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Biomedical subjects

V De Stefano

Publications and source records attributed to V De Stefano.

At least 73 records · Page 4Linked to original sources

Failure of fibrinolytic endoventricular treatment to prevent neonatal post-haemorrhagic hydrocephalus. A case-control trial.

Post-haemorrhagic hydrocephalus is assumed to result from obstruction of the cerebrospinal fluid (CSF) pathways by blood clots and subsequent chronic infiltration with collagen. The aim of this work was to evaluate the possibility of preventing permanent shunt dependence by enhancing the endoventricular fibrinolysis by means of an endoventricular streptokinase infusion in babies affected by posthaemorrhagic ventricular dilation. A case-control trial was carried out in 12 neonates affected by intraventricular haemorrhage and subsequent progressive ventriculomegaly. Six of them were treated with 20,000 U/day of streptokinase infused over 96 h through a percutaneous ventricular catheter. Our results show that the percentage of shunted babies was identical in treated and control patients despite the enhancement of endoventricular fibrinolysis obtained in all treated patients. On the basis of our results we do not recommend intraventricular streptokinase infusion for routine use in post-haemorrhagic ventricular dilatation.

Case-Control Studies↗

Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases.

Four cases of pleomorphic xanthoastrocytoma (PXA) were collected from among 688 glioma patients who underwent operation at the Institute of Neurosurgery, University of Naples "Federico II" between January 1973 and December 1994. Three were females and one male, ranging in age from 10 months to 65 years. Three tumors were superficial in location, appearing as a meningo-cerebral mass in the temporo-parietal region. In one case, the tumor was situated deep within the brain (capsulo-thalamic region), without contact with leptomeninges. Three patients had experienced epileptic seizures, whereas one patient presented with an ictal episode. Tumor excision was grossly total in two cases, and subtotal in the remaining two. In three cases, histological examination demonstrated a "typical" PXA; conversely one tumor (subtotal excised) was an "atypical" PXA. The two patients with incomplete surgical resection were postoperatively treated with fractionated brain radiation therapy. Of the two patients who had grossly total removals, one showed tumor recurrence 6 years after surgery, and underwent operation (the recurrent neoplasm did not exhibit malignant transformation); the second patient was free of tumor at 14 months following craniotomy. Of the two patients who had undergone subtotal removals, one died because of massive regrowth of the lesion 22 months after surgery, whereas the second patient was asymptomatic at 1 year follow up.

Adolescent↗

Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with Budd-Chiari syndrome or portal vein thrombosis.

Budd-Chiari syndrome is a severe disease characterized by occlusion of large hepatic veins leading to death if untreated. Using the classical criteria for the diagnosis of polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (IMF), overt PV was the underlying cause in about 10% of the cases and ET or IMF in only a very few. Using spontaneous endogenous erythroid colony (EEC) formation in vitro and/or bone marrow biopsies, a primary myeloproliferative disorder (PMD) was present in 78% of the patients with apparently idiopathic Budd-Chiari syndrome and in about half of the patients with portal, splenic, and/or mesenteric vein thrombosis. The diagnoses in 40 reported cases with hepatic vein thrombosis and spontaneous EEC were overt PV in 25 and latent unclassified PMD in 15 patients. The diagnoses of 40 reported cases with splanchnic vein thrombosis and spontaneous EEC were overt PV in 12, ET in 2, IMF in 2, and latent unclassified PMD with the presence of EEC in 24 patients. Thrombocytosis as a manifestation of myeloproliferative disease was recorded in 34 of 80 (42.5%) patients with spontaneous EEC and Budd-Chiari syndrome or portal vein thrombosis. Thrombocythemia was present in 15 of 41 patients with a proven and in 19 of 39 patients with a latent myeloproliferative disorder. Patients with hepatic vein or splanchnic vein thrombosis associated with a PMD are predominantly females younger than 45. It is concluded that both spontaneous EEC and histopathology from bone marrow biopsy provide specific information as sensitive clues to the diagnosis of all variants of overt and latent myeloproliferative disorders. The association of hepatic and splanchnic vein thrombosis and PMD is not fully understood. Therapeutic options of Budd-Chiari syndrome include anticoagulation with heparin, fibrinolysis followed by oral anticoagulation, and appropriate treatment of the underlying PMD. In case of failure, invasive options include local procedures such as angioplasty or stenting, venous decompression by portal-systemic shunts, or liver transplantation.

Biomarkers↗

Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry.

