Severe deep vein thrombosis in a 2-year-old child with protein S deficiency.
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Child, Preschool↗
Biomedical subjects
G Leone
Publications and source records attributed to G Leone.
Further characterization of a pathological isoantithrombin with no affinity for heparin (antithrombin III Roma).
A molecular antithrombin III variant (Antithrombin III Roma) with an abnormal pattern of crossed immunoelectrofocusing was further investigated in order to identify the pathological isoforms. AT III crossed immunoelectrofocusing of the whole plasma from the affected patients showed a loop overlapping the peak normally present at pH 4.8-4.6. Affinity chromatography demonstrated the presence of an AT III fraction totally lacking in heparin affinity. Crossed immunoelectrophoresis on heparin-agarose (H-CIE) and crossed immunoelectrofocusing (CIEF) runs performed on the fractions obtained by heparin-agarose affinity chromatography confirmed that the functional defect was exclusively related to the pathological isoantithrombin (pH 4.8-4.6), which was also devoid of any progressive activity. The AT III fraction with normal affinity to heparin displayed H-CIE and CIEF patterns identical to the control AT III.
Antithrombin III↗
[Use of ticlopidine in a group of patients with obliterative arteriopathy of the lower extremities].
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Arterial Occlusive Diseases↗
Antithrombin III in full-term and pre-term newborn infants: three cases of neonatal diagnosis of AT III congenital defect.
Antithrombin III (AT III) plasma levels were investigated in 18 full term neonates and 14 healthy preterm neonates. A control group of 20 healthy adults was also studied. AT III was measured as antigen concentration (Ag) and antithrombin or anti-factor Xa heparin cofactor (H.C.) activities. Crossed immunoelectrophoresis on heparin-agarose (H-CIE) was carried out on plasma samples; moreover the distribution of isoantithrombins was investigated on whole plasma by a technique of crossed immunoelectrofocusing (CIEF). AT III plasma levels in full term infants were significantly lower as compared to the adult values. The preterm newborns group showed a further significant decrease in AT III levels as compared to the full term neonates. In all infants AT III H-CIE runs displayed a single fast moving anodal peak, so that a normal binding to heparin was demonstrated. The CIEF AT III plasma pattern of the adults as well as of all neonates displayed three major peaks at pH range 5.2-4.9, a small amount of AT III at pH 4.9-4.8 and a minor peak at pH 4.8-4.6, so that it was concluded that the isoantithrombins plasma distribution in neonatal age is identical to that of the adult subjects. Four neonates whose mothers were affected by AT III congenital defect were also investigated: diagnosis of congenital deficiency was established in three cases.
Adult↗
Identification of blast cells in peripheral blood through automatic assessment of nuclear density: a new tool for monitoring patients with acute leukaemia.
The Technicon H*1 haematology system is provided with a new method for basophil count, neutrophil lobularity assessment and detection of blasts in peripheral blood through automated measurement of nuclear density. We compared the results of the H*1 blast flag with those of the microscope examination in 131 peripheral blood samples from 43 patients with acute leukaemia in different phases of their disease, to determine the degree of sensitivity and specificity of the system in this setting. In six patients at diagnosis or in overt relapse, all having large percentages of blasts at the manual differential count, a typical deformation of the profile of the mononuclear cell population on the display was consistently observed, regardless of the morphological subtype of leukaemic cell which was involved. Amongst 34 samples with 4-95% morphologically recognizable blasts on the peripheral blood film, the sensitivity of the system was 100% with no false negatives at all. In 43 samples, on the other hand, the H*1 blast flag was positive in the absence of any morphological evidence of blasts on the smear. These 'false positives', however, were always obtained from leucopenic patients who had more than 12% blast infiltration in the bone marrow, compared to the 0-6% value that was found in patients with a negative H*1 blast flag. These results suggest that the H*1 system is a highly sensitive tool for the detection in peripheral blood of even small concentration of leukaemic cells, which escape morphological identification.
Acute Disease↗
Antithrombin III Pescara: a defective AT III variant with no alterations of plasma crossed immunoelectrophoresis, but with an abnormal crossed immunoelectrofocusing pattern.
An abnormal AT III variant was found in five members from a family where a high incidence of thromboembolism occurred. In all the affected subjects AT III antigen concentration was normal, whereas antithrombin and antifactor Xa progressive activities as well as heparin cofactor activities were low. Crossed immunoelectrophoresis performed either in absence or in presence of heparin showed a normal plasma pattern. Further chromatographic investigations showed a normal affinity to heparin. An abnormal plasma pattern was evidentiated by crossed immunoelectrofocusing throughout all the AT III pH range. These data are consistent with the presence of an abnormal AT III variant with a defective binding to serine proteases and clearly identifiable only by crossed immunoelectrofocusing. This variant appeared different from the other qualitative AT III defects so far described and was named 'Antithrombin III Pescara'.
Adolescent↗
Aggressive chemotherapy in the treatment of subacute myelomonocytic leukaemia with skin infiltration.
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Aged↗
Neonatal diagnosis of antithrombin III congenital defect in a premature newborn.
