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Biomedical subjects

L Santoro

Publications and source records attributed to L Santoro.

At least 127 records · Page 7Linked to original sources
PubMed1992

[Microcirculatory changes in mitral prolapse as an expression of a systemic change in the connective tissue].

Many authors hypothesize that mitral valve prolapse (MVP) can be, in most cases, only a clinical sign of a primitive and systemic disorder of the connective tissue, like in Marfan Syndrome (MS). In our previous works we supported the presence of morphological and functional alterations of the microcirculation in patients affected by MS. In order to characterize a possible common denominator between these pathologies we have studied the cutaneous microcirculation in a group of patients affected by MVP, divided into 2 groups: anatomic MVP (MVP) and MVP syndrome (MVPS). The morphologic parameters have been investigated by nailfold capillaroscopy while digital laser-Doppler was used to study skin flowmetry. The results have been compared with a control group. Capillaroscopic remarkers showed an architecturally disorganized microvasculature with aspects related to a reduced compactness of the microvasculature unit with a significatively higher score compared with controls (7.3 +/- 2.9 vs 3.6 +/- 1 p less than 0.0005). Laser-Doppler flowmetry showed a significatively reduced rest flow; ischemic test showed: spike time 48.9 +/- 36.9 vs 15.3 +/- 7.7 s (p less than 0.0005), hyperemic acme 6.6 +/- 2.7 vs 12.5 +/- 8.4 UP (p less than 0.002); % increase 32.1 +/- 20.2 vs 51.5 +/- 15.4 (p less than 0.002). Thermic test showed a significatively higher thermic acme 8.7 +/- 4.2 vs 12.6 +/- 9.11 UP (p less than 0.05). These results appeared to be correlated with stage pathology as it was observed a severe microvasculature disorders in MVPS. Therefore we suppose that a phenotypic continuum may exist between MS and MVP.

Adult↗
PubMed1991

Pitfalls in the determination of absolute dimensions using angiographic catheters as calibration devices in quantitative angiography.

Using catheter outer diameter as a scaling device, quantitative coronary arteriography allows the precise and objective measurement of change in absolute dimensions of coronary arteries after mechanical or pharmacologic intervention. Because of variable density in the wall of the catheter, automated systems might vary in the determination of the outer catheter diameter. To examine this premise, catheters in a variety of French sizes from 6 manufacturers were injected with radiographic contrast and used as scaling devices for arterial phantoms of known geometric dimension. Radiographic diameters of the catheters were determined by applying the quantitative coronary arteriographic algorithm to the catheters using a calibration grid in the same field of view. The varying composition of the catheters resulted in differing x-ray attenuation and, subsequently, automated edge-detection algorithms varied widely in determining the actual catheter diameter to be used as a scaling factor. For instance, a Lucite "artery" with a minimal luminal diameter of 1.50 mm (image calibrated using the micrometer-determined outside diameter of a Baxter 8Fr guiding catheter) resulted in a quantitative angiographic diameter of 2.03 mm (overestimation by 35%). If the diameter of a similar size Shiley catheter was used to calibrate the image, a luminal diameter of 1.60 mm was determined: a difference of 0.43 mm based solely on differences in scaling catheter attenuation. These data suggest that a specific "fingerprint" for each catheter material and catheter French size exists, rendering generalizations about catheter size questionable. These observations are important for quantitative angiography where many brands and sizes of angiographic catheters are being used clinically.(ABSTRACT TRUNCATED AT 250 WORDS)

Calibration↗
PubMed1991

Production and characterization of two immunotoxins specific for M5b ANLL leukaemia.

We describe the production and functional characterization of 2 monocytic-cell-lineage-specific immunotoxins constructed with saporin emitoxin (SAP) from Saponaria officinalis. Interest in the production of these immunotoxins, of possible clinical relevance, has been raised by the availability of 2 MAbs of high specificity for circulating monocytes and M5b ANLL, thus envisaging their potential use in bone-marrow purging. SAP emitoxin was selected on the basis of the low cytotoxicity in unconjugated form, as opposed to highly specific cytotoxicity and favourable pharmacokinetical properties in the conjugated form. SPDP conjugation produced immunotoxins which retained serological specificity and protein-synthesis-inhibitory activity. The 2 immunotoxins did not interfere with bone-marrow progenitor-cell growth in a CFU-GM colony assay. On the contrary, they were capable of killing monocytic cells selectively, as demonstrated in phenotypical and functional assays. Thus these 2 novel immunotoxins appear to be promising reagents in purging autologous bone marrow prior to transplantation in patients suffering from monocytic leukaemia.

