RESUMO
INTRODUCTION: Given the lack of data, we aimed to explore which therapeutic endpoints pediatric patients with eosinophilic esophagitis (EoE) and their parents consider to be relevant. METHODS: We created an educational brochure on EoE and a questionnaire, both of which were content-validated by pediatric patients and parents. Validated documents were sent to 112 patients and parents. They ranked the importance (5 levels) of short (during next 3 months) and long-term (≥1 year) treatment effect on symptoms, quality of life, endoscopic inflammation, stricture formation, histological inflammation, and fibrosis. RESULTS: A total of 45 parents and 30 pediatric patients ≥11 years completed the questionnaires. Pediatric patients identified improvement in the following domains as most important in the short- and long-term, respectively: symptoms (73% vs. 77%), QoL (53% vs. 57%), histologic inflammation (47% vs. 50%), histologic fibrosis (40% vs. 33%), endoscopic inflammation (47% vs. 40%), and strictures (33% vs. 40%). Parents of children ≥11 years old classified improvement in the following domains as most important in the short- and long-term, respectively: symptoms (70% vs. 83%), QoL (63% vs. 80%), histologic inflammation (67% vs. 77%), histologic fibrosis (47% vs. 63%), endoscopic inflammation (77% vs. 80%), and strictures (40% vs. 53%). Agreement between caregiver and children on the short-term importance of treatment outcomes was as follows: symptoms (77%), QoL (40%), histologic inflammation and fibrosis (47% and 43%), endoscopic inflammation and strictures (50% and 40%). CONCLUSION: Pediatric patients and parents attributed most importance to improvement in symptoms and QoL. Agreement between parents and patients regarding therapy goals is limited.
Assuntos
Esofagite Eosinofílica , Pais , Qualidade de Vida , Humanos , Esofagite Eosinofílica/terapia , Esofagite Eosinofílica/diagnóstico , Pais/psicologia , Criança , Inquéritos e Questionários , Masculino , Feminino , Resultado do Tratamento , Adolescente , Pré-EscolarRESUMO
OBJECTIVES: Inflammatory bowel disease (IBD) requires long-term drug therapy in most patients, posing a risk for adverse drug events with the need for discontinuation. In this study, we investigated adverse events (AE) necessitating drug discontinuation in pediatric and adolescent IBD patients. METHODS: We used data prospectively collected from IBD patients below the age of 18 enrolled in the Swiss Inflammatory Bowel Disease Cohort Study (SIBDCS), namely demographic variables, medical characteristics, drug treatments, and related AE. We analyzed the frequency, type, and risk factors for AE necessitating drug discontinuation. RESULTS: A total of 509 pediatric IBD patients fulfilled the inclusion criteria of which 262 (51.5%) were diagnosed with Crohn disease (CD), 206 (40.5%) with ulcerative colitis (UC), and 41 (8%) with IBD-unclassified (IBD-U). In total, 132 (25.9%) presented with at least 1 drug-related AE that required drug cessation. Immunomodulators [methotrexate 29/120 (24.2%), azathioprine 57/372 (15.3%)] followed by tumor necrosis factor (TNF)-alpha antagonists [adalimumab 8/72 (11.1%), infliximab 22/227 (9.7%)] accounted for the highest proportions of AE necessitating treatment discontinuation. Treatment schemes with at least 3 concomitant drugs significantly amplified the risk for development of drug-related AE [odds ratio = 2.50, 95% confidence interval (1.50-4.17)] in all pediatric IBD patients. CONCLUSIONS: Drug-related AE necessitating discontinuation are common in pediatric and adolescent IBD patients. Caution needs to be taken in the case of concomitant drug use.
