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Selected AbstractsDopaminergic deficit is not the rule in orthostatic tremor,MOVEMENT DISORDERS, Issue 12 2008Jean-Marc Trocello MD Abstract Involvement of the dopaminergic system in orthostatic tremor is controversial. The aim of this study was to detect possible dopaminergic denervation in primary orthostatic tremor (OT). Twelve consecutive patients with a firm diagnosis of primary orthostatic tremor were compared with age-matched normal controls. All the patients had a neurological examination, surface polymyography, and quantification of striatal dopamine transporters with 123I-FP-CIT SPECT imaging. There was no significant difference in 123I-FP-CIT SPECT findings between controls and patients with OT. Longstanding primary orthostatic tremor is not necessarily associated with 123I-FP-CIT SPECT abnormalities, as 8 of our patients had more than a 10-year history of OT. Primary orthostatic tremor without dopaminergic denervation remains a valid entity, although representing only a subtype of high-frequency OT. A new role may emerge for 123I-FP-CIT SPECT in distinguishing between patients whose symptoms will be restricted to OT throughout the disease course and patients at an increased risk of developing PD. © 2008 Movement Disorder Society [source] Histological diagnosis of mediastinal lymph node metastases from renal cell carcinoma by endobronchial ultrasound-guided transbronchial needle aspirationRESPIROLOGY, Issue 2 2007Takahiro NAKAJIMA Abstract: Evaluation of mediastinal lymphadenopathy in patients with an intrathoracic nodule post malignancy is crucial for the determination of further treatment. Different radiological modalities are available for the detection of mediastinal lymph node metastases such as multidetector helical CT, PET-scan and PET-CT. However, tissue sampling is required for a firm diagnosis. A minimally invasive method of tissue sampling of mediastinal and hilar lymph nodes using direct real-time endobronchial ultrasound-guided transbronchial needle aspiration has been reported. This method is appropriate not only for cytodiagnosis but also for histological diagnosis. This current study reports a case of mediastinal lymph node metastases from renal cell carcinoma successfully diagnosed histologically by endobronchial ultrasound-guided transbronchial needle aspiration. [source] Anosacral cutaneous amyloidosis: a study of 10 Chinese casesBRITISH JOURNAL OF DERMATOLOGY, Issue 6 2000W-J. Wang Background,Primary cutaneous amyloidoses are rare in Western countries, but are relatively common in Taiwan. Anosacral cutaneous amyloidosis is a rare type of primary cutaneous amyloidoses, first reported in Japanese patients. Patients/methods,In the present study, we investigated the age of onset, sites of involvement, associated systemic diseases, and histopathological findings in 10 cases of anosacral cutaneous amyloidosis seen during the past 27 years. Results,In previous reports the aetiology of anosacral cutaneous amyloidosis was thought to be a senile change, but half of our patients developed the disease before the age of 60 years. Based on our histopathological findings, apoptosis may be the initial event causing amyloid deposition, although the precise mechanism causing apoptosis needs further investigation. Three patients were found to have diabetes mellitus, but any relationship to anosacral cutaneous amyloidosis is unclear. Conclusions,No cases of this cutaneous disorder have been reported in the Western literature; there seems to be a racial difference accounting for the disease, although the precise factor is not clarified yet. The disease could easily be misdiagnosed as lichen simplex chronicus, postinflammatory hyperpigmentation or tinea cruris; therefore, a thorough history, a careful physical examination and a skin biopsy is needed to establish a firm diagnosis. [source] Management of uveal tumoursACTA OPHTHALMOLOGICA, Issue 2009B DAMATO Purpose The purpose of this presentation is to describe the management of uveal melanomas and the other most common uveal tumours. Methods Choroidal melanomas are treated with plaque radiotherapy if possible, with proton beam radiotherapy, stereotactic radiotherapy, trans-scleral local resection, trans-retinal endoresection, phototherapy and enucleation being reserved for patients who cannot be managed with a plaque. Increasingly, tumour biopsy is performed for histological grading of malignancy and for cytogenetic studies aimed at determining the genomic tumour type so that risk of metastatic disease can be