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BACKGROUND: Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. AIM: To create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. METHODS: A survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. RESULTS: A total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient's transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1-2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. CONCLUSION: This study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient's chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed.
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Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Transição para Assistência do Adulto , Humanos , Nutrição Parenteral no Domicílio/métodos , Insuficiência Intestinal/terapia , Doença Crônica/terapia , Transição para Assistência do Adulto/normas , Adulto , Criança , Inquéritos e Questionários , Europa (Continente)RESUMO
This study investigated the neurodevelopmental impact of pathogenic adenomatous polyposis coli (APC) gene variants in patients with familial adenomatous polyposis (FAP), a cancer predisposition syndrome. We hypothesized that certain pathogenic APC variants result in behavioral-cognitive challenges. We compared 66 FAP patients (cases) and 34 unaffected siblings (controls) to explore associations between APC variants and behavioral and cognitive challenges. Our findings indicate that FAP patients exhibited higher Social Responsiveness Scale (SRS) scores, suggesting a greater prevalence of autistic traits when compared to unaffected siblings (mean 53.8 vs. 47.4, Wilcoxon p = 0.018). The distribution of SRS scores in cases suggested a bimodal pattern, potentially linked to the location of the APC variant, with scores increasing from the 5' to 3' end of the gene (Pearson's r = 0.33, p = 0.022). While we observed a trend toward lower educational attainment in cases, this difference was not statistically significant. This study is the first to explore the connection between APC variant location and neurodevelopmental traits in FAP, expanding our understanding of the genotype-phenotype correlation. Our results emphasize the importance of clinical assessment for autistic traits in FAP patients, shedding light on the potential role of APC gene variants in these behavioral and cognitive challenges.
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BACKGROUND: Therapy-resistant constipation often is a frustrating clinical entity recognised by the persistence of infrequent and painful bowel movements faecal incontinence and abdominal pain despite intensive treatment. It is important to clearly define therapy-resistant constipation before children are subjected to invasive diagnostic and therapeutic procedures. AIM: To conduct a systematic review determining how paediatric interventional studies define therapy-resistant constipation. METHOD: We searched CENTRAL, MEDLINE, Embase, WHO ICTR and ClinicalTrials.gov. Studies that included patients with therapy-resistant constipation were identified. Data were extracted on criteria used for defining therapy-resistant constipation and reported using a meta-narrative approach highlighting areas of convergence and divergence in the findings. RESULTS: A total of 1553 abstracts were screened in duplicate, and 47 studies were included in the review. There were at least seven definitions used in the paediatric literature to define medically resistant constipation. The term intractable was used in 24 articles and 21 used the term refractory to describe therapy-resistant constipation. Out of them, only 14 articles have attempted to provide an explicit definition including a predefined time and prior therapy. There were 10 studies without a clear definition for therapy-resistant constipation. The duration before being diagnosed as therapy-resistant constipation varied from 1 months to 2 years among studies. Seven studies employed the Rome criteria (Rome III or Rome IV) to characterising constipation while five adopted the Rome III and European and North American paediatric societies definition of paediatric gastroenterology, hepatology and nutrition guideline of management of constipation in children. CONCLUSION: The current literature has no explicit definition for therapy-resistant constipation in children. There is a need for a detailed consensus definition to ensure consistency of future research and to avoid unnecessary and maybe even harmful, invasive diagnostic and therapeutic interventions.
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Constipação Intestinal , Humanos , Constipação Intestinal/terapia , Constipação Intestinal/diagnóstico , Constipação Intestinal/tratamento farmacológico , Criança , Adolescente , Pré-EscolarRESUMO
Parenteral nutrition (PN) is recognized as a complex high-risk therapy. Its practice is highly variable and frequently suboptimal in pediatric patients. Optimizing care requires evidence, consensus-based guidelines, audits of practice, and standardized strategies. Several pediatric scientific organizations, expert panels, and authorities have recently recommended that standardized PN should generally be used over individualized PN in the majority of pediatric patients including very low birth weight premature infants. In addition, PN admixtures produced and validated by a suitably qualified institution are recommended over locally produced PN. Licensed multi chamber bags are standardized PN bags that comply with Good Manufacturing Practice and high-quality standards for the finished product in the frame of their full manufacturing license. The purpose of this article is to review the practical aspects of PN and the evidence for using such multi-chamber bags in pediatric patients. It highlights the safety characteristics and the limitations of the different PN practices and provides some guidance for ensuring safe and efficient therapy in pediatric patients.
