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1.
ObjectiveAlthough a staccato uroflow pattern is considered representative of dysfunctional voiding (DV), we recently found that only a third of children with staccato flow had an active pelvic floor electromyography (EMG) during voiding. Here, we analyzed the reverse, that is, how often a staccato flow pattern occurs in children with documented DV. In addition, we reviewed what other flow patterns are prevalent in this condition.Materials and methodsWe reviewed our LUT dysfunction registry for children with EMG-confirmed DV. Uroflow patterns were categorized as staccato, interrupted, mixed (i.e., staccato and interrupted patterns), or grossly normal.ResultsOf 596 children who underwent a uroflow/EMG examination, 121 had an active pelvic floor EMG during voiding, that is a finding consistent with the diagnosis of DV. The flow patterns identified in those diagnosed with DV were staccato in 70 (58%), interrupted in 22 (19%), mixed in 12 (10%), and a bell-shaped or depressed curve in 17 (14%). Staccato pattern became normal in 96% following successful treatment with biofeedback.ConclusionsWhile a staccato uroflow pattern was the most common pattern seen in children diagnosed with DV by a uroflow/EMG, nearly a third had an interrupted or mixed flow pattern underscoring the importance of performing simultaneous pelvic floor EMG during a uroflow study, especially when trying to rule out DV. Failure of the staccato flow pattern to normalize after therapy strongly suggests either inadequate therapy or an incorrect diagnosis.  相似文献   

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Among congenital malformations, congenital diaphragmatic hernia (CDH) is distinguished by its relatively low occurrence rate, need for resource intensive, integrated multidisciplinary care, and widespread variation in practice and outcome. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence, they are poorly suited to the study of a condition like CDH due to challenges in illness severity adjustment, unpredictability in clinical course and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for CDH is the patient registry, which aggregates multi-institutional condition-specific patient level data into a large CDH-specific database for the dual purposes of collaborative research and quality improvement across participating sites. This article discusses patient registries from the perspective of structure, data collection and management, and privacy protection that guide the use of registry data to support collaborative, multidisciplinary research. Two CDH-specific registries are described as illustrative examples of the “value proposition” of registries in improving the evidence basis for best practices for CDH.  相似文献   

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Background

High-resolution peripheral quantitative computed tomography (HR-pQCT) evaluates different components of bone fragility. The positioning and length of the region of interest (ROI) in growing populations remain to be defined.

Methods

Using HR-pQCT at the ultradistal tibia, we compared a single-center cohort of 28 teenagers with chronic kidney disease (CKD) at a median age of 13.6 (range, 10.2–19.9) years to local age-, gender-, and puberty-matched healthy peers. Because of the potential impact of short stature, bone parameters were assessed on two different leg-length-adjusted ROIs in comparison to the standard analysis, namely the one applied in adults. The results are presented as median (range).

Results

After matching, SDS height was ?0.9 (?3.3;1.6) and 0.3 (?1.4;2.0) in patients and controls, respectively (P < 0.001). In younger children (e.g., prepubertal, n = 11), bone texture parameters and bone strength were not different using standard analysis. However, using a height-adjusted ROI enabled better characterization of cortical bone structure. In older patients (e.g., pubertal, n = 17), there were no differences for height between patients and controls: with the standard evaluation, cortical bone area and cortical thickness were significantly lower in CKD patients: 85 (50–124) vs. 108 (67–154) mm2 and 0.89 (0.46–1.31) vs 1.09 (0.60–1.62) mm, respectively (both P < 0.05).

Conclusions

Adapting the ROI to leg length enables better assessment of bone structure, especially when height discrepancies exist between controls and patients. Larger cohorts are required to prospectively validate this analytic HR-pQCT technique.  相似文献   

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Total IgE levels are usually elevated in allergic diseases, being highest in atopic eczema, followed by atopic asthma and allergic rhinitis. Genetic factors are believed to play a role in total IgE levels, with higher levels seen in Black African subjects. Total IgE is also raised in parasite infection. Thus, the higher total IgE levels in Black Africans could be because of environmental rather than genetic factors. Few studies have investigated the usefulness of total IgE levels in the evaluation of atopy in Black Africans. The objective of this study was to determine the total IgE levels in unselected urban Black African high school children and to correlate this with atopy and ascaris sensitization. Atopic status was assessed by means of specific allergen sensitization (skin prick tests to eight inhalant and four food allergens), self-reported asthma and bronchial hyper-responsiveness measured by methacholine challenge. Ascaris sensitization was assessed by means of ascaris IgE measured by CAP-RAST. Total IgE levels were markedly skewed toward the left and were not distributed in a Gaussian or a log-normal distribution. Skin prick tests were positive for aeroallergens in 32.3% of subjects. Thirty four percent had elevated ascaris IgE. Total IgE was higher in atopic vs. non-atopic subjects and correlated with the number of positive skin prick tests, self-reported asthma and bronchial hyper-responsiveness. Subjects without allergy (or) atopy had a median total IgE of 80–90 kU/I. In addition total IgE correlated with ascaris IgE. Subjects with no ascaris sensitization had median total IgE of 77.1 kU/l. Subjects with neither atopy/asthma nor ascaris sensitisation had a median total IgE of 69.9 kU/I, similar to the levels seen in people of other genetic origins. This study suggests that helminthic infection rather than genetic differences, may be the major determining factor of IgE levels in certain populations.  相似文献   

