271 research outputs found
The Effect of Income on Demand for Micronutrients in Poor Rural Mexico
We estimate income elasticities for a variety of macro- and micro-nutrients using a sample of poor rural households in Mexico. The nutrient-income elasticity is estimated using a linear regression model controlling both for the clustered nature of our data and for the bias due to measurement error in nutrient consumption at the household level. Our preferred estimates (instrumental variable-fixed effect specification for the sample of all households) show a sizeable positive elasticity for some nutrients (especially vitamin A 0.8, vitamin C 0.69 and calcium 0.45). For other nutrients the effect of income on the consumption is still significant but very small (elasticity for fiber is only 0.09 and for iron 0.08). We also test for the robustness of our estimates using a semi-parametric estimator (partially linear model) and whether the presence of zero consumption for specific micronutrients in our sample, such as cholesterol and heme iron, can be a source of bias for our estimates.vitamin A , vitamin C; folate; iron; zinc; calcium; calories; protein; fat; carbohydrates; Income elasticity; partially linear model.
Progressive familial intrahepatic cholestasis
Progressive familial intrahepatic cholestasis (PFIC) refers to heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. The exact prevalence remains unknown, but the estimated incidence varies between 1/50,000 and 1/100,000 births
Defining the natural history of rare genetic liver diseases:Lessons learned from the NAPPED initiative
While rare diseases collectively affect similar to 300 million people worldwide, the prevalence of each disease concerns a relatively small number of patients. Usually, only limited data with regard to natural history are available. Multicenter initiatives are needed to aggregate data and answer clinically relevant research questions. In 2017, we launched the NAtural course and Prognosis of PFIC and Effect of biliary Diversion (NAPPED) consortium. In three years, NAPPED created a global network focused on rare genetic liver diseases in the Progressive Familial Intrahepatic Cholestasis (PFIC) spectrum. During these years, we have learned important lessons which we feel should be taken into account when initiating and leading a global consortium.First, it is essential to 'keep it simple' from the start. Research questions, case report forms (CRFs) and data acquisition should be limited and clear to stay focused and keep the workload low for new participants. Secondly, early rewards and research output are needed to keep momentum and motivation. Quick output can only follow a clean and simple design. Thirdly, the leading team should be in touch and accessible. Ideally, an involved PhD-candidate is appointed as primary contact person. Lastly, be inclusive and actively involve all participants the consortium's course.Global consortia are critical for personalized medicine in rare diseases. Also, they are essential for setting up trials to investigate generic drugs and personalized therapies. We hope to herewith stimulate others that are starting (or are planning to start) a global consortium, ultimately to help improve the care for patients with a rare disease.</p
CD25 Appears Non Essential for Human Peripheral Treg Maintenance In Vivo
Background: IL-2 has been reported to be critical for peripheral T reg survival in mouse models. Here, we examined T reg maintenance in a series of paediatric liver transplant recipients who received basiliximab, a therapeutic anti-CD25 monoclonal antibody. Methodology/Principal Findings: FoxP3 + CD4 T cells were analyzed by flow cytometry before liver grafting and more than 9 months later. We found that in vivo CD25 blockade did not lead to Treg depletion: the proportion of FoxP3 + cells among CD4 T cells and the level of FoxP3 expression were both unchanged. IL-2Rb expression was enhanced in FoxP3 + cells both before and after basiliximab treatment, while the level of IL-2Rc expression was similar in Tregs and non-Tregs. No significant change in the weak or absent expression of IL-7Ra and IL-15Ra expression on FoxP3 + cells was observed. Although the proportion of FoxP3 + cells among CD4 T cells did not vary, food allergies occurred more rapidly after liver grafting in patients who received basiliximab, raising questions as to T reg functionality in vivo in the absence of functional CD25. Conclusions: CD25 appears non essential for human Treg peripheral maintenance in vivo. However, our results rais
Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency
Compound heterozygosity; Genotype; PhenotypeHeterocigosidad compuesta; Genotipo; FenotipoHeterozigositat composta; Genotip; FenotipBackground & Aims
Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship.
Methods
From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS.
Results
The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001).
Conclusions
Individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment.
