IGLIAS: A new experimental set-up for low temperature irradiation studies at large irradiation facilities.

We designed and built a mobile experimental set-up for studying the interaction of ion beams with solid samples in a wide temperature range from 9 to 300 K. It is either possible to mount up to three samples prepared ex situ or to prepare samples by condensation of molecules from gases or vapours onto IR or Visible-ultraviolet (Vis-UV) transparent windows. The physico-chemical evolution during irradiation can be followed in situ with different analysis techniques including Fourier transform infrared spectroscopy, Vis-UV, and quadrupole mass spectrometry.

Advances in the surgical management of nephrolithiasis.

The surgical management of urinary calculus disease has undergone a dramatic evolution over the past 2 decades. Twenty years ago, open surgical procedures for urinary calculi were some of the most frequently performed urologic procedures. Since then, however, stone management has been at the forefront of "minimally invasive" intervention. Specifically, the initiation and refinement of percutaneous and ureteroscopic access to the upper tracts, along with the rapid and nearly simultaneous development of both extracorporeal and intracorporeal lithotripsy techniques, has limited the role of open surgery to less than 1% of patients undergoing intervention for their stone disease. This manuscript will review the current indications for the surgical management of urinary calculi, the basic physics of the most frequently utilized vehicles for both extracorporeal and intracorporeal lithotripsy, and the respective roles of extracorporeal and intracorporeal lithotripsy with percutaneous or ureteroscopic access and open surgery. In addition, the results and complications associated with each of these forms of intervention will be reviewed. Finally, a discussion of specific clinical challenges to the urologist will be presented.

Ureteroscopic management of lower-pole renal calculi: technique of calculus displacement.

PURPOSE: We review our technique of ureteroscopic management of lower pole renal calculi with Nitinol basket displacement and holmium laser stone fragmentation. METHODS: Lower pole calculi are identified with a 7.5F flexible ureteroscope. In patients in whom the laser fiber reduces ureteroscopic deflection, precluding reentry into the lower pole, a Nitinol basket or grasper is used to displace the calculi into an upper pole calix for easier fragmentation. RESULTS: The Nitinol device can be passed into the lower pole through the fully deflected ureteroscope without any loss of deflection. Irrigation is significantly reduced by the basket, but this factor does not impede stone retrieval. CONCLUSIONS: Ureteroscopic management of lower pole stones is a reasonable alternative to SWL or percutaneous nephrolithotomy in low-volume stone disease. If the stone cannot be fragmented in situ, Nitinol basket capture through a fully deflected ureteroscope into a less dependent position facilitates stone fragmentation.

Simplified ureteral stent placement with the assistance of a ureteral access sheath.

PURPOSE: When using a ureteral access sheath following a ureteroscopic procedure, placement of an internal ureteral stent can be simplified by inserting the stent through the sheath without the need to reinsert the cystoscope. MATERIALS AND METHODS: An indwelling ureteral stent with the pull string attached is inserted over the guide wire into the access sheath followed by the pusher. The guide wire is partially withdrawn allowing the stent to form a coil in the renal pelvis, using the pull string to adjust the stent position. The fluoroscopy unit is then focused onto the bladder and the guide wire is slowly withdrawn until its tip is at the level of pubic symphysis. The pusher and guide wire are then removed and the pull string is cut at the urethral meatus. RESULTS: Among 71 cases studied 60 required ureteral stent placement. In 43 of the 60 cases (72%) the ureteral access sheath greatly facilitated ureteroscopy, and a stent was placed through the access sheath in 34 (79%). Stent placement through the access sheath was successful in all cases, with an average time saving of 2.3 minutes per case, compared to placing the stent by reinserting a cystoscope. CONCLUSIONS: If an access sheath has already been placed during a ureteroscopic procedure and stent insertion is deemed necessary, the stent can be easily placed through the access sheath under fluoroscopic guidance without the need to reinsert the cystoscope. Our experience suggests that all urologists who routinely perform ureteroscopic procedures can easily master this timesaving technique.

[Neural determination in Xenopus laevis embryos: control of early neural gene expression by calcium]. / La détermination neurale dans l'embryon de Xenopus laevis: contrôle de l'expression des gènes neuraux précoces par le calcium.

