Objective: With increasing life span osteoporosis becomes a more recognized problem in patients with cystic fibrosis (CF). The aim of this cross-sectional study in 75 adult patients with CF (mean age 25.3 years) was to assess the prevalence of low bone mineral density (BMD) by dual-energy x-ray absorptiometry (DEXA) and, for the first time, by quantitative ultrasound (QUS), and to identify predicting factors. Design and Methods: Bone status was assessed at the lumbar spine (L2-L4) and the femoral neck by DEXA, and at the calcaneus by QUS (stiffness index). These data were correlated with a variety of clinical and anthropomorphic variables. Biochemical markers of bone turnover such as osteocalcin, bone-specific alkaline phosphatase, crosslinks in urine, 25-hydroxy vitamin D (25-OH vitamin D), parathyroid hormone, calcium and free testosterone were determined by standard assays. Results: The mean BMD T score (^S.E.M.) was 21:4^0:17 at the lumbar spine, and 20:54^0:16 at the femoral neck. The mean T score of the calcaneal stiffness index was 20:83^0:19: Based on a lumbar spine T score ,22.5 by DEXA, 27% of the patients had osteoporosis. Multiple regression analysis showed that the forced expiratory volume in one second (FEV 1 ) and the use of oral glucocorticoids were independent predictors of low lumbar spine BMD, whereas body mass index (BMI) and the use of oral glucocorticoids were independent predictors of low femoral neck BMD. The stiffness index correlated moderately with BMD (0.49-0.62, P , 0:0001). QUS had a sensitivity and specificity of only 57% and 89% respectively for diagnosing 'osteoporosis' (based on a femoral neck T score ,22.5 by DEXA). Positive and negative predictive values were 36% and 95% respectively. Conclusions: Low BMD is frequent in adults with CF and is most strongly correlated with disease severity (BMI, FEV 1 ) and the use of glucocorticoids. Calcaneal QUS might help to screen out patients with a normal BMD, but sensitivity and specificity were not sufficiently high to replace DEXA in these patients.

Die inhalative Applikation von Medikamenten ist die bedeutsamste Form der Behandlung von obstruktiven Atemwegserkrankungen [30,75,99]. Gründe hierfür sind die hohe lokale Wirkstoffkonzentration bei geringer Gesamtdosis und das damit verbundene günstige Wirkungs-/Nebenwirkungsverhältnis (z. B. Glukokortikoide) sowie der rasche Wirkungseintritt (z. B. Beta-2-Adrenergika) [2,73,113,114]. Neben Medikamenten zur topischen Therapie wird zunehmend die Inhalation verschiedener Substanzen zur systemischen Behandlung geprüft [17,19,59,60,65,93]

Summary Experience with vascular pathology and vascular surgery, especially with the use of grafts, is limited in horses. Only one case of a venous aneurysm has been described in the horse, but without successful surgical correction. This case report describes the treatment of a 3‐year‐old female pony presented with an intermittent localised swelling of the left jugular vein after a previous thrombophlebitis. Ultrasonographic examination revealed a large jugular vein pseudoaneurysm. Treatment consisted of a partial aneurysmectomy preserving the healthy medial wall of the jugular vein. An autologous saphenous vein patch technique was used to reconstruct the vein with the largest possible lumen to minimise thrombus formation. Anticoagulants, antimicrobial and anti‐inflammatory drugs were administered pre‐ and post‐operatively. Follow‐up ultrasound examinations initially showed local narrowing of the graft. At 2 and 8 months post‐operatively, no external deformation of the jugular vein was visible and ultrasonography revealed patent blood flow with only a slight difference in lumen diameter. The patch venoplasty proved a viable surgical technique for jugular vein reconstruction resulting in a sufficient lumen, no thrombus formation and a good cosmetic and functional outcome.

Evaluation of health-related quality of life (HRQL) has become steadily more essential during the last two decades in research and health care practice in order to evaluate the human and financial costs and benefits of modern medical techniques. HRQL in its definition is based on different components of health including physical state, psychological well-being, social relations and functional capacities that are influenced by a persons experience, beliefs, expectations, and perceptions. For the purpose of assessment of HRQL several instruments have been developed. Generic instruments are not specific to any particular disease and are therefore most commonly used for general survey research on health allowing comparisons between disease states. In contrast, disease-specific questionnaires which are necessary in order to focus on domains most relevant to a particular disease are thought to be more sensitive than generic instruments following therapeutic interventions. Home mechanical ventilation (HMV) delivered noninvasively by a facial mask is a well established treatment for chronic hypercapnic respiratory failure. It is widely accepted that survival improves following institution of HMV in most patients with chest wall deformities or neuromuscular diseases while this is still controversially discussed in patients with COPD. However, patients receiving HMV usually have severe respiratory insufficiency with a past medical history of several years or decades, and suffer from end stage disease with objectively severe limitations of daily living. In addition, HMV is a time consuming and cost intensive therapy. Therefore, several studies have been conducted in the last decade to evaluate HRQL in patients receiving HMV. Recent studies using generic questionnaires have shown impairments in HRQL in patients receiving HMV compared to normals. This was primarily attributed to severe limitations in physical health, but not in mental health indicating that if severe physical limitation occurs in advanced respiratory disease this will not necessarily lead to mental limitation. In addition, limitations in HRQL in patients with HMV were not substantially higher than in patients with different chronic disease being not dependent on HMV. Improvements in HRQL following the institution of HMV were only mild or even insignificant in patients with COPD, but patients with restrictive ventilatory disorders are suspected to have more benefits. However, well validated disease-specific questionnaires which are designed to be more sensitive in the assessment of changes in HRQL than generic instruments have been introduced recently for patients with severe respiratory failure, but the influence of HMV to HRQL remains still unclear, since prospective studies using these questionnaires have yet not been finished.

No abstract