PURPOSE: To assess the natural history and risk factors for thrombosis in a large cohort of unselected patients with antiphospholipid antibodies. PATIENTS AND METHODS: Three hundred sixty consecutive patients (118 males, 242 females, median age 39 years [range 2 to 78]) fulfilling the currently accepted criteria for diagnosis of lupus anticoagulant (LAC) (n = 326) and/or raised immunoglobulin G anticardiolipin antibodies (IgG ACA) (n = 185) were collected from 16 Italian institutions and prospectively observed for a median of 3.9 years (range 0.5 to 5). Main endpoints were the occurrence of arterial or venous thrombosis, the outcome of pregnancies, and any severe complications leading to hospitalization or death. RESULTS: Thirty-four patients developed a thrombotic complication, with a total incidence of 2.5% patient-years. Multivariate logistic regression analysis identified two independent risk factors for thrombotic events: a previous thrombosis (RR 4.9; 95% CI, 1.76 to 13.7; P < 0.005) and IgG ACA titer above 40 units (RR 3.66; 95% CI, 1.24 to 10.8; P < 0.01). A total of 28 pregnancies were observed in 25 women and 11 (39%) were abortive. Adverse pregnancy outcomes were significantly more frequent in women with a history of miscarriage or vascular occlusion (9/16, 56%) than in asymptomatic women (2/12, 17%) (P = 0.035). Four patients developed non-Hodgkin's lymphoma during the follow-up. Eighteen patients died. Vascular events and hematological malignancies represented the most frequent causes of death (n = 5 for each). CONCLUSIONS: The present study shows that: (a) previous thrombosis and ACA titer > 40 U are independent predictors of thrombosis; (b) history of miscarriage or vascular disease is significantly associated with adverse pregnancy outcome; (c) hematological malignancies can develop during follow-up in patients with antiphospholipid antibodies.

Adolescent↗

Hypereosinophilia during 2-chlorodeoxyadenosine treatment for hairy cell leukaemia.

A 43-year-old male with newly diagnosed hairy cell leukaemia underwent a single course of 2-chlorodeoxyadenosine (2-CdA). Skin rash, facial swelling and marked eosinophilia developed 20 d after treatment and were resolved by 7 d of steroid therapy. Eosinophil peak in peripheral of the eosinophil population showed a high expression of the IL-2 receptor alpha-chain (CD25), representing up to 94% of gated cells. HLA-DR and CD4 antigens were constantly negative; eosinophils strongly reacted with the secretory form of the eosinophil cationic protein (ECP), recognized by EG2 monoclonal antibody. IL-5 serum levels were markedly elevated at the onset of eosinophilia, returned to normal levels after its disappearance and positively correlated with eosinophil count (r = 0.94, P = 0.016). Eosinophilia is an uncommon finding after treatment with 2-CdA. It is unclear whether these phenomena represented a true allergic reaction to the drug or the effect of massive tumour cell lysis and haemopoietic pancytopenia with immunosuppression, which induced the release of IL-5 and possibly other cytokines.

Adult↗

Instrument effect on the activated protein C resistance plasma assay performed by a commercial kit.

In order to evaluate the influence of the coagulation instrument on the activated protein C (APC)-resistance plasma assay performed by a commercial kit, we tested 70 plasma samples on 4 different instruments during a simultaneous session run using a same lot of Coatest APC-resistance (Chromogenix). The results were analyzed employing three different modes of expression (aPTT prolongation in the presence of APC, APC-sensitivity ratio, normalized APC-sensitivity ratio) and three different diagnostic threshold values (below the control mean--2 standard deviations or the lowest control value or the 5th percentile of the control values). The inter-instruments variability in the mean values of the control individuals can be limited expressing the results as normalized-APC-sensitivity ratio (range 0.99-1.05). The overall diagnostic yield in thrombotic patients and their relatives depended mainly on the instrument employed and only in some cases on the mode of expression of the results and on the diagnostic threshold value. The sensitivity of the commercial assay on heterozygotes for factor V Leiden diagnosed by gene analysis was overall satisfactory (75-100%) but in some cases a lower diagnostic yield was noticed, depending on the type of instrument employed and/or the type of expression of the results and/or the diagnostic threshold values. Thus the instrument system adopted should be carefully considered in the interpretation of the results using the commercial kit.

Blood Coagulation Tests↗

[Absence of an association of the D allele of the ACE gene with arterial pressure in mild-moderate essential arterial hypertension].

