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Summary: Background: Provision of feeds containing hydrolysed, peptide-based proteins and medium-chain triglycerides (MCT), can help mitigate gastrointestinal (GI) intolerance in adults with impaired GI function, maldigestion and/or malabsorption. Aim: This study evaluated a high-protein, peptide-based, MCT-containing oral nutritional supplement (PEHP; 1.5 kcal/mL and 7.5 g protein/100 mL). Methods: Adults with impaired GI function were recruited by their managing dietitian and took PEHP orally for 28-days, with GI tolerance, compliance, weight, energy and protein intake assessed via non-validated questionnaires and a 24-hour dietary recall at baseline and at intervention end. Results: Fifteen, adults (56 years (16), 67 kg (26.0), 24 kg/m2 (7.6)) took part in this study. Intensity of nausea (Z= -2.070, p=0.038, n=15) and abdominal pain (Z= -2.236, p=0.025, n=15) improved significantly compared to baseline. Reductions in the intensity of diarrhoea, constipation, vomiting, flatulence, and burping were observed but were not statistically significant (p>0.05 for all). Compliance was higher with PEHP (81% (24)) than baseline feeds (63% (42)) but not significantly. Weight remained stable between baseline (67 kg (26)) and at intervention end (67 kg (27), p=0.414, n=15). Compared to baseline, total energy intake increased with PEHP albeit not significantly (1661 kcal/day (572) vs 1981 kcal/day (592), p=0.137, n=15). Increases in total protein intake were also observed, this time significantly (61 g/day (23) vs 78 g/day (29), p=0.042, n=15). Conclusions: This study in adults with impaired GI function found that PEHP improved GI tolerance and protein intake compared to feeds taken at baseline (including both polymeric and peptide-based feeds).

Building computational models of agents in dynamic, partially observable and stochastic environments is challenging. We propose a cognitive computational model of sugarcane growers’ daily decision-making to examine sugarcane supply chain complexities. Growers make decisions based on uncertain weather forecasts; cane dryness; unforeseen emergencies; and the mill’s unexpected call for delivery of a different amount of cane. The Belief-Desire-Intention (BDI) architecture has been used to model cognitive agents in many domains, including agriculture. However, typical implementations of this architecture have represented beliefs symbolically, so uncertain beliefs are usually not catered for. Here we show that a BDI architecture, enhanced with a dynamic decision network (DDN), suitably models sugarcane grower agents’ repeated daily decisions. Using two complex scenarios, we demonstrate that the agent selects the appropriate intention, and suggests how the grower should act adaptively and proactively to achieve his goals. In addition, we provide a mapping for using a DDN in a BDI architecture. This architecture can be used for modelling sugarcane grower agents in an agent-based simulation. The mapping of the DDN’s use in the BDI architecture enables this work to be applied to other domains for modelling agents’ repeated decisions in partially observable, stochastic and dynamic environments

The HTML and PDF versions of this Article were updated after publication to remove images of one individual from Figure 1.

Chromodomain Helicase DNA-binding (CHD) proteins have been implicated in neurodevelopmental processes. Here, the authors identify missense variants in CHD3 that disturb its chromatin remodeling activities and cause a neurodevelopmental disorder with macrocephaly and speech and language impairment.

Autoimmune lymphoproliferative syndrome (ALPS) is caused by germline or somatic loss of function FAS mutations resulting in impaired apoptosis and consequent expansion of T-lymphocytes causing organomegaly and autoimmune anemia, neutropenia and thrombocytopenia. Herein, we report on a case of disseminated varicella zoster infection after post-partum vaccination in a patient found to have CD4 lymphopenia and eventually diagnosed with ALPS caused by a novel germline missense mutation in FAS death-domain. A subsequent retrospective analysis of 169 patients of the NIH ALPS-FAS cohort, revealed that CD4-T-cells lymphopenia (< 300 cells/μl) may occur in 5% of ALPS-FAS patients irrespectively of the underlying genetic defect, organomegaly or immunosuppressive treatment. Although immunophenotyping did not show depletion of specific CD4-T-cells subpopulations, CD4-lymphopenic ALPS-FAS subjects had an expansion of a subset of circulating T-follicular-helper (cTfh) cells, associated with autoantibody production (CCR7lowPD-1high). Furthermore, autoantibodies binding on CD4-T-cells were detected in 50% of the CD4-lymphopenic ALPS-FAS patients and caused cytotoxicity in a natural killer (NK)-mediated antibody-dependent-cellular cytotoxicity assay. Such autoantibodies can therefore be associated with CD4-T-cell death, impaired activation induced proliferation or impaired trafficking. The expansion of autoreactive T-cells in ALPS-FAS is known to be associated with autoimmune clinical manifestations, however our study reveals that ALPS-FAS can also be associated with a paradoxical depletion of CD4-T-cells due to the presence of autoantibodies on the surface of CD4-T-cells which can in turn result in increased susceptibility to opportunistic infections. These novel findings have implications for the diagnosis, clinical monitoring, and management of patients with ALPS-FAS.

Fas drives apoptosis and mutations in this receptor can cause autoimmunity through failure of cell death. Here, the authors uselpr/lprmice with palmitoylation-defective mutant Fas to provide evidence that Fas might limit spontaneous autoimmunity through a non-apoptotic mechanism.