Assuntos
Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Humanos , Criança , Adolescente , Estudos de Coortes , Infliximab/efeitos adversos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doença de Crohn/tratamento farmacológico , Colite Ulcerativa/tratamento farmacológico , Adalimumab/efeitos adversos , Fator de Necrose Tumoral alfa , Inibidores do Fator de Necrose TumoralRESUMO
Digestive and nutritional problems of children with cerebral palsy put them at risk of malnutrition. Identification of these problems through measurements of weight, height, and body composition is essential. Feeding difficulties may be caused by a combination of oral and digestive problems, such as swallowing difficulties, gastroesophageal reflux, and constipation. If oral feeding is difficult or unsafe, a nasogastric tube or gastrostomy may be necessary. Once the feeding regimen has been established, energy needs must be assessed on an individual basis. This nutritional management involves a multidisciplinary team of health care professionals, the child, and the family.
Les problématiques digestives et nutritionnelles des enfants avec infirmité motrice cérébrale les mettent à risque de malnutrition. L'identification de ces troubles par les mesures de poids, taille, et composition corporelle, est primordiale. Les difficultés alimentaires peuvent être causées par une combinaison de problèmes bucco-dentaires et digestifs, tels que les difficultés de déglutition et le reflux gastro-Åsophagien ou la constipation. Si l'alimentation per os est difficile ou dangereuse, il peut être nécessaire de mettre en place une sonde nasogastrique ou une gastrostomie. Une fois le mode d'alimentation établi, les besoins énergétiques doivent être évalués individuellement. Cette prise en charge nutritionnelle implique une équipe multidisciplinaire composée de professionnels de la santé, de l'enfant et de sa famille.
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Paralisia Cerebral , Transtornos de Deglutição , Desnutrição , Distúrbios Nutricionais , Paralisia Cerebral/complicações , Paralisia Cerebral/terapia , Criança , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/terapia , Gastrostomia/efeitos adversos , Humanos , Desnutrição/complicações , Distúrbios Nutricionais/complicações , Estado NutricionalRESUMO
AIM: To assess different techniques to measure body composition in paediatric patients with inflammatory bowel disease using dual energy X-ray absorptiometry as a reference method. We hypothesised that a three-compartment model may demonstrate superiority over other methods as skinfold thickness equations and bioelectrical impedance analysis. METHODS: Body composition was assessed using skinfold thickness equations, bioelectrical impedance analysis and the three-compartment model. Data obtained with these methods were compared to the results obtained by dual energy X-ray absorptiometry. Statistical analysis was performed using Spearman's correlation and Bland-Altman's limits of agreement method. RESULTS: Twenty-one paediatric patients with inflammatory bowel disease were included: 11 females and 10 males; mean age for the entire group: 14.3 years, range 12-16 years. In children with inflammatory bowel disease, skinfold thickness equations, bioelectrical impedance analysis and the three-compartment model showed reliable measurements with small differences in the percentage of total body fat and good limits of agreements. CONCLUSION: The assessment of body composition using bioelectrical impedance analysis provides a valid and accurate method in children with inflammatory bowel disease as compared to dual energy X-ray absorptiometry. In the future, superiority of 3-compartment model in research and clinical settings of nutritional intervention and disease status in children with inflammatory bowel disease remains to be demonstrated.
Assuntos
Composição Corporal , Doenças Inflamatórias Intestinais , Absorciometria de Fóton , Tecido Adiposo , Adolescente , Índice de Massa Corporal , Criança , Impedância Elétrica , Feminino , Humanos , Masculino , Dobras CutâneasRESUMO
The vegan diet, although beneficial for adults in the prevention of metabolic diseases, raises questions about its benefits for a growing child : adequate caloric and protein intake, quality of essential amino acids, presence of essential fatty acids, inhibition of absorption of trace elements (including iodine, iron and zinc) and supply of various vitamins. Whereas vegan food is vitamin B12 deprived, the biggest challenge remains the vitamin B12 substitution in pregnant women, breastfeeding mothers and children at any age. Specific management by pediatricians using dietetic support and blood analyzes are required for children under vegan diet with respect of moral and ethical values related to this lifestyle choice.
