Síndrome de hiper IgE, una enfermedad poco frecuente en edad adulta: Reporte de un caso / Hyper-immunoglobulin E syndrome: Report of one case

The Hyperimmunoglobulin E syndrome (HIES) is a rare sporadic or autosomal dominant immune and connective tissue disorder characterized by chronic eczema, cutaneous abscesses, pneumonias, invasive infections, high levels of Immunoglobulin E, primary teeth retention and bone abnormalities. We report a 24-year-old male with a history of cutaneous abscesses and esophageal candidiasis. He was admitted due to a left gluteal cellulitis. During the fifth day of hospitalization he presented a distal necrosis of the fourth finger of the right hand. Laboratory results showed high levels of IgE and positive cryoglobulins. The patient was discharged and was admitted again five days later with a new gluteal abscess. IgE levels were even higher. Applying Grimbacher scale, the diagnosis of Hyperimmunoglobulin E syndrome was reached.

Catecholamines-evoked cytosolic Ca2+ rise in endothelial cells from bovine adrenal medulla.

The effects of catecholamines on intracellular Ca2+ concentrations ([Ca2+]i) in single acutely dissociated bovine adrenal medulla endothelial cells (BAMECs) were measured using the intracellular fluorescent probe Fluo-3 AM. 100 microm epinephrine or norepinephrine induced a biphasic [Ca2+]i rise with an initial peak followed by a delayed phase. 10 microm phenylephrine (alpha1-adrenergic agonist) caused a [Ca2+]i rise similar to that evoked by catecholamines. The increase in [Ca2+]i induced by 10 microm phenylephrine was reverted by 10 microm phenoxybenzamine (alpha-adrenergic antagonist). Neither isoproterenol (beta-adrenergic agonist) nor clonidine (alpha2-adrenergic agonist) induced [Ca2+]i rise. The initial peak was insensitive to zero external Ca2+ and it was abolished after Ca2+ internal storages were emptied by 10 mM caffeine. The delayed phase was reduced to near zero by external Ca2+ removal. These results indicate that BAMECs possess alpha1-adrenergic receptors associated to both the release of caffeine-sensitive intracellular Ca2+ stores and the entry of extracellular Ca2+. We suggest that chromaffin cell secretion may activate BAMECs in vivo through an increase in [Ca2+]i which could induce the secretion of vasoactive factors allowing a rapid entry of hormones into the circulation.

L- and T-type voltage-gated Ca2+ currents in adrenal medulla endothelial cells.

We investigated voltage-dependent Ca2+ channels of bovine adrenal medulla endothelial cells with the whole cell version of the patch-clamp technique. Depolarization elicited an inward current that was carried by Ca2+ and was composed of a transient (T) current, present in all the cells tested, and a sustained (L) current, present in 65% of them. We separated these currents and measured their individual kinetic and gating properties. The activation threshold for T current was approximately -50 mV, and its maximum amplitude was -49.8 +/- 4.8 pA (means +/- SE, n = 19) at 0 mV. The time constant was 10.2 +/- 1.5 ms (n = 4) for activation and 18.4 +/- 2.8 ms (n = 4) for inactivation. The L current activated at -40 mV, and it reached a plateau at -20.1 +/- 2.3 pA (n = 6). Its activation time course was a single exponential with an activation time contant of 26.8 +/- 2.3 ms (n = 4). Current-voltage curves, kinetics, gating, response to BAY K 8644, nifedipine, amiloride, and different selectivity for Ba2+ and Ca2+ indicated that the underlying channels for the observed currents are only of the T- and L-types that resemble those of the endocrine secretory cells.

Comparison of LR White and Unicryl as embedding media for light and electron immunomicroscopy of chromaffin cells.