Autoimmune lymphoproliferative syndrome is a rare genetic disorder characterized by defective FAS-mediated apoptosis, autoimmune disease, accumulation of mature T-cell receptor alpha/beta positive, CD4 and CD8 double-negative T cells and increased risk of lymphoma. Despite frequent hematologic abnormalities, literature is scarce regarding the bone marrow pathology in autoimmune lymphoproliferative syndrome. We retrospectively reviewed 3l bone marrow biopsies from a cohort of 240 patients with germline FAS mutations. All biopsies were performed for the evaluation of cytopenias or to rule out lymphoma. Clinical information was collected and morphological, immunohistochemical, flow cytometric and molecular studies were performed. Bone marrow lymphocytosis was the predominant feature, present in 74% (23/31) of biopsies. The lymphoid cells showed several different patterns of infiltration, most often forming aggregates comprising T cells in 15 cases, B cells in one and a mixture of T and B cells in the other seven cases. Double-negative T cells were detected by immunohistochemistry in the minority of cases (10/31; 32%); significantly, all but one of these cases had prominent double-negative T-lymphoid aggregates, which in four cases diffusely replaced the marrow space. One case showed features of Rosai-Dorfman disease, containing scattered S-100+ cells with emperipolesis and double-negative T cells. No clonal B or T cells were detected by polymerase chain reaction in any evaluated cases. Classical Hodgkin lymphoma was identified in three cases. Our results demonstrate that infiltrates of T cells, or rarely B cells, can be extensive in patients with autoimmune lymphoproliferative syndrome, mimicking lymphoma. A multi-modality approach, integrating clinical, histological, immunohistochemical as well as other ancillary tests, can help avoid this diagnostic pitfall. This study is registered at Clinicaltrials.gov ID # NCT00001350

From the 1850s to 1917, members of the Community of True Inspiration produced and sold patterned indigo-dyed cotton cloth to support their closed communal society in Iowa, known as the Amana Colonies. Past published accounts of what is now called "Amana calico" lack an understanding of indigo-dyeing processes, use various labels for the patterned textiles, and skim over the evolution of production. This paper adds new information about the history of the Amana cotton fabrics by reviewing indigo-dyeing methods, searching online archives of newspapers for articles and advertising, and examining fabric samples, artifacts, and documents at the Amana Heritage Society Museum. The greatest production and sales of Amana cotton fabrics occurred in the 1890s at a time when many quilters across the country pieced blue and white quilts. An investigation of textiles used for bedding found a few remnants of traditional blue wholecloth German-style featherbeds and comforters. However, even before the calico factory closed in 1917, Amana women began to buy solid-color pastel sateen fabrics for their covers, stitch the layers together in large original patterns, and apply the term "quilt." The larger history of American textiles should include the Amana fabrics and the distinctive twentieth-century quilts from the Colonies. [ABSTRACT FROM AUTHOR]

Analyzing anonymized query and click-through logs leads to a better understanding of user behaviors and intentions, and provides opportunities to create an improved search experience. As a large-scale provider of SaaS services that returns search results against a single unified index, Serials Solutions is uniquely positioned to learn from the dataset of queries issued to its Summon® service by millions of users at hundreds of libraries around the world. In this paper, we describe the Relevance Metrics Framework that we use to analyze our query logs and provide examples of insights we have gained during development and implementation. We also highlight the ways our analysis is inspiring changes to the Summon® service to improve the academic research experience.

The article provides information on the "Circuit Rider's Quilt," an album on quilts which had been donated to the Art Institute of Chicago by Emma Blanxius Hodge. It discusses the topics of reality and romance portrayed in the quilt's story. It contains knowledge derived from genealogical materials, local records, county histories, museum collections, published sources to be able to render a more factual and less romantic account of the quilt's story. It is stated that the Circuit Rider was designed with botanical pictures which conveyed romance.

Lucile and Ben Taylor.filled their two-story brick house in Fairfield, Iowa, with numerous collections and saved letters, photographs, and clippings on many subjects. After their deaths, the ephemera that Lucile assembled pertaining to quilts and the two special friends who shared her interest in the subject came into the author's possession. This study surveys the Taylor archives to uncover information about how Lillian Walker, Maxine Teele and Lucile Taylor influenced each other's quilt activities after the late 1950s. This primary material reveals the conditions quilters faced prior to the flowering of the current revival in the 1970s, the on-going quilt activity in southeast Iowa that bridged the two revivals of the twentieth century, and the details of a mutually beneficial three-way friendship that brought public recognition to Walker late in her life and produced two new quilt experts who contributed to the contemporary quilting scene on regional and national levels. [ABSTRACT FROM AUTHOR]

The name Hope Winslow in the title of a 1933 catalog; is probably better known than the little recognized man who produced the book and designed some unique twentieth-century pieced quilt patterns. Hubert Ver Mehren of Des Moines, Iowa, marketed his needlework items through magazines, newspapers, and mail order catalogs. Examination of a dozen news papers and periodicals and seven versions of his books revealed the growth of Ver Mehren's work in the 1920s and 1930s. The research provides information about some of the by-lines associated with the patterns and shows how Home Art Studios, the title usually associated with the quilt patterns, functioned in relation to Ver Mehren's primary business, the Iowa Button and Pleating Company. The results credit Hubert Ver Mehren with being an artist, entrepreneur, and marketing expert whose name should be attached hence forth to all his original designs. [ABSTRACT FROM AUTHOR]

Carlie Sexton, a quilt writer and pattern producer in the 1920s and 1930s, ran a home-based business during the quilt revival taking place at that time. This paper examines the beginning, development, and operation of the business and describes Sexton's products and marketing methods. Sources for the paper include a collection of Sexton's published articles, books, leaflets, and patterns; a collection of unsigned patterns and catalogs from two farm magazines; public records, newspapers, and letters; and interviews with people who knew her. The study reveals a remarkable self-made business woman whose work was far more extensive and influential than previously known. [ABSTRACT FROM AUTHOR]