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Nutrição Parenteral , Humanos , Recém-Nascido , Nutrição Parenteral/normas , Nutrição Parenteral/métodos , Lactente , Criança , Pré-Escolar , Adolescente , Soluções de Nutrição Parenteral/normas , Recém-Nascido Prematuro , Guias de Prática Clínica como Assunto , Recém-Nascido de muito Baixo PesoRESUMO
BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. RESULTS: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. TYPE OF STUDY: Clinical consensus statement. LEVEL OF EVIDENCE: 3a.
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BACKGROUND: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands. METHODS: We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality. RESULTS: A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI. CONCLUSION: Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center.
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Infecções Relacionadas a Cateter , Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Infecções Estafilocócicas , Staphylococcus aureus , Taurina/análogos & derivados , Tiadiazinas , Humanos , Nutrição Parenteral no Domicílio/métodos , Nutrição Parenteral no Domicílio/efeitos adversos , Masculino , Infecções Relacionadas a Cateter/prevenção & controle , Infecções Relacionadas a Cateter/microbiologia , Infecções Relacionadas a Cateter/epidemiologia , Estudos Retrospectivos , Feminino , Criança , Pré-Escolar , Lactente , Infecções Estafilocócicas/prevenção & controle , Adolescente , Países Baixos , Insuficiência Intestinal/terapia , Recém-Nascido , Cateteres Venosos Centrais/efeitos adversos , Cateteres Venosos Centrais/microbiologia , Doença Crônica , Incidência , Remoção de Dispositivo , Estudos de Coortes , Cateterismo Venoso Central/efeitos adversos , Cateteres de Demora/efeitos adversos , Cateteres de Demora/microbiologia , Bacteriemia/prevenção & controle , Bacteriemia/epidemiologia , Bacteriemia/etiologiaRESUMO
OBJECTIVES/BACKGROUND: High-output stoma is one of the most common major morbidities in young children with an enterostomy that could lead to intestinal failure. Management of high-output enterostomy in children is mostly based on personal experience. This systematic review aims to clarify the evidence-based therapeutic approach of high-output enterostomy in children. METHODS: A systematic review was performed using Pubmed, Embase (Ovid), and Cochrane Library to identify studies published until March 20, 2023, following the 2020 Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline. The study population comprised children (i.e., age <18 years) with high-output enterostomy (i.e., jejuno-, ileo-, and/or colostomy), regardless of underlying aetiology. Interventions comprised any (non)pharmacological and/or surgical treatment. Interventions were compared with each other, placebos, and/or no interventions. Primary outcome was reduction of enterostomy output. Secondary outcomes were morbidity, mortality, quality of life, associated healthcare costs, and adverse events. RESULTS: The literature search identified 4278 original articles of which 366 were screened on full text, revealing that none of the articles met the inclusion criteria. CONCLUSION: This first systematic review on management of high-output enterostomy in children revealed that any evidence on the primary and secondary outcomes is lacking. There is an urgent need for evidence on conservative treatment strategies including fluid restrictions, dietary advices, oral rehydration solution, chyme re-infusion, and pharmacological and surgical treatments of high-output enterostomy in children, aiming to reduce the risk for short- and long-term complications. Till more evidence is available, a systematic and multidisciplinary step-up approach is needed. Therefore, a therapeutic work-up is proposed that could guide the care.