Impact and implications
This manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mutation. Until now, it had always been assumed that the mild mutation would be enough to ensure a relatively good prognosis. However, our manuscript shows that the prognosis of these patients is just as poor as that of patients with two severe mutations. They do not respond to biliary diversion surgery and will likely not respond to the new IBAT (ileal bile acid transporter) inhibitors, which have recently been approved for use in BSEP deficiency.1. MD/PhD scholarship from the University of Groningen, Groningen, The Netherlands 2. ESPGHAN Networking Grant 2019 3. ChiLDReN and CTSA National Institutes of Health grants: Ann & Robert H. Lurie Children's Hospital, Chicago: U01DK062436; University of Colorado, Denver: U01DK62453, UL1 TR002535; Baylor college of Medicine, Houston: U01DK103149; Children's Hospital of Philadelphia, Philadelphia: U01DK062481, UL1TR000003; Children's Hospital of Pittsburgh, Pittsburgh: U01DK062466; University of California, San Francisco U01DK062500; University of California, San Francisco CTSI grant UL1TR001872; Riley Hospital for Children, Indianapolis: U01DK084536; Seattle Children’s Hospital, Seattle: DK084575; Children’s Hospital Los Angeles, California: U01DK084538. 4. Unrestrictive research grant from Albireo 5. Unrestrictive research grant from Mirum Pharmaceuticals. 6. C&W de Boer Stichting research grant
Clima laboral y compromiso organizacional en un centro médico de salud ocupacional, Chiclayo – 2022
La presente investigación de tipo descriptivo correlacional y diseño no
experimental, tiene como objetivo analizar la relación entre el Clima Laboral
y el Compromiso Organizacional, asà como determinar la categorÃa de clima
y el nivel de compromiso, además de determinar la relación entre los factores
del Clima laboral con las dimensiones del Compromiso en un Centro Médico
de Salud Ocupacional en la ciudad de Chiclayo. En esta investigación se
empleó la totalidad de su población. Los resultados obtenidos evidenciaron
que no existe relación significativa entre ambas variables, además se
comprobó que la categorÃa de clima laboral es favorable representado por el
47% y que el nivel de compromiso es bajo demostrado por el 74%.
Conjuntamente, se comprobó que no existe relación significativa alguna
entre los factores de clima (Autorrealización, Involucramiento Laboral,
Comunicación, Supervisión y Condiciones Laborales) y las dimensiones de
compromiso (Afectivo, Normativo y de Continuidad)
Reading Comprehension and Behavior in Children Using E-books vs. Printed Books
The purpose of this research is to investigate the influence that personalized, gamified, and PDF electronic reading practices have on the
attitudes which fifth-grade students possess toward e-reading experiences, as well as how these stances affect the students' motivation and
reading comprehension while they are learning English as a second/foreign language (EFL). For the purpose of the study, there were a
total of 84 fifth-grade kids from public schools in Greece, who participated. These students were split up into three different experimental
groups and a control one. Participants in the experimental groups read throughout the treatment period according to a preset schedule
using one of three diverse electronic reading formats (PDF, gamified, or customized), whilst participants in the control group read
utilizing a paper guided reading plan. The participants' experiences playing video games online were analyzed via a technique called the
quasi-experimental approach. According to the findings of the research, the experimental group and the control group did not significantly
vary from one another in terms of their levels of reading comprehension. On the other hand, in comparison to the participants in the
control group, those who took part in the experiments reported having more favorable sentiments regarding their electronic reading
experiences and were more inspired to read. As indicated from the research findings, kids may experience an increase in their desire to
read when they use electronic gadgets. This study has implications for educators and policymakers as they consider incorporating digital
reading practices into their teaching methods, particularly when it comes to improving students' motivation to read
Diagnóstico operativo empresarial de la planta Procesadora Industrial RÃo Seco
El trabajo de investigación desarrollado en la presente tesis es fruto de un profundo
análisis y revisión del estado de las operaciones de la Procesadora Industrial RÃo Seco S.A.,
que es una empresa subsidiaria de la CompañÃa de Minas Buenaventura S.A.A. El objetivo de
este trabajo es diagnosticar las actividades operativas de la empresa y contrastarlas con las
mejores prácticas y métodos para detectar oportunidades de mejora. Esto se logró mediante el
uso de conceptos y técnicas aprendidos durante la maestrÃa, tales como la comprensión de la
cultura organizacional hasta el análisis contable de los estados financieros de la empresa.
Gracias a ello, se identificaron puntos en los cuales el desempeño de la empresa es de gran
nivel y puntos en donde aún puede mejorar. Las recomendaciones generadas tras este estudio
buscan mejorar la rentabilidad de la empresa, asà como su desarrollo competitivo y
sostenibilidad.
En el presente trabajo de investigación se describe a la empresa y su contexto con un
especial énfasis en el proceso productivo de lixiviación de concentrado Pb-Ag-Mn, el cual es
la razón de ser del negocio. A continuación, detallamos aspectos tales como los productos
elaborados, dimensionamiento de planta, capacidad instalada, planificación, operación,
cadena de suministro, costos operacionales y la gestión de mantenimiento. El análisis de cada
uno de estos puntos se llevó a cabo haciendo uso de la bibliografÃa especializada en cada
tema en cuestión.