L'alimentation végétalienne, bien que bénéfique pour les adultes dans la prévention de maladies métaboliques, pose des questions quant à ses bénéfices pour un enfant en croissance : apports caloriques et protéiques suffisants, qualité en acides aminés essentiels et présence d'acides gras, inhibition de l'absorption des vitamines (notamment la D) et des éléments traces (par exemple, iode, fer, et zinc). Elle pose surtout la question de la supplémentation en vitamine B12 des futures mamans, des mères allaitantes et des enfants de chaque âge, alors que leur alimentation en est dépourvue. Des mesures particulières de surveillance diététiques et biologiques, ainsi qu'un accompagnement pédiatrique nutritionnel sont à proposer tout en respectant les valeurs morales et éthiques sous-jacentes à ce choix de vie.
Assuntos
Dieta Vegana , Dieta Vegetariana , Adulto , Criança , Dieta , Feminino , Humanos , GravidezRESUMO
BACKGROUND: In ulcerative colitis (UC) 5-aminosalicylic acid (5-ASA) is recommended as primary therapy for mild to moderate disease. Topical 5-ASA has been proven especially effective. In Crohn's disease (CD) the evidence for a beneficial role of 5-ASA is weak. We investigated the use of topical and systemic 5-ASA therapy in children and adolescents with inflammatory bowel disease. MATERIALS AND METHODS: Data of patients younger than 18 years, registered between April 2008 and December 2015 in the Swiss Inflammatory Bowel Disease Cohort, were analyzed. RESULTS: Three hundred twenty pediatric inflammatory bowel disease patients were included; 189 with CD and 131 with UC. Over one third of UC patients [51 (39%)] received topical 5-ASA therapy and 43 (33%) received combination therapy during their disease course. UC patients with left-sided colitis or proctitis were more likely to receive topical or combination therapy as compared with patients with pancolitis (P<0.001 and <0.001, respectively). An increase in the use of topical 5-ASA therapy in UC patients was noted over time from 5% to 38%. Forty-seven percent of CD patients were treated with oral 5-ASA during their disease course. The usage was stable over time at approximately 15% to 20%. CONCLUSIONS: In recent years a very positive trend showing an increase in topical 5-ASA therapy in children and adolescents with UC has been observed. However topical therapy is still used with relative low frequency, especially in patients with a more extensive disease. Conversely, despite weak evidence supporting 5-ASA use in CD patients it has been frequently prescribed. Physicians should continue to encourage their UC patients to use topical therapy.
Assuntos
Anti-Inflamatórios não Esteroides/administração & dosagem , Doenças Inflamatórias Intestinais , Mesalamina/administração & dosagem , Administração Cutânea , Administração Oral , Adolescente , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Prontuários Médicos , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
PURPOSE: During the first years of life, food preferences are shaped that might last throughout a person's entire life affecting his/her health in the long term. However, knowledge on early feeding habits is still limited for toddlers. Therefore, the goal of the present study was to: (1) assess toddlers' nutrient intake; (2) compare the findings to past studies as well as to national feeding recommendations and (3) identify major food sources for energy and macronutrients. METHODS: A food survey using a 4-day diary was conducted. The dietary software nut.s® was used to analyse the data. RESULTS: A cohort of 188 healthy toddlers (aged 1-3 years) was analysed. The energy intake of most toddlers was below the recommended daily intake (RDI) but in accordance with earlier studies. Protein intake was three- to fourfold higher than the RDI and reached the proposed upper limit of 15% of total energy intake. Fat intake was in accordance with the RDI, but the balance of saturated and unsaturated fatty acids should be improved. Carbohydrate intake met the RDI. For the micronutrients, iron and vitamin D intakes showed critical values. CONCLUSION: As in other European countries, the diet of Swiss toddlers in general seems adequate but does not meet all nutritional requirements. In particular, the quality of the fats and vitamin D supplementation should be improved. For proteins and iron, additional research is needed to gain more confidence in the recommendations.
