence of genetic risk factors must be considered while determining the optimal surgical approach for children with CP. Surgical outcomes for CP are variable and depend on the type of intervention. A multidisciplinary team approach is needed to assure that the best possible operation is selected for each patient, their recovery is optimized, and their immediate and long-term postoperative needs are well-met.
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Gastroenterologia , Pancreatite Crônica , Adulto , Criança , Humanos , América do Norte , Pâncreas/cirurgia , Pancreatectomia/efeitos adversos , Pancreatite Crônica/etiologia , Pancreatite Crônica/cirurgiaRESUMO
OBJECTIVES: Endoscopic retrograde cholangiopancreatography (ERCP) in adults has been extensively studied through multicenter prospective studies. Similar pediatric studies are lacking. The Pediatric ERCP Database Initiative (PEDI) is a multicenter collaborative aiming to evaluate the indications and technical outcomes in pediatric ERCPs. METHODS: In this prospective cohort study, data were recorded for pediatric ERCPs performed across 15 centers. A pre-procedure, procedure, 2-week post-procedure follow-up, and adverse event form were completed for each ERCP performed. Univariate and stepwise linear regression was performed to identify factors associated with technically successful procedures and adverse events. RESULTS: A total of 1124 ERCPs were performed on 857 patients from May 1, 2014 to May 1, 2018. The median age was 13.5 years [interquartile range (IQR) 9.0-15.7]. Procedures were technically successful in the majority of cases (90.5%) with success more commonly encountered for procedures with biliary indications [odds ratio (OR) 4.2] and less commonly encountered for native papilla anatomy (OR 0.4) and in children <3 years (OR 0.3). Cannulation was more often successful with biliary cannulation (95.9%) compared to pancreatic cannulation via the major papilla (89.6%, P < 0.0001) or minor papilla (71.2%, P < 0.0005). The most commonly identified adverse events included post-ERCP pancreatitis (5%), pain not related to post-ERCP pancreatitis (1.8%), and bleeding (1.2%). Risk factors for the development of each were identified. CONCLUSIONS: This large prospective study demonstrates that ERCP is reliable and safe in the pediatric population. It highlights the utility of PEDI in evaluating the technical outcomes of pediatric ERCPs and demonstrates the potential of PEDI for future studies in pediatric ERCPs.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatite , Humanos , Criança , Adulto , Adolescente , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Estudos Prospectivos , Estudos Retrospectivos , Cateterismo/efeitos adversos , Pancreatite/etiologiaRESUMO
BACKGROUND AND AIMS: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP. METHODS: ERCP on children <18âyears from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018. Data were retrospectively evaluated for outcome and quality measures. A univariate and step-wise linear regression analysis was performed to identify factors associated with increased FT. RESULTS: 1073 ERCPs performed in 816 unique patients met inclusion criteria. Median age was 12.2âyears (interquartile range [IQR] 9.3-15.8). 767 (71%) patients had native papillae. The median FT was 120âseconds (IQR 60-240). Factors associated with increased FT included procedures performed on patients with chronic pancreatitis, ERCPs with American Society of Gastrointestinal Endoscopy (ASGE) difficulty grade >3, ERCPs performed by pediatric gastroenterologist (GI) with adult GI supervision, and ERCPs performed at non-free standing children's hospitals. Hispanic ethnicity was the only factor associated with lower FT. CONCLUSION: Several factors were associated with prolonged FTs in pediatric ERCP that differed from adult studies. This underscores that adult quality indicators cannot always be translated to pediatric patients. This data can better identify children with higher risk for radiation exposure and improve quality outcomes during pediatric ERCP.
