RESUMEN
OBJECTIVES: This study described disease characteristics and long-term outcomes in patients diagnosed with very early onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age) and infantile-IBD (before 2 years). METHODS: Cases from 21 centers worldwide diagnosed with VEOIBD (2008-2018), with minimum 2 years of follow-up, were retrospectively reviewed. RESULTS: The cohort included 243 patients (52% males, median follow-up of 5.8 [range 2-18] years, including 69 [28%]) with infantile-IBD. IBD subtypes included Crohn's disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU) in 30%, 59%, and 11%, respectively. Among patients with CD, 94% had colonic involvement, and among patients with UC/IBDU, 75% had pancolitis. Patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels, and higher C-reactive protein, and had lower response rates to first-induction therapy and corticosteroids therapy (P < .05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population, 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention, and higher rates of major infections (P < .05 for all). CONCLUSIONS: Patients with VEOIBD, including infantile-IBD, exhibit low rate of complications and surgical interventions at the long term. Patients with monogenic IBD are at risk for more severe disease course.
Asunto(s)
Edad de Inicio , Humanos , Masculino , Femenino , Estudios Retrospectivos , Preescolar , Lactante , Adolescente , Niño , Enfermedades Inflamatorias del Intestino/genética , Enfermedades Inflamatorias del Intestino/diagnóstico , Enfermedades Inflamatorias del Intestino/epidemiología , Enfermedades Inflamatorias del Intestino/terapia , Estudios de Seguimiento , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/epidemiología , Enfermedad de Crohn/terapia , Enfermedad de Crohn/genética , Enfermedad de Crohn/cirugía , Colitis Ulcerosa/diagnóstico , Colitis Ulcerosa/terapia , Colitis Ulcerosa/genética , Colitis Ulcerosa/cirugía , Colitis Ulcerosa/epidemiologíaRESUMEN
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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Insuficiencia Intestinal , Nutrición Parenteral en el Domicilio , Transición a la Atención de Adultos , Humanos , Nutrición Parenteral en el Domicilio/métodos , Insuficiencia Intestinal/terapia , Enfermedad Crónica/terapia , Transición a la Atención de Adultos/normas , Adulto , Niño , Encuestas y Cuestionarios , Europa (Continente)RESUMEN
Celiac disease (CeD) is likely to be associated with growth impairment and poor weight gain. However, long-term growth patterns following diagnosis are poorly characterized. We evaluated long-term anthropometric changes in a large cohort of pediatric patients with CeD. A retrospective chart review of patients diagnosed with CeD between 1999 and 2018 was conducted. Demographic and clinical data were collected, and anthropometrics were analyzed from diagnosis and throughout follow-up. The study included 500 patients (59.8% females, median (IQR) age at diagnosis 5.7 (3.7-8.9) years), with a mean follow-up of 5.5 (range 1.5-16.2) years. Weight, height, and BMI Z-score-for-age (WAZ, HAZ, and BMIZ) increased significantly from a mean (± SD) of - 0.82 (± 1.21), - 0.73 (± 1.16), and - 0.32 (± 1.11) at diagnosis to - 0.41 (± 1.23), - 0.45(± 1.16), and - 0.17 (± 1.14) at last follow-up, respectively (p < 0.001 for WAZ and HAZ and p = 0.002 for BMIZ). The largest improvements were observed in patients diagnosed before 3 years of age (p < 0.01). Patients for whom the final adult height was available (n = 86) improved from HAZ mean (± SD) - 0.89 ± 1.37 at diagnosis to - 0.51 ± 1.28 at adulthood measurement, p < 0.05. Wasting was present in 19.7% and stunting in 16.4% of the cohort at diagnosis and normalized in 77.3% and 64.8%, respectively, within a median (IQR) time of 0.79 (0.42-4.24) and 2.3 (0.72-6.02) years, respectively. Gluten-free diet adherence and frequency of visits were not associated with normalization of wasting or stunting in all age groups. Conclusion: Over a long-term follow-up, pediatric patients with CeD demonstrate significant increases in weight, height, and BMI-for-age. Younger age at diagnosis is associated with greater improvement in weight and linear growth, emphasizing the importance of early diagnosis of CeD. What is Known: ⢠Celiac disease (СeD) is likely to be associated with growth impairment and poor weight gain. ⢠Long-term changes in anthropometric indices after diagnosis of CeD are not well characterized. What is New: ⢠Over a long-term follow-up, pediatric patients with CeD demonstrate significant increases in weight, height, and BMI-for-age. ⢠Young age at diagnosis is associated with larger improvement in weight and linear growth.