Al final, se determinan las buenas prácticas de la empresa, se calculan costos
adicionales en los que incurren y las nuevas herramientas que deben emplear para lograr un
desempeño de clase mundial. Por lo tanto, se señala que la implementación de las propuestas
de mejora requiere una inversión de USD 1’693,425 y como resultado se puede obtener una
rentabilidad estimada de USD 6’474,086 en cinco años.In this thesis, we are presenting a business operating diagnosis of the Industrial Plant
RÃo Seco after a rigorous data collection and analysis. The company is a subsidiary of
CompañÃa de Minas Buenaventura, one of the biggest mining companies in Peru. The
development of this diagnosis was possible because of the use of every concept learnt in our
MBA, like leadership, financial analysis and organizational behavior. Due to the application
of those concepts we were able to identify the critical points that generate economical losses
to the company and, therefore, propose ideas to make Rio Seco profitable and sustainable
across time.
In the present research, the company and its context are described, with a special
emphasis on the leaching of Pb-Ag concentrate, which is the core of the business. Then, we
detail aspects such as the elaboration of products, plant dimensions, total capacity installed,
planning, operation, supply chain, costs and maintenance management. The analysis of each
of these points was carried out by using the specialized bibliography on each subject in
question.
At the end, the good practices of the company are determined and the additional costs
incurred are determined. Finally, the tools that the company must use to achieve a world-class
performance are proposed. Therefore, it is pointed out that the implementation of the
improvement proposals requires an investment of USD 1’693,425 and as a result, an
estimated return of USD 6’474,086 in five years can be obtained.Tesi
Childhood Malnutrition and Association of Lean Mass with Metabolome and Hormone Profile in Later Life.
This study aimed to determine the associations of targeted metabolomics and hormone profiles data with lean mass index (LMI), which were estimated using bioelectrical impedance, in survivors of child severe malnutrition (SM) (n = 69) and controls (n = 77) in Malawi 7 years after being treated. Linear associations between individual metabolite or hormone and LMI were determined, including their interaction with nutrition status 7 years prior. Path analysis was performed to determine structural associations. Lastly, predictive models for LMI were developed using the metabolome and hormone profile by elastic net regularized regression (EN). Metabolites including several lipids, amino acids, and hormones were individually associated (p < 0.05 after false discovery rate correction) with LMI. However, plasma FGF21 (Control: ? = -0.02, p = 0.59; Case: ? = -0.14, p < 0.001) and tryptophan (Control: ? = 0.15, p = 0.26; Case: ? = 0.70, p < 0.001) were associated with LMI among cases but not among controls (both interaction p-values < 0.01). Moreover, path analysis revealed that tryptophan mediates the association between child SM and LMI. EN revealed that most predictors of LMI differed between groups, further indicating altered metabolic mechanisms driving lean mass accretion among SM survivors later in life
International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts.
BACKGROUND
Congenital portosystemic shunts (CPSS) are rare vascular malformations associated with the risk of life-threatening systemic conditions, which remain underdiagnosed and often are identified after considerable diagnostic delay. CPSS are characterized by multiple signs and symptoms, often masquerading as other conditions, progressing over time if the shunt remains patent. Which patients will benefit from shunt closure remains to be clarified, as does the timing and method of closure. In addition, the etiology and pathophysiology of CPSS are both unknowns. This rare disorder needs the strength of numbers to answer these questions, which is the purpose of the international registry of CPSS (IRCPSS).
METHOD
A retrospective and prospective registry was designed using secuTrial® by the ISO certified Clinical Research Unit. Given that a significant number of cases entered in the registry are retrospective, participants have the opportunity to use a semi-structured minimal or complete data set to facilitate data entry. In addition, the design allows subjects to be entered into the IRCPSS according to clinically relevant events. Emphasis is on longitudinal follow-up of signs and symptoms, which is paramount to garner clinically relevant information to eventually orient patient management. The IRCPSS includes also three specific forms to capture essential radiological, surgical, and cardiopulmonary data as many times as relevant, which are completed by the specialists themselves. Finally, connecting the clinical data registry with a safe image repository, using state-of-the-art pseudonymization software, was another major focus of development. Data quality and stewardship is ensured by a steering committee. All centers participating in the IRCPSS have signed a memorandum of understanding and obtained their own ethical approval.
CONCLUSION
Through state-of-the-art management of data and imaging, we have developed a practical, user-friendly, international registry to study CPSS in neonates, children, and adults. Via this multicenter and international effort, we will be ready to answer meaningful and urgent questions regarding the management of patients with CPSS, a condition often ridden with significant diagnostic delay contributing to a severe clinical course
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