Bullous pemphigoid in a 19-year-old woman. A case with unusual target antigens

Summary We report a 19-year-old woman with typical clinical histological and immunofluorescence features of bullous pemphigoid. By immunoblotting, the serum was shown to detect antigens at 240 and 138 kDa. Elevated serum IgK levels were present, and there was a marked peripheral blood eosinophilia. The degree of eosinophilia correlated with small changes in the severity of the skin lesions, and the IgE level showed correlation with marked changes in severity. Another unusual feature was an exacerbation of her disease at the time of her first menses after the onset of the bullous pemphigoid.     

Detection of Philadelphia Chromosome in Chronic Neutrophilic Leukemia

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferativedisorder, differentiated from chronic myelogenous leukemia byseveral features. A case of CNL which was found by long-termculture to involve the Philadelphia chromosome is reported.     

MYCOSIS FUNGOIDES WITH SIGNET-RING CELLS AND MONOCLONAL GAMMOPATHY

A 73-year-old white woman was admitted to our hospital for evaluation of a chronic dermatitis. Personal history was remarkable only for arterial hypertension and noninsulin-dependent6 diabetes mellitus. Clinical and histopathologic findings were consistent with a diagnosis of mycosis fundoides (mf and staging procedures including bolld cell counts, serum biochemistry, urinalysis, bone marrow biopsy, Sé zary cell counts in peripheral bolld, computerized tomography (ct scans, abdominal ultrasonography, chest roentgenograms, serum protein electrophoresis, and immunoelectrophoresis disclosed normal or negative results (T2 N0 M0 B0). After 2 years of standard treatment, the patient's condition had progressively worsened and she presented generalized erythematious plaques and a few, small, red-bluish tumors (Fig. 1). A biopsy specimen of a tumor showed an intense lymphoid infiltrate composed mainly of large and medium-sized cells, many of which exhibited clear interacytoplasmic vacuoles (Fig. 2). These vacuoles were negative for periodic acid-Schiff, alcian blue, and oil-red-O staining. Most of the neoplastic cells stained with uchl1 antibody, and none expressed B-cell markers. Some of the malignant cells reacted wih the Ber-h2 and mb3 monoclonal antibodies. No evidence of immunoglobulin libht chain production was observed. Negative staining with cd68 monoclonal antibody ruled out a monocyte-macrophage lineage of the malignant cells. On physical examination, no palpable lymph nodes were found. The quantitative immunoglobulin study showed an IgG level of 6,950 mg/dL (noral values, 800–1,800 mg/dL), IgA level of 126 mg/dL (normal, 90–450 mg/dL), IgM level of 130 mg/dk (normal, 70–280 gm/dL).Serum immunoelectrophoresis showed an IgG lambda monoclonal protein. Further studies including blood cell count, serum biochemistry, urinalysis, bone marrow biopsy, peripheral blood Sézary cell counts, Bence-Jones proteinuria, ct scans, abdominal ultrasonography, and chest roentgenograms yielded negaive or normal results. Thus, diagnosis of monoclonal gammopathy of unknown significance (mgus ) was made. Despite treatment with systemic chemotherapy, the diesease followed an aggressive course and the patient died from Staphylococcus epidermidis septicemia.     

Quality assessment of air pollutants concentration in epidemiologic time series on short-term effects of pollution on health

Meta-analyses of short-term effect of air pollution use environmental exposure measurements defined as the concentration average between selected monitors. A simple quality index, Pearson's correlation coefficient between each pair of monitors, has been used in sensitivity analyses and meta-regression. To better characterize the degree of homogeneity in population exposure we propose to use Lin's concordance coefficient and the correlation coefficient between the difference and the average of each pair of values. Using simulated data and real data from the city of Rome (1998-2001) and Milan (1996-2002) we identify three conditions responsible of exposure misclassification when averaged concentrations are used in the analysis.     