Assuntos
Dieta , Ingestão de Energia/fisiologia , Necessidades Nutricionais , Recomendações Nutricionais , Pré-Escolar , Dieta/normas , Gorduras na Dieta/administração & dosagem , Proteínas Alimentares/administração & dosagem , Europa (Continente) , Feminino , Humanos , Lactente , Masculino , MicronutrientesRESUMO
We evaluated the diagnostic delay (time from first symptoms to diagnosis) in 100 pediatric patients with Crohn disease (CD) and 75 patients with ulcerative colitis (UC). Median (interquartile range) diagnostic delay in patients with CD was 4 (2-8) (range 0-82) months compared with 2 (1-7) (range 0-52) months in patients with UC (Pâ=â0.003). The time interval from first physician visit to inflammatory bowel disease diagnosis was longer in patients with CD and UC when compared to the time interval from symptom onset to first physician visit (CD: median 3 vs 1 months, Pâ<â0.001; UC: median 2 vs 0 months, Pâ<â0.001). No specific risk factors were identified for the length of diagnostic delay. Measures should be taken to reduce diagnostic delay.
Assuntos
Colite Ulcerativa/diagnóstico , Doença de Crohn/diagnóstico , Diagnóstico Tardio/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Modelos de Riscos Proporcionais , Fatores de Risco , Suíça , Fatores de TempoRESUMO
BACKGROUND: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). METHODS: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. RESULTS: A total of 55 patients (16.7%) experienced 1-4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, Pâ=â0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, Pâ=â0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti-tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, Pâ=â0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). CONCLUSIONS: In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.
Assuntos
Colangite Esclerosante/etiologia , Colite Ulcerativa/complicações , Doença de Crohn/complicações , Artropatias/etiologia , Dermatopatias/etiologia , Uveíte/etiologia , Adolescente , Anti-Inflamatórios/uso terapêutico , Criança , Pré-Escolar , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/tratamento farmacológico , Colangite Esclerosante/epidemiologia , Colite Ulcerativa/diagnóstico , Colite Ulcerativa/tratamento farmacológico , Doença de Crohn/diagnóstico , Doença de Crohn/tratamento farmacológico , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Artropatias/diagnóstico , Artropatias/tratamento farmacológico , Artropatias/epidemiologia , Modelos Logísticos , Masculino , Prevalência , Estudos Retrospectivos , Dermatopatias/diagnóstico , Dermatopatias/tratamento farmacológico , Dermatopatias/epidemiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Uveíte/epidemiologiaRESUMO
PURPOSE: Little is known about disease-specific health-related quality-of-life (HRQoL) changes over time in paediatric patients with inflammatory bowel disease (IBD), and about their associations with baseline medical characteristics. METHODS: In this study, 153 paediatric patients with IBD from the multicentre prospective Swiss IBD cohort study were included at baseline. Of these, 90 patients were analysed at a 1-year follow-up. Medical data were extracted from hospital records, while HRQoL data were measured using the standardized, self-report disease-specific IMPACT-III questionnaire. RESULTS: The IBD diagnosis of the included children was made an average of 2.0 years before their baseline assessment. Over the 1-year follow-up period, a significant increase in overall HRQoL and in the HRQoL domain 'physical functioning' was evident. On multivariate analysis, overall HRQoL changes over time were predicted by baseline HRQoL, baseline disease activity, and disease activity changes over time. HRQoL improvements were significantly associated with decreases in physician-assessed disease activity. Children reporting a low baseline HRQoL and children with inactive or mildly-active disease experienced greater improvements. CONCLUSIONS: Children with more severe baseline disease activity had the greatest risk for HRQoL deterioration over the 1-year follow-up period. However, among possible factors that might influence HRQoL changes over time, the child's medical characteristics explained only a small proportion of their variability in our sample. We, therefore, recommend that researchers and clinicians focus on factors that are not incorporated within the multidimensional HRQoL concept if they seek to gain better insights into factors that influence HRQoL changes over time in children with IBD.
