Related Articles

Hereditary angioedema with C1 inhibitor deficiency: delay in diagnosis in Europe.

Allergy Asthma Clin Immunol. 2013 Aug 12;9(1):29

Authors: Zanichelli A, Magerl M, Longhurst H, Fabien V, Maurer M

Abstract
BACKGROUND: Hereditary angioedema (HAE) is a rare, debilitating, and potentially life-threatening disease characterized by recurrent edema attacks. Important advances in HAE treatment have been made, including the development of new therapies for treating or preventing attacks. Nevertheless, the disease is still frequently misdiagnosed and inappropriately treated, potentially exposing patients with laryngeal attacks to the risk of asphyxiation.
METHODS: The Icatibant Outcome Survey (IOS) is an international, observational study that documents the clinical outcome of HAE patients eligible for treatment with icatibant. Patient ages at first symptoms and at diagnosis were recorded at enrolment, and the delay between first symptoms and diagnosis was calculated.
RESULTS: The median [range] diagnostic delay in HAE type I and II patients across eight countries was 8.5 years [0--62.0]. The median delay in diagnosis was longer for HAE type II versus type I (21 versus 8 years, respectively), although this did not quite reach statistical significance.
CONCLUSIONS: Although it can be difficult to differentiate HAE symptoms from those of more common angioedema sub-types (e.g. idiopathic or acquired angioedema), our results show that HAE type I and II patients have an unacceptable delay in diagnosis, even those with a family history of the disease. Raising physician awareness of this disabling and potentially fatal disease may lead to a more accurate diagnosis and timely treatment.

PMID: 23937903 [PubMed - as supplied by publisher]

Development of a Modified BODE Index as a Mortality Risk Measure Among Older Adults With and Without Chronic Obstructive Pulmonary Disease.

Am J Epidemiol. 2013 Aug 8;

Authors: Roberts MH, Mapel DW, Bruse S, Petersen H, Nyunoya T

Abstract
The BODE index was developed as a prognostic mortality risk tool for persons with chronic obstructive pulmonary disease (COPD). It incorporates 4 measures: body mass index, lung obstruction, dyspnea, and exercise capacity. The intent of this study was to examine how well a BODE-like index constructed using a simpler lung function measure, peak expiratory flow, in combination with physical functioning and symptom information more readily found in survey data (a quasi-BODE index), performs in identifying persons at higher risk of mortality and whether it may be extended as an assessment of mortality risk to persons without diagnosed COPD. Using US national survey data from the Health Retirement Study for 2006-2010, each unit increase in the quasi-BODE index score was associated with a multiplicative 50% increase in mortality risk (odds ratio = 1.50, 95% confidence interval: 1.41, 1.59). The quasi-BODE index is a multidimensional health status instrument based on the BODE index, which is a good predictor of mortality. The quasi-BODE index was compiled using simple measures of physical and respiratory function. It is a potentially useful prognostic instrument for older adult populations with or without COPD, including those with severe physical limitations, particularly when combined with demographic factors and comorbid conditions.

PMID: 23928262 [PubMed - as supplied by publisher]

Related Articles

Mesenchymal stem cells in the treatment of pediatric diseases.

World J Pediatr. 2013 Aug;9(3):197-211

Authors: Zheng GP, Ge MH, Shu Q, Rojas M, Xu J

Abstract
BACKGROUND: In recent years, the incredible interests in mesenchymal stem cells have boosted the expectations of both patients and physicians. Unlike embryonic stem cells, neither their procurement nor their use is deemed controversial. Moreover, their immunomodulatory capacity coupled with low immunogenicity has opened up their allogenic use, consequently broadening the possibilities for their application. In May 2012, Canadian health regulators approved Prochymal, the first mesenchymal stem cells-based drug, for acute graft-versus-host diseases in children who have failed to respond to steroid treatment. The aim of this article is to review the recent advances in mesenchymal stem cells for pediatric diseases.
DATA SOURCES: A literature review was performed on PubMed from 1966 to 2013 using the MeSH terms "mesenchymal stem cells", "clinical trials" and "children". Additional articles were identified by a hand search of the references list in the initial search.
RESULTS: The following categories are described: general properties, mechanisms of action, graft-versus-host diseases, cardiovascular diseases, liver diseases, inflammatory bowel diseases, osteoarticular diseases, autoimmune diseases, type 1 diabetes, and lung diseases.
CONCLUSIONS: Mesenchymal stem cells, owing to their availability, immunomodulatory properties, low immunogenicity, and therapeutic potential, have become one of the most attractive options for the treatment of a wide range of diseases. It is expected to see more and more clinical trials and applications of mesenchymal stem cells for pediatric diseases in the near future.

PMID: 23929252 [PubMed - in process]

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Physical Activity and Arterial Stiffness in Chronic Obstructive Pulmonary Disease.

Respir Physiol Neurobiol. 2013 Aug 7;

Authors: Stickland MK, Vogan N, Petersen SR, Wong EY, Bhutani M

Abstract
Arterial stiffness is predictive of cardiovascular events and is elevated in chronic obstructive pulmonary disease (COPD). As physical inactivity and exercise intolerance are associated with elevated arterial stiffness in health, we hypothesized that lower physical activity would be related to increased arterial stiffness in COPD; and that active COPD patients would have reduced arterial stiffness compared to sedentary counterparts. Arterial stiffness was evaluated using pulse wave velocity (PWV) in 33 COPD patients (FEV1 =65% predicted) and 10 controls. FEV1%pred, peak oxygen consumption (V. O2peak), and physical activity data were obtained. The inactive COPD group had higher PWV than controls (9.6 vs. 8.3 m•s(-1), p<0.05); while there was no difference in PWV between the active COPD group and controls. Within the COPD patients, V. O2peak (r=-0.44, p=0.01) and physical activity (r=-0.38, p=0.03) were the best predictors of PWV. Physical inactivity and exercise intolerance appear to be related to arterial stiffness in COPD, and may contribute to increased cardiovascular disease risk in COPD.

PMID: 23933008 [PubMed - as supplied by publisher]