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Biomedical subjects

V De Angelis

Publications and source records attributed to V De Angelis.

At least 19 recordsLinked to original sources
PubMed1992

The relationship between allergy, clinical symptoms and bronchial responsiveness in atopic dermatitis.

Atopic Dermatitis (AD) and asthma are closely associated with respect to epidemiology, hereditary factors and occurrence in the same individuals. Bronchial Hyperresponsiveness (BH), the hallmark of asthma, can also be a physiopathological feature of AD, even in the absence of clinical asthma. We studied 78 subjects with AD. A follow-up study was performed in 27 of these. Data on respiratory and dermatologic symptoms were collected by means of a standardized questionnaire. Skin reactivity was evaluated by prick testing, and in 57 subjects BH was assessed with a methacholine test (Mch). Twenty-one subjects had asthma and 36 showed a positive skin reaction. A PC20 FEV1 was measurable in 38 subjects. Males were found more likely to be Mch responders than females (p < 0.05). Mch responders also showed an earlier age at onset of AD than nonresponders (2.1 yrs vs. 6.2, p = 0.03). Determinants of the degree of BH were evaluated by a stepwise multiple regression analysis, taking the log of the slope of the concentration response curve as dependent variable. In the final model we found that the degree of BH was directly related to wheezing (p = 0.0017) and coughing (p = 0.04) and inversely related to lung function (p = 0.0082) and age (p = 0.0008). Neither skin reactivity nor grading of AD were statistically significant. The longitudinal study demonstrated that the courses of AD and BH seem to run parallel only in skin-negative subjects, whereas an increase in BH was observed in skin-positive subjects.

Adolescent↗
PubMed1991

Role of spleen in hereditary spherocytosis: evidence for increased in vitro proteolysis of red cell membrane.

In vitro proteolysis of red cell membranes has been studied by means of electrophoretic separation on SDS-polyacrylamide gel of solubilized ghost proteins and subsequent densitometry of separated, stained bands; the amounts of major membrane proteins were measured in ghosts either with inhibited or with allowed proteolysis in the following cases: 15 patients suffering from hereditary spherocytosis (HS) with variable degree of spleen enlargement, eight cirrhotic patients with spleen enlargement and 12 healthy blood donors as control group. Proteolysis was present to a greater extent in HS patients with larger splenomegaly, lesser in HS with smaller splenomegaly, and was comparable to healthy controls both in splenectomized HS and in patients with spleen enlargement due to liver cirrhosis. The results suggest the involvement of splenomegaly in the enhancement of in vitro proteolysis in HS red cell membrane; it is probably attributable to joint effects of the damage induced in red cells by prolonged retention within haemolysing spleen together with the abnormalities genetically affecting the structure of HS red cell membrane.

Electrophoresis, Polyacrylamide Gel↗
PubMed1990

Normal fibronectin levels after surgical treatment of portal hypertension in liver cirrhosis.

We found a significantly higher plasma fibronectin concentration in a group of nine cirrhotic patients who underwent surgical treatment for portal hypertension (either shunting and non shunting procedures) when compared to twenty non operated patients. Significantly shorter prothrombin time and activated partial thromboplastin time in the operated patients were found as well. These results might be related to an increased breakdown of fibronectin during consumption coagulopathy taking place in the extended collaterals and reversed in part by surgical treatment of portal hypertension complicating liver cirrhosis.

Blood Coagulation Tests↗
PubMed1989

A new congenital platelet abnormality characterized by spontaneous platelet aggregation, enhanced von Willebrand factor platelet interaction, and the presence of all von Willebrand factor multimers in plasma.

A case is reported of a 49-year-old woman with a mild bleeding tendency. Her bleeding time, platelet count and size, plasma ristocetin cofactor activity, von Willebrand factor (vWF) antigen, and vWF multimeric pattern are all within normal limits. Spontaneous platelet aggregation is observed when citrated platelet-rich plasma (PRP) is stirred in an aggregometer cuvette. This aggregation is completely is only slightly diminished by an antiglycoprotein (GP) IIb/IIIa or by an anti GPIb monoclonal antibody. The patient's PRP shows increased sensitivity to ristocetin. The distinct feature of this patient, also present in two family members studied, is that platelet aggregation is initiated by purified vWF in the absence of any other agonist. The vWF-induced platelet aggregation is abolished by anti-GPIb and anti-GPIIb/IIIa monoclonal antibodies and by EDTA (5 mmol/L). Apyrase inhibits the second wave of aggregation. Patient's platelets in PRP are four to six times more reactive to asialo vWF-induced platelet aggregation than normal platelets. The amount of radiolabeled vWF bound to platelets in the presence of either low concentration of ristocetin or asialo vWF was increased 30% compared with normal. The patient's platelet GPIb was analyzed by SDS page and immunoblotting and by binding studies with anti-GPIb monoclonal antibodies showed one band with slightly increased migration pattern and a normal number of GPIb molecules. Unlike the previously reported patients with pseudo or platelet-type von Willebrand disease, this patient has normal vWF parameters.