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Antithrombin III Deficiency↗
Effect of oral anticoagulant treatment on plasma and serum antithrombin III: a study on 172 patients at different levels of anticoagulation.
Antithrombin III (AT III) functional levels are much lower in serum than in plasma; during oral anticoagulation this difference is reduced. Plasma and serum of 172 patients taking vitamin K antagonists were tested for AT III antigen and both AT III heparin cofactor and anti-Xa heparin cofactor. Crossed immunoelectrophoresis of AT III on heparin-agarose was also carried out in plasma and serum. The patients were divided into four groups: (1) international normalized ratio (INR) 9.3-4.1, n = 25; (2) INR 4.0-2.5, n = 73; (3) INR 2.4-2.0, n = 40, and (4) INR 1.9-1.5, n = 34. 66 healthy subjects were used as controls. Plasma levels of AT III antigen, AT III heparin cofactor, and anti-Xa heparin cofactor were the same in all groups. In all groups all serum AT III parameters were higher than in controls; crossed immunoelectrophoresis of AT III on heparin-agarose indicated that this finding was due to a lower formation of complexed AT III in serum. AT III heparin cofactor serum values were the same whatever the INR over a large range (9.3-1.5); the highest anti-Xa heparin cofactor serum levels were noted in the groups treated more intensely (groups 1 and 2).
Administration, Oral↗
Discrimination and spectral resolution of ultradian and circadian periodicities for 24-hour blood pressure patterns on sodium manipulation in normotensive humans.
The effects of a severe (10-20 mEq/24-h) but short-term (7-days) restriction in dietary sodium on 24-h blood pressure (BP) patterns were investigated in 20 normotensive volunteers (10 men, 10 women; 20-25 years old) by means of non-invasive automatic sphygmomanometric monitoring (recordings at 1-h interval) at home, with subjects in bedrest. Statistical methods used include bivariate Gaussian distribution analysis for systolic and diastolic BP bivariate discriminant analysis, periodic regression analysis with a 24-h period (cosinor method) and spectral analysis of time series. Casual BP measurements were unable to detect the tensinogenic effects, while the bivariate Gaussian analysis documented a total rearrangement in the pattern distribution to show discriminated values of BP after sodium restriction. A significant decrease (p less than 0.01) in the 24-h mean level (mesor) for systolic BP was observed. An increase in the extent of fluctuation (amplitude) for the circadian components was documented by the spectral analysis, mainly in diastolic BP patterns. Such a phenomenon of 'amplitude magnification' was not accompanied by a change in the repartition of the ultradian and circadian harmonic components. The extensive changes in the time structure support the hypothesis of an interaction of sodium regimen with the centrally-located oscillators which physiologically organize the circadian rhythmicity of BP.
Adult↗
Spinal fluid procoagulant activity in leukaemic patients treated with intrathecal methotrexate.
A procoagulant activity was found in cerebrospinal fluid (CSF) of patients with myelo- or lymphoproliferative diseases on intrathecal therapy with methotrexate, independently of leukaemic CNS involvement. This activity did not correlate with the cell count in CSF and disappeared on storage at -40 degrees C or after filtration with 0.22 nm filters. Dosage of coagulation factors revealed a strong increase in Factor V activity (F. V:C), an increase in Factor VIII procoagulant activity (F. VIII:C) without a correspondent increase in Factor VIII related antigen (F. VIII R:Ag), and an inconstant increase in Factor IX activity (F. IX:C). These activities all disappeared after filtration with 0.22 micron filters but not with 1.2 micron filters. It is concluded that complexes formed by membrane phospholipids and Factor V were responsible for the procoagulant activity lost after storage. The F. VIII and F. IX.-like procoagulant activity was not lost after storage; it was considered unspecific and attributed to thromboplastin-like substances.
Adult↗
Cerebrospinal fluid protein findings during prophylaxis and treatment of neuromeningosis in leukaemic patients.
Methotrexate and Aracytin commonly employed in prophylaxis and treatment of leukaemic neuromeningosis, can induce neuromeningeal injury. Cerebrospinal fluid (CSF) protein disturbance may reflect the damage. In this study we have examined 141 CSF samples collected by lumbar puncture from 51 subjects: 15 normal controls and 36 leukaemic patients during the prophylaxis or the Central Nervous System (CNS) involvement treatment. In all the CSF samples, total proteins, IgG and IgA were examined. CSF total protein and albumin levels increased in the presence of leukaemic neuromeningosis and also after a prolonged CNS prophylaxis or therapy. The same behaviour was found in IgG levels. The IgA levels did not show any modification. We conclude that these parameters are not reliable indices of leukaemic CNS involvement.
Adult↗
Antithrombin III loss in patients with nephrotic syndrome or receiving continuous ambulatory peritoneal dialysis. Evidence of inactive antithrombin III in urine of patients with nephrotic syndrome.
