[Familial dysgammaglobulinemia in a case of lupous erythematovisceritis].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to L Fontana.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The overall purpose of the research reported in this article is two-fold: firstly, to describe the efforts of a regional health planning agency in the United States to reduce the size of acute care facilities in its planning area; and, secondly, to frame these events into a general theory of structural problems of state interventions in the American health care sector. Specifically, a case study is presented that documents--over time--the process of decision making, in seeking to close a community hospital against strong, and vocal, local interests. This analysis supports the view that, in the real world of health planning, power is exercised through a process of bargaining between health care providers and government, and between health care providers. Overall, the study illustrates the constraints upon the American health planning agencies to steer a relatively autonomous health care sector.
An in-depth analysis of clinical and laboratory findings in 58 patients affected by hereditary angioneurotic edema (HANE) is reported with special focus on problems related to the diagnosis of the disorder. The functional C1 inhibitor (C1INH) assay is the method of choice in the diagnosis of HANE, as it is capable of revealing the disorder with 100% accuracy. The immunochemical assay of C1INH detected HANE in 84.5% of the cases, i.e., immunochemical deficiency of C1INH (type I HANE). C4 was markedly reduced in both type I and type II HANE; thus, C4 levels can be particularly useful when C1INH functional tests are not available. CH50 testing is of little diagnostic value since total hemolytic complement activity is reduced in a variety of other congenital or acquired pathologies involving the complement system. The CH50 assay after incubation in low ionic strength buffer may be utilized in mass screening programs for qualitative evaluation. However, the test has the drawback of not being applicable in cases of frank hypocomplmentemia. While a depletion of the complement classical pathway was detected in most cases, no alteration in the complement alternative pathway was recorded, nor there was any reduction in immunoglobulin levels. Family history was positive in 100% of the cases. Attacks were almost always brought on by stress and/or trauma, though the causes were sometimes unknown. Edema could be cutaneous (non-pitting and non-pruritic) in 94.2%, laryngeal (often life-threatening) in 48% and abdominal (almost always painful) in 88.4% of patients. Associated pathologies were found in 2 patients, i.e., lupus rash and C3NeF-positive chronic membranoproliferative glomerulonephritis, respectively.
Hydatid cyst fluid of Echinococcus granulosus was found to interact with human complement. This interaction resulted in the depletion of hemolytic complement in normal serum and in the electrophoretic conversion of C3 and factor B. The results provided evidence of an activation of complement via the alternative pathway when hydatid cyst fluid was used in the mixtures. Also purified hydatid antigen preparations were found to interact with complement in vitro; this interaction resulted in the consumption of complement not only via the alternative pathway, but also via the classical pathway.
Polymorphonuclear leukocytes (PMN) from 11 patients with acute non-lymphoid leukemia (ANLL) in complete remission and off therapy for at least 22 months were studied for their oxidative metabolism and motility. In all patients, PMN motility tests revealed no consistent abnormality, whereas cells from 2 patients showed a selective impairment of oxidative metabolism, as assessed by the low capacity to reduce nitroblue tetrazolium and/or to generate chemiluminescence when stimulated with phorbol myristate acetate. No morphological change was detectable in PMN from these patients. However, the functional alterations appeared related to an early tendency to relapse, as both patients developed an acute phase after 5 and 9 months from testing, respectively. This suggests that abnormal PMN may circulate in ANLL patients in complete remission in spite of a normal differential count and that functional studies may be useful for detecting such a population.
We have studied complement activity, total leukocyte counts, PO2 and acid-base balance during a single hemodialysis with cuprophan membranes in a patient with hereditary angioedema and C3NeF-positive chronic membranoproliferative glomerulonephritis. Before, during and after the dialytic procedure plasma complement activity (total hemolytic complement, classical and alternative pathway activities) was not detectable and no C3-conversion occurred, while profound leukopenia (from 8,500 to 1,800 leukocytes/microliter) and hypoxemia (from 101.8 to 86 mmHg PO2) were found within 20 min from the initiation of hemodialysis. Similar results were obtained by studying the same parameters during two additional hemodialytic procedures. In vitro experiments showed that the patient's C3 could not be converted, either by cuprophan or zymosan, a specific and potent complement activator, even under optimal experimental conditions. Our data demonstrate that complement activation is not the only possible mechanism responsible for early leukopenia (as well as hypoxemia) during dialysis with cuprophan membranes.