Antibodies, Monoclonal↗
PubMed1989

Time-related shape control modifications during erythrocyte storage with additive solutions.

To obtain more detailed information on the reversibility of shape alterations in blood bank stored erythrocytes, we have studied shape recovery after chemical crenation and rheological properties in 8 PAGGS-sorbitol preserved erythrocyte concentrates during a five week storage period under blood bank conditions. Our results show that red cell capability to regain a normal discoid shape after chemical crenation decreases during storage but is not lost over a five week period. Moreover there is a significant but weak correlation between red cell ATP content and both shape recovery capability and viscosity. Our results confirm suspicious that red cell shape perturbations following blood bank storage are widely reversible. Two different mechanisms may be involved in reducing shape recovery capability during storage, namely an ATP-dependent mechanism and an energy-independent one. The energy dependent mechanism may be preserved by the previous addition of solutions which maintain higher energy levels during storage.

Adenine↗
PubMed1989

Modulation of NK activity by thymic hormones: in vitro effects of thymostimulin.

Plastic-adherent depleted or not depleted peripheral mononuclear blood cells (PMBC) from healthy donors showed enhanced lytic activity against 51Cr-labelled K562 target cells when exposed to thymostimulin (TS), 1 microgram ml-1, for 3 h, washed and incubated in TS-free medium before testing for natural killer (NK) cytotoxicity. No modification of NK cell activity was seen when effector cells were treated with placebo (splenic extract). The NK boosting activity of TS was lost when effector cells were treated for 3 h immediately before the performance of the cytotoxic test or when this thymic extract was added directly to the mixture of effector and target cells during the lytic phase of 51Cr release assay.

Cell Survival↗
PubMed1989

A phase I study of human natural interferon-beta in cancer patients.

In this phase I study 15 patients with metastatic tumors were given interferon (IFN)-beta by i.v. bolus injections. Twelve individual doses of 1, 2, 3.3, 5, 7, 9, 12, 16, 21, 27, 35, and 46 x 10(6) IU were administered every other day. The single maximal tolerated dose ranged from 9 to 46 x 10(6) IU. Eight patients tolerated the dose of 46 x 10(6) IU without side effects. Disturbances of cardiac rhythm were observed, but were closely related temporally to severe chills and appeared to be the consequence of adrenergic stimulation associated with this side-effect. In addition, no significant variations in the left ventricular function as assessed by nuclear stethoscope were observed. Neurotoxicity was not a major side-effect. The toxicity of IFN-beta given as scheduled in this study was significant, but acceptable.

Adult↗
PubMed1989

Platelet membrane abnormalities in myeloproliferative disorders: decrease in glycoproteins Ib and IIb/IIIa complex is associated with deficient receptor function.

The number and functional activity of membrane glycoproteins (GP) Ib and IIb/IIIa were investigated in platelets from 11 patients with myeloproliferative disorders (MPD). Three patients had essential thrombocythaemia, two had chronic myeloid leukaemia and six had polycythaemia vera. The numbers of GPIb and GPIIb/IIIa molecules were detected on the platelet surface using different 125I-labelled monoclonal antibodies. The functional properties of GPIb and GPIIb/IIIa were evaluated using purified 125I-labelled asialo von Willebrand factor (vWF) and purified 125I-labelled fibrinogen, respectively, in a binding assay. Binding of the anti-GPIIb/IIIa antibody was decreased by 40% in almost all patients studied and, when measured, it was accompanied by decreased fibrinogen binding to activated platelets. Binding of anti-GPIb antibodies to platelets was also slightly decreased or virtually the same in eight out of 11 patients. The decrease correlated with decreased binding of asialo vWF. The increased plasma glycocalicin levels, measured in four patients, depended on the high platelet count. Scatchard analysis revealed normal receptor binding affinity for all ligands tested in all but one patient. In this report we demonstrate that abnormalities in the concentrations of GPIIb/IIIa membrane proteins are commonly present in patients with MPD, while a decrease in GPIb concentration is also seen, although in fewer patients. These abnormalities are accompanied by a concurrent decrease in the respective receptor functions. These findings may explain part of the haemorrhagic tendency often encountered in MPD.

Antibodies, Monoclonal↗
PubMed1989

Bronchial hyperresponsiveness in atopic dermatitis.

We investigated the prevalence of bronchial asthma, immediate skin reactivity and bronchial hyperresponsiveness in 40 patients with atopic dermatitis (AD). Eight patients reported to have asthma while 29 were found responders to methacholine test (PC20 Mch less than 64 mg/ml) and 21 were skin reactors. The prevalence of skin positive reactions did not differ in responder and non-responder (PC20 Mch greater than 64 mg/ml) groups. Males were more likely both to have bronchial asthma and be responders to methacholine test than females. Moreover, responders had an earlier age of onset of AD than the non-responders. We suggest that bronchial responsiveness should be evaluated in children with AD.

Adolescent↗
PubMed1987

Rheological abnormalities of erythrocyte deformability and increased glycosylation of hemoglobin in the nephrotic syndrome.

