Hepatic amyloidosis increases liver stiffness measured by transient elastography.

Liver stiffness values in transient elastography (TE) have to be interpreted with caution. Steatosis, congestion, acute inflammation and extrahepatic cholestasis can indeed influence measurements. Obtained stiffness values in the cirrhotic range can also be present in the absence of fibrosis as in hepatic amyloidosis. Here we report two cases of systemic amyloidosis with hepatic involvement where high stiffness values were measured at TE. In fact, deposits of amyloid may increase the rigidity of the liver parenchyma resulting in higher liver stiffness values. Therefore, results of TE should always be interpreted in their clinical context and if inconsistent, the performance of a liver biopsy might be necessary.

Insuffisance rénate chronique: attitudes et pratiques de dépistages en l'absence d'etudes randomisées. [Chronic kidney disease: screening attitude and practice in the absence of randomized controlled trials].

Au vu de l'augmentation de la prévalence de l'insuffisance rénale chronique (IRC), une détection précoce a été proposée. Certaines organisations de santé proposent des mesures de détection précoce (par exemple : taux de filtration glomérulaire). L'efficacité du dépistage de l'IRC n'est cependant pas connue puisqu'aucune étude randomisée contrôlée n'a été conduite. Si le test de dépistage de l'IRC est simple et peu onéreux, un dépistage n'est justifié que s'il améliore le pronostic par rapport à l'absence de dépistage avec un rapport risques-bénéfices favorable et un rapport coût-efficacité acceptable. Sur la base d'études observationnelles et de modèles de rapport coût-efficacité, le dépistage de l'IRC doit être proposé chez les patients hypertendus et/ou diabétiques mais pas dans la population générale. [Abstract] Given the increasing prevalence of chronic kidney disease (CKD), early detection has been proposed. Some organizations recommend CKD screening. Yet, the efficacy of CKD screening is unknown given the absence of randomized controlled trial conducted so far. While CKD screening tests (e.g., glomerular filtration rate) are simple and inexpensive, CKD screening can only be justified if it reduces CKD-related mortality and/or CKD-related morbidity compared to no screening. In addition, CKD screening must provide more benefits than risks to the participants and must be cost-effective. Based on observational studies and cost-effectiveness models, CKD screening has to be proposed to high risk population (patients with hypertension and/or diabetes) but not to the general population.

Amyloïdose rénale [Renal amyloidosis].

Amyloidosis is defined as the extracellular deposition of proteins that have the capacity to form beta-pleated sheets and become insoluble. More than 17 types of amyloidosis have been described. Systemic light chain amyloid (AL) and AA amyloid (secondary to chronic inflammatory process) are by far the most frequent forms of amyloidosis. In these systemic forms, organs involved are the kidneys, the heart and the gastrointestinal tract in AL amyloidosis. The diagnostic can be established only by tissue biopsy. Treatment of primary amyloidosis (AL) aims at suppressing the responsible clone whereas treatment of secondary amyloidosis relies on controlling the underlying inflammatory process. Prognosis is globally poor and depends on the extend of organs involvement particularly cardiac and renal. The prognosis is even worse in patients requiring dialysis.

Epibulbar osseous choristoma: a clinicopathological case series and review of the literature [Epibulbäre knöcherne Choristome: eine klinisch-pathologische Fallserie und Überblick der Literatur]

BACKGROUND: Choristomas are benign, congenital tumours composed of normal tissue in an abnormal location. Osseous choristomas represent the rarest form of epibulbar choristomas, with now 65 cases reported in the literature. We did a retrospective clinicopathological study of all patients with epibulbar osseous choristoma observed at our institution since 1982 and updated the last review of the literature. HISTORY AND SIGNS: Three Caucasian male patients, aged between 3 months and 11 years, were identified. All osseous choristomas were located under the superotemporal bulbar conjunctiva of the right eye. THERAPY AND OUTCOME: All lesions were managed with surgical excision. Histopathology revealed the presence of lamellar bone in all cases, one of which was associated with a dermolipoma. CONCLUSIONS: We report a small rare case series of 3 epibulbar osseous choristomas and did a review of the literature. In one patient, the osteoma was associated with a dermolipoma, corresponding to the fourth reported complex choristoma of this type, in an otherwise normal eye, in the literature.

Congenital diaphragmatic hernia: current status and review of the literature.