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Enterostomia , Humanos , Enterostomia/métodos , Criança , Pré-Escolar , Insuficiência Intestinal/terapia , Insuficiência Intestinal/cirurgia , Medicina Baseada em Evidências , Qualidade de Vida , LactenteRESUMO
INTRODUCTION: To date, no international guidelines have been published for the treatment of paediatric functional abdominal pain disorders (FAPDs), subcategorised into functional abdominal pain-not otherwise specified (FAP-NOS), irritable bowel syndrome (IBS), functional dyspepsia and abdominal migraine (AM). We aim for a treatment guideline, focusing on FAP-NOS, IBS and AM, that appreciates the extensive array of available therapies in this field. We present the prospective operating procedure and technical summary protocol in this manuscript. METHODS: Grading of Recommendations, Assessment, Development and Evaluation (GRADE) will be followed in the development of the guideline, following the approach as laid out in the GRADE handbook, supported by the WHO. The Guideline Development Group (GDG) is formed by paediatric gastroenterologists from both the European Society for Pediatric Gastroenterology, Hepatology and Nutrition, as well as the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Also, one clinical psychologist with expertise in FAPDs is a voting member in the GDG. A final consensus list of treatment options is translated into 'patient, intervention, comparison, outcome' format options. Prospective agreement on the magnitude of health benefits or harms categories was reached through a Delphi process among the GDG to support grading of the literature.There will be a detailed technical evidence review with randomised controlled trial data that will be judged for risk of bias with the Cochrane tool. Recommendations are preferably based on GRADE but could also be best practice statements following the available evidence. A full Delphi process will be used to make recommendations using online response systems. This set of procedures has been approved by all members of the GDG.
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Dispepsia , Gastroenterologia , Síndrome do Intestino Irritável , Transtornos de Enxaqueca , Criança , Humanos , Dor Abdominal , Dispepsia/tratamento farmacológico , Estudos Prospectivos , Ensaios Clínicos Controlados Aleatórios como Assunto , Guias de Prática Clínica como AssuntoRESUMO
OBJECTIVES: The objective of this study is to assess the psychopathology and medical traumatic stress in children with intestinal failure (IF) and identify associated risk factors. METHODS: Two-center study, performed from September 2019 until April 2022 (partly during COVID-19 pandemic), including children (1.5-17 years) with IF, dependent on parenteral nutrition (PN) or weaned off PN, treated by a multidisciplinary IF-team. Psychopathology in children was evaluated with a semi-structured interview assessing psychiatric classifications and validated questionnaires assessing emotional (internalizing) and behavioral (externalizing) problems. Medical traumatic stress was assessed with a validated questionnaire. Problem scores were compared with normative data. Associations between clinical characteristics and outcomes were analyzed with linear regression analyses. RESULTS: Forty-one (of 111 eligible) children were included [median age 8.9 years (interquartile range, IQR 5.5-11.8), 54% female, 73% born preterm]. Median PN-duration was 17.3 months (IQR 6.9-54.0); 17 children (41%) were still PN-dependent. One third of the children met criteria for at least 1 psychiatric classification (compared with 14% in age-matched general population). Anxiety disorders and attention deficit hyperactivity disorder were most common. In school-aged children (n = 29, 6-17 years), significantly increased emotional problems were consistently reported by children ( P = 0.011), parents ( P < 0.001), and teachers ( P = 0.004). In preschool children (n = 12, 1.5-5 years), no significant differences with normative data were found. Subclinical or clinical emotional problems were reported in 19 children (46%). Medical traumatic stress was present in 14%, and 22% of children had received psychological help for trauma before. Lower gastrointestinal related quality of life was associated with more emotional problems, but not PN-duration. CONCLUSIONS: Children with IF, particularly school-aged children, are at risk for psychological problems which is reflected by the high rate of received psychotherapy and the high rate of emotional problems and psychiatric classifications.