Assuntos
Doenças Inflamatórias Intestinais/psicologia , Qualidade de Vida/psicologia , Adolescente , Criança , Doença Crônica , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Estudos Prospectivos , Inquéritos e Questionários , Fatores de TempoRESUMO
PURPOSE: In obesity prevention, understanding psychosocial influences in early life is pivotal. Reviews reported contradictory results and a lack of longitudinal studies focusing on underlying lifestyle factors. This study tested whether psychosocial Quality-Of-Life (QOL) was associated with pre-schoolers' lifestyle and adiposity changes over one school year and whether lifestyle moderated the latter. It was hypothesised that QOL might not impact adiposity in everybody but that this might depend on preceding lifestyle. METHOD: Longitudinal data from 291 Swiss pre-schoolers (initially 3.9-6.3 years) was available. The following measures were used in longitudinal regressions: psychosocial QOL by PedsQL, adiposity (BMI z-score, waist, fat%), diet (food frequency), sedentary time and accelerometer-based activity. RESULTS: Concerning lifestyle, low psychosocial QOL was only related to unfavourable changes in diet (less fruit ß = 0.21 and more fat intake ß = -0.28) and lower physical activity (ß = 0.21). Longitudinal QOL-adiposity relations appeared only after moderation by lifestyle factors (beta-range 0.13-0.67). Low psychosocial QOL was associated with increased adiposity in children with an unhealthy diet intake or high sedentary time. By contrast, low psychosocial QOL was associated with decreasing adiposity in high fruit consumers or more physically active pre-schoolers. CONCLUSION: Results emphasise the need for testing moderation in the QOL-adiposity relation. An unhealthy diet can be a vulnerability factor and high physical activity a protective factor in QOL-related adiposity. Consequently, QOL and lifestyle should be targeted concurrently in multi-factorial obesity prevention. The environment should be an 'activity encouraging, healthy food zone' that minimises opportunities for stress-induced eating. In addition, appropriate stress coping skills should be acquired.
Assuntos
Adiposidade , Dieta , Estilo de Vida , Obesidade/prevenção & controle , Adaptação Psicológica , Criança , Pré-Escolar , Ingestão de Alimentos , Exercício Físico , Comportamento Alimentar , Feminino , Humanos , Estudos Longitudinais , Masculino , Obesidade/psicologia , Qualidade de VidaRESUMO
AIM: To assess the agreement of commonly used skinfold thickness equations to estimate percentage of Fat Mass (FM%) in paediatric patients with IBD, in comparison with dual energy X-ray absorptiometry (DEXA). METHODS: Twenty-one paediatric IBD patients were included: 11 females and 10 males; mean age for the entire group: 14.3 years, range 12-16 years, 16 with Crohn's disease, five with ulcerative colitis. The FM% was calculated using six established paediatric skinfold thickness equations and compared to the results obtained by DEXA. The statistical analysis was performed using Spearman's correlation, Lin's concordance correlation coefficient and corresponding 95% confidence interval, Bland-Altman's limits of agreement method and the Bradley-Blackwood test. RESULTS: Correlation between skinfold and DEXA values ranged between 0.85 (Deurenberg) and 0.92 (Durnin and Rahaman and Johnston), all P < 0.001. Lin's concordance correlation coefficients and (95% confidence interval) ranged between 0.702 (0.512-0.891) for the Deurenberg equation and 0.876 (0.779-0.972) for the Brook equation. Average differences between skinfold and DEXA values ranged between -3.6% (Deurenberg) and 2.5% (Weststrate). Bland-Altman limits of agreement were wide, spanning over 10%. Finally, the Bradley-Blackwood test of equality of means and variances was significant in all but the Durnin and Rahaman equation. CONCLUSION: In adolescents with IBD, fat mass calculated from six skinfold thickness equations showed good correlation but poor agreement with reference values from DEXA. Assessment of body composition using skinfold thickness equations cannot be recommended in paediatric patients with IBD.
