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Treatment with continuous subcutaneous insulin infusion (CSII) allows a large degree of treatment individualization and intensification in children with diabetes. The study's aim was to evaluate the impact of treatment with CSII on glycated haemoglobin level (HbA1c) in children with diabetes and investigate whether introduction of CSII is associated with an increased risk of acute complications of diabetes. Patients treated throughout the recruitment period exclusively with multiple daily injections (MDI) were matched for duration of diabetes and HbA1c level at baseline with patients treated exclusively with CSII in a 1:1 group ratio (n?=?223 and 231 for MDI and CSII, respectively). The CSII group showed lower HbA1c after the observation period (7.98?±?1.38 vs. 7.56?±?0.97; P?=?0.002). HbA1c variability measured as standard deviations of average values was also lower in the CSII group (0.73?±?0.45 vs. 0.84?±?0.54; P?=?0.049). The rate of hospitalization due to acute events was similar in both groups (14.7/100 vs. 14.0/100 person/years in the MDI and CSII group, P?=?0.72). Duration of hospital stay per year was on average 1.25?days shorter in the CSII group (P?=?0.0004), but the risk of acute complications resulting in hospitalization did not differ between the groups (hazard ratio (HR) 1.16; 95% confidence interval (95% CI) 0.68-1.63). The most significant risk factor for hospitalization due to acute complications was baseline HbA1c concentration (HR 1.25; 95% CI 1.14-1.37). In conclusion, CSII treatment may improve glycemic control and reduce its variability. Change of MDI to CSII does not alter the risk of hospitalization and may reduce the annual duration of hospitalization in children with diabetes.  相似文献   
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Hypertension (HTN) is common in pediatric recipients following kidney transplantation (KT). We retrospectively assessed the impact of HTN on long‐term (>10‐yr) outcomes in pediatric KT recipients (aged < 18 yr) at our center. Two hundred and ninety‐three pediatric KT recipients (83% living donor [LD]) with graft survival (GS) for ≥5 yr were studied. HTN was defined by antihypertensive medication use at five yr post‐KT. One hundred and sixty (55%) recipients did not have HTN, and 133 (45%) had HTN at five yr post‐KT. There were no differences in actuarial patient survival between cohorts. Actuarial GS at 15 and 20 yr was 68% and 53% for recipients without HTN, and 53% and 33% for recipients with HTN (p = 0.006). Among LD recipients using one antihypertensive, GS at 15 yr was 100% for those using an angiotensin‐converting enzyme inhibitor (ACEI) and 44% for those not using an ACEI (p = 0.04). Among these recipients, HTN treated with no ACEI was a significant risk factor for graft failure at >5 yr (hazard ratio [HR] = 2.5, p = 0.02), but HTN treated with an ACEI was not (HR = 0.6, p = 0.7). HTN at five yr post‐KT is associated with poorer long‐term GS in pediatric recipients, but ACEI therapy may enable better outcomes and should be studied further.  相似文献   
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Background

The incidence of infected urolithiasis is unknown, and evidence describing the optimal management strategy for obstruction is equivocal.

Objective

To examine the trends of infected urolithiasis in the United States, the practice patterns of competing treatment modalities, and to compare adverse outcomes.

Design, setting, and participants

A weighted estimate of 396 385 adult patients hospitalized with infected urolithiasis was extracted from the Nationwide Inpatient Sample, 1999–2009.

Outcome measurements and statistical analysis

Time trend analysis examined the incidence of infected urolithiasis and associated sepsis, as well as rates of retrograde ureteral catheterization and percutaneous nephrostomy (PCN) for urgent/emergent decompression. Propensity-score matching compared the rates of adverse outcomes between approaches.

Results and limitations

Between 1999 and 2009, the incidence of infected urolithiasis in women increased from 15.5 (95% confidence interval [CI], 15.3–15.6) to 27.6 (27.4–27.8)/100 000); men increased from 7.8 (7.7–7.9) to 12.1 (12.0–12.3)/100 000. Rates of associated sepsis increased from 6.9% to 8.5% (p = 0.013), and severe sepsis increased from 1.7% to 3.2% (p < 0.001); mortality rates remained stable at 0.25–0.20% (p = 0.150). Among those undergoing immediate decompression, 113 459 (28.6%), PCN utilization decreased from 16.1% to 11.2% (p = 0.001), with significant regional variability. In matched analysis, PCN showed higher rates of sepsis (odds ratio [OR]: 1.63; 95% CI, 1.52–1.74), severe sepsis (OR: 2.28; 95% CI, 2.06–2.52), prolonged length of stay (OR: 3.18; 95% CI, 3.01–3.34), elevated hospital charges (OR: 2.71; 95%CI, 2.57–2.85), and mortality (OR: 3.14; 95%CI, 13–4.63). However, observational data preclude the assessment of timing between outcome and intervention, and disease severity.

Conclusions

Between 1999 and 2009, women were twice as likely to have infected urolithiasis. Rates of associated sepsis and severe sepsis increased, but mortality rates remained stable. Analysis of competing treatment strategies for immediate decompression demonstrates decreasing utilization of PCN, which showed higher rates of adverse outcomes. These findings should be viewed as preliminary and hypothesis generating, demonstrating the pressing need for further study.  相似文献   
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The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013–2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5–116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60–79 %), 6 (26 %) had moderate (FVC% 40–59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (r = 0.31, p = 0.02 and r = 0.39, p = 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.  相似文献   
100.
Arthrogryposes – multiple joint contractures – are a clinically and etiologically heterogeneous class of diseases, where accurate diagnosis, recognition of the underlying pathology and classification are of key importance for the prognosis as well as for selection of appropriate management. This treatment remains challenging and optimally in arthrogrypotic patients should be carried out by a team of specialists familiar with all aspects of arthrogryposis pathology and treatment modalities: rehabilitation, orthotics and surgery. In this comprehensive review article, based on literature and clinical experience, the authors present an update on current knowledge on etiology, classifications and treatment options for skeletal deformations possible in arthrogryposis.  相似文献   
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