Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review

Familial Mediterranean fever (FMF) (OMIM #249100) is the most common hereditary autoinflammatory disease in the world. FMF is caused by gain of function mutations of MEFV gene which encodes an immune regulatory protein, pyrin. Over the last few years, we have witnessed several new developments in the pathogenesis, genetic testing, diagnosis, comorbidities, disease related damage and treatment approaches to FMF. Elucidation of some of the pathogenic mechanisms has led to the discovery of pathways involved in inflammatory, metabolic, cardiovascular and degenerative diseases. The use of next generation sequencing in FMF has revealed many new gene variants whose clinical significance may be clarified by developing functional assays and biomarkers. Clinically, although FMF is considered an episodic disease characterized by brief attacks, recent systematic studies have defined several associated chronic inflammatory conditions. Colchicine is the mainstay of FMF treatment, and interleukin (IL)-1 antagonists are the treatment of choice in refractory or intolerant cases. Experience of IL-1 antagonists, anakinra and canakinumab, is now available in thousands of colchicine resistant or intolerant FMF patients. In this contemporary review, we surveyed current FMF knowledge in the light of these recent advances

Effects of Vitamin D levels on asthma control and severity in pre-school children

OBJECTIVE: Prevalence of asthma and Vitamin D deficiency has been increasing and leading to significant morbidities. This study aimed to compare the Vitamin D levels in the pre-school children with asthma and in healthy controls and to assess the relationship between Vitamin D levels and asthma clinical parameters and control. PATIENTS AND METHODS: Vi tamin D [25(OH)D3] levels were measured in 102 preschool children, aged 1-4 years with asthma and 102 healthy controls in winter. The patients with asthma were grouped according to serum Vitamin D levels as sufficient, insufficient and deficient. Asthma control was classified according to the Global Initiative for Asthma (GINA) guidelines and the Test for Respiratory and Asthma Control in Kids (TRACK) in 1-4 years-old children. RESULTS: Serum Vitamin D levels were 22.64 (9.96) ng/ml in the asthma group and 32.11 (14.74) ng/ml in the control group (p = 0.001). Total number of exacerbations during the previous year were significantly lower in the Vitamin D sufficient group, compared to the deficient and insufficient groups (p = 0.03). Frequency of patients with controlled asthma was higher in the sufficient group compared to the deficient and insufficient groups (p = 0.001 and p = 0.001, respectively). There was a positive correlation between serum Vitamin D levels and asthma control. CONCLUSIONS: The frequency of Vitamin D deficiency and insufficiency was higher in children with asthma, compared to the controls. Therefore, we suggest that lower levels of Vitamin D are associated with poor asthma control and increased asthma severity

State of the Knowledge for Gender in Breeding: Case Studies for Practitioners

Debates around gender-responsive agricultural research, particularly plant and animal breeding, invariably circulate around similar topics: the recognition that considering gender is important to developing varieties that lead to equitable benefits, coupled with questions around an evidence base that proves this point. Without convincing evidence—exemplified by case studies across commodities and countries—our arguments for gender-responsive research fall on deaf ears. This synthesis seeks to compile available cases from two workshops organized by the CGIAR Gender and Agriculture Research Network: “Gender, Breeding and Genomics” (18–21 October 2016) and “Innovation in Gender-Responsive Breeding” (5–7 October 2017). While by no means comprehensive, with these 10 cases we hope to emphasize the point that considering gender in breeding program design, working with women in the breeding process, and acting on these findings can have dramatic consequences on breeding programs. We begin the synthesis by setting the scene with a chapter reflecting on how taking gender into account matters for the success of plant or animal breeding programs with welfare or development goals and a focus on smallholders. This chapter illustrates how the use of a conceptual framework for gender analysis can help breeding programs make sense of gender-differentiated traits and tease out the likely impact of taking gender into account in program-level policies and strategies. The following case studies are structured around steps of a plant breeding cycle (see Figure 1.1), examining cases that consider gender in setting breeding priorities, selection, testing experimental varieties, and seed production and distribution. The cases cover a wide range of commodities: beans, cassava, forage grasses, poultry, maize, sorghum, matooke, barley, and groundnuts. Although cases mostly focus on sub-Saharan Africa (Nigeria, Mali, Tanzania, Uganda, Ethiopia, Malawi), we also present cases from China and Syria. What is particularly compelling about these cases is that they not only provide evidence that men and women have different trait preferences; access to resources; or opportunities to engage in production, processing, and marketing of diverse commodities. They also illustrate steps taken by breeding programs to address these issues. These steps range from incorporating “cooking time” as a must-have trait in bean breeding to creating opportunities for maize seed production and sale for women; from changing the structure of matooke breeding programs to add participatory processing for food quality, new breeding targets for adaptation, to nutrient poor soils in sorghum. These are powerful illustrations and positive examples that documenting differences is a means to an end—the real focus should be on change. The synthesis ends with a chapter drawing lessons from the case studies for future action aiming to integrate gender and gender analysis in breeding. We hope that these cases, together with the companion publications from the GBI on design principles1, gender and social targeting2, breeding decisions3, and uptake pathways4, compel and challenge breeding programs to become truly gender responsive