Assuntos
Composição Corporal/fisiologia , Doenças Inflamatórias Intestinais , Dobras Cutâneas , Adolescente , Criança , Feminino , Humanos , Masculino , Modelos Estatísticos , Valores de Referência , Reprodutibilidade dos TestesRESUMO
BACKGROUND: Growth failure and delayed puberty are well known features of children and adolescents with inflammatory bowel disease (IBD), in addition to the chronic course of the disease. Urinary metabonomics was applied in order to better understand metabolic changes between healthy and IBD children. METHODS: 21 Pediatric patients with IBD (mean age 14.8 years, 8 males) were enrolled from the Pediatric Gastroenterology Outpatient Clinic over two years. Clinical and biological data were collected at baseline, 6, and 12 months. 27 healthy children (mean age 12.9 years, 16 males) were assessed at baseline. Urine samples were collected at each visit and subjected to ¹H Nuclear Magnetic Resonance (NMR) spectroscopy. RESULTS: Using ¹H NMR metabonomics, we determined that urine metabolic profiles of IBD children differ significantly from healthy controls. Metabolic differences include central energy metabolism, amino acid, and gut microbial metabolic pathways. The analysis described that combined urinary urea and phenylacetylglutamine-two readouts of nitrogen metabolism-may be relevant to monitor metabolic status in the course of disease. CONCLUSION: Non-invasive sampling of urine followed by metabonomic profiling can elucidate and monitor the metabolic status of children in relation to disease status. Further developments of omic-approaches in pediatric research might deliver novel nutritional and metabolic hypotheses.
Assuntos
Doenças Inflamatórias Intestinais/metabolismo , Doenças Inflamatórias Intestinais/urina , Adolescente , Criança , Colite Ulcerativa/metabolismo , Colite Ulcerativa/urina , Doença de Crohn/metabolismo , Doença de Crohn/urina , Feminino , Humanos , Espectroscopia de Ressonância Magnética , Masculino , MetabolômicaRESUMO
BACKGROUND AND AIMS: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. RESULTS: A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. Paediatric cohort: median age at diagnosis 12.5 years (range 1-17, interquartile range (IQR) 8-15), 16 (53%) girls, 6 (32%) with type 2 autoimmune hepatitis, 8 (27%) with autoimmune sclerosing cholangitis, 1 with primary biliary cholangitis variant syndrome, 4 (15%) with inflammatory bowel disease and 10 (41%) with advanced liver fibrosis at diagnosis. Adult cohort: median age at diagnosis 54 years (range 42-64, IQR 18-81), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis. The median follow-up time for the entire cohort was 5.2 years (IQR 3-9.3 years). Treatment in children: 29 (97%) children were initially treated with corticosteroids, 28 of whom received combination treatment with azathioprine. Budesonide was used in four children, all in combination with azathioprine. Mycophenolate mofetil was used in five children, all of whom had previously received corticosteroids and thiopurine. Treatment in adults (data available for 228 patients): 219 (96%) were treated with corticosteroids, mostly in combination with azathioprine. Predniso(lo)ne was the corticosteroid used in three-quarters of patients; the other patients received budesonide. A total of 78 (33%) patients received mycophenolate mofetil, 62 of whom had previously been treated with azathioprine. Complete biochemical response was achieved in 13 of 19 (68%) children and 137 of 182 (75%) adults with available follow-up data. All children were alive at the last follow-up, and none had undergone liver transplantation. Five (2%) adults underwent liver transplantation, two of whom had a fulminant presentation. Four (2%) adults with autoimmune hepatitis died (two from liver-associated causes). CONCLUSION: Patients with autoimmune hepatitis in Switzerland had clinical features similar to those in other cohorts. The proportion of patients diagnosed with primary biliary cholangitis variant syndrome was higher than expected. Autoimmune hepatitis was managed according to guidelines, except for the use of budesonide in a small proportion of paediatric patients. The outcomes were excellent, but the findings must be confirmed over a longer follow-up period.