To assess the relationship between the angiotensin converting enzyme (ACE) gene I/D polymorphism, blood pressure (BP) and family history of hypertension, 133 hypertensive subjects (mean age 50 +/- 9 years, 78 males, 55 females) were selected according to both casual supine BP > 140/90 mmHg and ambulatory BP > 134/88 mmHg. Drug treatment was discontinued 2 weeks before entering the study. Subjects with myocardial ischemia, as well as those with "white coat" hypertension, were excluded. The study population was subclassified according to age < or = 50 years. Polymerase chain reaction was used to detect the I/D polymorphism of the ACE gene, and the DD genotype was analysed twice. The frequencies of the I and D allele were 42 and 58%, and the distribution of the ID+ II and DD genotypes were 69 and 31% respectively. No significant relation was found among ACE genotypes (DD vs ID+ II) and casual systolic or diastolic BP as well as ambulatory BP, both in the whole study population and in the subpopulation < 50 years old. No difference was found also in the distribution of dippers and no dippers, as well as in the distribution of subjects with a positive family history in the whole sample and hypertensives < 50 years old.

Adult↗

Prognostic significance of nucleolar organizer regions in meningiomas.

The argyrophilic method for the demonstration of nucleolar organizer region-associated proteins (AgNOR's) was applied to paraffin-embedded histologic sections of 30 meningiomas. These were divided into three groups: 1) group I comprising 10 primary tumors which subsequently recurred within five years; 2) group II including the relative recurrences of the above mentioned primary tumors; 3) group III consisting of 10 nonrecurrent tumors over a follow-up of at least five years. The means of AgNOR counts were 1.58 with a SD of 0.67 for the group I, 1.47 with a SD of 1.04 for the group II, and 1.42 with a SD of 0.62 for the group III. A statistical analysis of these data using the Student's "t" test showed no significant difference between primary tumors and relative recurrences, as well as between primary and nonrecurrent tumors. However, when the AgNOR number was > 2.0 there was a statistically significant difference between primary and nonrecurrent meningiomas. These results are discussed in light of the findings previously reported by the literature.

Humans↗

Effect of all-trans retinoic acid on procoagulant and fibrinolytic activities of cultured blast cells from patients with acute promyelocytic leukemia.

The mechanisms underlying acute promyelocytic leukemia (APL) coagulopathy and its reversal by administration of all-trans retinoic acid (ATRA) have been investigated. Bone marrow promyelocytic blasts from nine patients with APL were cultured with or without ATRA 1 mumol/L. Cultured blasts (days 0, 3, 6, and 9) were washed, resuspended in phosphate buffer, lysed by freezing and thawing, and then assayed for procoagulant activity (PCA), elastase activity, tissue factor (TF) antigen, tissue-type plasminogen activator (t-PA) antigen and urokinase-type plasminogen activator (u-PA) antigen. PCA was determined by a recalcification assay. Elastase was measured by an amidolytic assay (S-2484). TF, t-PA, and u-PA antigens were measured by an enzyme-linked immunosorbent assay (ELISA). Malignant promyelocytes isolated from the patients had increased levels of PCA and TF as compared with the control polymorphonucleates, and low levels of elastase, t-PA, and u-PA; the patient blast PCA level was significantly related to the degree of hypofibrinogenemia. In this system, blast PCA depended on the tissue factor and was significantly correlated to the TF antigen values. In the cultures without ATRA, PCA, TF, and u-PA progressively increased, whereas elastase and t-PA levels remained essentially unchanged. In the presence of ATRA, all parameters (except u-PA) decreased during the culture time. Thus, a major role of the promyelocytic blast cell PCA in the pathogenesis of M3-related coagulopathy is suggested; the ATRA effect on coagulopathy seems mainly mediated by a downregulation of the PCA.

Adolescent↗

[Solitary capillary hemangioblastoma, cellular variant. Clinical, radiological, and anatomo-pathological study of 2 cases].

Two cases of the rare cellular variant of the solitary capillary haemangioblastoma are reported. On MR study both tumors appeared as cerebellar contrast-enhancing masses, without evidence of intra- or perilesional blood vessels. Histologically, they showed compact groups of polygonal or rectangular cells separated by compressed small capillaries. There were no reticulin fibres among cell clusters. The stromal cells were found to be immunopositive for neuron-specific enolase (NSE), factor VIII-related antigen (von Wille-brand factor), Ulex europaeus lectin, and glial frillary acidic protein (GFAP). The findings are discussed in light of the pertinent literature.

Aged↗