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E Barra

Publications and source records attributed to E Barra.

At least 19 recordsLinked to original sources

Analysis of exocrine pancreatic function in cystic fibrosis: one mild CFTR mutation does not exclude pancreatic insufficiency.

BACKGROUND: Cystic fibrosis (CF) is the most common cause of exocrine pancreatic insufficiency in childhood. The aim of the present study is to evaluate the correlation between genotype and exocrine pancreatic insufficiency in CF patients. The special emphasis was put on the analysis of mild CFTR mutations. DESIGN: The study comprised 394 CF patients and 105 healthy subjects (HS). Elastase-1 concentrations were measured in all subjects. RESULTS: Severe pancreatic insufficiency was associated with the presence of two CFTR gene mutations (DeltaF508, N1303K, CFTR dele 2,3 (21kb), G542X, 1717-1G-A, R533X, W1282X, 621GT, 2183AAG, R560T, 2184insA and DeltaI507, G551D, 895T) and mild insufficiency with the presence of at least one mutation (R117H, 3171insC, A155P2, 138insL, 296 + 1G-A, E92GK, E217G, 2789 + 5G-A. 3849 + 1kbC-T/3849 + 1kbC-T) genotype resulted in high elastase-1-values. However, in case of patients with genotype DeltaF508/3849 + 10kbC-T, 1717-1GA/3849 + 10kbC-T as well as with DeltaF508/R334W, both high and low elastase-1 concentrations were found. Low E1 values were found in a patient with DeltaF508/R347P genotype. CONCLUSION: Patients who carry two 'severe' mutations develop pancreatic insufficiency, whereas those who carry at least one 'mild' usually remain pancreatic sufficient. However, the presence of one mild mutation does not exclude pancreatic insufficiency.

Adult↗

P53 immunohistochemistry can identify bronchial dysplastic lesions proceeding to lung cancer: a prospective study.

Dysplasia is an important step in bronchial carcinogenesis and smokers present more dysplastic lesions than nonsmokers. These lesions not always lead to malignancy, so there is a need for additional, preferentially objective, diagnostic markers. To verify whether immunohistochemical overexpression of p53 protein in dysplastic areas could be a predictive marker of the development of lung cancer, we investigated p53 overexpression in 22 bronchial dysplastic lesions obtained by fibrebronchoscopy from heavy smokers who were not diagnosed as having lung cancer and were followed for a 4-yr period. Nine (41%) lesions showed p53-positivity. Seven lung cancers (78%), mostly squamous cell carcinomas, were detected within the follow-up in these patients and 3 in 13 (23%) patients with p53-negative lesions. Lung cancer occurred in all seven patients with dysplastic lesions showing >10% p53 positive nuclei. The positive predictive value of p53 immunostaining for lung cancer was 78%. The negative predictive value of p53 was 77%. p53 staining was not detected in squamous metaplasia lesions without atypia and in normal bronchial epithelium. Our findings provide evidence that p53-overexpression in bronchial dysplastic areas may be a clinically useful marker for identifying patients proceeding to, at least, squamous cell carcinoma and, in addition, may facilitate the detection of occult tumours.

Bronchi↗

Involvement of calcium in the cardiac depressant actions of a garlic dialysate.

In order to elucidate a possible role for calcium on the negative cardiotropic effects of a garlic (Allium sativum L., Liliaceae) dialysate in rat atria we studied: (a) the effects of our extract 15 min after preincubation with high and low concentrations of extracellular calcium ([Ca2+]o) on left and right activity of rat atria. The negative inotropism of garlic dialysate increased with calcium 0.75 mM; in contrast, high level of calcium (4.5 mM) induced a significant reduction of this depressant effect. None of these treatments modified the negative chronotropism of garlic; (b) nifedipine (10(-9) to 10(-7) M, verapamil (10(-9) to 10(-7) M) and diltiazem (10(-9) to 10(-7) M) induced a concentration-dependent synergism of the log concentration-effect of garlic dialysate on left atria. Verapamil and diltiazem (10(-7)M), but not nifedipine increased the inhibitory chronotropism of garlic in right atria; (c) negative inotropic and chronotropic effects demonstrated by nifedipine (1 x 10(-10) to 1.1 x 10(-6) M) were antagonized as expected by preincubation with Bay K-8644. Depressant actions of garlic were not modified with this pretreatment. These results suggest that the negative inotropic effect of our garlic dialysate is related to [Ca2+]o availability. It is possible that a restriction of intracellular calcium contributes to this effect. However, the negative chronotropic effect of garlic is scarcely affected by these modifications.