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Enfermedad Celíaca , Humanos , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/fisiopatología , Enfermedad Celíaca/dietoterapia , Femenino , Masculino , Niño , Estudios Retrospectivos , Preescolar , Estudios de Seguimiento , Adolescente , Trastornos del Crecimiento/etiología , Trastornos del Crecimiento/diagnóstico , Índice de Masa Corporal , Estatura , Antropometría/métodos , Aumento de Peso/fisiología , Peso CorporalRESUMEN
Background and Objectives: Potential Celiac Disease (PCD) is defined by positive celiac serology without villous atrophy. We aimed to describe the short-term outcome of pediatric PCD while consuming a gluten-containing diet (GCD). Materials and Methods: Retrospective analysis of pediatric PCD patients continuing GCD, between December 2018-January 2022. Baseline demographics, celiac serology and duodenal biopsy results were reviewed. Follow-up data included repeated serology and biopsy results when performed. Minimum follow-up was 12 months unless celiac disease (CeD) was diagnosed earlier. Results: PCD was diagnosed in 90 children (71% females) with a mean age of 7.2 (range 1.8-16.5) years. Baseline anti-tissue transglutaminase (TTG) levels were above 10 times the upper limit of normal (ULN) in 17/90 (18.9%), 3-10 × ULN in 56/90 (62.2%) and 1-3 × ULN in 17/90 (18.9%). During follow-up, the mean time was 17.6 (range 5-35) months, TTG normalized in 34/90 (37.8%), was stable in 48/90 (53.3%), and increased or remained >10 × ULN in 8/90 (8.9%). In 20/90 (22.2%) patients, a repeat endoscopy was performed, leading to CeD diagnosis in 12/20 (60%). Thus, at the end of follow-up, CeD was diagnosed in 12/90 (13.3%). In patients with TTG >10 × ULN at diagnosis, TTG normalized in 5/17, decreased to 3-10 × ULN in 8/17, and remained above 10 × ULN in 4/17. Conclusions: During the short-term follow-up of pediatric PCD patients, less than 15% progressed to CeD. A third had normalized TTG levels, including children with TTG >10 × ULN, indicating the need for periodic serological and histological follow-up among PCD patients.
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Enfermedad Celíaca , Femenino , Niño , Humanos , Lactante , Preescolar , Adolescente , Masculino , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/epidemiología , Transglutaminasas , Proteína Glutamina Gamma Glutamiltransferasa 2 , Estudios Retrospectivos , Autoanticuerpos , Proteínas de Unión al GTP , Biopsia , Glútenes , Inmunoglobulina ARESUMEN
OBJECTIVES: Inadequate caloric intake is common in Crohn disease (CD) and nutrition support plays an important role in remission induction and in meeting nutritional needs. For nutritional planning, determining resting metabolic rate (RMR) is essential in pediatric patients with CD. STUDY AIM: To measure RMR in CD pediatric patients using indirect calorimetry, and compare it with estimated RMR (eRMR) calculated by Schofield equation. METHODS: Children with CD treated in a tertiary pediatric inflammatory bowel disease center in Israel participated in the cross-sectional study. At study visits, weight, height, clinical and laboratory assessments, bioelectrical impedance, and RMR measured by indirect calorimetry were done. Additionally, disease severity was determined by the Pediatric Crohn Disease Activity Index and eRMR was calculated using the Schofield equation. The ratio of the measured RMR to eRMR was performed in addition to the Spearman correlation test. RESULTS: The study group included 73 children (49 boys) with mean age of 13.9 ± 2.3 years. Children with moderate or severe disease had lower weight for age z score, lower BMI- z , and lower RMR compared to children with mild disease. However, when RMR was adjusted for fat free mass (n = 50), the association between RMR and disease severity disappeared. Variance in RMR was high between subjects. CONCLUSIONS: Our data suggest that the Schofield equation is inadequate for determining RMR in pediatric CD and that RMR should be measured to guarantee the most appropriate nutritional intervention.
