A systematic review of food protein-induced enterocolitis syndrome from the last 40 years.

OBJECTIVE: To provide a complete, exhaustive summary of current literature relevant to food protein-induced enterocolitis syndrome (FPIES). DATA SOURCES: Data have been extracted from PubMed and Science Direct databases. STUDY SELECTIONS: Following the Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines, a literature search for peer-reviewed journal articles in English through January 1975 with updates through October 2016 was conducted. Relevant publications were reviewed that included pediatric and adult populations. Information on the study design, sample, intervention, comparators, outcome, timeframe, and risk of bias were abstracted for each article. RESULTS: Of 135 reviewed reports, 52 were included in this systematic review. In accordance with the age at onset, clinical features, and offending foods, it is possible to distiguish different types of FPIES. An immune systemic involvement can occur in patients with FPIES. In addition to the most common causative foods (cow's milk, soy, and rice), any food can potentially cause FPIES. Although specific diagnostic tests are not available, open food challenge remains the gold standard for FPIES diagnosis. Moreover, because of the lack of randomized clinical trials and of use of different adopted methods, confounding factors might mask critical findings, leading to poor knowledge of this pleiotropic clinical entity. CONCLUSION: Multicenter studies are needed to better develop an evidence-based approach to pathophysiology, prevalence, diagnosis, and natural history of the disease.

Electroclinical Features of Early-Onset Epileptic Encephalopathies in Congenital Disorders of Glycosylation (CDGs).

Congenital disorders of glycosylation (CDG) are a constantly growing group of genetic defects of glycoprotein and glycolipid glycan synthesis. CDGs are usually multisystem diseases, and in the majority of patients, there is an important neurological involvement comprising psychomotor disability, hypotonia, ataxia, seizures, stroke-like episodes, and peripheral neuropathy. To assess the incidence, among early-onset epileptic encephalopathies (EOEE), of patients with identified congenital disorders of glycosylation (CDG), we made a review of clinical, electrophysiological, and neuroimaging findings of 27 CDG patients focusing on seizure onset, semiology and frequency, response to antiepileptic drugs (AED), and early epileptic manifestations. Epilepsy was uncommon in PMM2-CDG (11%), while it was a main concern in other rare forms. We describe a series of patients with EOEE and genetically confirmed CDG (ALG3-CDG, ALG6-CDG, DPM2-CDG, ALG1-CDG). Epileptic seizures at onset included myoclonic and clonic fits and focal seizures. With time, patients developed recurrent and intractable seizures principally tonic-clonic seizures, infantile spasms, and myoclonic seizures. Electrophysiological correlates included focal and multifocal epileptic discharges, slowed background rhythm, and generalized epileptic activity including burst suppression pattern and status epilepticus. We propose a diagnostic flowchart for the early diagnosis of CDG in patients presenting with EOEE and suggest to perform serum transferrin IEF (or capillary zone electrophoresis) as a first-line screening in early-onset epilepsy.

Comparison of complications and outcomes to one year of transcatheter aortic valve implantation versus surgical aortic valve replacement in patients with severe aortic stenosis.

Comparisons of transcatheter aortic valve implantation (TAVI) to surgical aortic valve replacement (SAVR) in patients with severe aortic stenosis remain sparse or limited by a short follow-up. We sought to evaluate early and midterm outcomes of consecutive patients (n = 618) undergoing successful TAVI (n = 218) or isolated SAVR (n = 400) at 2 centers. The primary end point was incidence of Valvular Academic Research Consortium-defined major adverse cerebrovascular and cardiac events (MACCEs) up to 1 year. Control of potential confounders was attempted with extensive statistical adjustment by covariates and/or propensity score. In-hospital MACCEs occurred in 73 patients (11.8%) and was more frequent in patients treated with SAVR compared to those treated with TAVI (7.8% vs 14.0%, p = 0.022). After addressing potential confounders using 3 methods of statistical adjustment, SAVR was consistently associated with a higher risk of MACCEs than TAVI, with estimates of relative risk ranging from 2.2 to 2.6 at 30 days, 2.3 to 2.5 at 6 months, and 2.0 to 2.2 at 12 months. This difference was driven by an adjusted increased risk of life-threatening bleeding at 6 and 12 months and stroke at 12 months with SAVR. Conversely, no differences in adjusted risk of death, stroke and myocardial infarction were noted between TAVI and SAVR at each time point. In conclusion, in a large observational registry with admitted potential for selection bias and residual confounding, TAVI was not associated with a higher risk of 1-year MACCEs compared to SAVR.