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Colangiopancreatografia Retrógrada Endoscópica , Exposição à Radiação , Adulto , Criança , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Estudos de Coortes , Fluoroscopia/efeitos adversos , Humanos , Exposição à Radiação/efeitos adversos , Estudos RetrospectivosRESUMO
OBJECTIVES: Differences in the initial management of pediatric eosinophilic esophagitis (EoE) by practice setting have not been well characterized. We aimed to characterize these differences for sites in the Carolinas EoE Collaborative (CEoEC), a multicenter network of academic and community practices. METHODS: We performed a retrospective cohort study of pediatric EoE patients at five CEoEC sites: University of North Carolina (UNC) Hospital, Charlotte Asthma and Allergy Specialists, Greenville Health Systems, Wake Forest Baptist Medical Center, and the Medical University of South Carolina Hospital. Cases of EoE were defined by consensus guidelines. Data were extracted from electronic medical records. We tested for differences among sites and used a multinomial model (polytomous regression) to assess associations between treatment and site, adjusting on patient factors. RESULTS: We identified 464 children with EoE across the CEoEC sites. The median age was highest at Wake Forest (11.4 years), the median eosinophil count was highest at UNC (69 eos/hpf), and UNC had the most male patients (82%). UNC used topical steroids for initial treatment in 86% of cases, compared with <1% in Greenville (P < 0.01). Greenville used dietary elimination more frequently than UNC (81% vs 2%, P < 0.01). Differences in treatment approach held after adjusting for potential baseline confounders. There was no significant association between patient factors and initial treatment approach. CONCLUSIONS: Significant differences in EoE patient factors and treatment approaches were identified across CEoEC sites and were not explained by patient or practice factors. This suggests that institutional or provider preferences drive initial treatment approaches, and that more data are needed to drive best practice decisions.
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Esofagite Eosinofílica/diagnóstico , Padrões de Prática Médica/estatística & dados numéricos , Projetos de Pesquisa/tendências , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Registros Eletrônicos de Saúde , Esofagite Eosinofílica/epidemiologia , Feminino , Humanos , Masculino , North Carolina/epidemiologia , Estudos Retrospectivos , South Carolina/epidemiologiaRESUMO
OBJECTIVE: The aim of the study was to assess the safety and efficacy of high- and low-dose oral, delayed-release mesalamine in a randomized, double-blind, active control study of children with mild-to-moderately active ulcerative colitis. METHODS: Patients ages 5 to 17 years, with a Pediatric Ulcerative Colitis Activity Index (PUCAI) score of ≥ 10 to ≤ 55 and a truncated Mayo Score of ≥ 1 for both rectal bleeding and stool frequency, were enrolled. They received body weight-dependent doses of oral, delayed-release mesalamine for 6 weeks in a low- (27-71 mg · g(-1) · day(-1)) or high-dose group (53-118 mg · g(-1) · day(-1)). The primary endpoint was treatment success, defined as the proportion of patients who achieved remission (PUCAI score <10) or partial response (PUCAI score ≥ 10 with a decrease from baseline by ≥ 20 points). Secondary endpoints included truncated Mayo Score and global assessment of change of disease activity. RESULTS: The modified intent-to-treat population included 81 of 83 patients enrolled. Treatment success by PUCAI was achieved by 23 of 41 (56%) and 22 of 40 (55%) patients in the mesalamine low- and high-dose groups, respectively (P = 0.924). Truncated Mayo Score (low-dose 30 [73%] and high-dose 28 [70%] patients) and other efficacy results did not differ between the groups. The type and severity of adverse events were consistent with those reported in previous studies of adults with ulcerative colitis and did not differ between groups. CONCLUSIONS: Both low- and high-dose oral, delayed-release mesalamine doses were equally effective as short-term treatment of mild-to-moderately active ulcerative colitis in children, without a specific benefit or risk to using either dose.