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Metabolismo Basal , Enfermedad de Crohn , Masculino , Humanos , Niño , Adolescente , Estudios Transversales , Ingestión de Energía , Calorimetría Indirecta , Composición CorporalRESUMEN
BACKGROUND AND AIMS: To investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN). METHODS: Period of observation: March 1st, 2020 March 1st, 2021. INCLUSION CRITERIA: patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up. RESULTS: Sixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths. CONCLUSIONS: In patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death.
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COVID-19 , Enfermedades Intestinales , Insuficiencia Intestinal , Nutrición Parenteral en el Domicilio , Humanos , COVID-19/epidemiología , Enfermedades Intestinales/epidemiología , Enfermedades Intestinales/terapia , Nutrición Parenteral en el Domicilio/efectos adversosRESUMEN
OBJECTIVE: There is no gold standard to assess adherence to gluten-free diet (GFD) among patients with celiac disease (CeD). Gluten immunogenic peptides (GIPs) in urine and stool were suggested as novel markers for evaluating adherence to GFD. Our aim was to assess the presence of GIP in pediatric patients with CeD, and to compare the results with alternative methods for evaluating GFD adherence. METHODS: Pediatric patients diagnosed with CeD, who were on GFD for at least 1 year, were enrolled and followed prospectively between November 2018 and January 2021. Study visits included clinical assessment, a dietitian interview, Biagi score, food questionnaires, anthropometric and laboratory measurements, and urine and stool samples obtained for laboratory GIP analysis. RESULTS: The study included 74 patients (63.5% females), with median (interquartile range, IQR) age of 9.9 (7.8-11.7) years, and median (IQR) duration on GFD of 2.5 (2-5.5) years. Good GFD adherence, assessed by Biagi score, was reported in 93.1% of cases. GIP was evaluated during 134 visits, with GIP detected in 27 of 134 (20.1%) of the visits (16.3% of stool samples and 5.3% of urine samples). Positive GIP results were significantly more common in males compared to females (30.6% vs 14.1%, respectively, P < 0.05). Detection of positive GIP was not associated with dietary assessment of GFD adherence, celiac serology results, or reported symptoms. CONCLUSIONS: Stool and urine GIP can be detected in children with CeD, even when dietary assessment indicate good adherence to GFD. The role of GIP testing in clinical practice should be further explored.
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Enfermedad Celíaca , Glútenes , Masculino , Femenino , Humanos , Niño , Enfermedad Celíaca/diagnóstico , Dieta Sin Gluten , Cooperación del Paciente , PéptidosRESUMEN
OBJECTIVES: The glucagon-like peptide-2 analog Teduglutide has been shown to enhance intestinal absorption and decrease parenteral nutrition (PN) requirements in short bowel syndrome (SBS). As data in children is limited, we evaluated nationwide real-life experience and treatment outcome in children with SBS. METHODS: Longitudinal data of children treated with Teduglutide for ≥3 months was collected. Data included demographic and medical background, anthropometrics, laboratory assessments and PN requirements. Treatment response was defined as >20% reduction in PN requirement. RESULTS: The study included 13 patients [54% males, median (interquartile range {IQR}) age of 6 (4.7-7) years]. The most common SBS etiology was necrotizing enterocolitis (38%), and median (IQR) small bowel length was 20 (15-40) cm. Teduglutide treatment ranged between 3 and 51 months [median (IQR) of 18 (12-30) months], with 10 patients (77%) treated >1 year. Response to treatment was observed in 8 patients (62%), with a mean [±standard deviation (SD)] treatment duration of 5.9 (±3.2) months. Among responders, 2 patients were weaned off PN and additional 4 decreased PN needs by >40%. There was a median (IQR) reduction in PN volume/kg of 36% (15%-55%) and in PN energy/kg of 27% (6%-58%). Response was not associated with patients' background, and no correlation was found with bowel length or PN dependency at baseline. CONCLUSIONS: Real-life response to Teduglutide is highly variable among children with SBS. While most patients did reach 20% reduction in PN, less achieved further significant reduction or enteral autonomy. No predictive factors of response to treatment were identified, and large multicenter studies are needed to elucidate predictive factors and long-term outcome.