Characterization of renal tubular damage in preterm infants with renal failure

This study attempts to clarify the characteristics of renal tubular damage in preterm infants with renal failure. Sixty-one neonates (17 term and 44 preterm infants) were divided into three groups: 15 infants with intrinsic renal failure (IRF), five term and 10 preterm; 19 with pre-renal renal failure (PRF), five term and 14 preterm; and 27 without renal dysfunction (control), seven term and 20 preterm. Urine was collected for an 8 h period on the 2nd or 3rd day of life to determine the following parameters: creatinine clearance (Ccr), fractional excretion of sodium (FENa), urinary N-acetyl-beta-D-glucosaminidase (NAG) index and endothelin-1 (ET-1) excretion. Parameters of renal tubular function and/or renal tubular damage such as FENa, NAG index and ET-1 excretion were considered as a useful marker to differentiate IRF from PRF in preterm infants. However, these parameters were significantly elevated in preterm infants with PRF. These findings led us to make the following speculations: (i) renal tubular damage may easily occur in preterm infants; and (ii) there still remains difficulty in differentiation between IRF and PRF using Ccr instead of the fluid challenge test.     

A significant correlation between clonogenic radiosensitivity and the simultaneous assessment of micronucleus and apoptotic cell frequencies

Purpose: To study the relationship between clonogenic radiosensitivity and a simultaneous assessment of the percentages of radiation-induced micronuclei (MN) and apoptotic cells. Materials and methods: Five mammalian cell lines were studied. Following irradiation, measurements were made of the percentages of MN, apoptotic cells and floating cells. Each factor was compared, both alone and as the simple sum of the factors, with cell survival. The factors were also evaluated using a multiple linear regression analysis. Results: The simple sum of the MN and apoptotic cell percentages, the sum of the MN and floating cell percentages, and the sum of all three factors correlated with cell survival. However, the multiple linear regression analysis was the best method of predicting cell survival. Conclusions: These results suggest the usefulness of the simultaneous assessment of the MN frequency and the detection of floating cells in culture medium or apoptotic cells among attached cells to predict cellular intrinsic radiosensitivity. In order to predict cell survival after irradiation from these factors, the use of multivariate analyses is more useful than simple summation.     

Overexpression of p53 protein correlates with a high risk of malignant transformation of adenomas in patients with multiple colorectal adenomas

To assess the correlation of p53 oncoprotein expression with the high risk of developing carcinomas in patients with multiple colorectal adenomas, 25 cases with histologic carcinoma in adenoma (CIA) were examined by immunohistochemistry using a monoclonal antibody specific to human p53 protein (wild and mutant). The 25 cases were classlfied into multiple and single groups. The former contained 13 cases with synchronous multiple colorectal adenomas (one to six adenomas) and adenocarcinoma. The latter included 12 cases with single CIA only. This study revealed an overall incidence of 57.14% of p53 overexpression in carcinomatous lesions and 31.9% in adenomatous lesions, which was statistically significant ( P <0.05). The carcinomatous lesions showed a diffuse staining pattern, whereas the adenomatous lesions showed a focal pattern. A significant finding was that the incidence of p53 overexpression was slgnificantly higher in multiple groups (81.25%) than in single groups (31.43%) in the carcinomatous ( P <0.01) rather than in the adenomatous ( P >0.05) lesions. There were no correlations between p53 overexpression and proliferation activity or carcinoembryonic antigen expression. The results indicate that p53 abnormality may be an important genetic factor responsible for the high risk of developing carcinomas in patients with multiple adenomas.     

Chemical synthesis and biological activities of the EGF-like domain of mouse schwannoma-derived growth factor (SDGF)

Schwannoma-derived growth factor (SDGF), an epidermal growth factor (EGF) family peptide recently discovered, has an EGF-like domain in the carboxyl terminal portion. In this study, we synthesized mSDGF(38-80) corresponding to the EGF-like domain of mouse SDGF by means of stepwise solid-phase method using Fmoc chemistry in order to evaluate the biological function of the EGF-like domain in SDGF. The linear peptide of mSDGF(38-80) was folded by direct oxidation with reduced and oxidized glutathione to form intramolecular disulfide bridges in synthetic peptide. On the biological activity, we examined mitogenic activity induced by mSDGF(38-80) in NIH/3T3 fibroblast cells and interaction with EGF receptor in A431 cells. In the results, mSDGF(38-80) was confirmed to form three disulfide linkages that were similar in pattern to EGF by amino acid and sequence analysis of fragments obtained after thermolytic digestion. However, mSDGF(38-80) possessed weak mitogenic activity in NIH/3T3 cells and weak binding affinity for the EGF receptor in A431 cells compared with those of human EGF. These results suggest that the EGF-like domain of SDGF may have little effect upon mitogenic activity and the EGF receptor binding of SDGF. © Munksgaard 1995.