We measured beta-endorphin (BE) and beta-lipotropin (BLPH) plasma concentrations (by means of an HPLC-RIA coupled method) during attacks as well as during symptom-free periods in a group of 28 patients with immunochemical (21) or functional (7) Cl inhibitor deficiency. Thirteen patients suffering from chronic urticaria served as controls. Three orders of considerations prompted us to initiate the present study: the clinical relationship between stress and the onset of acute episodes, the possible effects of repeated stressful situations, as are the attacks themselves, on the patients' neuroendocrine system and the well-known existence of close links between the immune system and endogenous opioids. The results show that plasma BE (and, to a lesser extent, BLPH) is dramatically increased during the attacks. In symptom-free periods many patients show very high BE concentrations, often in the presence of slightly elevated concentrations of BLPH and of ACTH. These observations suggest that patients with hereditary angioneurotic edema show a modified pro-opiomelanocortin-synthesizing cell activity that can result in a massive release of BE from the readily disposable pool present in the pituitary and/or an increase in the turnover of the peptide as evaluated by the BLPH/BE ratio.
We measured peripheral blood mononuclear cells (PBMC) beta-endorphin (BE) in patients suffering from hereditary angioneurotic edema (HANE), a disease attributed to C1-esterase inhibitor (C1INH) deficiency inherited as an autosomal dominant trait. Two orders of considerations prompted us to undertake the study reported herein: the presence of elevated plasma BE concentrations in HANE and the demonstration of BE-immunoreactivity in human unstimulated peripheral blood leukocytes obtained from healthy volunteers. Our results show that patients suffering from HANE have a very high BE presence in uncultured, unstimulated PBMC and in unstimulated PBMC cultured for 48 h. At this time high BE concentrations are detected in the culture supernatants. These observations suggest that in HANE patients the same factor(s) involved in causing increased secretion and release of BE from the pituitary (and, in turn, increased plasma BE levels) can play a relevant role also in the determination of high BE presence in PBMC and BE release from the cells.
OBJECTIVE: As in other countries, Moroccan health-care workers are exposed to occupational blood exposure (OBE) hazards. The purpose of this study was to estimate the occupational safety and hygiene conditions determining the OBE risk for health-care workers. WORKERS AND METHODS: In March 2000, a multicentric study was carried out in Morocco on 420 health-care workers, with an anonymous questionnaire. The study included health-care workers in the Taza and Temara hospitals and health centers, as well as in a Rabat public medical analysis laboratory. RESULTS: The participation rate was 67.8% (285/420). The population was mainly female (61%) with a mean age of 41.4 years (+/-7 years). Health-care workers answered that: occupational hygiene and safety were inadequate (55.1%); wearing single-use gloves was rare (34.5%); resheathing used needles was frequent (74.5%); safe containers were often missing (67%). In 1999, the annual average incidence of OBE was 1.5 (+/-4.3) per capita. If we consider the whole career of health-care workers, the incidence reached about 14.3+/-28.1 per capita. The absence of post-exposure chemoprophylaxis was the rule. CONCLUSION: The study shows that there is a need to improve occupational hygiene and safety conditions for Moroccan health-care workers in order to reduce OBE hazards.
Explore the source record for details and available documents.
The capacity of tobacco infusion and cigarette smoke condensate to activate the complement pathway was investigated in vitro by hemolytic and immunological assays. Complement activity was studied using samples of normal human serum incubated at 37 degrees C with different dilutions of tobacco infusion and cigarette smoke condensate. In all mixtures containing tobacco infusion or cigarette smoke condensate at a concentration of 0.1: 1, total hemolytic complement showed an average progressive decrease of 90% and 50%, respectively, after 60 min of incubation. This decrease was associated with a decrease of both classical and alternative pathway activities. In addition, cleavage fragments of C3, C4, and factor B were identified with immunochemical analyses. This study suggests that tobacco infusion and cigarette smoke condensate are activators of complement either through the classical or alternative pathway.