Hereditary renal amyloidosis associated with a novel mutation in the apolipoprotein AII gene.
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Adult↗
Biomedical subjects
E J Fernández
Publications and source records attributed to E J Fernández.
[Treatment of acute lithium intoxication with high-flux haemodialysis membranes].
Lithium carbonate is commonly prescribed for the treatment of bipolar (manic-depressive) disorders. However, because of its narrow therapeutic index an excessive elevation of serum lithium concentration, either during chronic maintenance therapy or after an acute overdose, can result in serious toxicity. In addition to supportive care, the established treatment of severe lithium toxicity is haemodialysis. Conventional haemodialysis can reduce serum lithium rapidly, but post-dialysis rebound elevations with recurrent toxicity have been documented in old publications. High-flux membranes should be capable of removing more lithium per hour of haemodialysis, but published values are not available. We report here three patients with acute lithium intoxication who were treated successfully with bicarbonate and high-flux haemodialysis membranes. Our patients presented with a severe degree of intoxication, based on the amount of drug ingested, the initial serum lithium level, the severity of neurologic symptoms and systemic manifestations. Two patients developed acute renal failure probably as a result of volume depletion since it was rapidly reversible by haemodialysis and infusion therapy. In addition, consecutive haemodialysis sessions and improvement of renal function allowed a rapid decrease in serum lithium levels without haemodynamic instability or rebound elevations in lithium concentration. The effectiveness of the procedure in these cases can be attributed to the use of bicarbonate dialysate and high-efficiency dialysers. This is the first report describing the effect of high-efficiency dialysers on lithium pharmacokinetic. Using this technique the elimination rate of lithium was found to be greater than previously reported with haemodialysis.
Acute Disease↗
[Arachnoid cysts in autosomal dominant polycystic kidney].
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Adult↗
Adaptive-optics ultrahigh-resolution optical coherence tomography.
Merging of ultrahigh-resolution optical coherence tomography (UHR OCT) and adaptive optics (AO), resulting in high axial (3 microm) and improved transverse resolution (5-10 microm) is demonstrated for the first time to our knowledge in in vivo retinal imaging. A compact (300 mm x 300 mm) closed-loop AO system, based on a real-time Hartmann-Shack wave-front sensor operating at 30 Hz and a 37-actuator membrane deformable mirror, is interfaced to an UHR OCT system, based on a commercial OCT instrument, employing a compact Ti:sapphire laser with 130-nm bandwidth. Closed-loop correction of both ocular and system aberrations results in a residual uncorrected wave-front rms of 0.1 microm for a 3.68-mm pupil diameter. When this level of correction is achieved, OCT images are obtained under a static mirror configuration. By use of AO, an improvement of the transverse resolution of two to three times, compared with UHR OCT systems used so far, is obtained. A significant signal-to-noise ratio improvement of up to 9 dB in corrected compared with uncorrected OCT tomograms is also achieved.
Equipment Design↗
Ability of a Au(III)-N unit to bond two aurophilically interacting gold(I) centers.
The monohapto neutral 2-(diphenylphosphino)aniline (PNH(2)) complexes [Au(C(6)F(5))(2)X(PNH(2))] (X = C(6)F(5) (1), Cl (2)) have been obtained from [Au(C(6)F(5))(3)(tht)] or [Au(C(6)F(5))(2)(micro-Cl)](2) and PNH(2), and the cationic [Au(C(6)F(5))(2)(PNH(2))]ClO(4) (3) has been similarly prepared from [Au(C(6)F(5))(2)(OEt(2))(2)]ClO(4) and PNH(2) or from 2 and AgClO(4). The neutral amido complex [Au(C(6)F(5))(2)(PNH)] (4) can be obtained by deprotonation of 3 with PPN(acac) (acac = acetylacetonate) or by treatment of the chloro complex 2 with Tl(acac). It reacts with [Ag(OClO(3))(PPh(3))] or [Au(OClO(3))(PPh(3))] to give the dinuclear species [Au(C(6)F(5))(2)[PNH(MPPh(3))]]ClO(4) (M = Ag (5), Au (6)). The latter can also be obtained by reaction of equimolar amounts of 3 and [Au(acac)(PPh(3))]; when the molar ratio of the same reagents is 1:2, the trinuclear cationic complex [Au(C(6)F(5))(2)[PN(AuPPh(3))(2)]]ClO(4) (7) is obtained. The crystal structures of complexes 2-4 and 7 have been established by X-ray crystallography; the last-mentioned displays an unusual Au(I)-Au(III) interaction.
Journal Article↗
Heteropolynuclear phosphide complexes: phosphorus as unique atom bridging coinage metal centres.