LR White and Unicryl are members of the same family of acrylic embedding resins and are very suitable for "on grid" postembedding immunogold labeling. We studied the ultrastructure of LR White- and Unicryl-embedded cultured chromaffin cells and the immunolocalization of three chromaffin cell proteins, the enzymes dopamine-beta-hydroxylase (DbetaH) and tyrosine hydroxylase (TH), and the membrane fusion and Ca2+ channel protein synexin (annexin VII). We report here that Unicryl is preferable to LR White as an embedding medium for electron microscopy when osmium tetroxide fixation is omitted. The basis for this distinction is better ultrastructural preservation and improved immunodetection efficiency.

Design methodology for a multifunctional hand prosthesis.

The first part of this paper reviews different approaches to define the motion of the fingers and the thumb in order to obtain prehension. The last part presents the design of a hand prosthesis based on a new plane of action for the thumb and on proposed design specifications and functional characteristics. The design methodology consists of two steps: the morphology design of the hand prosthesis and the 4-bar mechanism design for each finger. A 3-D computer-aided design (CAD) interactive program was used as a design tool to obtain the hand morphology. This CAD technique was also used to check the geometry, the relative motions of the fingers, and the possibility of interference for the proposed model with two prehension patterns (tridigital and lateral). It is noted that identical flexion angles of the finger joints were obtained for these two prehension patterns, the difference being in the inclination angle of the thumb's plane of flexion. This finding greatly simplified the design of the internal mechanisms of the fingers. CAD was a powerful tool in the design process of this hand prosthesis and will be more and more useful in the future.

Effects of dietary fats, alpha-tocopherol and beta-carotene supplementation on aortic ring segment responses in the rat.

We examined the effects of vitamin supplement on the vascular smooth muscle response of rats fed four different oil diets, after a 20-day feeding period. Male Sprague Dawley rats were fed diets containing 15% of each hazelnut, corn, olive or fish oils, with/without 30 mg/kg beta-carotene and 500 mg/kg dl-alpha-tocopherylacetate. After the feeding period, plasma retinol, alpha-tocopherol, cholesterol, HDL and triacylglyceride concentrations and aortic ring segment responses to KCl (70 mM), phenylephrine and acetylcholine (maximal tension, maximal relaxation and pD2 or -log ED50) were measured. The intake of vitamin-supplemented diets increased plasma alpha-tocopherol levels in rats fed hazelnut or fish oil, while retinol concentration was unaffected. Also, the vitamin supplement counteracted the specific hypercholesterolemic effect of hazelnut oil intake. The addition of a vitamin supplement augmented acetylcholine pD2 values in aortic ring segments of rats fed corn oil (p < 0.001), revealing that arteries were more prone to induced relaxation under this dietary condition. It also lowered the maximal ring tension in response to phenylephrine in rats fed hazelnut or olive oil. These results indicate that the intake of alpha-tocopherol and beta-carotene supplement can modulate the effect of dietary fat type on aortic ring segment responses to pharmacological agents in the rat.

Interactions between Ca2+, PCA50941 and Bay K 8644 in bovine chromaffin cells.

We describe here the effects of PCA50941 (a novel 1,4-dihydropyridine derivative) comparatively with Bay K 8644 on various parameters in bovine adrenal chromaffin cells. The binding of [3H](+)-isradipine to bovine adrenal medulla plasma membranes was inhibited similarly by PCA50941 and Bay K 8644 at various [Ca2+]o suggesting a common binding site for both compounds on the dihydropyridine receptor. In voltage-clamped chromaffin cells PCA50941 (1 microM) and Bay K 8644 (1 microM) shifted the I-V relationship of whole-cell Ca2+ currents by about 5-10 mV towards more hyperpolarizing potentials. At -20 mV, PCA50941 enhanced ICa by 195 +/- 16% and Bay K 8644 by 288 +/- 51%. Stimulation of fura 2-loaded chromaffin cell suspensions with 17.7 K+/0.5 Ca2+ increased 3-fold the basal [Ca2+]i. PCA50941 increased further the K(+)-evoked peak to 655 nM, and Bay K 8644 to 1129 nM. In the presence of 5 mM Ca2+, PCA50941 or Bay K 8644 increased the [Ca2+] peaks to 427 and 350 nM, respectively. PCA50941 potentiated the release of catecholamines from perfused bovine adrenal glands evoked by 30 s pulses of 17.7 mM K+ in a manner dependent on the [Ca2+]o. Thus at 1, 2.5, 5 and 10 mM Ca2+, secretion was 2.3-, 3.8-, 5- and 4-fold greater than in control glands. Bay K 8644 enhanced the K(+)-induced response 3- and 9-fold at [Ca2+]o of 0.25 or 0.5 mM, respectively; at higher [Ca2+]o the potentiation was similar to that of PCA50941.(ABSTRACT TRUNCATED AT 250 WORDS)

