Alstrupmarkussen7698
To translate and cross-culturally adapt the Childhood Bladder and Bowel Dysfunction Questionnaire (CBBDQ) for use in Brazilian Portuguese. The CBBDQ is an 18-item tool covering 10 bladder and 8 bowel symptoms that was developed for use with children of 5 to 12 years of age with bowel and bladder dysfunction (BBD). The instrument has already been validated for use in Dutch and English.
In the process of translation and cultural adaptation from English to Portuguese, the CBBDQ was submitted to undergo the required steps as established by the international methodological criteria forward translation, synthesis, back-translation, expert panel review and pre-testing.
Ninety-three parents of children with lower urinary tract dysfunction answered the questionnaire. The mean age of the children was 7.6±2.1 years and 54 were female. Internal consistency was excellent, with a Cronbach's alpha of 0.91 to 0.96. Additionally, reliability was high, with an intraclass correlation coefficient of 0.94 (95%CI 0.85-0.93; p<0.0001).
The translation and cultural adaptation of the CBBDQ enabled a quantitative evaluation of bladder and bowel symptoms to be performed in Brazilian children. The scores achieved allow the severity of BBD to be evaluated, as well as the patient's progress during treatment. Selleckchem BIIB129 The use of this questionnaire in clinical practice and research will allow more consistent data on BBD to be obtained.
The translation and cultural adaptation of the CBBDQ enabled a quantitative evaluation of bladder and bowel symptoms to be performed in Brazilian children. The scores achieved allow the severity of BBD to be evaluated, as well as the patient's progress during treatment. The use of this questionnaire in clinical practice and research will allow more consistent data on BBD to be obtained.
Inborn Errors of Immunity (IEI), also known as primary immunodeficiencies, correspond to a heterogeneous group of congenital diseases that primarily affect immune response components. The main clinical manifestations comprise increased susceptibility to infections, autoimmunity, inflammation, allergies and malignancies. The aim of this article is to review the literature on combined immunodeficiencies (CIDs) focusing on the diagnosis and treatment and the particularities of the clinical management of these patients.
Critical integrative review, aimed to present articles related to primary immunodeficiencies combined with a searchin the PubMed and SciELO databases, with evaluation of publications from the last twenty years that were essential for the construction of knowledge on this group of diseases.
We highlight the main characteristics of CIDs, dividing them according to their pathophysiological mechanisms, such as defects in the development of T cells, TCR signaling, co-stimulatory pathways, cytokine signaling, adhesion, migration and organization of the cytoskeleton, apoptosis pathways, DNA replication and repair and metabolic pathways. In CIDs, clinical manifestations vary widely, from sinopulmonary bacterial infections and diarrhea to opportunistic infections, caused by mycobacteria and fungi. Neonatal screening makes it possible to suspect these diseases before clinical manifestations appear.
The CIDs or IEI constitute a complex group of genetic diseases with T-cell involvement. Neonatal screening for these diseases has improved the prognosis of these patients, especially in severe ones, known as SCIDs.
The CIDs or IEI constitute a complex group of genetic diseases with T-cell involvement. Neonatal screening for these diseases has improved the prognosis of these patients, especially in severe ones, known as SCIDs.
To meta-analyze health-related quality of life in pediatric patients with chronic kidney disease in comparison to healthy patients according to the dimensions of the PedsQL instrument.
A systematic review was performed with meta-analysis for the mean difference in each of the health-related quality of life dimensions. The authors searched for ten scientific databases including PubMed, Scopus, SciELO, Science Direct, ProQuest, Google Scholar. Reproducibility by the Kappa index was evaluated, and Dersimonian and Laird's tests, RI coefficient, Begg statistic, Forest Plot, and sensitivity analysis were carried out.
17 investigations were included in the qualitative synthesis and 7 in the quantitative synthesis with a population of 1214 of both healthy and sick pediatric patients with 3-5 chronic kidney disease stages. The health-related quality of life in pediatric chronic kidney disease patients presented lower scores in all the evaluated dimensions in the physical dimension the difference is of 13.6 pointted to strengthen the affected dimensions, including adjustments to daily life and prevention of complications related to the disease.Health is usually defined as the absence of pathology. Here, we endeavor to define health as a compendium of organizational and dynamic features that maintain physiology. The biological causes or hallmarks of health include features of spatial compartmentalization (integrity of barriers and containment of local perturbations), maintenance of homeostasis over time (recycling and turnover, integration of circuitries, and rhythmic oscillations), and an array of adequate responses to stress (homeostatic resilience, hormetic regulation, and repair and regeneration). Disruption of any of these interlocked features is broadly pathogenic, causing an acute or progressive derailment of the system coupled to the loss of numerous stigmata of health.In the germarium of the Drosophila ovary, developing germline cysts are surrounded by a population of somatic escort cells that are known to function as the niche cells for germline differentiation;1 however, the underlying molecular mechanisms of this niche function remain poorly understood. Through single-cell gene expression profiling combined with genetic analyses, we here demonstrate that the escort cells can be spatially and functionally divided into two successive domains. The anterior escort cells (aECs) specifically produce ecdysone, which acts on the cystoblast to promote synchronous cell division, whereas the posterior escort cells (pECs) respond to ecdysone signaling and regulate soma-germline cell adhesion to promote the transition from 16-cell cyst-to-egg chamber formation. The patterning of the aEC and pEC domains is independent of the germline but is dependent on JAK/STAT signaling activity, which emanates from the posterior. Thus, a heterogeneous population of escort cells constitutes a stepwise niche environment to orchestrate cystoblast division and differentiation toward egg chamber formation.