3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethy↗

Lung cancer metastatic cells detected in blood by reverse transcriptase-polymerase chain reaction and dot-blot analysis.

PURPOSE: We analyzed the blood of patients with lung cancer at different stages of presentation for the presence of carcinoembryonic antigen (CEA) mRNA detected by reverse transcriptase-polymerase chain reaction (RT-PCR) combined with the dot-blot procedure as an indicator of micrometastatic malignant cells. PATIENTS AND METHODS: We studied 24 lung cancer patients (10 with distant metastases and 14 with no evidence of distant metastases), eight age- and sex-matched patients affected by nonneoplastic respiratory diseases (four smokers), and eight healthy subjects. We used immunohistochemistry and RT-PCR dot-blot analysis to evaluate CEA expression in the neoplastic tissue, and the RT-PCR dot-blot procedure to analyze CEA mRNA in circulating cells. RESULTS: The RT-PCR dot-blot procedure was highly sensitive aspecific: it detected CEA mRNA in samples of RNA from lung cancer diluted 10(6)-fold with RNA extracted from normal blood cells, and sequence analysis confirmed that the amplified product was CEA. CEA mRNA was found in circulating cells from eight of 10 lung cancer patients with distant metastases (diagnostic sensitivity, 80%) and in four of 14 patients with no evidence of distant metastases. Two of the latter had distant metastases within 6 months of analysis. Thus, the diagnostic specificity of the analysis toward lung cancer without distant metastases was 86%. The analysis was negative in the eight nonneoplastic patients and in the eight healthy controls. CONCLUSION: The RT-PCR dot-blot analysis of CEA mRNA in blood cells seems to be a promising tool for the early detection of micrometastatic circulating cells in patients with lung cancer.

Aged↗

Serum and salivary antigliadin antibodies and serum IgA anti-endomysium antibodies as a screening test for coeliac disease.

Serum and salivary IgA and IgG antigliadin antibodies were determined by an enzyme-linked immunosorbent assay in 18 children with villous atrophy and 30 children on a gluten-free diet for coeliac disease in whom normal intestinal mucosa was found. Serum IgA anti-endomysium antibodies were also determined by an immunofluorescence method in these children. Serum IgG antigliadin and IgA anti-endomysium antibodies had the highest sensitivity (100 and 94.4%, respectively), followed by serum IgA antibodies to gliadin (72.2%), salivary IgA antigliadin (61.2%) and IgG antigliadin (50%) antibodies. The highest specificity was found for serum IgA anti-endomysium (100%) and IgA antigliadin (96.6%) antibodies and salivary IgA and IgG antigliadin antibodies (93.3%), while serum IgG antigliadin antibodies were found to be least specific (63.3%).

Adolescent↗

Fibronectin, laminin, type IV collagen distribution, and myofibroblastic stromal reaction in aggressive and nonaggressive basal cell carcinoma.

The stromal myofibroblastic reaction and extracellular matrix in a series of 19 morphologically and clinically aggressive basal cell carcinomas (BCC) were examined by an immunohistochemical staining panel for myofibroblastic markers (vimentin, actin, and desmin), for basement membrane components (laminin and type IV collagen), and for fibronectin. Results were compared with a series of 19 nonaggressive BCC. The aggressive BCC showed discontinuous staining for laminin and type IV collagen, a marked myofibroblastic response, and increased stromal fibronectin. Conversely, the nonaggressive BCC showed a continuous basement membrane and a total lack of staining for fibronectin and myofibroblastic markers in all but three cases. This report suggests that a combined analysis of extracellular matrix components and myofibroblastic markers may be useful for evaluating the clinical behavior including the metastatic capability of aggressive BCC.

Aged↗

p53 protein in aggressive and non-aggressive basal cell carcinoma.

Basal cell carcinoma (BCC) is the most frequent cutaneous neoplasm, with a generally favorable clinical behavior. Sometimes, indeed, it recurs after therapy and/or metastasizes. As point mutations in the coding sequence of the p53 tumor suppressor gene have been implicated in the progression of many human tumors, we studied the expression of p53 protein on this neoplasia. We tested immunohistochemically the positivity for p53 protein (NCL-p53-CM1, YLEM) on 19 cases of morphologically "non aggressive" BCC (BCC1) and on 19 "aggressive" BCC (BCC2), all with one or more relapses and 3 with distant metastases also. Results were related to clinico-pathological and follow-up data. All but one BCC2 were found positive for p53 protein. Conversely, only 2 cases of BCC1 exhibited low immunoreactivity for p53 protein, with high statistical differences between the two groups. No correlation was found between the immunoreactivity, age of patients, and site of the lesions. The availability of immunohistochemistry and the relatively easy interpretation of the results make screening for p53 protein a possibly useful tool in the prognostic evaluation of BCC.