determined. Choroidal metastases usually respond to external beam radiotherapy. Biopsy may be needed to confirm the diagnosis. Choroidal haemangiomas are treated by photodynamic therapy, with good response in most patients. Results In the large majority of patients, it is possible to conserve the eye and vision. Patients need life-long follow-up in case tumour recurrence occurs. After radiotherapy of choroidal and ciliary body melanomas some patients develop exudative and neovascular complications needing treatment. Conclusion Successful management of uveal tumours is based on a firm diagnosis, accurate staging of disease, reliable prognostication and adequate aftercare. [source] Oral challenges are needed in the diagnosis of ,-lactam hypersensitivityCLINICAL & EXPERIMENTAL ALLERGY, Issue 1 2008P. J. Bousquet Summary Background ,-lactams continue to remain the most commonly involved drug family in allergic drug reactions. They are often essential and there is a cost-effective and favourable risk-benefit ratio for the exploration of all suspicions of ,-lactam allergy. A firm diagnosis is always based on skin tests and sometimes on provocation tests. Recommendations have been published by allergy societies and distinguished scientists but they are not always concordant and can lead to some confusion for the practicing allergologist. The situation has even worsened since the world wide withdrawal of these penicillin determinants and since the predominance of amoxicillin and cephalosporin prescriptions in most countries. Objective , Method In a recent article, it was stated that patients with a penicillin allergy history and negative skin tests to major and minor penicillin determinants are at a low risk of relapse (0,5%) when receiving a ,-lactam. In this paper, our Drug Allergy and Hypersensitivity Database, a cohort database, was used to demonstrate that this statement is false. Standardized European Network for Drug Allergy questionnaires, skin test and challenge procedures were followed. Results One-thousand two-hundred and eighteen subjects, 69.8% of female, 51.7% of atopics, were included. 21.1% had a true ,-lactam allergy confirmed by skin tests (178, 69.3%) or by drug provocation (79, 30.7%). 17.4% of the patients with negative skin tests to major and minor penicillin determinants were positive for a ,-lactam. Conclusion In the diagnosis of ,-lactams allergy, if all skin tests are negative, skin tests with other determinants and provocation tests under strict surveillance are mandatory. [source] Drug allergy claims in children: from self-reporting to confirmed diagnosisCLINICAL & EXPERIMENTAL ALLERGY, Issue 1 2008E. Rebelo Gomes Summary Background Poorly documented self-reported drug allergy (DAll) is a frequent problem in daily clinical practice and has a considerable impact on prescription choices. The diagnostic work-up of drug hypersensitivity (DHs) allows a better classification of the reactions and provides patients with more reliable information and recommendations for future treatments. Objective To assess the prevalence of self-reported adverse drug reactions (ADRs) and DAll in a paediatric population and to investigate children reporting suspected DAll in order to achieve a firm diagnosis. Design The first phase was based on a cross-sectional survey assessing the life occurrence of ADRs and self-reported DAll carried out at the outpatient clinic of a paediatric hospital. The second phase was based on the diagnostic work-up in children with parent-reported DAll, including detailed anamnesis and in vitro and in vivo investigations (skin and provocation tests). Participants One thousand four hundred and twenty-six parents responded to the initial survey. Sixty of the 67 patients with reported DAll were evaluated at the allergy clinic. Results The prevalences of self-reported ADRs and DAll were 10.2% and 6.0%, respectively. Most of the suspected allergic reactions were non-immediate cutaneous events attributable to ,-lactam antibiotics and occurred in very young children. Thirty-nine of the 60 patients consulting for evaluation had a plausible clinical history and were recommended further investigation. DHs was diagnosed in three children only, based on positive responses in skin (n=1) and oral provocation (n=2) tests. Conclusion ADRs are frequently reported in children, and many children are classified as having a DAll. After complete evaluation, only a few of these reactions can be attributed to DHs and DAll. Most of the patients (94% in this study) could actually tolerate the initially suspected drug. [source] |