The Effects of Gluten-free Diet on Body Mass Indexes in Adults with Celiac Disease: A Systematic Review and Meta-analysis of Observational Studies.

GOALS AND BACKGROUND: Gluten-free diet (GFD) includes a higher intake of sugars and fats. Previous studies have investigated its effect on body mass index (BMI) in celiac disease (CD) patients but had contradictive conclusions. Thus, we conducted a systematic review and meta-analysis examining the effect of GFD on BMI in CD patients. STUDY: Systematically, we conducted literature research using Medline, Scopus, and Embase, and we identified 1565 potential studies/abstracts. Only studies of patients with CD under a GFD with recorded BMI before and after dietary intervention were included. Subgroup analyses based on study design and BMI categories were performed. We calculated the pooled odds ratios (ORs) and 95% confidence intervals (Cls) for the number of patients in each BMI group according to the World Health Organization (WHO) definitions after GFD using fixed and random effect meta-analysis. RESULTS: The analysis included 10 studies and 38 sub-studies/data sets, which encompassed 2450 patients from 5 countries. We found nonsignificant odds for changing the BMI group (pooled OR 0.972, 95% CI: 0.858-1.101, P=0.65) after GFD. However, looking specifically at BMI subgroups, we found higher odds for BMI category change after GFD in underweight patients (OR 0.588, 95% CI: 0.479-0.723, P <0.001), and overweight patients,25

Infantile-onset inflammatory bowel disease has variable long-term outcomes.

Objective and aim: Infantile-onset inflammatory bowel disease (IO-IBD), defined as IBD diagnosed at age 2 years or younger, tends to be more severe and refractory to conventional treatment than IBD diagnosed at a later age. However, data about IO-IBD and its long-term follow up are limited. We thus aimed to evaluate the presentation and long-term outcomes of patients with IO-IBD in a retrospective multicenter study. Methods: Medical records of patients diagnosed with IO-IBD in eight medical centers during 2000-2017 with at least 1-year follow up were reviewed. Demographics and disease characteristics at diagnosis including age of onset, disease phenotype and location, surgeries, medical therapy, and comorbid conditions were recorded. Results: Twenty-three patients with IO-IBD (16 males, 70%) were identified and followed for a median (range) of 51.2 (26.0-110.3) months. The mean ages at presentation and at the last follow up were 14 ± 9.8 and 101 ± 77 months, respectively. Six (26%) patients needed ileostomy already at the time of diagnosis and 20 (87%) were treated with corticosteroids. During long-term follow up, remission was achieved in 16 (73%) patients; of whom, 3 (14%) were without medications and 7 (32%) were in remission with the use of 5-aminosalicylic acid only. One patient needed hemicolectomy and one developed a severe EBV related infection. Conclusion: The majority of patients with IO-IBD achieved long-term remission, despite a severe disease presentation at diagnosis. Surgery rate however is high, mainly during the first months from diagnosis.

Correction to: Ambulatory blood pressure profiles in familial dysautonomia.

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Knowledge of disease and self-management of adolescents with inflammatory bowel diseases.

AIM: To describe factors that might affect successful transition in young adult patients with inflammatory bowel disease (IBD). METHODS: Questionnaires regarding the personal disease history, treatment and diseases specific knowledge and patients' self-efficacy were completed by teenaged patients with IBD aged 14-18 years, during routine clinic visits. Patient answers were then compared with information obtained from the medical records and information provided by treating physician. RESULTS: The study included 80 patients with IBD, 54 (67.5%) with Crohn's disease. Patients demonstrated good knowledge in their personal disease history and current treatment. Knowledge gaps were observed in medication dosages, knowledge of potential side effects and effects of smoking on disease, with only 53/80 (66%), 12/80 (15%) and 6/36 (16.7%) of patients providing correct answers. Only 25/36 (69.4%) of the patients aged 16-18 had read about their disease. All patients in 16-18 age group reported needing assistance to schedule a gastroenterologist appointment. CONCLUSION: Knowledge and self-efficacy skills of IBD teenagers are suboptimal regarding medications, smoking and appointment management. Medical providers should work with parents and teenaged patients with IBD to allow them to attain missing information and promote self-efficacy skills, in order to reinforce readiness towards transition.

Long-term Outcomes of Paediatric Patients Admitted With Acute Severe Colitis- A Multicentre Study From the Paediatric IBD Porto Group of ESPGHAN.