La vacuna BCG no modifica las subpoblaciones linfocitarias de lactantes a los 3 meses de edad / The BCG vaccine does not modify lymphocite subpopulations in three months old infants

Los efectos de la vacuna BCG sobre el sistema inmune han dado resultados contradictorios y no se dispone en la actualidad de datos que demuestren fehacientemente su mecanismo de acción. Por estos antecedentes decidimos estudiar las modificaciones de las subpoblaciones linfocitarias T y la respuesta blastogénica en 60 lactantes de 3 meses de edad randomizados en 3 grupos 20 niños vacunados con PPD >8mm (BCG+PPD+), 20 niños vacunados anérgicos al PPD(BCG+PPD-) y 20 no vacunados con PPD negativo (BCG-PPD-). Los siguientes fueron los resultados obtenidos (xñDS) en el recuento de CD3, CD4, CD8 en los diferentes grupos: BCG+PPD+:56ñ10; 42ñ8.6; 33ñ7.3 porciento respectivamente. BCG+PPD-: 49ñ9.6 porciento; 45ñ10.6; 33ñ7.7 porciento. BCG-PPD-: 47ñ7.1 porciento; 40ñ7.4; 30ñ5.3 porciento (p:n.s.). Por su parte los índices de estimulación linfocitaria guardaron estrecha correlación con la respuesta in vivo al PPD. Nuestros resultados permiten concluir que el BCG no modifica los niveles de subpoblaciones linfocitarias T

Effects of chronic ethanol consumption on alpha-adrenergic-induced contractions in rat thoracic aorta.

1. The effects of chronic oral administration of ethanol (EtOH, 80 mM daily during 15-18 days) on the modulation exerted by the endothelium on the contractions induced by phenylephrine (Ph) and clonidine (C) were studied in rat aortic rings with and without endothelium. 2. The maximal contraction induced by a 70 mM KCl depolarizing solution was similar in control and EtOH treated-rings. 3. EtOH pretreatment significantly enhanced the contractile response to Ph in vessels with intact endothelium, but did not significantly affect the response of endothelium-denuded rings. 4. This effect reflects a reduction of the modulation exerted by the endothelial cells on alpha 1-adrenergic vasoconstriction. 5. C did not contract control aortic rings with endothelium, but after EtOH treatment, the response reached about 20% of the maximal KCl-induced contraction. 6. EtOH pretreatment significantly enhanced the contractile response to C both in endothelium-denuded and intact aortic rings. 7. The results indicate that chronic EtOH consumption significantly potentiates alpha-adrenergic-induced contractions in rat aortic rings, probably through interference with the production and/or the release of EDRF.

Modulation of alpha-adrenergic-induced contractions by endothelium-derived relaxing factor in rat aorta.