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Anti-Inflamatórios não Esteroides , Colite Ulcerativa/tratamento farmacológico , Mesalamina/administração & dosagem , Adolescente , Criança , Pré-Escolar , Preparações de Ação Retardada , Método Duplo-Cego , Feminino , Humanos , Masculino , Mesalamina/efeitos adversosRESUMO
INTRODUCTION: Kidney ureter bladder radiography (KUB) is widely used for the evaluation of constipation in children with bladder and bowel dysfunction (BBD); however, there is varying evidence to support its routine diagnostic use. One drawback to KUB is radiation exposure. The dangers of radiation in children are well-documented, and per As Low As Reasonably Achievable, non-beneficial radiation should be avoided. This risk is especially high in children who undergo repeated imaging in the follow up of constipation treatment. OBJECTIVE: We sought to assess the utility of KUB in diagnosing children with BBD by comparing it to four diagnostic tests and/or validated instruments: the Dysfunctional Voiding Symptom Score (DVSS), Rome IV criteria, rectal diameter on ultrasound (RD), and the Bristol Stool Form Score (BSFS). STUDY DESIGN: We prospectively enrolled a cohort of patients presenting to an academic pediatric urology practice with symptoms of BBD. Severity of stool burden on KUB (mild, moderate, or severe), RD on ultrasound (≥3.4 cm), DVSS, Rome IV, and BSFS were obtained for each patient. All imaging was interpreted by a pediatric radiologist and pediatric urologist. Primary outcomes were the association between the four diagnostic tests and KUB stool burden. Bivariate analysis of all individual variables versus KUB was performed, as well as multivariate regressions to determine if multiple measures were predictive of KUB stool burden when combined. RESULTS: Between October 2020 and May 2022, 50 patients were enrolled. All children were under the age of 18, with a median age of 8 years (IQR 3-13). 38 % were male. Median BMI-for-age-percentile was 80.8 (IQR 50.3-98.3). When comparing individual variables to KUB in bivariate analyses, it was found that RD on ultrasound is predictive of significant stool burden on KUB (p = 0.03). No other individual variables were predictive. In the multivariate analyses, no combination of tests was found to be predictive of KUB. DISCUSSION: We compared the effectiveness of four commonly used diagnostic tests in children with BBD to validate the use of KUB. In conclusion, our results support the use of RD on ultrasound as a non-radiating alternative to KUB to assess stool burden. Data also suggest that KUB for fecal load does not correlate with urinary (DVSS) or bowel (Rome IV, BSFS) symptoms in BBD, and that symptoms scores should still be used independently for diagnosis and monitoring of treatment response. CONCLUSION: In conclusion, KUB has a limited role in the diagnosis of BBD.
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Gastroenterologia , Pancreatite , Criança , Humanos , Pâncreas , Troca Plasmática , Plasmaferese , Estados UnidosRESUMO
Autoimmune pancreatitis (AIP) is a rare autoimmune disorder that resembles pancreatic neoplasia and occurs primarily in adults. Management strategies and diagnostic criteria are being revised for adult patients; there are no clear diagnostic criteria for pediatric patients. We describe 3 cases of AIP in children, on the basis of clinical and pathology records. We also performed a literature review to determine the incidence of biliary obstruction in pediatric patients with pancreatic tumors. We found that children with AIP present with a variety of symptoms, and that diagnostic and therapeutic strategies also vary. Furthermore, on the basis of the many studies published on pediatric patients with pancreatic tumors, only a small percentage of the patients have biliary obstructions. Cytologic analysis of samples collected by fine-needle aspiration cytology does not accurately identify AIP in children. However, frozen section needle core biopsy samples can be used to distinguish children with AIP from those with neoplasia. Children with pancreatic mass and biliary obstruction are more likely to have AIP than neoplasms.