Assuntos
Hepatite Autoimune , Doenças Inflamatórias Intestinais , Cirrose Hepática Biliar , Adulto , Humanos , Criança , Feminino , Lactente , Pré-Escolar , Adolescente , Pessoa de Meia-Idade , Masculino , Azatioprina/uso terapêutico , Estudos Retrospectivos , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/tratamento farmacológico , Estudos Prospectivos , Suíça/epidemiologia , Estudos de Coortes , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/tratamento farmacológico , Ácido Micofenólico/uso terapêutico , Cirrose Hepática , Doenças Inflamatórias Intestinais/tratamento farmacológico , Budesonida/uso terapêuticoRESUMO
Growth retardation, associated with delayed puberty, is a frequent feature in pediatric patients with inflammatory bowel disease (IBD), especially with Crohn's disease. It is mainly induced by malnutrition and the effects of the inflammatory process on the growth hormone/insulin-like growth factor-1 axis or on the growth plate. Therefore, control of disease activity and mucosal healing are paramount to promote growth and adequate pubertal onset. Current therapeutic strategies for maintenance in IBD include anti-inflammatory drugs, immunosuppressives, and, more recently, biologic agents. Although these treatments are efficient in minimizing inflammation and inducing prolonged remission, their long-term effects on growth and final height remain controversial. Furthermore, glucocorticoid therapy, even though very efficient in inducing remission, clearly shows deleterious effects on growth, which is not the case for exclusive enteral nutrition showing comparable results regarding induction of remission. Thus regular assessment of weight, height and pubertal stage is essential in children and adolescents with chronic disease, namely IBD.
Assuntos
Glucocorticoides/efeitos adversos , Transtornos do Crescimento/etiologia , Imunossupressores/efeitos adversos , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/tratamento farmacológico , Puberdade Tardia/etiologia , Adolescente , Criança , Nutrição Enteral , Transtornos do Crescimento/metabolismo , Hormônio do Crescimento/metabolismo , Humanos , Inflamação/complicações , Inflamação/etiologia , Inflamação/metabolismo , Doenças Inflamatórias Intestinais/metabolismo , Fator de Crescimento Insulin-Like I/metabolismo , Desnutrição/complicações , Desnutrição/etiologia , Desnutrição/metabolismo , Puberdade Tardia/metabolismoRESUMO
BACKGROUND: Intestinal spirochetosis is an unusual infection in children and its clinical significance in humans is uncertain. The presence of these microorganisms in humans is well-known since the late 1800's and was first described in 1967 by Harland and Lee by electron microscopy. CASE PRESENTATION: This article reports the findings of one pediatric case, review of the current literature, and an overview of therapeutic options. CONCLUSION: A high degree of suspicion is required in cases presenting with abdominal pain, chronic diarrhoea and/or hematochezia associated with a normal endoscopic examination, thus emphasizing the importance of multiple biopsies throughout the colon.
Assuntos
Doenças Inflamatórias Intestinais/diagnóstico , Enteropatias/diagnóstico , Enteropatias/microbiologia , Infecções por Spirochaetales/diagnóstico , Adolescente , Diagnóstico Diferencial , Humanos , MasculinoRESUMO
BACKGROUND: In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin's and non-Hodgkin's lymphomas. CASE PRESENTATION: We report an unusual case of sporadic Burkitt's lymphoma (BL) presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome. CONCLUSION: Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor.