This report is an attempt to gain more complete knowledge of the causes of the tendency to thrombosis in the nephrotic syndrome. Our purpose was to detect some rheological abnormalities in a group of nephrotic patients having normal renal function. Plasma and blood viscosity, erythrocyte filtration flow (EFF) and some indices of the carbohydrate metabolic state (glycosylated hemoglobin A1c; HbA1c, fasting glycemia and oral glucose tolerance test) were investigated in 10 patients with newly diagnosed primary nephrotic syndrome and 10 healthy subjects. EFF was determined in order to avoid variables depending upon plasma, white cells and hematocrit. The mean level of HbA1c was higher in the nephrotic group, in which we found 4 patients with impaired glucose tolerance according to the criteria of the National Diabetes Data Group. EFF was lower in nephrotic patients than in controls and correlated inversely with HbA1c. These findings indicate that nephrotic patients have a reduced red cell deformability which seems to be related to the abnormal carbohydrate metabolism commonly observed in this syndrome.

Adult↗
PubMedDate not supplied

IFN-beta induced biochemical and immunological modifications in hairy cell leukemia patients.

BACKGROUND: Although IFN-beta is 30-40% homologous with IFN-alpha, its intrinsic biological properties are not identical. Compared with IFN-alpha, IFN-beta exerts greater in vitro antiproliferative activity on many cell lines, stimulates peripheral blood stem cells of hairy-cell leukemia (HCL) patients to differentiate to erythroid burst forming cells, has higher specific type I IFN receptor affinity and modulates the expression of class II histocompatibility antigens. IFN-beta would, therefore, be expected to have a greater, or at least similar, antitumor activity as that of the various types of IFN-alpha. METHODS: We have treated 12 patients affected by HCL with IFN-beta and have investigated the biological and immunological changes induced by such treatment. RESULTS: A rise in beta 2-microglobulin and neopterin values throughout IFN-beta therapy was documented in most patients. An increase in NK activity was observed only in clinical responders whose CD57+/CD16+ cell ratio dropped below baseline. There was also a modulation in IFN-gamma synthesis that was dependent on baseline levels and in line with the clinical response. IFN-beta provoked a reduction in CD3+ and CD4+ cell subsets in patients with WBC greater than or equal to 10.0 x 10(9)/1 and greater than or equal to 50% circulating HCs, an expansion in absolute number of CD3+ and CD8+ cell fractions and a slight rise in the absolute values of CD2+ and CD4+ cell subpopulations in patients with WBC less than or equal to 5.0 x 10(9)/1 and less than or equal to 50% circulating HCs. There was no correlation between either the IFN-beta induced increase in beta 2-M or Np levels and clinical response. Most immunological parameters improved or normalized later during the course of IFN-beta treatment, when pathological-hematological signs of disease remission were already evident. CONCLUSIONS: The relevance of the IFN-beta induced changes as well as that of the IFN-alpha induced biological effects in the clinical control of HCL remain unclear.

Adult↗
PubMedDate not supplied

Phase I-II trial on natural beta interferon in chemoresistant and relapsing multiple myeloma.

One Waldenström's disease and 16 multiple myeloma patients were administered IFN-beta I.V. at the dose of 6 x 10(6) IU/m2 over 6 hours for 7 days on alternate weeks for a total of 3 cycles, and then continued at the same dose, twice a week, for an additional 24 weeks. Four patients had initially proved to be, and 6 became, resistant to chemotherapy. Disease progressed after chemotherapy was discontinued in another 7 patients: 3 SD and 4 PR. One of the 16 evaluable patients achieved an MR, and 11 experienced brief stabilization of disease (median 14 weeks, range 6-34). In addition, cellular response to IFN-beta was documented by increased B2-microglobulin and neopterin levels, even as early as 24 hours after the 1st IFN injection of the 1st and 2nd cycles. Hemtological and extrahematological toxicity was low despite the fact that 8/16 patients had severely or moderately reduced bone marrow reserve at the beginning of treatment. Since vincristine-adriamycin-dexamethasone and etoposide-dexamethasone-cytarabine-cisplatin combinations achieve high response rates in resistant and relapsing multiple myeloma patients, IFNs alone should be reserved as third-line therapy for those subjects who are resistant to, or not candidates for, these chemotherapeutic regimens. The low toxicity and the modulation of B2-microglobulin and neopterin should encourage studies aimed at defining the optimal antitumor dose of IFN-beta that could be used in combination with conventional chemotherapeutic regimens to improve the response rate in multiple myeloma patients.

Adult↗
PubMedDate not supplied

Combined factor VIII and factor XI congenital deficiency: a case report.

Combined congenital defect involving both Factor VIII and XI is a very rare disorder. We describe a case of combined Factor VIII and XI deficiency in which the propositus has a mild hemophilia A and inherited a Factor XI deficiency from the father. Moreover, we report on the benefit of DDAVP administration to the patient during a bleeding episode.

Adult↗