Antibodies, Monoclonal↗
PubMed1991

Intrafamilial phenotype variation in Friedreich's disease: possible exceptions to diagnostic criteria.

Three families are described which include members with "typical" Friedreich's disease (FD) and others who are ataxic but do not satisfy all the diagnostic criteria for that disease. In family A two patients have an early-onset, rapidly progressive FD, while two others have a late-onset, more benign form. In families B and C one member has "typical" FD, and another has a similar ataxic syndrome, except for preservation of knee jerks. Laboratory evaluation is consistent with the diagnosis of FD in all cases. FD diagnosis appears justified in secondary cases with late onset or preserved tendon reflexes, provided that the index case fulfils all diagnostic criteria. Whether the diagnosis of FD is tenable in sporadic "atypical" cases remains to be seen. Echocardiographic and neurophysiological examination may be valuable in classifying such cases.

Adolescent↗
PubMed1991

Motor and somatosensory evoked potentials in hereditary spastic paraplegia.

Motor evoked potentials (MEPs) from the arms and legs to transcranial stimulation of the motor cortex and somatosensory evoked potentials (SSEPs) from stimulation of the nerves of the arms and legs, were recorded in 11 patients with hereditary spastic paraplegia. Electrophysiological abnormalities were found to be distributed differently among the systems examined; the longer the pathway, the higher the incidence and severity of impairment. MEPs from the leg were either absent or clearly reduced or prolonged in all patients. Eight patients showed abnormal cortical SSEPs on stimulation of the leg (absent or reduced responses in four, slowed central conduction velocity in seven), but only two of these patients had abnormal MEPs from the arm (absent responses). Cortical SSEPs on stimulation of the median nerve were reduced in two patients. Mean values of amplitude and central conduction velocity for MEPs and SSEPs from the leg were significantly different between patients and controls. Such differences were not found for either MEPs or SSEPs from the arm. This distribution of abnormalities, which suggests a differential involvement of the spinal pathways, parallels the reported pathological pattern in which degeneration of axons is more common and severe in the motor and sensory fibres supplying the leg.

Adolescent↗
PubMed1991

Pyridoxine neuropathy. A four-year electrophysiological and clinical follow-up of a severe case.

In a 54-year-old male a severe sensory neuropathy was observed during treatment for pulmonary tuberculosis with isoniazid (400 mg/day) and pyridoxine (600 mg/day). Eight months after withdrawal from isoniazid the sensory symptomatology was still progressing, although muscle strength was never reduced. A sural nerve biopsy revealed marked loss of large myelinated fibres. Only when pyridoxine treatment was interrupted did a slow improvement begin. A clinical and electrophysiologic follow-up showed a very slow and still incomplete recovery after four years. The possibility of an unusual individual susceptibility to toxic effects of pyridoxine is considered.

Electrophysiology↗
PubMed1990

Electrophysiological and histological follow-up study in 15 Friedreich's ataxia patients.

A clinical and electrophysiological follow-up was carried out for 3 to 7 years on 15 patients with Friedreich's ataxia (FA). Sural nerve biopsy was performed once in all patients, and a second time 6-7 years later in three of them. Clinical worsening and progression of disturbance were evaluated according to IAP and IACR scales. Sensory orthodromic conduction along median and tibial nerves was typical of FA and did not change between first and last examinations, nor were there morphological changes between the first and the second sural nerve biopsies. Peripheral nerve involvement is thought to be a result of defective development of the largest neurons and to remain stable from a very early stage of the disease; the clinical worsening may then be due to a progressive involvement of the pyramidal tracts and the cerebellar pathways.

Adolescent↗
PubMed1990

A family with tomaculous neuropathy mimicking Charcot-Marie-Tooth disease.

The appearance of Guillain-Barré syndrome in a 9-year-old girl led to the detection of a hereditary neuropathy in her family. This neuropathy showed clinical and electrophysiological characteristics of Charcot-Marie-Tooth disease. Only nerve biopsy performed in a sister of the proband allowed diagnosis of tomaculous neuropathy which presented unusual clinical, electrophysiological and bioptic aspects.

Biopsy↗
PubMed1990

Clinical and genetic heterogeneity in early onset cerebellar ataxia with retained tendon reflexes.