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Síndrome del Intestino Corto , Niño , Femenino , Fármacos Gastrointestinales/uso terapéutico , Humanos , Recién Nacido , Masculino , Nutrición Parenteral , Péptidos/uso terapéutico , Síndrome del Intestino Corto/tratamiento farmacológicoRESUMEN
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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Enfermedades Intestinales , Insuficiencia Intestinal , Nutrición Parenteral en el Domicilio , Síndrome del Intestino Corto , Adulto , Niño , Enfermedad Crónica , Estudios Transversales , Femenino , Humanos , Enfermedades Intestinales/epidemiología , Enfermedades Intestinales/terapia , Masculino , Síndrome del Intestino Corto/terapiaRESUMEN
OBJECTIVE: Tissue-transglutaminase antibodies (TGA) may be used to diagnose celiac disease (CD) without biopsy in selected cases. We aimed to investigate real-life performance of a CD serology automated analyzer (Bioplex2200), and to explore the correlation between TGA levels and intestinal biopsies in children. METHODS: A retrospective review was performed in 2 pediatric gastroenterological centers, between November 1, 2018 and April 1, 2020 and included patients with both TGA serology testing and duodenal biopsies. Retrieved data included patients' demographics, medical background, TGA levels, and biopsy results. RESULTS: Overall, 538 children were evaluated, 256 with positive TGA (68.4% girls, median age 6.4âyears), and 282 with negative TGA (53.9% girls, median age 13.4âyears). Among patients with positive TGA, intestinal biopsies confirmed CD in 219 (85.5%). Overall, positive serology with normal histology was found in 14.5% of the cohort, with 52%; 21.6%; 21.1%; and 4.2% in TGA ranges of 1 to 3 times upper limit of normal (ULN); 3 to 5 ULN; 5 to 10 ULN; and above 10 times ULN, respectively, Pâ<â0.001. Area under the receiver-operating characteristic curve (AUC) was 0.963 (95% CI 0.947-0.980). Among patients with positive TGA, 216 (84.4%) had positive anti-endomysial antibodies. In this sub-group, the overall diagnostic performance was inferior, with AUC of 0.737 (95% CI 0.834-0.839). CONCLUSIONS: The Multiplex TGA assay had a very high diagnostic accuracy in real-life. Among patients with positive TGA, adding EMA did not improve the diagnostic performance of the test. False-positive rates differed between different ranges of TGA and were low with TGA above 10 times ULN.
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Enfermedad Celíaca , Adolescente , Autoanticuerpos , Biopsia , Enfermedad Celíaca/patología , Niño , Femenino , Proteínas de Unión al GTP , Humanos , Inmunoglobulina A , Masculino , Proteína Glutamina Gamma Glutamiltransferasa 2 , TransglutaminasasRESUMEN
BACKGROUND: Long-term parenteral nutrition (PN) has been associated with renal complications, including hypercalciuria, nephrocalcinosis, proteinuria and reduced glomerular filtration rate (GFR). Pediatric data are scarce and mostly short-term. Our study aimed to evaluate renal complications in children with intestinal failure (IF) receiving long-term PN. METHODS: A cross-sectional study was performed in a tertiary pediatric IF clinic of patients receiving home-PN treatment for more than 1 year. Data regarding medical background, anthropometrics, laboratory investigations and abdominal sonography were retrieved. RESULTS: Complete data were available for 15 children (67% males), with a median age of 6 (range 1.5-15) years and a median (IQR) PN duration of 4 (1.5-6) years. Low-grade proteinuria was identified in 61% and microalbuminuria in 30% of the cohort. Hypercalciuria and hyperoxaluria were present in 50% and 46%, respectively. One patient had nephrocalcinosis. The estimated GFR was normal in all but one patient who had pre-existing kidney disease. CONCLUSIONS: Pediatric IF patients can present with preserved kidney function after years of PN treatment. Despite the high prevalence of hypercalciuria, nephrocalcinosis was not common. Base line and long-term monitoring of various aspects of renal function would be essential to characterize the effects of prolonged PN on kidney functions in pediatric patients.