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新生儿缺血缺氧性脑病(hypoxic-ischemic encephalopathy,HIE)是一种新生儿较常见的影响脑功能的疾病。目前,亚低温、高压氧舱是治疗新生儿HIE的主要手段,然而二者互相独立,无法实现二者结合同步治疗,疗效单一,且未对新生儿进行脑功能相关的生理信息监测。此外,高压氧舱及亚低温床垫的参数设置依赖医疗师的经验,且在整个过程不变。该文提出了一种针对新生儿HIE治疗的新型装置,同时具有高压氧舱及亚低温床垫模块,使新生儿可以根据不同情况接受高压氧舱和/或亚低温床垫的治疗;还具有多种生理信息的实时监测功能,包括振幅整合脑电图、心电监护、近红外光谱监测,可以实时监测新生儿的脑功能状态、心率、心律、心肌供血、脑组织血红蛋白浓度和血氧饱和度;并且结合智能调控算法,根据新生儿的生理信息智能调控参数并提供后续治疗的建议。  相似文献   

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Summary Twenty-three children with diabetes mellitus, their ages ranging from 0.2–9.8 years, but with no sign of diabetic microvascular disease were investigated by M-mode and Doppler echocardiography, along with a comparable group of control subjects. In the diabetics, the fractional shortening and the mean velocity of fractional shortening were 14 and 18% higher, respectively, whereas the left ventricular end-systolic wall stress, an indicator of left ventricular afterload, was markedly reduced (22%). Assuming an unchanged preload in the two groups, this indicates a reduced afterload in these children. Systolic and diastolic time intervals, heart rate, and blood pressure were similar in diabetics and controls. Doppler-derived transmitral left ventricular filling indices were also similar. Thus, in these diabetic children no signs of left ventricle is considered to be due to a reduced afterload in early insulin-dependent diabetes.  相似文献   

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Background

National advisory panels (NAPs) have been established for the care of children and young people (CYP) with cancer in the United Kingdom since 2011, with an increase in panel number in recent years. Their practice has not previously been reviewed; therefore, we sought to evaluate the role, practice and impact of six selected NAPs offering expertise in ependymoma, histiocytosis, leukaemia, neuroblastoma, renal tumours and sarcoma.

Procedure

This service evaluation used mixed methodology, including review of NAP documentation, semi-structured interviews with the NAP chairs and an analysis of the cases referred for discussion.

Results

Total 1110 referrals were analysed. Results demonstrated the significant scope and amount of work undertaken by the NAPs, largely testament to the commitment of the panel members. Specific roles fulfilled have been highlighted, and NAP recommendations have been shown to influence clinical decision-making and be implemented in the majority of cases. Despite widespread good practice, areas to address have been identified; these include clarity regarding NAP membership, consistency in recommendations, the consideration of holistic information to promote personalised management and the exploration of wider multidisciplinary team roles.

Conclusions

In the context of increasing demand and the escalating number of NAPs, it is timely to consider how service improvement can be facilitated. Best practice guidelines have been formulated as a product of this study, to promote a sustainable and effective model for NAPs. Review and benchmarking national panel performance against these guidelines will drive high standards of care going forward and they should be embedded as standard practice.  相似文献   

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Five groups of full-term newborn infants divided on the basis of the subtypes of M and one group of full-term newborn infants belonging to the phenotype M1S have been studied.A decrease in alpha-1-antitrypsin level in the M1S newborn infants was observed (P<0.001). A similar decrease was observed in a group of M1S preterm newborn infants in comparison with M1M1 and M2M2 newborn infants of the same gestational age.Moreover, full-term infants belonging to the subtype M1M2 also showed a decrease in the level of this inhibitor in comparison to the full-term infants belonging to other subtypes of M (P<0.01). Preterm newborn infants belonging to subtypes M1M2 and M1M1 showed identical circulating alpha-1-antitrypsin levels.When the phenotype is taken into account, data obtained from newborn infants free from perinatal pathology show that alpha-1-antitrypsin levels—but not alpha-2-macroglobulin levels—increase with fetal age.Moreover the specific compensatory response to the deficiency, consisting in an increase in alpha-2-macroglobulin levels, is absent in M1S and M1M2 neonates.The wide distribution of the M1M2 subtypes and, in some regions of the MS phenotype, suggests a need for genetic typing of the alpha-1-antitrypsin molecule in investigating the behaviour of this inhibitor in the perinatal period.Supported in part by a grant from Consiglio Nazionale delle Ricerche  相似文献   