Treatment of congenital diaphragmatic hernia (CDH) challenges obstetricians, pediatric surgeons, and neonatologists. Persistent pulmonary hypertension (PPHT) associated with lung hypoplasia in CDH leads to a high mortality rate at birth. PPHT is principally due to an increased muscularization of the arterioles. Management of CDH has been greatly improved by the introduction of prenatal surgical intervention with tracheal obstruction (TO) and by more appropriate postnatal care. TO appears to accelerate fetal lung growth and to increase the number of capillary vessels and alveoli. Improvement of postnatal care over the last years is mainly due to the avoidance of lung injury by applying low peak inflation pressure during ventilation. The benefits of other drugs or technical improvements such as the use of inhaled nitric oxide or extracorporeal membrane oxygenation (ECMO) are still being debated and no single strategy is accepted worldwide. Despite intensive clinical and experimental research, the treatment of newborn with CDH remains difficult.

Performance of the 47-Kilodalton Membrane Protein versus DNA Polymerase I Genes for Detection of Treponema pallidum by PCR in Ulcers.

Treponema pallidum PCR (Tp-PCR) is a direct diagnostic method for primary and secondary syphilis, but there is no recommendation regarding the best choice of target gene. In this study, we sequentially tested 272 specimens from patients with sexually transmitted ulcers using Tp-PCR targeting the tpp47 and then polA genes. The two methods showed similar accuracies and an almost-perfect agreement.

Lésions cutanées et infiltrats pulmonaires d'évolution défavorable sans cause infectieuse [Progressive cutaneous and pulmonary lesions without infectious etiology: two cases reports of sweet syndrome with pulmonary involvement].

Sweet syndrome is a non infectious febrile disease with a neutrophilic infiltrate of dermis. Extracutaneous involvement can occur. We report two cases of Sweet syndrome with cutaneous and pulmonary involvement and give a short review of the literature of pulmonary involvement in Sweet syndrome.

Calprotectine fécale: outil diagnostique dans les maladies inflammatoires chroniques de l'intestin [Fecal calprotectin: a diagnostic tool for inflammatory bowel disease].

Fecal calprotectin (FC) is a valid biomarker to discriminate with a good sensitivity and specificity the presence of mucosal lesions of the gastrointestinal tube (e.g. ulcers in the context of inflammatory bowel disease (IBD)) from functional disorders (e.g. irritable bowel syndrome). FC is not specific for IBD and can be elevated also in gastrointestinal infections, ischemic colitis or neoplasia. An elevated FC should stimulate further investigations, notably an endoscopic workup. The level of FC correlates with the endoscopic score in Crohn's disease and ulcerative colitis. The correlation of FC and the endoscopic severity is better than the one of CRP or blood leukocytes. Thus, FC can also be used in the follow-up of IBD patients.

Idiopathic sclerochoroidal calcification: new observations.

Two cases of idiopathic sclerochoroidal calcification are reported with follow-up of two and ten years. In addition we have reviewed 102 cases of choroidal osteoma, including six misleading case reports which actually described idiopathic sclerochoroidal calcification and not choroidal osteoma. Clinical manifestation and the angiographic features of idiopathic sclerochoroidal calcification are outlined. The differential diagnosis of intraocular deposition of calcium salts is discussed in detail.

Pure sensory neuropathy in patients with primary Sjogren's syndrome: clinical, immunological and electromyographic findings

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.

Evaluation of the caloric test by combining 3 response parameters.

The slow-phase velocity of nystagmus is one of the most sensitive parameters of vestibular function and is currently the standard for evaluating the caloric test. However, the assessment of this parameter requires recording the response by using nystagmography. The aim of this study was to evaluate whether frequency and duration of the caloric nystagmus, as measured by using a clinical test with Frenzel glasses, could predict the result of the recorded test. The retrospective analysis of 222 caloric test results recorded by means of electronystagmography has shown a good association between the 3 parameters for unilateral weakness. The asymmetry observed in the velocity can be predicted by a combination of frequency and duration. On the other hand, no relationship was observed between the parameters for directional preponderance. These results indicate that a clinical caloric test with frequency and duration as parameters can be used to predict the unilateral weakness, which would be obtained by use of nystagmography. We propose an evaluation of the caloric test on the basis of diagrams combining the 3 response parameters.