From traits to typologies: Piloting new approaches to profiling trait preferences along the cassava value chain in Nigeria

Breeding programs are increasing efforts towards demand-led breeding approaches to ensure that cultivars released meet the needs of end users including processors, traders, and consumers, and that they are adopted by farmers. To effectively deploy these approaches, new tools are required to better understand and quantify the degree of preference differences among alternative trait changes competing for measurement and selection effort. The purpose of this study was to present a method of quantifying preferences and developing typologies according to breeding priorities by applying an online trait preference survey approach to cassava (Manihot esculenta Crantz). This paper presents a conjoint analysis based on Potentially All Pairwise RanKings of all possible Alternatives (PAPRIKA) to help guide breeding programs in understanding trait preferences across value chain roles and social contexts and set breeding priorities that represent diverse interests. Trait preferences were assessed using a comprehensive survey and analysis package incorporating a core adaptive conjoint method (1000minds, 2020). Trait selection was based on a trade-off of 11 cassava traits carried out with 792 cassava value chain actors in four geopolitical regions in Nigeria. Principal component and cluster analyses revealed three clusters (typologies) of respondents according to their trait preferences. The results demonstrate the usefulness of this methodology that innovates on previous trait preference approaches to address the expanding needs of plant breeding programs within smallholder contexts. © 2021 The Authors. Crop Science © 2021 Crop Science Society of Americ

Giant cervicothoracic extradural arachnoid cyst: case report

The pathogenesis, etiology, and treatment of the spinal arachnoid cyst have not been well established because of its rarity. A 57-year-old male was presented with spastic quadriparesis predominantly on the left side. His radiological examination showed widening of the cervical spinal canal and left neural foramina due to a cerebrospinal fluid - filled extradural cyst that extended from C2 to T2 level. The cyst was located left anterolaterally, compressing the spinal cord. Through a C4–T2 laminotomy, the cyst was excised totally and the dural defect was repaired. Several features of the reported case, such as cyst size, location, and clinical features make it extremely unusual. The case is discussed in light of the relevant literature

Magnetic tunnel junctions with metastable bcc Co3Mn electrodes

We studied magnetic tunnel junctions (MTJs) with a MgO(001) barrier and metastable bcc Co3Mn(001) disordered alloy electrodes. A tunnel magnetoresistance (TMR) ratio was approximately 200{250% observed at room temperature.We successfully observed the TMR ratio greater than 600% at 10 K which was higher than the past reported value of MgO-based MTJs with ultrathin bcc Co(001) electrodes. However our experimental value was still much lower than the past theoretical prediction in bcc Co/MgO/Co(001) MTJs. We discuss some differences in the bulk band structure affecting the TMR effect for bcc Co and bcc Co3Mn

Cassava trait preferences of men and women farmers in Nigeria: implications for breeding

Nigeria is the world’s largest cassava producer, hosting a diverse array of cassava farmers and processors. Cassava breeding programs prioritize “common denominator” traits in setting breeding agendas, to impact the largest possible number of people through improved varieties. This approach has been successful, but cassava adoption rates are less than expected, with room for improvement by integrating traits in demand by farmers and processors. This paper aims to inform breeding priority setting, by examining trait and varietal preferences of men and women cassava farmer/processors. Men and women in eight communities in Southwest and Southeast Nigeria were consulted using mixed methods. Women and men had significantly different patterns of cassava use in the Southwest. Fifty-five variety names were recorded from the communities demonstrating high genetic diversity maintained by growers, especially in the Southeast. High yield, early maturity, and root size were most important traits across both regions, while traits women and men preferred followed gender roles: women prioritized product quality/cooking traits, while men placed higher priority on agronomic traits. Trait preference patterns differed significantly between the Southeast and Southwest, and showed differentiation based on gender. Patterns of access to stem sources were determined more by region and religion than gender

Existence of a lens-shaped cluster of surfaces self-shrinking by mean curvature

We rigorously show the existence of a rotationally and centrally symmetric "lens-shaped" cluster of three surfaces, meeting at a smooth common circle, forming equal angles of 120 degrees, self-shrinking under the motion by mean curvature.Comment: 22 pages, 2 figure