Angioma en penacho: A propósito de un caso clínico / Tufted angioma: A case report

Se presenta el caso de una paciente de un año de edad, con una lesión tumoral congénita en dorso, eritemato-violácea. Se evidencia al cabo de un año durante el control evolutivo, aumento del volumen y cambio de coloración, por lo que se decide su exéresis completa. El examen histopatológico de la pieza concluyó con el diagnóstico de angioma en penacho (AP). El AP es un tumor vascular benigno, poco frecuente. Aparece sin predilección racial y es igual en ambos sexos. Puede ser congénito o adquirido en la primera infancia, ocasionalmente se presenta en la edad adulta. Su patogenia está poco dilucidada.

Presented the case of a patient of one year old with a congenital, erythematous-violaceous tumoral lesion on back. During the control evolutionary is evidence after a year increase of the volume and change of coloration by what is decides his removal complete. The histopathological examination of the piece ended with a diagnosis of tufted angioma (TA). The TA is a rare, benign vascular tumor. Appears no predilection racial and is equal in both sexes. It can be congenital or acquired in early childhood, it occurs occasionally in adulthood. Its pathogenesis is shortly to become.

Accuracy of MRI criteria for dissemination in space for the diagnosis of multiple sclerosis in patients with clinically isolated syndromes.

The MRI Barkhof-Tintoré criteria have proved to be highly specific for predicting conversion to clinically definite multiple sclerosis in patients with clinically isolated syndromes (CIS), but lacked an optimal sensitivity. In order to improve the accuracy of early multiple sclerosis diagnosis, new imaging criteria have been proposed by Swanton et al. We aimed to evaluate the accuracy of both MRI criteria for dissemination in space to predict conversion from CIS to clinically definite multiple sclerosis. We studied 79 CIS patients with baseline MRI performed within the first 3 months after onset. The sensitivity and specificity of both MRI criteria to predict conversion to clinically definite multiple sclerosis were analysed. The time to develop clinically definite multiple sclerosis from CIS onset, according to each imaging criteria, was studied by Kaplan-Meier survival curves. The overall conversion rate was 75.7% with a median follow-up of 57 months. Barkhof- Tintoré's criteria showed a sensitivity of 71.9% and a specificity of 77.2%. Swanton's criteria had a sensitivity of 91.2% and a specificity of 68.1%. Both MRI criteria identified CIS patients with higher risk and faster conversion to clinically definite multiple sclerosis. Swanton's criteria are simpler and more sensitive than Barkhof-Tintoré's criteria, with a slight decrease in specificity. These results reinforce their use in multiple sclerosis diagnosis.

Ischemia induces a selective biphasic response in brain mitochondrial mRNA levels.

We determined the independent effects of hypoxia, glucose deprivation and ischemia (hypoxia plus glucose deprivation) on steady-state levels of mRNA coding for specific nuclear and mitochondrially encoded enzymes of oxidative metabolism in cultured rat neurons and glia. Neither hypoxia nor low glucose alone changed steady-state message levels for any transcript. However, ischemia induced a biphasic effect on mitochondrially encoded transcripts for cytochrome oxidase subunit two (CO2) and the subunits 8 and 6 of ATPase (A 8/6), initially decreasing and then increasing mRNA levels to or above the levels recorded prior to ischemia. In contrast, three nuclear encoded transcripts for mitochondrial proteins were decreased by ischemia. These data demonstrate a lack of coordination between the expression of nuclear and mitochondrial genes in the initial response to ischemia and suggest that a selective, primary reaction to brain cell insults exists within the mitochondrion.

Regulation of brain glucose transporters by glucose and oxygen deprivation.

