Ultrastructural pathology of primary ciliary dyskinesia: report about 125 cases in Germany

<p>Abstract</p> <p>Background</p> <p>Primary ciliary dyskinesia (PCD) is a rare genetically induced disorder of cilia inducing mainly respiratory diseases. Transmission electron microscopy (TEM) analysis of ciliary ultrastructure is classically used for diagnosis. We report our experience of TEM investigations in a large series of patients.</p> <p>Methods</p> <p>TEM analysis performed of 742 biopsies from patients with suspected PCD was reviewed retrospectively. Ultrastructural defects were analysized further in 125 cases with changes typical for PCD.</p> <p>Results</p> <p>In 18.1% of patients diagnosis of PCD was made because of morphological alterations, in 68.2% secondary changes were seen. In 13.7% material was not feasible for analysis. Mostly defects of dynein arms were detected in PCD (96.8%). In particular defects of the inner arms (51.2%) and combined dynein defects (37.6%) were found. Total loss of dynein arms was dominant. Only in 3.2% deficiencies of central structures were found alone. Associated situs inversus or dextracardia was reported clinically in 21.4%.</p> <p>Conclusions</p> <p>TEM analysis is possible in most patients and a useful tool for diagnosis of PCD. Functional and genetic analysis should be done additionally. Registers should be installed to collect all available informations and push further research.</p

The evolution of the student as a customer in Australian higher education: a policy perspective

In 2014, the Australian Federal Government attempted to de-regulate higher education fees so as to allow universities to set their own tuition fees. The associated public debate offer critical insights into how the identity of a student as a ‘customer’ of higher education is understood and deployed when developing higher education policy. This paper uses the 2014 Australian higher education reforms as a lens through which to further scholarly research into the student-as-customer metaphor and to see how it is influenced by the perceptions and understandings of policy actors external to the higher education sector. These include politicians, special interest groups, the students and their parents and prospective employers. This study reveals that the public/private nexus—both of funding and benefit— problematizes traditional conceptualisations of students and others as higher education customers. In turn, this restricts the ability or desire of policy actors to describe how the student functions as a customer as a consequence of market reform. This inability compromises the development of effective and sustainable higher education polic

Correlation between disability and MRI findings in lumbar spinal stenosis: A prospective study of 109 patients operated on by decompression

Background and purpose MRI is the modality of choice when diagnosing spinal stenosis but it also shows that stenosis is prevalent in asymptomatic subjects over 60. The relationship between preoperative health-related quality of life, functional status, leg and back pain, and the objectively measured dural sac area in single and multilevel stenosis is unknown. We assessed this relationship in a prospective study. Patients and methods The cohort included 109 consecutive patients with central spinal stenosis operated on with decompressive laminectomy or laminotomy. Preoperatively, all patients completed the questionnaires for EQ-5D, SF-36, Oswestry disability index (ODI), estimated walking distance and leg and back pain (VAS). The cross-sectional area of the dural sac was measured at relevant disc levels in mm(2), and spondylolisthesis was measured in mm. For comparison, the area of the most narrow level, the number of levels with dural sac area < 70 mm(2), and spondylolisthesis were studied. Results Before surgery, patients with central spinal stenosis had low HRLQoL and functional status, and high pain levels. Patients with multilevel stenosis had better general health (p = 0.04) and less leg and back pain despite having smaller dural sac area than patients with single-level stenosis. There was a poor correlation between walking distance, ODI, the SF-36, EQ-5D, and leg and back pain levels on the one hand and dural sac area on the other. Women more often had multilevel spinal stenosis (p = 0.05) and spondylolisthesis (p < 0.001). Spondylolisthetic patients more often had small dural sac area (p = 0.04) and multilevel stenosis (p = 0.06). Interpretation Our findings indicate that HRQoL, function, and pain measured preoperatively correlate with morphological changes on MRI to a limited extent

Femoral nerve palsy due to iliacus hematoma occurred after primary total hip arthroplasty

PubMed ID: 18026742The perforation of the medial acetabular wall during total hip arthroplasty due to drilling is not uncommon. But, it has rarely been associated with serious adverse events. Here, we present a case report describing an iliacus hematoma with subsequent femoral nerve palsy after primary total hip arthroplasty in a 67-year-old woman who underwent primary total hip arthroplasty due to painful hip osteoarthritis. The diagnosis was made by pelvic magnetic resonance imaging. Conservative treatment was employed and the symptoms were resolved within 3 months. It should be borne in mind that femoral nerve palsy may occur after total hip arthroplasty. It may be due to a treatable cause, such as iliacus hematoma. So, pelvic MRI is recommended in such a condition, rather than just observation. © Springer-Verlag 2007

Vertebral osteomyelitis after cardiac surgery

Background. Mediastinitis after open-heart surgery is a serious complication that has a decreasing incidence but still a significantly high mortality rate. Back pain may develop during the course of treatment for mediastinitis, and this should suggest vertebral osteomyelitis in the differential diagnosis. Diagnosis of vertebral osteomyelitis may be difficult because of the insidious onset and delayed diagnosis, and treatment may result in serious neurologic compromise and even death of the patient

Pulmonary cement embolism: A complication of percutaneous vertebroplasty

Background: Percutaneous vertebroplasty ( PV) has recently become a very common procedure for vertebral compression fractures. Extravasation of cement, a common event associated with vertebroplasty, may lead to cement emboli in the lungs

The Effects of Riluzole on Neurological, Brain Biochemical, and Histological Changes in Early and Late Term of Sepsis in Rats

Objective. One of the underlying mechanisms of sepsis is thought to be the oxidative damage due to the generation of free radicals. Glutamate, the major excitatory amino acid in the brain, is known to play an important role in blood brain barrier (BBB) permeability, brain edema, and oxidative damage in pathological conditions. Riluzole, a glutamate release inhibitor, has been shown to have neuroprotective effects in several animal models. The aim of our study was to investigate the putative protective effect of riluzole against sepsis-induced brain injury