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Transtorno do Deficit de Atenção com Hiperatividade , Transtornos do Comportamento Infantil , Insuficiência Intestinal , Recém-Nascido , Pré-Escolar , Criança , Humanos , Feminino , Masculino , Transtornos do Comportamento Infantil/epidemiologia , Qualidade de Vida , Pandemias , Transtorno do Deficit de Atenção com Hiperatividade/complicaçõesRESUMO
OBJECTIVE: To summarize available data on defecation frequency and stool consistency of healthy children up to age 4 in order to estimate normal references values. STUDY DESIGN: Systematic review including cross-sectional, observational, and interventional studies published in English, that reported on defecation frequency and/or stool consistency in healthy children 0-4 years old. RESULTS: Seventy-five studies were included with 16â393 children and 40â033 measurements of defecation frequency and/or stool consistency. Based on visual inspection of defecation frequency data, a differentiation was made between two age categories: young infants (0-14 weeks old) and young children (15 weeks-4 years old). Young infants had a mean defecation frequency of 21.8 per week (95 % CI, 3.9-35.2) compared with 10.9 (CI, 5.7-16.7) in young children (P < .001). Among young infants, human milk-fed (HMF) infants had the highest mean defecation frequency per week (23.2 [CI, 8.8-38.1]), followed by formula-fed (FF) infants (13.7 [CI 5.4-23.9]), and mixed-fed (MF) infants (20.7 [CI, 7.0-30.2]). Hard stools were infrequently reported in young infants (1.5%) compared with young children (10.5%), and a reduction in the frequency of soft/watery stools was observed with higher age (27.0% in young infants compared with 6.2% in young children). HMF young infants had softer stools compared with FF young infants. CONCLUSIONS: Young infants (0-14 weeks old) have softer and more frequent stools compared with young children (15 weeks-4 years old).
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Defecação , Leite Humano , Lactente , Humanos , Criança , Pré-Escolar , Recém-Nascido , Estudos Transversais , Diarreia , Alimentos Formulados , FezesRESUMO
OBJECTIVES: Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines. METHODS: An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams. RESULTS: In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse. CONCLUSIONS: Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed.
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Insuficiência Intestinal , Pseudo-Obstrução Intestinal , Criança , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/genética , Pseudo-Obstrução Intestinal/terapia , Nutrição Parenteral , Inquéritos e QuestionáriosRESUMO
INTRODUCTION: A stoma will cause nutrients loss which could result in impaired growth. Impaired growth can negatively impact long-term development. This study aims to evaluate: (1) the effect of stomas on growth comparing small bowel stoma versus colostomy and (2) if early closure (within 6 weeks), proximal small bowel stoma (within 50 cm of Treitz), major small bowel resection (≥ 30 cm), or adequate sodium supplementation (urinary level ≤ 30 mmol/L) influences growth. METHODS: Young children (≤ 3 years) treated with stomas between 1998 and 2018 were retrospectively identified. Growth was measured with weight-for-age Z-scores. Malnourishment was defined using the World Health Organization's definition. Comparison between changes in Z-scores at creation, closure, and a year following closure was done by Friedman's test with post hoc Wilcoxon's signed rank test or Wilcoxon's rank-sum test when necessary. RESULTS: In the presence of a stoma in 172 children, 61% showed growth decline. Severe malnourishment was seen at the time of stoma closure in 51% of the patients treated by small bowel stoma and 16% of those treated by colostomy. Within a year following stoma closure, 67% showed a positive growth trend. Having a proximal small bowel stoma and undergoing major small bowel resection led to significantly lower Z-scores at closure. Adequate sodium supplementation and early closure did not lead to significant changes in Z-scores. CONCLUSION: Stomas have a negative impact on growth in the majority of children. This impact might be decreased by preventing small bowel stomas when possible, specifically proximal stomas, and limiting small bowel resection. Since stoma closure is essential in reversing the negative effect on growth, we opt that early closure might result in an early shift to catch-up growth.