BACKGROUND AND AIM: Acute severe colitis [ASC] is associated with significant morbidity in paediatric patients with ulcerative colitis [UC]. Most outcome studies in ASC since tumour necrosis factor alpha [TNFα] antagonists became available have focused on the first year after admission. The aim of this study was to characterise the longer-term outcomes of paediatric patients admitted with ASC. METHODS: This retrospective study was conducted in 25 centres across Europe and North America. Data on patients with UC aged <18 years, admitted with ASC (defined as paediatric ulcerative colitis activity index [PUCAI] score ≥65) between 2009 and 2011, were collected at discharge and 1, 3 and 5 years after admission. The primary outcome was colectomy-free rates at each time point. RESULTS: Of the 141 patients admitted with ASC, 137 [97.1%] were treated with intravenous corticosteroids. Thirty-one [22.6%] patients were escalated to second-line therapy, mainly to infliximab. Sixteen patients [11.3%] underwent colectomy before discharge. Long-term follow-up showed colectomy-free rates were 71.3%, 66.4% and 63.6% at 1, 3 and 5 years after initial ASC admission, respectively, and were similar across different age groups. Sub-analysis of colectomy rates in patients with new-onset disease [42.5% of the cohort] yielded similar results. In a multivariate analysis, use of oral steroids in the 3 months before admission, erythrocyte sedimentation rate >70 mm/h, and albumin <2.5 g/dL, were significantly associated with 5-year colectomy risk. CONCLUSIONS: High colectomy rates were demonstrated in paediatric UC patients admitted with ASC. Additional studies are required to determine whether intensification of anti-TNFα treatment, close therapeutic drug monitoring, and use of new drugs alter this outcome.

Ambulatory blood pressure profiles in familial dysautonomia.

OBJECTIVE: Familial dysautonomia (FD) is a rare genetic disease that involves extreme blood pressure fluctuations secondary to afferent baroreflex failure. The diurnal blood pressure profile, including the average, variability, and day-night difference, may have implications for long-term end organ damage. The purpose of this study was to describe the circadian pattern of blood pressure in the FD population and relationships with renal and pulmonary function, use of medications, and overall disability. METHODS: We analyzed 24-h ambulatory blood pressure monitoring recordings in 22 patients with FD. Information about medications, disease severity, renal function (estimated glomerular filtration, eGFR), pulmonary function (forced expiratory volume in 1 s, FEV1) and an index of blood pressure variability (standard deviation of systolic pressure) were analyzed. RESULTS: The mean (± SEM) 24-h blood pressure was 115 ± 5.6/72 ± 2.0 mmHg. The diurnal blood pressure variability was high (daytime systolic pressure standard deviation 22.4 ± 1.5 mmHg, nighttime 17.2 ± 1.6), with a high frequency of a non-dipping pattern (16 patients, 73%). eGFR, use of medications, FEV1, and disability scores were unrelated to the degree of blood pressure variability or to dipping status. INTERPRETATION: This FD cohort had normal average 24-h blood pressure, fluctuating blood pressure, and a high frequency of non-dippers. Although there was evidence of renal dysfunction based on eGFR and proteinuria, the ABPM profile was unrelated to the measures of end organ dysfunction or to reported disability.

The steroid-sparing effect of intravenous immunoglobulin in patients with autoimmune diseases.

This review covers the major advances in the therapeutic potential of intravenous immunoglobulin (IVIg) as a steroid-sparing agent in autoimmune diseases utilizing a structured search of Medline (1992-2007). IVIg is a potent biological drug, utilized routinely for idiopathic thrombocytopenic purpura, Kawasaki's diseases, Guillain-Barre syndrome and dermatomyositis. In addition, however, IVIg is an adjunct second-line therapy in neuroimmunologic, infectious, dermatologic, hematologic, obstetric, autoimmune, inflammatory and idiopathic disorders. Compared with immunosuppressive agents administered routinely for systemic autoimmune diseases, IVIg is advantageous, owing to its few and transient minor adverse effects. Hence, it is logical to deliver IVIg with steroids as a sparing agent. All the published material on IVIg and its steroid-sparing effect was reviewed. Currently, there is insufficient evidence to confirm that IVIg has a significant steroid-sparing effect. Based on the available information, IVIg has the potential to act as a steroid-sparing agent in systemic lupus erythematosus and autoimmune blistering diseases, but its effect in other autoimmune diseases remains uncertain. Further investigation is warranted where this issue will be addressed as a primary endpoint and in controlled trials.