In amphibian embryos the central nervous system derives from the dorsal region of the ectoderm. Molecular studies led to the formulation of the "neural default model" in which neural development is under the inhibitory control of members of the BMP family. These growth factors also act as epidermis inducers. The neural fate is revealed by factors secreted by the Spemann Organizer such as noggin, chordin, follistatin, Xnr3 and cerberus which act by blocking BMP signalling. We propose a new model for neural cell determination in which a signalling pathway controlled by an increase in intracellular calcium suppresses the epidermis fate and activates the neural fate instead. This increase in calcium is due to an influx through calcium channels of the L-type, expressed in ectodermal cells during gastrulation. The possible involvement of a calcium-dependent phosphatase (calcineurin) to inhibit the epidermis fate and a calcium-calmodulin kinase (CaMkinase II) which activates the neural fate is discussed.

Amyloid arthropathy revealed by RS3PE syndrome.

Amyloid arthropathy is a form of primary AL amyloidosis with a monoclonal component in the blood and/or urine, and RS3PE syndrome is acute edematous polysynovitis in subjects older than 60 years. A 74-year-old man was diagnosed with both disorders. He was admitted for benign acute polyarthritis of the hands and feet and reported carpal tunnel symptoms predominating on the right. A synovial biopsy at the right wrist disclosed deposits that stained with Congo red even after potassium permanganate treatment (positive Wright's test). Articular AL amyloidosis was diagnosed. The symptoms resolved under glucocorticoid therapy alone, casting some doubt on their relationship with the amyloidosis. Roentgenograms showed geodes, a feature not present in RS3PE. Whether RS3PE may be among the possible presentations of articular amyloidosis is discussed.

Pneumococcal polyarticular septic arthritis in a patient with rheumatoid arthritis.

Rheumatoid arthritis is the most commonly reported host-related risk factor for septic arthritis. This risk is highest in severe, seropositive, long-standing (mean, 10 years) rheumatoid arthritis responsible for extraarticular symptoms and treated with systemic glucocorticoids. The clinical presentation of the joint infection is often atypical, leading to diagnostic wanderings. In 25% of cases, the infection is polyarticular, with 3.5 involved joints on average. Staphylococcus aureus is the most common causative organism. Streptococcus pneumoniae causes 5% of all cases of septic arthritis and is more often responsible for polyarticular infections than other organisms. Polyarticular septic arthritis carries a poor prognosis, with a mortality rate of 50% in rheumatoid arthritis patients. Despite its low incidence, polyarticular septic arthritis should be routinely considered in the differential diagnosis of rheumatoid flares. We report a case of pneumococcal septic arthritis involving five joints in a patient with known rheumatoid arthritis. Three other cases with involvement of more than four joints have been published.

HLA-DRB1 alleles and shared amino acid sequences in disease susceptibility and severity in patients from eastern France with rheumatoid arthritis.

OBJECTIVE: To examine the effects of HLA-DRB1 alleles and amino acid sequences that carry the shared epitope (SE) upon rheumatoid arthritis (RA) susceptibility and disease severity in patients from Eastern France. METHODS: HLA-DRB1 alleles were determined in 120 patients and 104 healthy controls by polymerase chain reaction/sequence specific oligonucleotide probes. Subtyping of DRB1*01 and *04 were performed using sequence specific primers. Patients were retrospectively evaluated for disease duration, age at disease onset, presence of rheumatoid factors, subcutaneous nodules, vasculitis and other extraarticular diseases, for the need for arthroplasty and immunosuppressive/immunoregulatory agents, and for radiographic damage. RESULTS: The prevalence of HLA-DRB1*04 was significantly higher in patients (46.6%) than in controls (17.3%) (Pcorr = 0.000003). HLA-DRB1*0101 and *0401 were the most prominently associated subtypes in patients with RA (33.3%, Pcorr = 0.011, and 28.3%, Pcorr = 0.00008, respectively). A significant fraction of patients (72.5%) expressed one or 2 copies of the SE (p < 0.0000001; OR 4.77, CI 2.61-8.78). The presence of double SE was associated with a higher risk of developing RA (OR 4.83, CI 1.91-12.71; p = 0.0001). No significant differences in the clinical records among patients expressing no RA linked alleles, one and 2 copies of the SE, were observed. However, analyzing the specific effect of each amino acid sequence, we observed a significant association of the QKRAA motif with vasculitis (p = 0.03) and history of joint replacement surgery (p = 0.05), suggesting a role for lysine in position 71 of the shared sequence. CONCLUSION: These findings differ from those of previous HLA-DRB1 allele studies in patients with RA from other regions of France. Thus, the heterogeneity in both the expression of DRB1 alleles and the association of these alleles with disease severity could be relevant within a country such as France.