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Functional constipation is a common problem in childhood worldwide and has a great impact on social, physical, and emotional functioning of affected children and their caregivers. It is a clinical diagnosis based on the Rome IV criteria. Non-pharmacological treatment involves education, demystification, lifestyle advice, and toilet training. Pharmacological treatment consists of disimpaction, maintenance treatment, and eventually weaning if possible. Polyethylene glycol is considered as the first choice of laxative for both disimpaction and maintenance treatment. Different osmotic laxatives, stimulant laxatives, lubricants, and enemas are available as alternative pharmacological treatment options. Novel drugs are emerging but evidence to support the widespread application of these drugs in the pediatric population is often lacking and more high-quality research is needed in this field. If children remain symptomatic despite optimal pharmacological treatment, botulinum toxin injections in the anal sphincter can be considered as an alternative, more invasive treatment option. This review provides an update on currently available literature concerning the pharmacologic treatment of functional constipation in children.
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Constipação Intestinal , Laxantes , Criança , Humanos , Laxantes/uso terapêutico , Constipação Intestinal/tratamento farmacológico , Polietilenoglicóis/uso terapêuticoRESUMO
BACKGROUND: In research on pediatric chronic intestinal failure, heterogeneity in reported definitions and outcomes exists. This leads to a risk of reporting bias and impossibility of evidence synthesis. Also, reported outcomes should be relevant to both healthcare providers and patients and their parents. Therefore, the aim of this study is to create a core outcome set (COS) to be used in studies on pediatric chronic intestinal failure. METHODS: Candidate outcomes were selected from a recent systematic review. A three-round Delphi study among key stakeholders and a consensus meeting with an expert panel were undertaken to achieve consensus on the COS. RESULTS: Seventy-two stakeholders (79%) completed all three rounds of the Delphi process. Ninety-eight outcomes were assessed, and five new outcomes were added after the first round. Ten outcomes were included in the final COS: weaning from parenteral nutrition, growth, mortality, central line-related infection, central line longevity, sepsis not related to central line infection, central line-related thrombosis, intestinal failure-associated liver disease, (serious) adverse events, and health-related quality of life. CONCLUSION: This pediatric chronic intestinal failure COS consists of 10 outcomes important for all key stakeholders. Usage of this set in future research should minimize outcome heterogeneity and enhance the value of evidence synthesis. This will lead to better management in this field of rare gastrointestinal conditions.
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Enteropatias , Insuficiência Intestinal , Falência Hepática , Humanos , Criança , Qualidade de Vida , Projetos de Pesquisa , Técnica Delphi , Doença Crônica , Avaliação de Resultados em Cuidados de Saúde , Resultado do TratamentoRESUMO
Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1−4 and 14−18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.
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Enteropatias , Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Adulto , Criança , Doença Crônica , Estudos Transversais , Feminino , Humanos , Enteropatias/epidemiologia , Enteropatias/terapia , Masculino , Síndrome do Intestino Curto/terapiaRESUMO
Background: Children with intestinal failure (IF) require parenteral nutrition (PN). Transition to oral and enteral nutrition (EN) can be difficult also due to abnormal gastrointestinal motility. The gut hormone ghrelin is increased in states of negative energy balance, functioning to preserve euglycemia, and also has appetite stimulating and prokinetic properties. We aimed to evaluate and compare ghrelin levels in children with IF, and to assess the relationship with PN-dependency. Methods: In this exploratory prospective multicenter study, plasma acylated (AG) and unacylated (UAG) ghrelin levels were measured in children with short bowel syndrome (SBS) and with functional IF (pseudo-obstruction or any enteropathy) and compared with healthy control subjects. Spearman's rho (rs) was used to assess correlations of AG and UAG with PN-dependency (%PN) and parenteral glucose intake. Results: Sixty-four samples from 36 IF-patients were analyzed. Median baseline AG and UAG levels were respectively 279.2 and 101.0 pg/mL in children with SBS (n = 16), 126.4 and 84.5 pg/mL in children with functional IF (n = 20) and 82.4 and 157.3 pg/mL in healthy children (n = 39). AG levels were higher in children with SBS and functional IF than in healthy children (p = 0.002 and p = 0.023, respectively). In SBS, AG positively correlated with %PN (rs = 0.5, p = 0.005) and parenteral glucose intake (rs = 0.6, p = 0.003). These correlations were not observed in functional IF. Conclusion: Children with IF had raised AG levels which could be related to starvation of the gut. The positive correlation between AG and glucose infusion rate in SBS suggests an altered glucoregulatory function.