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Doenças Autoimunes/diagnóstico , Colestase Extra-Hepática/etiologia , Neoplasias Pancreáticas/diagnóstico , Pancreatite/diagnóstico , Adolescente , Doenças Autoimunes/complicações , Doenças Autoimunes/patologia , Biópsia/métodos , Criança , Colestase Extra-Hepática/patologia , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/patologia , Pancreatite/complicações , Pancreatite/patologiaRESUMO
BACKGROUND: Crohn's disease (CD) has a multitude of complications including intestinal strictures from fibrostenotic disease. Fibrostenotic disease has been reported in 10%-17% of children at presentation and leads to surgery in 20%-50% of cases within ten years of diagnosis. When symptoms develop from these strictures, the treatment in children has primarily been surgical resection. Endoscopic balloon dilation (EBD) has been shown to be a safe and efficacious alternative to surgery in adults, but evidence is poor in the literature regarding its safety and efficacy in children. AIM: To evaluate the outcomes of children with fibrostenosing CD who underwent EBD vs surgery as a treatment. METHODS: In a single-center retrospective study, we looked at pediatric patients (ages 0-18) who carry the diagnosis of CD, who were diagnosed after opening a dedicated Inflammatory Bowel Disease clinic on July 1, 2012 through May 1, 2019. We used diagnostic codes through our electronic medical record to identify patients with CD with a stricturing phenotype. The type of intervention for patients' strictures was then identified through procedural and surgical billing codes. We evaluated their demographics, clinical variables, whether they underwent EBD vs surgery or both, and their clinical outcomes. RESULTS: Of the 139 patients with CD, 25 (18%) developed strictures. The initial intervention for a stricture was surgical resection in 12 patients (48%) and EBD in 13 patients (52%). However, 4 (33%) patients whom initially had surgical resection required follow up EBD, and thus 17 total patients (68%) underwent EBD at some point in their treatment process. For those 8 patients who underwent successful surgical resection alone, 4 of these patients (50%) had a fistula present near the stricture site and 4 (50%) had strictures greater than 5 cm in length. All patients who underwent EBD had no procedural complications, such as a perforation. Twenty-two (88%) of the treated strictures were successfully managed by EBD and did not require any further surgical intervention during our follow up period. CONCLUSION: EBD is safe and efficacious as an alternative to surgery for palliative management of strictures in selected pediatric patients with CD.
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OBJECTIVES: There are limited data on the role of regulatory T cells (Treg) in the disease pathology of eosinophilic esophagitis (EoE). We tested the differences in Treg in subjects with EoE compared with those with gastroesophageal reflux disease (GERD) and healthy controls (HC). PATIENTS AND METHODS: Pediatric patients evaluated by endoscopy were recruited for our study. Participants were categorized into 3 groups: EoE, GERD, and HC. RNA purified from esophageal biopsies were used for real-time quantitative polymerase chain reaction assays and tested for forkhead box P3 (FoxP3) mRNA expression. Treg were identified as CD4+CD25hiCD127lo cells in peripheral blood and as CD3+/FoxP3+cells in esophageal tissue. RESULTS: Forty-eight subjects were analyzed by real-time quantitative polymerase chain reaction: EoE (n = 33), GERD (n = 7), and HC (n = 8). FoxP3 expression was higher by up to 1.5-fold in the EoE group compared with the GERD and HC groups (P < 0.05). Protein levels of FoxP3 in blood and tissue were then investigated in 21 subjects: EoE (n = 10), GERD (n = 6), and HC (n = 5). The percentage of Treg and their subsets in peripheral blood were not significant between groups (P > 0.05). The amount of Treg in esophageal tissue was significantly greater in the EoE group (mean 10.7 CD3+/FoxP3+cells/high power field [HPF]) compared with the other groups (GERD, mean 1.7 CD3+/FoxP3+cells/HPF and HC, mean 1.6 CD3+/FoxP3+cells/HPF) (P < 0.05). CONCLUSIONS: We show that Treg are increased in esophageal tissue of EoE subjects compared with GERD and HC subjects. The present study illustrates another possible mechanism involved in EoE that implicates impairment of immune homeostasis.
