A flexible auditory research platform using acoustic or electric stimuli for adults and young children

A user-friendly and versatile research platform for use in auditory experiments, referred to as APEX (Application for PsychoElectrical eXperiments), is described. The platform takes care of automatic stimulus presentation and collection of the subject's responses. Acoustical auditory, as well as electrical auditory experiments with CI recipients can be conducted. The platform currently supports LAURA, Nucleus CI22 and Nucleus CI24 cochlear implants. The graphical user interface for the subjects has been extended to allow for testing very young children, by embedding the psychophysical procedures in a computer game. The research platform is available free of charge.     

Congenital analbuminaemia: biochemical and clinical implications. A case report and literature review

Congenital analbuminaemia was diagnosed in a small-for-gestational-age neonate presenting with placental and body oedema, an unusual presentation of this rare autosomal recessive disorder. A review of 39 reported cases in the literature shows that the clinical symptoms are always remarkably mild and that the diagnosis is rarely made in infancy. The absence of albumin appears to be partly counterbalanced by high levels of non-albumin proteins and circulatory adaptations. However, congenital analbuminemia can have important complications: lipodystrophy and hypercholesterolaemia, possibly leading to atherosclerosis. Other possible complications reported in literature are hypercoagulability, osteoporosis, respiratory tract infections, intrauterine growth retardation and intrauterine death. Moreover, albumin-binding drugs should be used with caution. Conclusion:congenital analbuminaemia is a rare disorder with remarkably mild signs and symptoms at all ages. Although often thought to be innocent, this disorder may have important clinical complications.     

Molecular diagnostic tools in Creutzfeldt-Jakob disease and other prion disorders

Clinical criteria and cerebrospinal fluid biomarkers for the diagnosis of human prion diseases (sporadic, iatrogenic or variant Creutzfeldt-Jakob disease and genetic inherited transmissible spongiform encephalopathies) are now widely available and show a sensitivity and specificity of approximately 98%. Final diagnosis of prion diseases is obtained by post-mortem examination upon identification of the pathological conformer of the prion protein (PrPSc) in the brain. Several diagnostic kits are now available that facilitate the immunochemical measurement of PrPSc. Several new molecular diagnostic techniques, aimed at increasing the sensitivity and specificity of PrPSc detection and at identifying markers of disease other than PrPSc, are the subject of ongoing studies. The aim of these studies is to develop preclinical screening tests for the identification of infected but still healthy individuals. These tests are also essential to investigate the safety of blood or blood-derived products and to ensure meat safety in European countries.     

Debridement arthroplasty for osteoarthritis of the elbow (Outerbridge-Kashiwagi procedure)

The Outerbridge-Kashiwagi (O-K) procedure has been described to treat mild to moderate osteoarthritis of the elbow. We treated a consecutive series of 15 patients (16 elbows) with the O-K procedure. All patients were reviewed after a mean follow-up of twenty months (range 3-64 months). The purpose of this study was to evaluate the results of the O-K procedure. Assessment included evaluation of elbow pain, range of motion and the Mayo elbow Performance Index (MPI). The mean arc of motion improved from 94 degrees (range, 65 degrees-120 degrees) preoperatively to 114 degrees (range 93 degrees-128 degrees) at the time of follow-up (p < 0.01). At the latest follow-up evaluation the total MPI improved from 63 (range, 50-75) to 88 (range, 45-100) (p < 0.01), eight elbows (50%) had no pain, seven (43.8%) mild pain and one (6.2%) moderate pain. The result was excellent for 8 elbows (50%), good for 6 (37.5%), fair for one (6.2%) and poor for one (6.2%). Patients' satisfaction showed that thirteen (86.6%) were much better or better and two (13.3%) were not improved compared with the preoperative situation.     

Trends in the incidence of treated end-stage renal failure in The Netherlands: hope for the future?

This study shows that in The Netherlands there is a changing pattern in incidence of renal replacement therapy over time when adjusted for age and sex. There has been an almost linear increase in crude incidence rates during four decades. However, unlike the situation elsewhere in the Western world, a flattening of the curve has been observed during the last six years. The growing incidence of end-stage renal disease (ESRD) due to especially hypertensive renovascular disease and diabetes mellitus type 2 has been neutralized by a decrease in ESRD due to glomerulonephritis, urologic interstitial nephritis, and diabetes mellitus type 1. The latter observations suggest that renoprotective interventions can be successful on a population level, and thus, provide hope for the future. To battle the increase in ESRD due to atherosclerosis and diabetes mellitus type 2-related renal disease, it is important that screening programs are being developed that enable us to identify in an early phase patients at risk for development of ESRD who may benefit from preventive strategies. Population screening for albuminuria will be helpful in this respect.     