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Intestinos/fisiopatología , Riñón/fisiopatología , Nutrición Parenteral Total , Adolescente , Aminoácidos/metabolismo , Niño , Preescolar , Creatinina/orina , Femenino , Humanos , Lactante , Masculino , Proteinuria/complicaciones , Proteinuria/orinaRESUMEN
Vitamin C is an essential nutrient that serves as antioxidant and plays a major role as co-factor and modulator of various pathways of the immune system. Its therapeutic effect during infections has been a matter of debate, with conflicting results in studies of respiratory infections and in critically ill patients. This comprehensive review aimed to summarize the current evidence regarding the use of vitamin C in the prevention or treatment of patients with SARS-CoV2 infection, based on available publications between January 2020 and February 2021. Overall, 21 publications were included in this review, consisting of case-reports and case-series, observational studies, and some clinical trials. In many of the publications, data were incomplete, and in most clinical trials the results are still pending. No studies regarding prevention of COVID-19 with vitamin C supplementation were found. Although some clinical observations reported improved medical condition of patients with COVID-19 treated with vitamin C, available data from controlled studies are scarce and inconclusive. Based on the theoretical background presented in this article, and some preliminary encouraging studies, the role of vitamin C in the treatment of patients with SARS-CoV-2 infection should be further investigated.
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Ácido Ascórbico/uso terapéutico , Tratamiento Farmacológico de COVID-19 , Vitaminas/uso terapéutico , Adulto , Anciano , Antioxidantes/administración & dosificación , Antioxidantes/uso terapéutico , Ácido Ascórbico/administración & dosificación , Niño , Ensayos Clínicos como Asunto , Suplementos Dietéticos , Vías de Administración de Medicamentos , Femenino , Humanos , Masculino , Persona de Mediana Edad , SARS-CoV-2 , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vitaminas/administración & dosificaciónRESUMEN
OBJECTIVES: Celiac disease (CD) is a common intestinal autoimmune disorder with diverse presenting features. We aimed to determine age-dependent patterns in CD presentation, diagnosis and management at a large tertiary referral center. METHODS: A retrospective review of electronic medical records of pediatric patients diagnosed with CD between January 1999 and December 2018 at Schneider Children's Medical Center of Israel. We compared demographics, clinical and laboratory parameters between four age groups at CD presentation. RESULTS: A cohort of 932 children was divided into four groups by age (in years) at diagnosis: 0-3 (17.9%), 3-6 (31.8%), 6-12 (34.5%), 12-18 (15.8%). The youngest age group presented more frequently with diarrhea, weight loss, abdominal distention, vomiting and lower weight z scores, Pâ<â0.01. Hypoalbuminemia and zinc deficiency were also more frequent in this age group, compared to older patients (Pâ<â0.05, each). Rates of anemia were higher in younger age groups (0-3 and 3-6âyears), compared to older age groups, Pâ<â0.05. Patients in the younger age groups (0-3 and 3-6âyears) presented more frequently with tissue transglutaminase (TTG) levels above 10 times the upper limit of normal (ULN; Pâ<â0.05), and more often normalized their CD serologies by 24âmonths of gluten-free diets (GFD) compared to older age groups (Pâ<â0.05). CONCLUSION: There is an age-dependent variation in CD presentation during childhood. Younger patients present more often with malabsorptive features, and higher TTG levels, yet normalize TTG while on GFD more rapidly than older patients. Clinicians should be aware of the diversity in CD presentation and course at the various presentation age.
Asunto(s)
Enfermedad Celíaca , Anciano , Autoanticuerpos , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/epidemiología , Niño , Dieta Sin Gluten , Humanos , Israel/epidemiología , Estudios Retrospectivos , TransglutaminasasRESUMEN
OBJECTIVES: Chronic inflammation of Crohn disease (CD) is associated with reduced bone mineral density (BMD). As bone mass is almost exclusively accrued during childhood, early recognition of osteopenia is especially important in pediatric CD. We aimed to identify variables associated with osteopenia to guide dual-energy X-ray absorptiometry (DXA) scan screening to those who most need it. METHODS: This was a retrospective inception cohort study of children newly diagnosed with CD, and routinely referred to DXA scans. Demographic and explicit clinical data were recorded along with whole-body less head BMD, adjusted for age, sex, and height by z-scores. RESULTS: Of the 116 included children (mean age 13â±â3.1 years, 67 [58%] boys, mean body mass index [BMI] 16.7â±â2.6), 63 (54%) had normal BMD (z-scoreâ>â-1) or borderline osteopenia (-1 ≥ z-scoreâ>â-2) and 53 (46%) had osteopenia (z-scoreâ≤â-2). Osteopenia was associated with lower BMI z-score (-0.8â±â1.2 vs -1.8â±â1.1, Pâ<â0.001) and higher PCDAI (33.7â±â15.2 vs 25.7â±â16.5; Pâ=â0.009) than those with BMD z-score >-2. In total, 59% of children with BMI z-score <-0.5 had moderate-severe osteopenia and only 18% of those with higher z-scores. Multivariate logistic regression identified BMI z-score as the sole risk factor (OR 1.28 [95% CI 1.08-1.52], Pâ=â0.005). BMI z-score ≥-0.5 excludes osteopenia with a sensitivity 87%, specificity 49%, NPV 82%, and PPV 59%. CONCLUSIONS: Osteopenia was found in nearly half of children with newly onset CD. BMI z-score <-0.5 should prompt referral to DXA screening.