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Esofagite Eosinofílica/imunologia , Esôfago/imunologia , Fatores de Transcrição Forkhead/metabolismo , Refluxo Gastroesofágico/imunologia , Linfócitos T Reguladores/metabolismo , Adolescente , Adulto , Antígenos CD/metabolismo , Estudos de Casos e Controles , Criança , Pré-Escolar , Esofagite Eosinofílica/metabolismo , Esôfago/metabolismo , Feminino , Fatores de Transcrição Forkhead/genética , Refluxo Gastroesofágico/metabolismo , Humanos , Lactente , Masculino , RNA Mensageiro/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Adulto JovemRESUMO
The use of esophageal stents has been commonplace in adults for many years and for a variety of indications, including palliative care for malignant lesions involving the esophagus. The use of esophageal stents in the pediatric population, however, was limited by the inability to remove them and the implications this has for the growing child, especially for primarily benign esophageal lesions. With the advent of removable, covered stents, the potential uses for stents in children expanded to include treatment of a wide variety of congenital and acquired esophageal strictures. Stenting offers tremendous potential advantage over more traditional pneumatic or bougie dilation in its ability to provide continuous, radially oriented dilation pressure sustained over a period of time. This review examines the published pediatric literature on stents, discusses the indications for their use, outlines the types of stents available, offers technical guidance for proper placement, and reviews subsequent management and complications.
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Estenose Esofágica/cirurgia , Cuidados Paliativos/tendências , Stents , Criança , Pré-Escolar , Humanos , Complicações Pós-OperatóriasRESUMO
PURPOSE: There have been many efforts to develop generalizable severity markers in children with acute pancreatitis (AP). Expert opinion panels have developed consensus guidelines on management but it is unclear if these are sufficient or valid. Our study aims to assess the effect of clinical and laboratory variables, in addition to treatment modality on hospital length of stay (LOS) as a proxy variable for severity in pediatric patients admitted with AP. METHODS: We conducted a retrospective chart review of patients between ages of 0-18 years, who were admitted with AP at 2 institutions between 2013-2018, John R. Oishei Children's Hospital (Buffalo, NY, USA) and Medical University of South Carolina Children's Hospital (Charleston, SC, USA). We constructed three linear regression models to analyze the effect of clinical signs of organ dysfunction, laboratory markers and fluid intake on hospital LOS. RESULTS: Ninety-two patients were included in the study. The mean age was 12 years (range, 7.6-17.4 years), 55% were females, and median LOS was 3 days. The most frequent cause of AP was idiopathic. Our study showed that elevated blood urea nitrogen (BUN) on admission (p<0.005), tachycardia that lasted for ≥48 hours (p<0.001) and need for fluid resuscitation were associated with increase LOS. Total daily fluid intake above maintenance did not have a significant effect on the primary outcome (p=0.49). CONCLUSION: Elevated serum BUN on admission, persistent tachycardia and need for fluid resuscitation were associated with increase LOS in pediatric AP. Daily total fluid intake above recommended maintenance did not reduce LOS.
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OBJECTIVES: : A multicenter, double-blind study was conducted to evaluate the safety, efficacy, and pharmacokinetics of balsalazide in pediatric patients with mild-to-moderate ulcerative colitis (UC). PATIENTS AND METHODS: : Sixty-eight patients, 5 to 17 years of age, with mild-to-moderate active UC based on the modified Sutherland UC activity index (MUCAI) were randomized to receive oral balsalazide 2.25 or 6.75 g/day for 8 weeks. The primary endpoint was clinical improvement (reduction of the MUCAI score by > or =3 points from baseline). Clinical remission (MUCAI score of 0 or 1 for stool frequency) and histological improvement after 8 weeks were also assessed. Pharmacokinetic parameters for balsalazide, 5-aminosalicylic acid, and N-acetyl-5-aminosalicylic acid were determined at 2 weeks. Adverse events and laboratory changes were monitored throughout the study. RESULTS: : Clinical improvement was achieved by 45% and 37% of patients and clinical remission by 12% and 9% of patients receiving 6.75 and 2.25 g/day, respectively. Improvement in histologic grade was achieved by 8 of 16 (50%) and 3 of 10 (30%) patients receiving 6.75 and 2.25 g/day, respectively. No significant differences were seen in efficacy. Pharmacokinetics in 12 patients were characterized by large interpatient variability and low systemic exposure. Adverse events were similar between the treatment groups, the most common being headache and abdominal pain. No clinically significant changes were observed in laboratory values, including those indicative of hepatic or renal toxicity. CONCLUSIONS: : Balsalazide is well tolerated and improves the signs and symptoms of mild-to-moderate active UC in pediatric patients 5 to 17 years of age.
