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Largest Series (largest + series)
Selected AbstractsOccupational immunologic contact urticaria from pine processionary caterpillar (Thaumetopoea pityocampa): experience in 30 casesCONTACT DERMATITIS, Issue 2 2004Jesús Vega Cutaneous lesions caused by the pine processionary caterpillar Thaumetopoea pityocampa (TP) are frequent in pinewood areas. In the present study, 30 patients diagnosed with occupational immunologic urticaria from this caterpillar were included. Immediate hypersensitivity was demonstrated by performing prick and IgE-immunoblotting tests. Workers were grouped according to their common tasks. Occupations at risk of exposure to TP were pine-cone collectors/woodcutters (14), farmers/stockbreeders (8), other forestry personnel (4), construction workers (2), residential gardeners (1) and entomologists (1). Besides contact urticaria, angioedema (60%), papular lesions of several days of evolution (30%) and anaphylactic reactions (40%) were also detected. The most frequently detected molecular weight bands by immunoblot were 15 (70%), 17 (57%) and 13 kDa (50%). The appearance of isolated bands corresponds with the least serious cases. Only 8 subjects had bands higher than 33 kDa, which was present in the 3 most severe cases of anaphylactic reactions. By presenting these cases, we wish to offer the largest series reported so far of occupational immunologic contact urticaria caused by TP. We include the first cases described in certain occupations, some of them not directly related to forestry work. Pine-cone or resin collectors, woodcutters, farmers and stockbreeders were the most frequently and severely affected workers. [source] Molecular and muscle pathology in a series of caveolinopathy patients,HUMAN MUTATION, Issue 1 2005Luigi Fulizio Abstract Mutations in the caveolin-3 gene (CAV3) cause limb girdle muscular dystrophy (LGMD) type 1C (LGMD1C) and other muscle phenotypes. We screened 663 patients with various phenotypes of unknown etiology, for caveolin-3 protein deficiency, and we identified eight unreported caveolin-deficient patients (from seven families) in whom four CAV3 mutations had been detected (two are unreported). Following our wide screening, we estimated that caveolinopathies are 1% of both unclassified LGMD and other phenotypes, and demonstrated that caveolin-3 protein deficiency is a highly sensitive and specific marker of primary caveolinopathy. This is the largest series of caveolinopathy families in whom the effect of gene mutations has been analyzed for protein level and phenotype. We showed that the same mutation could lead to heterogeneous clinical phenotypes and muscle histopathological changes. To study the role of the Golgi complex in the pathological pathway of misfolded caveolin-3 oligomers, we performed a histopathological study on muscle biopsies from caveolinopathy patients. We documented normal caveolin-3 immunolabeling at the plasmalemma in some regenerating fibers showing a proliferation of the Golgi complex. It is likely that caveolin-3 overexpression occurring in regenerating fibers (compared with caveolin-deficient adult fibers) may lead to an accumulation of misfolded oligomers in the Golgi and to its consequent proliferation. Hum Mutat 25:82,89, 2005. © 2004 Wiley-Liss, Inc. [source] Presentation and therapy of myelolipomaINTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2005ANDREAS MEYER Abstract, Background:, Adrenal myelolipoma is a rare and benign, hormonally inactive tumor frequently discovered incidentally. Because of the increasing rate of detection of adrenal myelolipoma, use of the correct diagnostic examination and treatment, with respect to surgical excision or regular controls, is continually gaining importance. We report herein on the largest series of surgically treated patients with adrenal myelolipoma from a single institute. Methods:, The clinical charts of 12 patients who underwent adrenal surgery for myelolipoma were reviewed. Follow up was carried out by means of re-examination or by personal contact with the primary physician. It was possible to perform a re-examination on eight of the 12 patients 6.9 years after the operation: one patient had died 5.2 years after surgery. Results:, Diagnosis of adrenal myelolipoma was made incidentally in seven patients, and during examination for reasons of unspecific abdominal or flank pain in five patients. A transabdominal approach was taken in five patients, a subcostal approach was taken in three patients and a translumbal approach