1. In rat thoracic aorta, endothelium removal produced a significant increase of the maximal contraction (Emax) and of the pD2 value (-log ED50) induced by norepinephrine, phenylephrine and clonidine, and did not affect the maximal contractile response to 70 mM KCl. 2. Clonidine did not induce a contraction in aorta with intact endothelium, but after endothelium removal, the contractile response was 94.8% of the Emax produced by norepinephrine in aorta with endothelium. 3. Pre-incubation with methylene blue (10(-5) M) and hemoglobin (0.02%), which inhibit EDRF effects, produced the same effects as the mechanical removal of endothelium on the contractile responses to alpha-adrenergic agonists. 4. These results suggest that EDRF formation and release is an important factor in the modulation of alpha-adrenergic-induced vasoconstriction.

Influence of extracellular Ca2+ on the modulation of alpha-adrenergic-induced contractions in rat aorta.

1. Rat thoracic aortic rings with and without endothelium incubated in a Ca2(+)-free solution showed a significant reduction of the maximal contraction (E max) induced by norepinephrine (NE) and phenylephrine (Ph), while the contraction induced by a 70 mM KCl depolarizing solution was completely abolished. 2. After Ca2+ removal, pD2 values (-log ED50) for NE and Ph were significantly reduced only in vessels without endothelium. 3. Under control conditions, clonidine (C) induced a contraction only in vessels without endothelium; this response was completely abolished by Ca2+ removal and by nifedipine (10(-8) M). 4. Pre-incubation with nifedipine (10(-8) M) produced an effect similar, although less pronounced, than the removal of Ca2+ in vessels with and without endothelium. 5. Nevertheless, aortic rings with intact endothelium showed a greater reduction of E max than rings without endothelium, suggesting that the endothelial layer may act in synergism with calcium channel blockers to inhibit the contractions induced by alpha-adrenergic agonists. 6. The modulation exerted by the endothelium on alpha 1- and alpha 2-adrenergic-induced contractions in rat aortic rings seems to depend both on extracellular Ca2+ concentration and on the release and action of endothelial relaxing factor(s).

Análisis clínico de 5 casos de intoxicación alcohólica en niños menores de 10 años / Alcohol intoxication in children younger than 10 years: a review of 5 cases

Se analizan cinco casos de intoxicación alcohólica en menores de 10 años egresados del Hospital de Temuco durante 1984-1986. La mayoría de los pacientes son de sexo masculino, residencia rural y la bebida fue ingerida con el consentimiento de los adultos responsables de ellos. En el cuadro clínico se destaca el compromiso de conciencia, hipertonía, trismus y convulsiones, las que sólo en un caso se relacionaron con hipoglicemia. La recuperación del estado psicomotor previa fue muy lenta en los menores de 3 años. Se comenta la necesidad de dar a conocer los peligros de la ingesta etílica infantil y de tener presente el diagnóstico de intoxicación alcohólica ante un paciente con compromiso de conciencia sin causa evidente

Endothelial role in alpha-adrenergic induced vasoconstriction in rat thoracic aorta

The effects of endothelial removal on vasoconstrictor responses elicited by phenilephrine (alpha-1 agonist), clonidine (alpha-2 agonist) and norepinephrine (alpha-1 and alpha-2 agomist) were studied on isolated rat thoracic aorta. The removal of vascular endothelium resulted in a significant reduction of the effective concentration 50% (EC 50) for norepinephrine from 9.12 x 10-9 M, without changes in the maximal response, which remained equal to the maximal concentration elicited by a solution of 70 mM K. Similary, the EC 50 for phenilephrine was significantly reduced from 2.19 x 10-8 M to 9.12 x 10-9 M, without changes in the maximal response. On the other hand, the maximal response to clonidine increased significantly in the arteries without andothelium from 5.5% to 35% of the maximal response to K, and the 50 was significantly reduced from 1.82 x 10-7 M to 1.10 x 10-7 . These result suggest that the presence of vascular endothelium increases the vasoconstriction induced by adrenergic, and that its action is predominantly manifested on alpha-2 dependent contractions, probably because these responses are highly dependent on extracellular calcium influx, which is modulated by the activity of the endothelial derived relaxing factor (EDRF)