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Anti-Inflamatórios/uso terapêutico , Colite Ulcerativa/tratamento farmacológico , Colo/efeitos dos fármacos , Mesalamina/uso terapêutico , Fenil-Hidrazinas/uso terapêutico , Adolescente , Ácidos Aminossalicílicos/efeitos adversos , Ácidos Aminossalicílicos/farmacocinética , Ácidos Aminossalicílicos/uso terapêutico , Anti-Inflamatórios/efeitos adversos , Anti-Inflamatórios/farmacocinética , Criança , Colite Ulcerativa/patologia , Colo/patologia , Método Duplo-Cego , Feminino , Humanos , Masculino , Mesalamina/efeitos adversos , Mesalamina/farmacocinética , Fenil-Hidrazinas/efeitos adversos , Fenil-Hidrazinas/farmacocinéticaRESUMO
IMPORTANCE: Alpha1-antitrypsin (AAT) deficiency is a common, but an underdiagnosed genetic condition, affecting 1 in 1500 individuals. It can present insidiously with liver disease in children. Although clinical practice guidelines exist for the management of AAT deficiency, especially with regards to pulmonary involvement, there are no published recommendations that specifically relate to the management of the liver disease and monitoring for lung disease associated with this condition, particularly in children. OBJECTIVE: To review the literature on the management of AAT deficiency-associated liver disease in adults and children. EVIDENCE REVIEW: A systematic search for articles indexed in PubMed and published was undertaken. Some earlier selected landmark references were included in the review. Search terms included: "alpha1-antitrypsin deficiency"; "liver disease"; "end-stage liver disease"; "liver transplantation" and "preventative management". Recommendations for the management of children with suspected or confirmed AAT deficiency were made according to the Strength of Recommendation Taxonomy scale. FINDINGS: Liver complications arising from AAT deficiency result from the accumulation of mutated AAT protein within hepatocytes. Liver disease occurs in 10% of children, manifested by cholestasis, pruritus, poor feeding, hepatomegaly, and splenomegaly, but the presentation is highly variable. A diagnostic test for AAT deficiency is recommended for these children. Baseline liver function tests should be obtained to assess for liver involvement; however, the only curative treatment for AAT deficiency-associated liver disease is organ transplantation. Conclusion and Relevance: There should be a greater vigilance for AAT deficiency testing among pediatricians. Diagnosis should prompt assessment of liver involvement. Children with AATdeficiency- associated liver disease should be referred to a liver specialist and monitored throughout their lifetimes for the symptoms of AAT-deficiency-related pulmonary involvement.
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Hepatopatias/terapia , Transição para Assistência do Adulto , Deficiência de alfa 1-Antitripsina/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Fígado/fisiopatologia , Hepatopatias/diagnóstico , Hepatopatias/etiologia , Transplante de Fígado/métodos , Masculino , Deficiência de alfa 1-Antitripsina/complicações , Deficiência de alfa 1-Antitripsina/diagnósticoRESUMO
BACKGROUND: Strictures are a common complication of inflammatory bowel disease (IBD) and are usually treated by surgical resection or strictureplasty. As an alternative to surgery, endoscopic balloon dilation and steroid injection have been used to relieve symptoms. GOALS: To assess patient or stricture characteristics that may predict a better outcome and duration of response as endoscopic therapy is not without its risks. STUDY: A retrospective review of patients with IBD strictures who underwent dilations between 1996 and 2005 was performed. The patients were followed in the adult and pediatric IBD clinics at a single center. Information was collected from medical records. RESULTS: Strictures were identified in the small and large bowel of 24 patients (22 adult and 2 pediatric). The majority had Crohn's disease (22/24). In total, 71 dilations were performed on 29 strictures; 46 dilations for 17 strictures were augmented with triamcinolone. Mean duration of follow-up was 32 months. This study included 1 stomal, 12 anastomotic, and 16 de novo strictures. Of 12 anastomotic strictures, 6 were complex. Endoscopic dilation was uneventful in 22/24 patients. Bleeding and perforation occurred on separate occasions in 1/6 complex stricture patients and rupture of a paracolonic abscess in another patient with a de novo sigmoid stricture. Surgery was performed on 2 patients, 1 for refractory disease and 1 for noncompliance with therapy. CONCLUSIONS: Endoscopic dilation can provide long-term effective palliation of symptoms with minimal risk in patients with simple strictures. Complex anastomotic strictures are technically more challenging compared with de novo strictures.