was taken in four patients. At the follow up, all formerly symptomatic patients were free of symptoms. No recurrence could be seen; however, in one patient a contralateral adrenal myelolipoma had developed. Conclusions:, Symptomatic tumors, growing tumors or tumors larger than 10 cm should be excised surgically by means of an endoscopic or conventional approach, depending on the size. Surgical indication should not be liberalized by the introduction of more gentle operative techniques such as the translumbal or the endoscopic approach. A close follow up should be maintained in the case of patients free of symptoms, and for those with a definite diagnosis from imaging procedures. [source] Gastrointestinal stromal tumours: A clinico-radiologic review from a single centre in South IndiaJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 6 2009A Singh Summary Gastrointestinal stromal tumours (GISTs) are rare tumours but are the commonest mesenchymal neoplasms in the gastrointestinal tract. To our knowledge, there is no large case series in Asian countries in which a clinico-radiological descriptive analysis of these tumours has been carried out. In this retrospective study, we analysed our experience of 70 patients with histopathologically proven GISTs, who were presurgically investigated by using CT, and describe the demography, anatomical distribution, imaging features and clinical course of the GIST. We found an unusually large predominance of males in our study, stomach and small bowel appeared to have been involved similarly and small bowel tumours had a higher rate of metastases. We also highlight some unusual CT features of these tumours that we encountered during the study, such as the presence of metastatic lymphadenopathy and satellite nodules, relapse in appendices epiploicae of the bowel, metachronous liposarcoma, adrenal and lung metastases, multiplicity of lesions and aneurysmal dilatation of the bowel. Two of our patients also had multiple neurofibromas, whose association with GIST has been seen in earlier reports. To the best of our knowledge, this article presents one of the largest series of articles on GISTs, to date, in Asian countries. We conclude with a differential diagnosis of GIST, with salient features distinguishing each entity. [source] Relative frequency of intra-oral minor salivary gland tumors: a study of 380 cases from northern California and comparison to reports from other parts of the worldJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 4 2007Amos Buchner Background:, The relative frequency of individual intra-oral minor salivary gland tumors (IMSGT) is not well documented in the literature. The aim of this study was to determine the relative frequency and distribution of IMSGT in an oral pathology biopsy service and to compare the data with similar studies from different parts of the world. Methods:, Files from the Pacific Oral and Maxillofacial Pathology Laboratory of the University of the Pacific, San Francisco, California served as a source of material for this study. Files were systematically searched for all cases of IMSGT during a 20-year period. Tumors were classified according to the 2005 WHO classification of salivary gland tumors. Results:, IMSGT were identified in 380 (0.4%) cases of 92 860 accessed. This is the largest series of IMSGT from one source reported in recent years. Of the 380 tumors, 224 (59%) were benign and 156 (41%) were malignant. Of the benign tumors, pleomorphic adenoma (PA) was the most common (39.2%), followed by cystadenoma (6.3%), canalicular adenoma (6.1%), ductal papillomas (4.4%), basal cell adenoma (1.6%), and myoepithelioma (1.3%). Of the malignant tumors, mucoepidermoid carcinoma was the most common (21.8%), followed by polymorphous low-grade adenocarcinoma (7.1%), adenoid cystic carcinoma (6.3%), adenocarcinoma, not otherwise specified (NOS; 2.1%), acinic cell carcinoma (1.6%), clear cell carcinoma, NOS (1.0%), and carcinoma ex PA (0.5%). Conclusions:, Studies related to the relative frequency of individual IMSGTs from different parts of the world are difficult to compare because many studies are outdated, the number of cases is small, the list of tumors is limited, and new entities are not included. To determine the true relative frequency, more studies should be conducted, on a large number of cases from one source, by experienced pathologists in the field of salivary gland tumors. [source] Response to pegylated interferon plus ribavirin in HIV-infected patients with chronic hepatitis C due to genotype 4JOURNAL