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Disorders of the gut-brain interaction negatively impact quality of life and carry a substantial socioeconomic burden. Irritable bowel syndrome (IBS) and functional abdominal pain-not otherwise specified (FAP-NOS) are common functional abdominal pain disorders in childhood. The pathophysiology is not fully understood, and high-quality intervention trials and international guidelines are missing. Therefore, the management of these disorders remains challenging. This review aims to provide an up-to-date overview of therapeutic possibilities for pediatric IBS or FAP-NOS and recommends management strategies. To prevent unnecessary referrals and extensive costs, it is fundamental to make a positive diagnosis of IBS or FAP-NOS in children with chronic abdominal pain with only minimal investigations. A tailor-made approach for each patient, based on the accompanying physical and psychological symptoms, is proposed to date. CONCLUSION: Shared decision-making including non-pharmacological and pharmacological interventions should be considered and discussed with the family. WHAT IS KNOWN: ⢠Irritable bowel syndrome and functional abdominal pain-not otherwise specified are common in childhood. ⢠Although the number of treatment options has grown recently, managing these disorders can be challenging and unsatisfactory, and no evidence-based international management guidelines are available. WHAT IS NEW: ⢠We suggest using a stepwise individualized approach to management, where after first-line management, both non-pharmacological and pharmacological interventions should be discussed.
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Síndrome do Intestino Irritável , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Dor Abdominal/terapia , Criança , Humanos , Síndrome do Intestino Irritável/complicações , Síndrome do Intestino Irritável/diagnóstico , Síndrome do Intestino Irritável/terapia , Qualidade de VidaRESUMO
Importance: Functional abdominal pain disorders (FAPDs) can severely affect the life of children and their families, with symptoms carrying into adulthood. Management of FADP symptoms is also a financial and time burden to clinicians and health care systems. Objective: To systematically review various randomized clinical trials (RCTs) on the outcomes of cognitive behavioral therapy (CBT), educational support, yoga, hypnotherapy, gut-directed hypnotherapy, guided imagery, and relaxation in the management of FAPDs. Data Sources: PubMed, MEDLINE, Embase, PsycINFO, and Cochrane Library. Study Selection: All RCTs that compared psychosocial interventions with any control or no intervention, for children aged 4 to 18 years with FAPDs. Data Extraction and Synthesis: Pairs of the authors independently extracted data of all included studies, using a predesigned data extraction sheet. One author acted as arbitrator. Risk of bias was assessed using the Cochrane risk of bias tool, and certainty of the evidence for all primary outcomes was analyzed using the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) framework. Main Outcomes and Measures: Primary outcomes were treatment success, pain frequency, pain intensity, and withdrawal owing to adverse events. Dichotomous outcomes were expressed as risk ratio (RR) with corresponding 95% CIs. Continuous outcomes were expressed as mean difference (MD) or standardized MD with 95% CI. Results: A total of 33 RCTs with 2657 children (median [range] age, 12 [7-17] years; 1726 girls [67.3%]) were included. Twelve studies compared CBT with no intervention, 5 studies compared CBT with educational support, 3 studiescompared yoga with no intervention, 2 studies compared hypnotherapy with no intervention, 2 studies compared gut-directed hypnotherapy with hypnotherapy, and 2 studies compared guided imagery with relaxation. Seven studies evaluated other unique comparisons (eg, visceral osteopathy vs normal osteopathy). Per the GRADE framework, owing to risk of bias, there was moderate certainty in evidence that CBT was associated with higher treatment success numbers (n = 324 children; RR, 2.37; 95% CI 1.30-4.34; number needed to treat [NNT] = 5), lower pain frequency (n = 446 children; RR, -0.36; 95% CI, -0.63 to -0.09), and lower pain intensity (n = 332 children; RR, -0.58; 95% CI, -0.83 to -0.32) than no intervention. Owing to high imprecision, there was low certainty in evidence that there was no difference between CBT and educational support for pain intensity (n = 127 children; MD, -0.36; 95% CI, -0.87 to 0.15). Owing to risk of bias and imprecision, there was low certainty in evidence that hypnotherapy resulted in higher treatment success compared with no intervention (n = 91 children; RR, 2.86; 95% CI, 1.19-6.83; NNT = 5). Owing to risk of bias and imprecision, there was low certainty in evidence that yoga had similar treatment success to no intervention (n = 99 children; RR, 1.09; 95% CI, 0.58-2.08). Conclusions and Relevance: Results of this systematic review and meta-analysis suggest that CBT and hypnotherapy may be considered as a treatment for FAPDs in childhood. Future RCTs should address quality issues to enhance the overall certainty of the results, and studies should consider targeting these interventions toward patients who are more likely to respond.