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Cateterismo/métodos , Endoscopia Gastrointestinal/métodos , Doenças Inflamatórias Intestinais/complicações , Adolescente , Adulto , Anti-Inflamatórios/uso terapêutico , Cateterismo/efeitos adversos , Constrição Patológica/etiologia , Constrição Patológica/terapia , Doença de Crohn/complicações , Endoscopia Gastrointestinal/efeitos adversos , Feminino , Seguimentos , Humanos , Intestino Grosso/patologia , Intestino Delgado/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Triancinolona/uso terapêuticoAssuntos
Estenose Esofágica/congênito , Esôfago/anormalidades , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/prevenção & controle , Diagnóstico Diferencial , Endossonografia , Estenose Esofágica/diagnóstico por imagem , Estenose Esofágica/fisiopatologia , Estenose Esofágica/cirurgia , Esôfago/diagnóstico por imagem , Esôfago/cirurgia , Feminino , Humanos , Lactente , Pneumonia Aspirativa/etiologia , Cirurgia Torácica Vídeoassistida , Resultado do TratamentoRESUMO
OBJECTIVES: Pancreatic enzyme concentrations are frequently elevated in children with diabetic ketoacidosis (DKA). We sought to determine the clinical and biochemical characteristics associated with patients with these elevations. Our hypothesis was that pancreatic enzyme elevations would be associated with biochemical markers of hypoperfusion. DESIGN: Prospective cohort study. SETTING: Three university-affiliated children's hospitals. PATIENTS: We collected data on consecutive children <18 yrs of age hospitalized with the diagnosis of DKA. INTERVENTIONS: Serum electrolyte and lactate concentrations and venous pH and Pco2 were measured every 3 hrs from hours 0 to 12 and then every 6 hrs until hour 24. Serum calcium, phosphate, and magnesium concentrations were measured every 6 hrs from hours 0 to 24. Serum amylase, lipase, and triglyceride concentrations were measured at hour 0 and then 12, 24, and 48 hrs after the initiation of therapy. MEASUREMENTS AND MAIN RESULTS: We performed multivariable analyses to test for associations between clinical variables and pancreatic enzyme elevation in 67 children with DKA. Lipase was elevated in 21 (31%) and amylase in 16 (24%) of the children. Pancreatic enzyme values peaked 12-24 hrs after admission. There was no significant correlation between pancreatic enzyme elevation and abdominal pain. In multivariable analyses, an elevated blood urea nitrogen (BUN) concentration was associated with elevated serum amylase (odds ratio 1.04 per unit increase; 95% confidence interval, 1.01-1.09; p = .02), and elevated BUN concentrations and hypophosphatemia were associated with elevated serum lipase (odds ratio 1.04 per unit increase; 95% confidence interval, 1.00-1.08; p = .04; and odds ratio 0.35 per unit increase; 95% confidence interval, 0.15-0.81; p = .01, respectively). CONCLUSIONS: Elevation of pancreatic enzymes is common in children with DKA, but clinical pancreatitis is rare. Pancreatic enzyme levels reach a peak 12-24 hrs after initiation of treatment for DKA. Pancreatic enzyme elevation is associated with increased BUN concentrations at presentation but is not associated with abdominal pain.