Rheumatoid arthritis and multiple sclerosis in the same patient. Two case-reports.

We report on two new patients with both rheumatoid arthritis and multiple sclerosis. In one patient, the first manifestations of multiple sclerosis occurred eight years after onset of seronegative rheumatoid arthritis without extraarticular manifestations. The other patient had a 20-year history of multiple sclerosis when she developed seropositive, nodular rheumatoid arthritis. Neither patient had evidence of systemic lupus erythematosus. A lip biopsy was done in one patient, with normal results; the other patient was free of clinical symptoms of sicca syndrome and had a negative Schirmer's test. The paucity of similar cases in the literature is surprising since multiple sclerosis and rheumatoid arthritis are both autoimmune diseases and share many pathophysiologic and etiologic features. Although chance alone may explain the occurrence of both conditions in the same patient, the existence of shared etiologic factors should in theory increase the likelihood of the association.

Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.

Prospective study of the prevalence of bronchiectasis in rheumatoid arthritis using high-resolution computed tomography.

A prospective study of the prevalence of bronchiectasis in rheumatoid arthritis was conducted over an 18-month period in 46 patients (34 women and 12 men with a mean age of 60.1 years) meeting 1987 American College of Rheumatology criteria for rheumatoid arthritis. All patients underwent high-resolution computed tomography of the chest, whose results were confronted with a number of clinical, laboratory test and lung function testing parameters. Bronchiectasis or bronchiolectasis was found in 23 patients (50%) and was the most common abnormality detected by high-resolution computed tomography. When the four patients with isolated bronchiolectasis were excluded, the prevalence was 41%. Eighteen of the 23 patients had not been diagnosed with bronchiectasis before the study and 13 were free of respiratory symptoms. No significant differences were found between the 23 patients with and the 23 patients without bronchiectasis for age at onset or duration of the rheumatoid arthritis, extraarticular involvement, positive rheumatoid factors, bony erosions, use of corticosteroids or immunosuppressives, respiratory manifestations, smoking, or spirometry parameters. Patients without bronchiectasis were more likely to have impaired diffusion of carbon monoxide across the alveolar-capillary membrane. Among the patients with bronchiectasis, those with respiratory symptoms (n = 10) were more likely to have a history of lung disease and those without respiratory symptoms (n = 13) were more likely to have a diagnosis of bronchiectasis secondary to rheumatoid arthritis; no other differences were found between these two subgroups. Routine use of high-resolution computed tomography, a technique capable of demonstrating silent bronchiectasis, showed that bronchiectasis was the most common lung change in rheumatoid arthritis in our study. At the time of the study, there was no evidence that presence of bronchiectasis was associated with more severe joint or lung symptoms.

Soluble intercellular adhesion molecule 1 in spondylarthropathies.

The aim of the study was to evaluate the soluble form of intercellular adhesion molecule 1 (sICAM-1), a ligand of LFA-1, in the serum of patients with spondylarthropathies (SpA) and to look for a correlation with several inflammatory parameters. sICAM-1 levels were measured by ELISA in 25 SpA patients, 20 healthy controls and 20 patients with rheumatoid arthritis (RA). Results showed that sICAM-1 levels were increased (but not significantly) in SpA patients compared with controls; high levels (> 400 ng/ml) where found in 28% of SpA patients but in none of the RA or control groups. In SpA, correlations were found between sICAM-1 and erythrocyte sedimentation rate, C-reactive protein and interleukin 6, but not with tumour necrosis factor alapha or IgA. These correlations were absent in RA. In conclusion, these results suggest that sICAM-1 levels in SpA may reflect the acute phase of inflammation.