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Biomedical subjects

G Gobbi

Publications and source records attributed to G Gobbi.

123 records · Page 7Linked to original sources

Benign partial epilepsy with centrotemporal (or rolandic) spikes and brain lesion.

We describe three patients with benign partial epilepsy with centrotemporal spikes (BECT) in association with proven brain lesion (agenesis of the corpus callosum, lipoma of the corpus callosum, and congenital toxoplasmosis, respectively). The age of onset, the ictal signs, the interictal electroencephalographic findings and the favorable outcome of epilepsy even after withdrawal of drug therapy led to the diagnosis of BECT although organic brain lesions were present. In such cases, the epilepsy should not be ascribed to the lesions but should be considered benign even though fortuitiously associated with brain lesions.

Action Potentials↗

Carnitine disposition before and during valproate therapy in patients with epilepsy.

Free and total carnitine and acylcarnitine in plasma and urine samples was measured in 22 epileptic patients before and after 15 and 45 days of valproate (VPA) therapy and in 16 healthy volunteers on a single occasion. Carnitine plasma concentration and renal excretion observed in epileptic patients before VPA therapy did not differ from control values. After VPA was started, free and total plasma concentration decreased significantly (p < 0.05) from 49 +/- 17 to 35 +/- 16 at 15 days and to 35 +/- 13 nmol/ml at 45 days of therapy (free carnitine) and from 60 +/- 18 to 50 +/- 18 at 15 days and to 55 +/- 14 nmol/ml at 45 days of therapy (total carnitine), whereas acylcarnitine increased significantly (p < 0.05) from 10 +/- 8 to 14 +/- 8 at 15 days and to 18 +/- 16 nmol/ml at 45 days of therapy. Free carnitine urinary excretion decreased significantly (p < 0.05) from 200 +/- 135 to 115 +/- 76 and 118 +/- 75 mumol/24 h, whereas acylcarnitine urinary excretion increased significantly (p < 0.05) from 78 +/- 56 to 154 +/- 98 and 155 +/- 89 mumol/24 h after VPA therapy was started. As a consequence, acylcarnitine renal clearance increased significantly (+30%, p < 0.05) whereas free carnitine renal clearance did not change during VPA therapy. No difference was detected between 15 and 45 days of therapy. No patients experienced symptoms of VPA toxicity. Our results suggest that VPA in patients increases both formation and renal clearance of acylcarnitine.

Acylation↗

Impaired diastolic function in active rheumatoid arthritis. Relationship with disease duration.

OBJECTIVE: Using digitized M-mode and Doppler echocardiography, we evaluated left ventricular (LV) function in 54 patients (43 women and 11 men; mean age 50 years) suffering from active rheumatoid arthritis (RA) without obvious cardiovascular disease, and compared them with 54 age- and sex-matched normal subjects. RESULTS: No differences were found in LV end-diastolic diameter, systolic function and parietal thickness between the patients and controls. However, a significant reduction in various indexes of LV diastolic function was found, including E/A (ratio of early to late filling waves of mitral inflow Doppler) and the peak lengthening rate of the LV diameter (an index of LV relaxation evaluated by M-mode echocardiography). The former was correlated with patient age and was independent of disease duration, while the latter was more markedly correlated with disease duration than with patient age. CONCLUSION: The relationship between diastolic impairment and disease duration in active RA may open new perspectives in the study of RA-associated cardiovascular disease.

Adult↗

Late onset familial Hallervorden-Spatz disease: MR findings in two sisters.

Two sisters affected by late onset Hallervorden-Spatz disease are described. In both patients, MR showed rings of decreased signal intensity surrounding hyperintense areas that gave a target-like appearance to the globi pallidi, a finding that corresponds with the known pathologic lesions in the disease. MR reflects the metabolic and anatomic evaluation of this disease.

Female↗

TNF-related apoptosis-inducing ligand (TRAIL) and erythropoiesis: a role for PKC epsilon.

The regulation of the hematopoietic stem cell pool size and the processes of cell differentiation along the hematopoietic lineages involve apoptosis. Among the different factors with a recognized activity on blood progenitor cells, TRAIL - a member of the TNF family of cytokines - has an emerging role in the modulation of normal hematopoiesis.PKC(epsilon) levels are regulated by EPO in differentiating erythroid progenitors and control the protection against the apoptogenic effect of TRAIL. EPO-induced erythroid CD34 cells are insensitive to the apoptogenic effect of TRAIL between day 0 and day 3, due to the lack of specific surface receptors expression. Death receptors appear after day 3 of differentiation and consequently erythroid cells become sensitive to TRAIL up to day 9/10, when the EPO-driven up-regulation of PKC epsilon intracellular levels inhibits the TRAIL-mediated apoptosis, via Bcl-2. In the time interval between day 3 and 9, therefore, the number of erythroid progenitors can be limited by the presence of soluble or membrane-bound TRAIL present in the bone marrow microenvironment.

Animals↗

[Sebaceous linear nevus syndrome with hemimegalencephaly. Report of a case].

The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.

Cerebral Cortex↗

Internal 131Cs dosimetry.

Because of the growing interest in 131Cs as a tracer for myocardium and thyroid scintigraphy, its internal disometry in man was reviewed and determined. The absorbed dose was calculated using the method of Loevinger and Berman, with minor modifications, i.e. taking into account the different activities of the body organs and their contribution to the absorbed dose. Special attention was also paid to the softer and non-penetrating radiations, namely L and M, capture events and Auger electrons, in relation to the decay of the nuclide. Dosimetric data for the thyroid were determined experimentally.

Cesium Radioisotopes↗