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Enfermedades Óseas Metabólicas , Enfermedad de Crohn , Absorciometría de Fotón , Adolescente , Densidad Ósea , Enfermedades Óseas Metabólicas/diagnóstico , Enfermedades Óseas Metabólicas/epidemiología , Enfermedades Óseas Metabólicas/etiología , Niño , Estudios de Cohortes , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/diagnóstico , Humanos , Masculino , Estudios RetrospectivosRESUMEN
Celiac disease (CD) is increasingly diagnosed without endoscopy. As such, the need for accurate serological markers to aid in the diagnosis and follow-up of CD has increased. Intestinal fatty acid binding protein (I-FABP) is a cytosolic protein present in enterocytes, whose blood levels reflect mucosal damage in a reliable and quantifiable way. The aim of this study was to compare I-FABP levels in newly diagnosed patients with CD and to examine changes in levels following 6 months of gluten-free diet (GFD). Methods: A prospective observational case control study of pediatric patients diagnosed with CD, with measurements of tissue transglutaminase IgA (TTG-IgA) and I-FABP levels at diagnosis and after 6 months of gluten free diet were compared to a control group of nonceliac patients. Results: This study included 35 patients and 32 controls. The CD group had higher I-FABP levels at diagnosis compared with the control group (median 641.7 pg/mL versus 334 pg/mL; P < 0.05). I-FABP levels significantly differed between patients presenting with TTG-IgA level 3-10 times the upper limit of normal (ULN) compared with those presenting with values >10 times ULN (median 432.2 pg/mL versus 796.2 pg/mL; P < 0.05). Patients with CD had a significant decrease in median I-FABP levels after 6 months of GFD (median 268.2 pg/mL), paralleling a decrease in TTG-IgA and GFD adherence. Conclusions: I-FABP levels are increased in patients with CD at diagnosis compared with controls and decrease significantly while patients adhere to GFD.
RESUMEN
OBJECTIVES: The aim of this study was to examine the effect of massive small bowel resection on proinflammatory cytokine intestinal expression and the effect of anti-TNF-α antibodies (ATA) on intestinal inflammation, epithelial cell turnover, and intestinal adaptation after bowel resection in rats. METHODS: Male Sprague-Dawley rats were divided into 4 experimental groups: Sham-rats underwent bowel transection; Sham-ATA rats underwent bowel transection and were treated with ATA; SBS-animals underwent 75% bowel resection; and SBS-ATA rats underwent bowel resection and were treated with ATA similarly to Group B. Parameters of intestinal adaptation, enterocyte proliferation, and apoptosis were determined at sacrifice. TNF-α and apoptosis-related gene and protein levels were determined by Illumina's Digital Gene Expression (DGE) analysis, Real Time PCR, Western blotting, and immunohistochemistry. RESULTS: From 25 genes related to TNF-α signalling that were investigated, 8 genes in the jejunum and 10 genes in the ileum were found to be up-regulated in resected versus sham animals. SBS rats demonstrated a significant increase in tissue and plasma TNF-α, IL-6 levels, intestinal mucosal TNF-α related gene expression, and microscopic parameters of inflammation. Treatment of resected animals with ATA resulted in a significant decrease in TNF-α levels, intestinal mucosal TNF-α-related gene expression, decreased number of intraepithelial lymphocytes and macrophages, and lower apoptotic index compared with SBS animals. CONCLUSIONS: In a rat model of SBS, ATA decreased plasma and tissue TNF-α levels, diminished mucosal inflammation, and inhibited cell apoptosis. Anti-apoptotic effects of ATA appear to be associated with an inhibited extrinsic apoptotic pathway.