A clinical and genetic study was performed on 20 patients affected by early onset cerebellar ataxia with retained tendon reflexes (EOCA). Mean age at onset was 8.8 (SD 6.0) years. The frequency distribution of age of onset significantly differed from the normal distribution. Consanguinity rate was 16.7% and segregation ratio 0.164. As well as ataxia, which was a constant feature, there were signs of involvement of the cortico-spinal tracts and/or peripheral nerves in most patients. Results of neurophysiological studies were not homogeneous, nor were morphological findings of the sural nerve biopsy. The data suggest that EOCA may be genetically and clinically heterogeneous.

Adolescent↗
PubMed1989

Late onset recessive ataxia with Friedreich's disease phenotype.

The Quebec Cooperative Study on Friedreich's ataxia required an onset before age 20 as an obligatory criterion of Friedreich's disease (FD). Harding included patients with onset before 25 years. We studied nine patients with FD phenotype but with onset ranging from 21 to 29 years (mean 24.4). Statistical analysis of the distribution and intrafamilial variation of onset age suggests that late onset Friedreich's disease (LOFD) is a distinct genetic entity or results from modifying genes in some families. Scoliosis was less common in LOFD than FD patients but otherwise the clinical picture was similar.

Adolescent↗
PubMed1987

Covalently bound pyruvate in phosphopantothenoylcysteine decarboxylase from horse liver.

Horse liver phosphopantothenoylcysteine decarboxylase (EC 4.1.1.36) incorporates nonexchangeable tritium from borotritide with a decrease of the activity. Substrate prevents both tritium incorporation and the decrease in activity. Acid and base hydrolysis of the tritiated protein releases labeled lactate identified by high-voltage paper electrophoresis, paper chromatography and silicic acid chromatography. These results indicate the presence of pyruvate covalently bound through an ester linkage to phosphopantothenoylcysteine decarboxylase which is then another example of a mammalian enzyme in which pyruvate is involved in a catalytic activity.

Amino Acid Transport Systems, Neutral↗
PubMed1987

Two murine monoclonal antibodies to peripheral blood monocyte differentiation antigens discriminate within M5 acute non-lymphoid leukemia (ANLL) cells.

Two murine monoclonal antibodies (MoAbs), LAM3 and LAM7 of the IgG1 isotype, which were produced by immunization with normal peripheral blood monocytes (PBM), were assayed in their specificity by indirect immunofluorescence against a panel of normal as well as leukemic cells. Both LAM3 and LAM7 were reactive with PBM while LAM3 also recognized platelets. Neither MoAb showed reactivity with erythrocytes, granulocytes, or resting and mitogen activated B and T lymphocytes. The reactivity with bone marrow cells correlated with the degree of monocyte contamination. Among the 62 cases of leukemia tested, which included three cases of B-CLL, 19 cases of ALL, and 40 cases of ANLL, both MoAbs reacted highly homogenously only with M5b ANLL cells. These findings indicate that the two MoAbs, which recognize two distinct epitopes, represent useful markers in the differential diagnosis of M5b ANLL.

Acute Disease↗
PubMed1987

A murine monoclonal antibody strictly reactive with human peripheral blood monocytes.

We have produced and characterized a novel murine monoclonal antibody (LAM7) of IgG1 isotype which appears specific for peripheral blood monocytes (PBM) on the basis of histochemical and functional studies. By indirect immunofluorescence, including FACS analysis, the antibody reacts with 90 +/- 6% of PBM and with monocytic leukemias, while it is totally unreactive with B and T lymphocytes, platelets, granulocytes, peripheral macrophages, dendritic cells, large granular lymphocytes, and nonmonocytic leukemias. The antigen-presenting capacity of peripheral blood mononuclear cells is abolished by treatment with MoAb LAM7 in an antiglobulin-complement-mediated cytotoxicity test, and restored by addition of purified PBM. The progressive disappearance of the antigen recognized by LAM7 from PBM within approximately 3 days in culture, and its absence from both bone marrow precursors and tissue macrophages, define it as a line-specific and stage-specific differentiation.

Animals↗
PubMed1987

A case of Dejerine-Sottas disease with prominent ataxia and brain stem involvement. A clinical, electrophysiological, otoneurologic, and ultrastructural study.

A case is presented of Dejerine-Sottas disease in a 12-year-old boy in which clinical signs made diagnosis of Friedreich's ataxia seem plausible. Based on marked slowing of motor conduction velocity, the sural nerve biopsy findings of a hypertrophic neuropathy with hypo- and demyelination of the nerve fibres, as well as the clinical history, the diagnosis of Dejerine-Sottas disease was made. ABR examination suggested involvement of brain stem at the roots and/or nuclei of the eighth cranial nerve, without involvement of higher structures.

Adolescent↗