Adult↗

Nucleolar organizer regions in aggressive and nonaggressive basal cell carcinoma of the skin.

Nucleolar organizer regions (NOR) were investigated on routine paraffin-embedded histologic sections of 11 aggressive basal cell carcinomas that recurred and/or metastasized (BCC2) and 11 nonaggressive basal cell carcinomas (BCC1). The absolute number of NOR per nucleus was higher in BCC2 than in BCC1, and their distribution pattern was also different. In fact, the means of argyrophilic staining of NOR (AgNOR) counts in the two groups of tumors by two observers were 6.56 with a SD of 1.98 for the nonaggressive and 9.48 with a SD of 2.12 for the aggressive basal cell carcinomas. A statistical analysis of these data using the Student's t test confirmed these observations (t = 64.49). Problems in the evaluation of NOR and possible comparison with other experiences are also discussed. The authors conclude that a quantitative assay of AgNOR and perhaps their distribution pattern may provide information useful to recognize BCC2 and hence may be of help in their prognostic prediction.

Adult↗

Comparative morphometric analysis of aggressive and ordinary basal cell carcinoma of the skin.

A morphometric analysis was performed on histologic sections of 11 cases of aggressive basal cell carcinoma that recurred and/or metastasized after the first diagnosis (BCC2) and on 11 ordinary basal cell carcinomas (BCC1). The considered parameters were as follows: nuclear area, perimeter, maximum diameter, feret x, feret y, form perimeter, and form area. The results show numeric differences between the two groups for the first five parameters. A statistical analysis was performed between BCC1 and BCC2 for all the considered parameters using a two-sided t test for independent samples. The test showed significant differences between the first five parameters except for the two form factors. A multivariate analysis was performed using area and perimeter values of the compared groups that amplified the discrimination threshold between the two groups. The authors conclude that a morphometric assessment may bring significant contribution in the knowledge and in the outcome prediction of basal cell carcinoma.

Aged↗

Fibromatosis of the larynx in the adult.

We describe the fourth case of aggressive fibromatosis of the larynx arising in an adult. Most cases of laryngeal fibromatosis have been described in children. In the larynx it may behave the same as in other sites, with local aggressiveness but without metastasis. The possible differential diagnoses are discussed.

Aged↗

Verrucous hemangioma and angiokeratoma circumscriptum: clinical and histologic differential characteristics.

It is important, from a prognostic and therapeutic point of view, to make a correct diagnosis between verrucous hemangioma (VH) and angiokeratoma circumscriptum. The former needs a large and deep exeresis, while the latter responds to the common means of physical therapy. Two cases of verrucous hemangioma are reported that have allowed us to review the clinical and histologic differential characteristics of the two forms.

Adolescent↗

Verruciform xanthoma of the vulva: case report.

A rare case of verruciform xanthoma of the vulva is reported. Diagnosis was made possible by histopathological examination and immunohistochemical staining. Verruciform xanthomas generally occur in the oral cavity. To the best of our knowledge, this is the third reported case of the tumour located on the genital mucosa. Immunohistochemical study supported the histiocytic origin of the lesion. Clinically, verruciform xanthomas may mimic other verrucous lesions of the vulva, such as seborrhoeic keratosis, verruca simplex, condyloma acuminatum, verrucous carcinoma, or erythropasia of Queyrat, or conditions such as histiocytosis, cutaneous lipidosis, or granular cell myoblastoma. The characteristics that differentiate those conditions from verruciform xanthoma, which can be seen only on histology, are given.

Aged↗

Multicentric Castleman's disease in association with Kaposi's sarcoma.

We have described the case of a 73-year-old female patient affected by multicentric Castleman's disease and complicated by Kaposi's sarcoma. Histologically the specimens of nodal biopsy showed combined patterns, both the hyaline-vascular type and the plasma cellular one. We have discussed the relationship between classic Castleman's disease and the multicentric type and the meaning of its association with Kaposi's sarcoma. Both diseases may present in immunologically depressed patients, but it is unknown whether Kaposi's sarcoma is a consequence of immunologic alterations caused by Castleman's disease or whether both processes are due to a primitive disorder of immunologic regulating factors.

Aged↗

[Twenty-nail dystrophy].

An idiopathic case of twenty-nail dystrophy observed in a 6 year old girl, is reported. Histopathological features obtained from a nail matrix biopsy specimen excluded lichen planus or other skin diseases. The various pathogenetic hypotheses are discussed.

Child↗