Brain cells are dependent on glucose and oxygen for energy. We investigated the effects of hypoxia, glucose deprivation, and hypoxia plus glucose deprivation on mRNA and protein levels of glucose transporter (GLUT1) and GLUT3 and 2-deoxyglucose (2-DG) uptake in primary cultures of rat neurons and astroglia. Hypoxia for 24 hours did not significantly affect cell viability but increased neuronal GLUT1 and GLUT3 mRNA up to 40-fold and fivefold, respectively, above control levels. Similar changes in GLUT1 mRNA were measured in glia. The effects of hypoxia on GLUT1 and GLUT3 mRNA were reversible. The increase in GLUT1 mRNA could be detected within 20 minutes of hypoxia and was blocked by actinomycin D. Nuclear runoff transcription assays showed that hypoxia did not alter the transcription rate of GLUT1. However, hypoxia enhanced the stability of GLUT1 mRNA in neurons (half-life [t(l/2)] > 12 hours) compared with normoxic conditions (t(1/2) approximately 10.4 hours), suggesting the existence of a posttranscriptional mechanism for the regulation of GLUT1 transcript levels. Twenty-four hours of normoxia and 1.0 mmol/L glucose increased neuronal GLUT1 mRNA less than threefold above basal, but 24 hours of glucose and oxygen deprivation increased GLUT1 over 111-fold above basal. Induction of neuronal GLUT1 mRNA was temporally associated with increased levels of GLUT1 protein and with stimulation of intracellular 2-DG accumulation. We conclude that hypoxia reversibly increases the transcript levels of GLUT1 and GLUT3 in rat brain cells and stimulates GLUT1 transcript levels by posttranscriptional mechanisms. Although glucose deprivation alone produces minimal effects on GLUT mRNA levels, hypoxia plus glucose deprivation synergize to markedly increase GLUT gene expression.

Lipoprotein-X fails to inhibit hydroxymethylglutaryl coenzyme A reductase in HepG2 cells.

Abnormalities of lipid composition and metabolism are frequently observed in patients with cholestatic liver disease. Both elevated low-density lipoprotein (LDL) levels and the appearance of lipoprotein-X (LP-X) in plasma underlie the high incidence of hypercholesterolemia in this population. We tested the hypothesis that the hypercholesterolemia of cholestasis may reflect a failure of normal feedback regulation of hepatic cholesterogenesis by determining the influence of LP-X on the rate-limiting enzyme of cholesterol synthesis, hydroxymethylglutaryl coenzyme A (HMG CoA) reductase. Cultured human hepatoma (HepG2) cells were incubated in purified lipoprotein for 24 hours, harvested, and then assayed for HMG CoA reductase activity and mass. LDL isolated from either normal controls or patients with cholestasis decreased reductase activity in a dose-dependent fashion (2 to 30 micrograms cholesterol/mL media) to a level approximately 50% of that measured in cells incubated in lipid-deficient serum. LP-X failed to downregulate enzyme activity compared with LDL, with little change in reductase activity at cholesterol concentrations (30 micrograms/mL media) that produced maximal reductase inhibition by LDL. Three distinct LP-X subspecies were purified from the plasma of a patient with primary biliary cirrhosis (PBC) and tested in an analogous manner. All LP-X subspecies were similar in their inability to decrease reductase activity as compared with LDL. HMG CoA reductase mass was increased approximately twofold in cells incubated with LP-X, as estimated by Western blot analysis. These results suggest that LP-X may contribute to hypercholesterolemia in the cholestatic patient by not effectively downregulating hepatic cholesterol synthesis.

[Type 4 echovirus meningitis in childhood. Epidemiologic and clinical aspects apropos of an epidemiologic outbreak]. / Meningitis por echovirus tipo 4 en la infancia. Aspectos epidemiológicos y clínicos a propósito de un brote epidémico.

In the first half of 1988 we observed 13 cases of lymphocytic meningitis from which echovirus type 4 was isolated in the CSF. We found a higher infection rate in the 6-9 years old group (54%) and in the masculine sex (69%). Almost all of the cases occurred during the springtime. The clinical and CSF characteristics of this epidemic do not differ much from those that have appeared in the literature. Throughout 1988, in our hospital, 46 cases of lymphocytic meningitis took place and in 20 of them an etiologic diagnosis was done. We think that the introduction of enterovirus cultures can help to know the etiology of many of these cases of meningitis since this virus is responsible for a great part of them.

[Unilateral asterixis associated with anatomic cerebral lesions]. / Asterixis unilateral asociada a lesiones anatómicas cerebrales.

Three patients with unilateral asterixis associated with different vascular lesions of thalamus, basal ganglia and internal and external capsules of contralateral hemisphere are described. Unilateral asterixis is a highly indicative sign of focal cerebral lesion. Its pathophysiology is still unknown. It has been postulated that asterixis is a myoclonic phenomenon resulting from malfunction within neuronal circuits of central nervous system responsible for the active maintenance of posture.