Assuntos
Linfoma de Burkitt/diagnóstico , Tumor de Krukenberg/diagnóstico , Neoplasias Ovarianas/diagnóstico , Adolescente , Diagnóstico Diferencial , Feminino , HumanosRESUMO
Given the paucity of data, we aimed to assess the impact of obesity on disease activity, complications, and quality of life (QoL) in pediatric inflammatory bowel disease (IBD) patients. Methods: Prospective analysis of pediatric IBD patients. Patients were categorized into 4 groups according to the World Health Organization (WHO) child growth standards: obese, overweight, normal weight, and underweight. Results: Three hundred twenty-seven pediatric patients were included (146 with Crohn's disease [CD], 181 with ulcerative colitis of whom 13 [4%] were underweight, 272 [83.2%] had normal weight, 22 [6.7%] were overweight, and 20 [6.1%] were obese). Compared with normal weight patients, obese ulcerative colitis had a significantly higher clinical but not biological disease activity nor severity. Compared with normal weight patients, overweight/obese CD patients did not have higher clinical or biological disease activity nor severity. Perianal abscesses and surgery for this purpose were more frequently observed in overweight/obese CD patients compared with normal weight controls. Overweight/obese IBD patients were similarly hospitalized in the last 12 months compared with normal weight controls. Conclusions: Prevalence of overweight/obesity was 12.8% in pediatric IBD patients. Obesity was not associated with a decrease in disease remission rates nor an increase in the risk of complicated disease progression in IBD pediatric patients, except for the occurrence of perianal abscesses and related surgery in CD patients.
RESUMO
Introduction: Given the lack of data, we aimed to assess the impact of the length of diagnostic delay on the natural history of ulcerative colitis (UC) in pediatric (diagnosed <18 years) and adult patients (diagnosed ≥18 years). Methods: Data from the Swiss Inflammatory Bowel Disease Cohort Study were analyzed. Diagnostic delay was defined as the interval between the first appearance of UC-related symptoms until diagnosis. Logistic regression modeling evaluated the appearance of the following complications in the long term according to the length of diagnostic delay: colonic dysplasia, colorectal cancer, UC-related hospitalization, colectomy, and extraintestinal manifestations (EIMs). Results: A total of 184 pediatric and 846 adult patients were included. The median diagnostic delay was 4 [IQR 2-7.5] months for the pediatric-onset group and 3 [IQR 2-10] months for the adult-onset group (p = 0.873). In both, pediatric- and adult-onset groups, the length of diagnostic delay at UC diagnosis was not associated with colectomy, UC-related hospitalization, colon dysplasia, and colorectal cancer. EIMs were significantly more prevalent at UC diagnosis in the adult-onset group with long diagnostic delay than in the adult-onset group with short diagnostic delay (p = 0.022). In the long term, the length of diagnostic delay was associated in the adult-onset group with colorectal dysplasia (p = 0.023), EIMs (p < 0.001), and more specifically arthritis/arthralgias (p < 0.001) and ankylosing spondylitis/sacroiliitis (p < 0.001). In the pediatric-onset UC group, the length of diagnostic delay in the long term was associated with arthritis/arthralgias (p = 0.017); however, it was not predictive for colectomy and UC-related hospitalization. Conclusions: As colorectal cancer and EIMs are associated with considerable morbidity and costs, every effort should be made to reduce diagnostic delay in UC patients.
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INTRODUCTION: Celiac disease typically presents with symptoms of malabsorption, but extraintestinal manifestations are increasingly reported. Aplastic anemia as the mode of celiac disease presentation is extremely rare in children. CASE PRESENTATION: We report a 2-year-old boy who presented with loose stools, loss of appetite, and bicytopenia with severe aregenerative normocytic anemia. Investigations, including bone marrow aspirate and biopsy, revealed aplastic anemia. Screening for malabsorption showed increased plasma concentrations of anti-transglutaminase and anti-gliadin antibodies. A duodenal biopsy confirmed the histologic features of celiac disease. The child received a packed red cell transfusion and was started on a gluten-free diet, with a very good prognosis and normalization of both his blood and histological parameters. To the best of our knowledge, our report is the sixth pediatric case in the literature. CONCLUSION: Screening for celiac disease should be performed in children with unexplained hematological abnormalities such as aplastic anemia with or without gastrointestinal symptoms.