Glucose regulates the expression of the farnesoid X receptor in liver

An increased prevalence of hypertriglyceridemia and gallbladder disease occurs in patients with diabetes or insulin resistance. Hypertriglyceridemia is positively associated to gall bladder disease risk. The farnesoid X receptor (FXR) is a bile acid-activated nuclear receptor that plays a key role in bile acid and triglyceride homeostasis. The mechanisms controlling FXR gene expression are poorly understood. This study evaluated whether FXR gene expression is regulated by alterations in glucose homeostasis. FXR expression was decreased in livers of streptozotocin-induced diabetic rats and normalized upon insulin supplementation. Concomitantly with diabetes progression, FXR expression also decreased in aging diabetic Zucker rats. In primary rat hepatocytes, D-glucose increased FXR mRNA in a dose- and time-dependent manner, whereas insulin counteracted this effect. Addition of xylitol, a precursor of xylulose-5-phosphate, to primary rat hepatocytes increased FXR expression to a comparable level as D-glucose. Finally, expression of the FXR target genes, SHP and apolipoprotein C-III, were additively regulated by D-glucose and FXR ligands. This study demonstrates that FXR is decreased in animal models of diabetes. In addition, FXR is regulated by glucose likely via the pentose phosphate pathway. Dysregulation of FXR expression may contribute to alterations in lipid and bile acid metabolism in patients with diabetes or insulin resistance.     

Tensionless Spigelian herniorrhaphy using a bilayered prosthetic patch: historical, anatomical, diagnostic, and operative perspectives

Spigelian hernias, which represent <2% of all hernias of the vellum abdominis (abdominal wall) anterior, can be a diagnostic challenge for clinicians. Noninvasive imaging techniques, including ultrasonography (US) and computerized axial tomography (CAT), substantially complement clinical inferences based on interrogation and physical examination. Successful definitive care mandates comprehension of the regional, topographical, and visceral anatomy in axial, coronal, and transverse planes. Reported herein is the successful use of a bilayered prosthetic patch, advantageous because of its unimodular and biplanar configuration, to perform a tensionless herniorrhaphy.     

Impact of Carotid Artery Angioplasty and Stenting on Management of Recurrent Carotid Artery Stenosis

Citing the higher perioperative risk of redo carotid surgery, balloon angioplasty and stenting of the carotid artery (CAS) has been advocated for recurrent carotid stenosis (RCS). To examine the impact of CAS on the management and outcome of recurrent stenosis, a retrospective review of a prospectively compiled database was performed. From a registry of patients treated for carotid disease, 105 procedures were performed from 1992 to 2002 for RCS. For comparison, two study groups were examined. Time I consisted of 77 reoperations performed through 1998, before CAS was introduced at our institution. Time II included 12 reoperations and 16 CAS procedures performed for RCS from 1999 through 2002. Using perioperative stroke as a measure of outcome, the results for time II were poorer than for time I (7.2% vs. 5.2%, p = NS). Overall, the risk of perioperative stroke was the same for reoperation (5/89) and CAS (1/16) (5.6% vs. 6.3%, p = NS). Although not statistically significant, there was a trend toward a higher risk of perioperative stroke for patients treated with reoperation during the latter time period (8.3% vs. 5.2%, p = NS). This probably relates to the finding that during time II, CAS was most likely to be used in asymptomatic patients (68.6% vs. 41.7%, p = NS) with early (<3 years) RCS (87.5% vs. 41.7%, p = 0.01). No patient with asymptomatic, early RCS had a perioperative stroke with either surgery or CAS (0/35 cases, 0%). The presence of preoperative neurologic symptoms was significantly predictive of a perioperative stroke among all procedures performed for RCS (13.6% vs. 0%, p = 0.004). Contrary to suggestions that CAS might improve the management of RCS, a review of our data shows the overall risk of periprocedural stroke to be no better since CAS has become available. The bias for using CAS for asymptomatic myointimal hyperplastic lesions, and reoperation for frequently symptomatic late recurrent atherosclerotic disease, makes direct comparisons of the two techniques for treating RCS difficult. It is expected that the overall risk for redo carotid surgery will increase, as fewer low-risk patients will be receiving open procedures. However, the increased risk among symptomatic patients undergoing reoperation suggests that endovascular techniques should be investigated among this group of cases as well.Presented at the Twenty-eighth Annual Meeting of the Peripheral Vascular Surgery Society, Chicago, IL, June 7, 2003.