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ObjectiveTo evaluate the effect of 15° forward (FW) seat inclination and foot-support in children with cerebral palsy (CP) on postural adjustments during reaching.DesignObservational study repeated-measures design; step two of two-step-project.SettingLaboratory unit within University Hospital and two special education schools.Participants19 children (ten unilateral spastic CP (US-CP); nine bilateral spastic CP (BS-CP); Gross Motor Function Classification System levels I-III; 6–12 years old). Participants were able to take part for one one-hour session.InterventionReaching while sitting in four seating conditions (FW or horizontal seat; with or without foot-support) applied in randomized order.Outcome measuresSimultaneously, surface electromyography (EMG) of neck, trunk and arm muscles and kinematics of head and reaching arm (step one of two-step-project) were recorded. Primary outcome parameters were the ability to modulate EMG-amplitudes at baseline and during reaching (phasic muscle activity). Other EMG-parameters were direction-specificity (1st control level), and 2nd level of control parameters: recruitment order, and anticipatory postural activity. Motor behaviour measures: ability to modulate EMG-amplitudes to kinematic characteristics of reaching and head stability.ResultsOnly foot-support was associated with increased tonic background EMG-amplitudes and decreased phasic EMG-amplitudes of the trunk extensors in children with US-CP and BS-CP (mixed-models analyses; p-values <0.01). The foot-support effect was also associated with better kinematics of reaching (Spearman's Rho; p-values <0.01).ConclusionIn terms of postural adjustments during forward reaching, foot-support enhanced the children's capacity to modulate trunk extensor activity, which was associated with improved reaching quality. FW-tilting did not affect postural muscle activity.  相似文献   

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Pinelli L, Zaffani S, Cappa M, Carboniero V, Cerutti F, Cherubini V, Chiarelli F, Colombini MI, La Loggia A, Pisanti P, Vanelli M, Lorini R. The ALBA Project: an evaluation of needs, management, fears of Italian young patients with type 1 diabetes in a school setting and an evaluation of parents' and teachers' perceptions. Objective: To determine how Italian parents and school personnel of 6–13‐year‐old children with type 1 diabetes (T1D) manage during school hours, including insulin administration, management of hypoglycemia, and glucagon use. A further aim was an investigation into the responsibilities and training of school personnel regarding diabetes. Research design and methods: After an initial qualitative phase, semi‐structured questionnaires were completed by a sample of parents and teachers. Results: 220 parent and 52 teacher questionnaires were completed. 43.6% of parents said diabetes had negatively influenced school activities. Children either self‐administer insulin, or have help from a parent, since there is very rarely a nurse present (3.6%) or a teacher who will take responsibility for the treatment (2.9%). Most parents (55.9%) stated either that the school had no refrigerator to store glucagon or that they did not know if the school was so equipped. A small percentage of teachers considered their schools to be equipped to manage an emergency (23%) and said they would use glucagon directly in an emergency (14.9%). Only 40.4% of teachers said that they had received any specific training. Conclusions: The study shows that people who are not directly involved have superficial knowledge of the different aspects of diabetes, even though no parents reported episodes of neglect/incorrect management. There is no legislation which clearly defines the role of the school in the care of children with T1D, and teachers are not trained to help them. Training sessions for school personnel and greater legislative clarity about the ‘insulin and glucagon question’ are key factors that may improve the full integration of the child with diabetes.  相似文献   

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The early and late introduction of complementary food, both prevalent in Ethiopia, are associated with morbidities, growth faltering and developmental risks in children. The interhousehold network around the primary caregiver''s intrahousehold network is critical in influencing the age of introducing complementary foods. This study examined the influence of household composition and structures on complementary food introduction. This is a secondary data analysis of four Ethiopian Demographic and Health Surveys conducted between 2000 and 2016. The household structure and composition variables were calculated from household members'' kinship status and attribute, respectively. The introduction of solid, semisolid or soft foods was dichotomised as whether the children within 6 to 8 months have been given complementary foods. Multivariable logistic regression with adjustment for the primary caregiver and household characteristics was run to examine the associations between household structure and composition variables and the introduction of complementary foods. The marginal effects (ME) were calculated to facilitate the practical interpretation of the study findings. Large households (>3 nonredundant contacts) with extended family or unrelated people (high effective size, ME = 6.01%, 95% confidence interval [CI]: −8.53, −3.49) lowered the proportion of children starting food within the recommended 6–8 months. Households with close kins (high constraint) (ME = 7.22%, 95% CI: −13.65, 28.09) and greater age diversity (ME = 0.65%, 95% CI: 0.15, 1.15) increased the proportion of children receiving complementary food at an appropriate age. This study revealed that interhousehold structure and composition influence the age of introduction of complementary foods. These factors, therefore, need to be considered in designing interventions to improve age at the introduction of complementary foods.  相似文献   

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