OF VIRAL HEPATITIS, Issue 10 2008L. Martín-Carbonero Summary., Hepatitis C virus (HCV) genotypes 1 and 4 respond less well to pegylated interferon (pegIFN) plus ribavirin (RBV) therapy. For this reason most studies merge these two genotypes when assessing virological response. However, in most trials the HCV genotype 4 population is rather small, and conclusions are mainly derived from what occurs in HCV-1 patients. All HCV-4 patients coinfected with HIV who received pegIFN plus RBV in two different multicentre studies, PRESCO and ROMANCE, conducted respectively in Spain and Italy, were retrospectively analyzed. Baseline plasma HCV-RNA, proportion of patients with HCV-RNA <10 IU,/,mL at week 4 (rapid virological response), and HCV-RNA declines >2 logs at week 12 (early virological response, EVR) were all assessed as predictors of sustained virological response (SVR). Overall, 75 patients (60 men) were evaluated. Median age was 40 years and median CD4 count 598 cells,/,mm3; 49% had plasma HIV-RNA <50 copies,,/,,mL; 71% had elevated liver enzymes and 31% had advanced liver fibrosis (Metavir F3,F4). Median serum HCV-RNA was 5.7 log IU,/,mL. Rapid virological response was attained by 10 (20%) patients and EVR by 26 (42%). Using intention-to-treat and on-treatment (OT) analyses, SVR was achieved by 21,/,75 (28%) and 21,/,62 (34%) of HCV-4 patients, respectively. In the multivariate analysis (OT), baseline HCV-RNA (OR 0.09 for every log increment; 95% CI: 0.01,0.7) and EVR (OR: 7.08; 95% CI: 1.8,27.2) were significantly and independently associated with SVR. This is the largest series of HIV-infected patients with chronic hepatitis C due to HCV-4 treated with pegIFN plus RBV examined so far and the results show that HCV-4 behaves similarly to HCV-1. Therefore, these patients should be considered as difficult to treat population. Baseline serum HCV-RNA and EVR are the best predictors of SVR in HCV-4,/,HIV-coinfected patients. [source] Childhood Psoriasis: A Clinical Review of 1262 CasesPEDIATRIC DERMATOLOGY, Issue 3 2001Anne Morris M.B., B.S. A total of 1262 patients seen consecutively in the dermatology department of the Royal Alexandra Hospital for Children, Sydney, Australia, between 1981 and 1995 are described and classified according to the pattern of psoriasis at the time of presentation. Additional information recorded included family history, facial involvement, and history of a psoriatic type of diaper rash in infancy. The ages of the children ranged from 1 month to 15 years. There was an equal gender distribution and a high rate of positive family history at 71%. Twenty-six percent of children had a history of a psoriatic diaper rash and facial involvement occurred in 38% of children. Plaque psoriasis was the most common type overall, affecting 430 patients (34%). Three hundred forty-five children were less than 2 years of age, and this is the largest series of children with psoriasis in this age group presented to date. An entity defined by us as psoriatic diaper rash with dissemination was the most common type of psoriasis in the less than 2-year age group, affecting 155 (45%) patients. This large series offers information on the manifestations of psoriasis in childhood, but is particularly useful in examining the previously less well-described infant age group. The classification used is proposed as a practical way to describe psoriasis in children, particularly with respect to future descriptive studies. [source] The presence of prostate cancer on saturation biopsy can be accurately predictedBJU INTERNATIONAL, Issue 5 2010Sascha A. Ahyai Study Type , Diagnostic (non-consecutive) Level of Evidence 3b OBJECTIVE To improve the ability of our previously reported saturation biopsy nomogram quantifying the risk of prostate cancer, as the use of office-based saturation biopsy has increased. PATIENTS AND METHODS Saturation biopsies of 540 men with one or more previously negative 6,12 core biopsies were used to develop a multivariable logistic regression model-based nomogram, predicting the probability of prostate cancer. Candidate predictors were used in their original or stratified format, and consisted of age, total prostate-specific antigen (PSA) level, percentage free PSA (%fPSA), gland volume, findings on a digital rectal examination, cumulative number of previous biopsy sessions, presence of high-grade prostatic intraepithelial neoplasia on any previous biopsy, and presence of atypical small acinar proliferation (ASAP) on any previous biopsy. Two hundred bootstraps re-samples were used to