Assuntos
Terapia Cognitivo-Comportamental , Hipnose , Dor Abdominal/terapia , Adulto , Criança , Feminino , Humanos , Intervenção Psicossocial , Resultado do TratamentoRESUMO
BACKGROUND: Functional abdominal pain disorders (FAPDs) are common among children and are associated with decreased quality of life and school attendance. Several dietary interventions have been suggested to improve symptoms of FAPDs. This systematic review assessed the efficacy and safety of dietary interventions for pediatric FAPDs. DESIGN AND METHODS: Electronic databases were searched (inception-October 2021). Systematic reviews or RCTs were included if children (4-18 years) with FAPDs were treated with dietary interventions and compared to placebo, no diet or any other diet. Data extraction and assessment of quality of evidence based on GRADE system was independently performed by two review authors. Outcomes were treatment success, pain intensity and frequency, and withdrawal due to adverse events. RESULTS: Twelve articles were included, representing data of 819 pediatric FAPD patients. Trials investigating fibers, FODMAP diet, fructans, fructose-restricted diet, prebiotic (inulin), serum-derived bovine immunoglobulin, and vitamin D supplementation were included. We found very low-certainty evidence that the use of fibers leads to higher treatment success (NNT = 5). CONCLUSION: Based on current evidence, the use of fibers can be discussed in daily practice. High-quality intervention trials are highly needed to investigate if other dietary interventions are effective in the treatment of pediatric FAPD.
Assuntos
Dor Abdominal , Qualidade de Vida , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Dor Abdominal/terapia , Animais , Bovinos , Criança , Humanos , Medição da Dor , Resultado do TratamentoRESUMO
OBJECTIVE: To develop a core outcome set for clinical studies assessing gastroesophageal reflux disease (GERD) in children. STUDY DESIGN: This core outcome set was developed using a 2-round Delphi technique and adhering to the Outcome Measures in Rheumatology Initiative (OMERACT 2.0) recommendations. Healthcare professionals (HCPs) and (parents of) children (age 1-18 years) with a GERD diagnosis (ie, the presence of bothersome symptoms), listed up to 5 harmful and/or beneficial outcomes that they considered important in the treatment of GERD. Outcomes mentioned by more than 10% of participants were put forward and rated and prioritized by HCPs, parents, and children in a second round. Outcomes with the highest rank formed the draft core outcome set. The final core outcome set was created during an online consensus meeting between an expert panel. RESULTS: The first round was completed by 118 of 125 HCPs (94%), 146 of 146 parents (100%), and 69 of 70 children (99%). A total of 80 of 118 HCPs (68%), 130 of 140 parents (93%), and 77 children (100%) completed round 2. "Adequate relief," "evidence of esophagitis," "feeding difficulties," "heartburn (≥4 years)," "hematemesis," "regurgitation," "sleeping difficulties," "vomiting," and "adverse events" were included in the final core outcome set for GERD in children aged 1-18 years. CONCLUSIONS: We identified a total set of 9 core outcomes and suggest these outcomes to be minimally measured in clinical studies assessing GERD in children. Implementation of this core outcome set is likely to increase comparison between studies and may thus provide future recommendations to improve treatment of GERD in children.