Antithrombin III (AT III) was measured as antigen (Ag) and as heparin cofactor (HC) in plasma and urine or dialysate from nine patients with nephrotic syndrome and nine patients receiving continuous ambulatory peritoneal dialysis (CAPD), respectively. Crossed immunoelectrophoresis on heparin-agarose (H-CIE) and crossed immunoelectrofocusing (CIEF) runs were carried out on plasma and urine or dialysate samples. AT III plasma levels of the patients receiving CAPD were in the normal range, whereas levels in the patients with nephrotic syndrome showed a significant reduction. Nevertheless the AT III Ag daily loss was the same in both patient groups, so that an additional AT III loss caused by renal metabolism was suggested in patients with nephrotic syndrome. No alteration in the isoantithrombin plasma distribution was found in any patient. The AT III recovered in urine was almost all inactive, as demonstrated by the quantitative assays and by the H-CIE runs; on the contrary, the findings obtained by functional assays, H-CIE, and CIEF runs on dialysate samples failed to demonstrate any major alteration in the AT III molecule. In urine the AT III CIEF pattern displayed a more acid distribution (pH 4.9 to 4.5) in respect to the plasma AT III (pH 5.2 to 4.6); this pattern was suggested to be related to the renal AT III functional inactivation, whose exact mechanism remains to be clarified.
Adult↗
[Cefotiam, a new cephalosporin. Microbiological research, preliminary evaluation of its effect on phagocytosis and clinical multicenter research].
After a brief review of the data on cefotiam in the literature the report presents the results of microbiological research, a preliminary study into the drug's possible actions on phagocytosis and a polycentric clinical study of 93 cases of broncho-pleuro-pulmonary pathology and one sinusitis of the jaw. In vitro cefotiam was found to have an excellent inhibitory effect on gram positive and gram negative bacteria with MICs50 and 90 respectively 0.2 and 0.8 mcg/ml V. Staph. aureus, Str. pyogenes. E. Coli, K. pneumoniae and Pr. mirabilis. A dose-dependent increase in phagocytosis was noted. The clinical response was excellent with 90.43% (88/94) of the cases achieving clinical and radiological cure or very much improved. Cefotiam was very well tolerated with the appearance of 2/94 skin rashes (2.12%). The liver and kidney parameters showed no change at the end of treatment. No increase in enzymuria was noted during treatment with cefotiam.
Absorption↗
Ovarian granulocytic sarcoma with central nervous system involvement.
The authors describe 2 cases of acute myeloid leukemia (AML) with ovarian granulocytic sarcoma (GS) and central nervous system (CNS) involvement. The patients had an unfavorable clinical course in a short period of time. It has been reported that GS or CNS involvement does not have a bad prognostic significance. We suggest that the association of these two complications worsens the prognosis of AML.
Adult↗
[Histomorphological study of the relation between mast cells and liver disease].
In order to report the eventual role of mastzellen in hepatic pathology, we recorded the histological count on 75 sections of transcutaneous hepatic biopsies. We observed that there is no difference between the number and the localization of the mastzellen of normal subjects and those of nonevolving pathologies (Chronic Persistent Hepatitis, Portal Fibrosis, Steatosis). We saw consisent increase in number in the evolving pathologies and in particular near the collagenopoietic cytolitic foci, indicating a possible role of these elements in inflammation and in the production of collagen in liver diseases.
Biopsy↗
[Neurological complications of acute myeloid leukemia in adults].
Neurological involvement in acute myeloid leukaemia has become more common in recent years. The increase seems to be related to the longer survival rates made possible by more intensive treatment protocols. The predictive elements appear to be the tumour mass, cytomorphological variety M5, splenomegaly and serum LDH. Prophylaxis with craniospinal radiotherapy or spinal chemotherapy does not modify the course of the leukaemia or diminish the frequency of neuromeningeal complications. Six cases of AML involving the central nervous system were examined, two at onset and 4 at first relapse. Neuromeningeal complications are to be feared since they are extremely difficult to eradicate completely and the prognosis is extremely unfavourable especially if a bone marrow relapse occurs simultaneously.
Adolescent↗
OKT4/OKT8 ratio and serum beta 2-microglobulin in mycosis fungoides and chronic benign dermatitis.
Pre-treatment Serum Beta 2-microglobulin (S B2-m) and OKT4/OKT8 Ratio (T4/T8 R) were studied in 15 patients with Mycosis Fungoides (MF) and in 10 subjects with Chronic Superficial Benign Dermatitis (CSBD) in order to verify whether these parameters may lend support to an earlier differential diagnosis. S B2-m levels and T4/T8 R showed no significant difference in CSBD as compared to normal controls. MF patients displayed elevated S B2-m and T4/T8 R values in comparison to healthy controls and subjects suffering from CSBD (P less than 0.001). After photochemotherapy (PUVA), markedly decreased S B2-m and T4/T8 R values were observed in all patients but two who proved to be unresponsive to PUVA treatment. On the basis of reported data, S B2-m and T4/T8 R can be regarded as an additional tool to discriminate CSBD and MF when clinical and histological features are not significantly diagnostic. Finally, these parameters seem to provide reliable information in monitoring response to treatment.
Adult↗