adjust for overfit bias. RESULTS Prostate cancer was diagnosed in 39.4% of saturation biopsies. Age, total PSA, %fPSA, gland volume, number of previous biopsies, and presence of ASAP at any previous biopsy were independent predictors for prostate cancer (all P < 0.05). The nomogram was 77.2% accurate and had a virtually perfect correlation between predicted and observed rates of prostate cancer. CONCLUSIONS We improved the accuracy of the saturation biopsy nomogram from 72% to 77%; it relies on three previously included variables, i.e. age, %fPSA and prostate volume, and on three previously excluded variables, i.e. PSA, the number of previous biopsy sessions, and evidence of ASAP on previous biopsy. Our study represents the largest series of saturation biopsies to date. [source] 52 Laparoscopic pyeloplasty , evolution of a new gold standardBJU INTERNATIONAL, Issue 2006D. MOON Objectives:, We report the largest series of laparoscopic dismembered pyeloplasty for treatment of primary and secondary uretero-pelvic junction (UPJ) obstruction, reviewing the current status of this procedure. Methods:, A total of 170 consecutive cases of laparoscopic pyeloplasty (156 for primary and 14 for secondary UPJ obstruction) were performed or supervised by a single surgeon (C.G.E). A four port extraperitoneal approach was used in all but three cases, which were performed transperitoneally. Results:, Median operative time was 140 min. The complication rate was 7.1% and conversion rate was 0.6% with no conversion in the last 161 cases. The median postoperative hospitalisation was 3 nights. Crossing vessels were encountered in 42% of cases and in 11 patients coexisting renal calculi were successfully removed. At a median follow-up of 12 months, the success rate was 96.2%. Conclusions:, Laparoscopic dismembered pyeloplasty produces functional results comparable to that of open surgery with the advantages of a minimally invasive procedure. Our results are consistent with previous series and support the view that laparoscopic pyeloplasty is moving rapidly towards replacing open surgery as the gold standard in treatment of UPJ obstruction. [source] Association of HLA DQ4-DR8 haplotype with papillary thyroid carcinomasCLINICAL ENDOCRINOLOGY, Issue 2 2006Teresa Porto Summary Objective, The association of the human leucocyte antigen (HLA) system with thyroid carcinomas is not clear. We sought to relate HLA alleles to susceptibility to papillary thyroid carcinoma (PTC) and also to clinical and pathological characteristics of PTC patients. Design and patients, The distribution of HLA in 181 unrelated Caucasian patients with PTC was compared to the HLA distribution in 315 normal controls, 31 patients with follicular carcinoma (FTC), 29 patients with lymphocytic thyroiditis (LT) and 50 patients with multinodular goitre (MNG), using a microlymphocytotoxicity assay. Results, Compared to normal controls, patients with PTC showed a significantly increased frequency of HLA-DQ4 [12·8%vs. 3·5%, P = 0·0005, Pcorrected (Pc) = 0·0032, odds ratio (OR) = 4·058, 95% confidence interval (95% CI) = 1·820,9·045] and HLA-DR8 (10·9%vs. 4·3%, P = 0·013, Pc > 0·05, OR = 2·752, 95% CI = 1·275,5·940). DQ4 and DR8 were also significantly increased in patients with MNG (DQ4, 16·3%; DR8, 16·3%) compared to controls (DQ4, P = 0·0019, Pc = 0·011, OR = 5·420, 95% CI = 1·978,14·852; DR8, P = 0·0044, Pc = 0·062). Linkage disequilibrium (LD) for these two alleles was present in controls (D = 0·0130, P = 9·7e-57) and in MNG patients (D = 0·0730, P = 4·6e-19) but not in PTC patients (D = 0·038, P > 0·05). In the subgroup of PTC subjects with concomitant nonthyroidal neoplasias (n = 27), there was a significant (P < 0·05) increase in the frequency of B57 (18·5%), DR11 (56·5%) and DQ3 (81·8%) compared to PTC patients without coexistent neoplasias (2·0%, 21% and 47%, respectively). No significant differences of HLA allele distribution was found in relation to PTC histology, age at diagnosis (> 45 or , 45 years), gender or tumour,node,metastasis (TNM) staging. In patients with FTC, the frequency of DR17 (FTC = 51·6%; controls = 22·5%; P = 0·0009; Pc = 0·0138) was significantly increased compared to controls. Patients with LT showed a higher frequency of the DR11 allele (48·3%) than controls (DR11 = 21·3%; P = 0·0028, Pc = 0·039, OR = 3·445, 95% CI = 1·568,7·567). Conclusions, We have typed the largest series of patients with thyroid carcinomas reported to date, and found that DR8 and DQ4 are independent susceptibility markers for PTC. [source] | ||||||||||||||||||||||