Search results for "rare"

Development and implementation of the AIDA International Registry for patients with Behçet's disease

2022

AbstractPurpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been rea…

Primary umbilical endometrioma: Analyzing the pathogenesis of endometriosis from an unusual localization

2015

Abstract Objective This report presents a rare case of symptomatic primary umbilical endometriosis and reviews the literature on the topic with the aim to clarify some questions on the origin of endometriosis. Case report A 33-year-old woman with cyclic umbilical bleeding was found to have umbilical endometriosis. She had no history of pelvic or abdominal surgery. There was no past history of endometriosis or endometriosis-associated symptoms. An omphalectomy was performed after explorative laparoscopy to carefully inspect the abdominopelvic cavity and assess any coexisting pelvic endometriotic lesions. Histological examination confirmed the diagnosis of umbilical endometriosis. Conclusion …

Hereditary angioedema in children and adolescents - A consensus update on therapeutic strategies for German-speaking countries.

2020

Background/methods At a consensus meeting in August 2018, pediatricians and dermatologists from German-speaking countries discussed the therapeutic strategy for the treatment of pediatric patients with type I and II hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) for Germany, Austria, and Switzerland, taking into account the current marketing approval status. HAE-C1-INH is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible and an optimal management of the disease are important to avoid ineffective therapies and to properly treat swelling attacks. This art…

Doppler and B-mode ultrasound for avascular nephrotomy.

1983

AbstractDoppler sonography for intraoperative localization of the intrarenal arteries combined with B-scan sonography for intraoperative visualization of stones allows complete stone removal via small radial nephrotomies for which clamping of the renal artery is no longer necessary. Since September 1980 we used this technique on 35 patients with staghorn or recurrent calculi. The main advantages of this technique are exact and quick stone localization, minimal loss of renal function owing to preservation of the intrarenal vascular system, and no need for renal ischemia and cooling.

Carcinoma of the neovagina: case report and review of the literature.

2001

Abstract Background. Carcinoma of the vagina is a rare disease, and it is even more rare in the neovagina. Nevertheless, it has been well described. The aim of this report was to analyze the reported cases and to add observations concerning a risk profile for this rare occurrence of carcinoma. Case report. The 29-year-old patient's history included congenital absence of vagina as a result of Rokitansky–Kuster syndrome. In 1987, when the patient was 17 years old, a neovagina was constructed by dissection between the bladder and the rectum, according to the Warthon method, and the apex of the neovagina was covered with Dura-mater. In 1990 the patient underwent radiation treatment with brachyt…

Accuracy of ASTER Level-2 thermal-infrared Standard Products of an agricultural area in Spain

2007

Abstract The Advanced Spaceborne Thermal Emission and Reflection Radiometer (ASTER) collects five-channel thermal-infrared images that are calibrated, corrected for atmospheric effects, and then converted to land surface temperature and emissivity products by the ASTER Temperature/Emissivity Separation (TES) algorithm. TES scales low- and high-contrast surfaces differently, and has been validated over water (low contrast) and rock (high contrast). Performance of TES over agricultural areas, however, has not been evaluated specifically. To address this issue, field measurements of “ground truth” were made over bare soil in addition to green grass, alfalfa and corn, at an agricultural researc…

Characterization of oligomeric compounds in secondary organic aerosol using liquid chromatography coupled to electrospray ionization Fourier transfor…

2009

The components of secondary organic aerosols (SOAs) generated from the gas-phase ozonolysis of two C(10)H(16)-terpenes (alpha-pinene; sabinene) and a cyclic C(6)H(10) alkene (cyclohexene) were characterized by the use of a Fourier transform ion cyclotron mass spectrometer equipped with an electrospray ionization source operated in the negative ion mode. Reversed-phase high-performance liquid chromatography was used to achieve chromatographic separation of highly oxidized organic compounds. In addition to the well-known group of low molecular weight oxidation products (monomers; e.g. dicarboxylic acids), higher molecular weight compounds (dimers) were also detected and their exact elemental …

2020

Motor control is associated with suppression of oscillatory activity in alpha (8–12 Hz) and beta (12–30 Hz) ranges and elevation of oxygenated hemoglobin levels in motor-cortical areas. Aging leads to changes in oscillatory and hemodynamic brain activity and impairments in motor control. However, the relationship between age-related changes in motor control and brain activity is not yet fully understood. Therefore, this study aimed to investigate age-related and task-complexity-related changes in grip force control and the underlying oscillatory and hemodynamic activity. Sixteen younger [age (mean ± SD) = 25.4 ± 1.9, 20–30 years] and 16 older (age = 56.7 ± 4.7, 50–70 years) healthy men were…

Synthesis and evaluation of zirconium-89 labelled and long-lived GLP-1 receptor agonists for PET imaging

2020

Contains fulltext : 220838.pdf (Publisher’s version ) (Open Access) INTRODUCTION: Lately, zirconium-89 has shown great promise as a radionuclide for PET applications of long circulating biomolecules. Here, the design and synthesis of protracted and long-lived GLP-1 receptor agonists conjugated to desferrioxamine and labelled with zirconium-89 is presented with the purpose of studying their in vivo distribution by PET imaging. The labelled conjugates were evaluated and compared to a non-labelled GLP-1 receptor agonist in both in vitro and in vivo assays to certify that the modification did not significantly alter the peptides' structure or function. Finally, the zirconium-89 labelled peptide…

Anomalous temperature dependence of the IR spectrum of polyalanine

1997

Abstract We have studied the temperature dependence of the infrared spectra of acetanilide (ACN), tryptophan–(alanine) 15 , and tyrosine–(alanine) 15 . No sidebands of the amide-I vibration were observed in the polypeptides, but two anomalous sidebands of the NH stretch with a similar temperature dependence as that of the anomalous amide-I vibrational mode at 1650 cm −1 of crystalline ACN were detected. Fermi resonance combined with the appearance of a red-shifted sideband of NH stretch through coupling to lattice modes seems to explain this band structure. Observations are indicative of excitons that may occur in polypeptides as well as in single crystals of ACN.