Cardiologie [Cardiology].

The present review provides a selected choice of clinical research in the field of interventional cardiology, electrophysiology and cardiac imaging. We also focused on the new guidelines published by the European society of cardiology in 2010 (revascularization, atrial fibrillation and device therapy in heart failure).

Laparoscopic surgery for coeliac artery compression syndrome: current management and technical aspects.

Objectives: The study aims to assess the feasibility and midterm outcome of trans-peritoneal laparoscopy for coeliac artery compression syndrome (CACS).Design: Retrospective chart review involving four European vascular surgery departments and two surgical teams.Materials and methods: charts for patients who underwent laparoscopy for symptomatic CACS between December 2003 and November 2009 were reviewed. Preoperative computed tomography (CT) angiography and postoperative duplex scan and/or CT angiography were performed.Results: Eleven consecutive patients (nine women) with a median age of 52 years (interquartile range: 42.5-59 years) underwent trans-peritoneal laparoscopy for CACS. All patients had a history of postprandial abdominal pain; weight loss exceeded 10% of the body mass in eight cases. Preoperative CT angiography revealed coeliac trunk stenosis >70% in all cases. One patient had additional aortitis and inferior mesenteric artery occlusion, while another patient presented with an occluded superior mesenteric artery. Two conversions occurred (one difficult dissection and one aorto-hepatic bypass needed for incomplete release of CACS). The median blood loss was 195 ml (range: 50-900 ml) and median operative time was 80 min (interquartile range: 65-162.5 years). Symptoms improved immediately in 10/11 patients (no residual stenosis) while one remained unchanged despite a residual stenosis treated by a percutaneous angioplasty. Symptoms reappeared in one patient due to coeliac axis occlusion. The mean follow-up period was 35 +/- 23 months (range: 12-78 months).Conclusion: Our study demonstrates that trans-peritoneal laparoscopy for treating median arcuate ligament syndrome is safe and feasible. Additional patients and a longer follow-up are needed for long-term assessment of this laparoscopic technique. (C) 2011 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

Aerosol immunization with NYVAC and MVA vectored vaccines is safe, simple, and immunogenic.

Each year, approximately five million people die worldwide from putatively vaccine-preventable mucosally transmitted diseases. With respect to mass vaccination campaigns, one strategy to cope with this formidable challenge is aerosol vaccine delivery, which offers potential safety, logistical, and cost-saving advantages over traditional vaccination routes. Additionally, aerosol vaccination may elicit pivotal mucosal immune responses that could contain or eliminate mucosally transmitted pathogens in a preventative or therapeutic vaccine context. In this current preclinical non-human primate investigation, we demonstrate the feasibility of aerosol vaccination with the recombinant poxvirus-based vaccine vectors NYVAC and MVA. Real-time in vivo scintigraphy experiments with radiolabeled, aerosol-administered NYVAC-C (Clade C, HIV-1 vaccine) and MVA-HPV vaccines revealed consistent mucosal delivery to the respiratory tract. Furthermore, aerosol delivery of the vaccines was safe, inducing no vaccine-associated pathology, in particular in the brain and lungs, and was immunogenic. Administration of a DNA-C/NYVAC-C prime/boost regime resulted in both systemic and anal-genital HIV-specific immune responses that were still detectable 5 months after immunization. Thus, aerosol vaccination with NYVAC and MVA vectored vaccines constitutes a tool for large-scale vaccine efforts against mucosally transmitted pathogens.

Improvement of cardiovascular risk prediction: time to review current knowledge, debates, and fundamentals on how to assess test characteristics.

Cardiovascular risk assessment might be improved with the addition of emerging, new tests derived from atherosclerosis imaging, laboratory tests or functional tests. This article reviews relative risk, odds ratios, receiver-operating curves, posttest risk calculations based on likelihood ratios, the net reclassification improvement and integrated discrimination. This serves to determine whether a new test has an added clinical value on top of conventional risk testing and how this can be verified statistically. Two clinically meaningful examples serve to illustrate novel approaches. This work serves as a review and basic work for the development of new guidelines on cardiovascular risk prediction, taking into account emerging tests, to be proposed by members of the 'Taskforce on Vascular Risk Prediction' under the auspices of the Working Group 'Swiss Atherosclerosis' of the Swiss Society of Cardiology in the future.