In this paper we describe the synthesis and reactivity of the diphenylphosphine derivatives [Au(C6F5)(PPh2H)] and trans-[Au(C6F5)2(PPh2H)2]ClO4. Reactions of the latter or the neutral [Au(C6F5)3(PPh2H)] with the appropriate Group 11 metal reagents (M = Au, Ag, Cu) in the presence of acetylacetonate afford a series of novel Au(III)-M phosphido-bridged complexes, which have been scarcely represented to date. The crystal structure of the tetranuclear [(Au(C6F5)2(mu-PPh2)2Ag)2] and the dinuclear [Au(C6F5)3(mu-PPh2)M(PPh3)] (M = Au,Ag) complexes were established by X-ray diffraction methods. The synthesis and deprotonating activity of the anionic gold(III) complex PPN[Au(C6F5)3(acac)] (PNN = [N(PPh3)2]+) was studied.
Journal Article↗
Experimental and theoretical studies of the d8-d10 interaction between Pd(II) and Au(I): bis(chloro[(phenylthiomethyl)diphenylphosphine]gold(I))- dichloropalladium(II) and related systems.
The reaction between thioether phosphine gold(I) precursors such as [AuCl(Ph2PCH2SPh)], 1, or [Au(Ph2PCH2SPh)2]CF3SO3 and PdCl2(NCPh)2 affords the new compounds [(AuCl(Ph2PCH2SPh)2PdCl2], 2, and [AuPdCl2(Ph2PCH2SPh)2]CF3SO3, 3. The crystal structure of complex 2 has the sterically unhindered Pd(II) and Au(I) at a distance of 314 pm. Quasirelativistic pseudopotential calculations on [AuPdCl3(PH2CH2SH)(SH2)] models give short Au-Pd distances at the second-order Møller-Plesset (MP2) level and long Au-Pd distances at Hartree-Fock (HF) level. A detailed analysis of the Au-Pd interaction shows dominant dispersion, some ionic contributions, and no net charge transfer between the metals.
Journal Article↗
[Importance of the benzylpenicillin nucleus and the side chain of the beta-lactams. Demonstration by skin tests and RAST in penicillin allergic patients].
We studied 30 patients with beta-Lactams allergy demonstrated by clinical findings. The aim of this work was to determine the capacity of the beta-Lactams nucleus and the side chain in the induction of specific IgE to BPO, Ax, Amp, performed by intradermal skin test and RAST. The patients were divided by clinical manifestations in: 1-Accelerated reactions (n:19); and 2-Immediate reactions (n:11). The Prick tests were performed with BPO-PL, Ax-PL, Amp-PL, MDM-BP, MDM-Ax, MDM-Amp. The accelerated group presented BPO-PL (+) in 2 cases, Ax-PL & Amp-PL (+) in 4 cases, and all of the reactives were (+) in 13 out of 19 cases. The immediate group presented MDM-BP (+) in 10 out of 11 cases and MDM-Amp was (+) in 1 out of 11 cases. The RAST's were performed in all patients(n:30). In accelerated group were (+) to BPO-PL in 13 out of 19 cases, to Ax-PL in 3 out of 19 cases, to Amp-PL in 1 out 19 cases, to BPO-PL and Ax-PL on overlap in 1 out of 19 cases, and 1 case was negative to all reactives. The immediate group presented RAST's negatives in 11 out of 11 cases. The control group(n:20) presented Prick (+) to Ax-PL in 1 out of 20 cases, and the others reactives were negatives in all cases. The RAST's to all reactives were (-) in 20 out of 20 subjects. These results indicate that BPO was the most important determinant, and the side chain of the Ax or Amp were others determinants of the beta-Lactams drugs. These determinants induced specific IgE, and in rare occasions appears specific IgE for two different determinants on overlap in the same patient. The intradermal skin testing is the method of choice to study the penicillin allergies, because non satisfactory RAST's have yet been developed for minor determinant-specific IgE antibodies.
Adult↗
[Radiofrequency treatment of tachyarrhythmias in children and adolescents].
INTRODUCTION: Radiofrequency ablation has been extensively used in adults to treat supraventricular and ventricular tachycardia. In children and adolescents few data are available on its safety and efficacy. METHODS: 28 patients (mean age 12.8) with symptomatic tachyarrhythmias underwent catheter ablation; 21 children had atrioventricular accessory pathways (11 right connections, 9 lef connections and one midseptal pathway), 3 had intranodal tachycardia, 2 had ventricular tachycardia and 2 had atrial tachycardia. Only four patients had associated structural anomalies. RESULTS: Success rate was 71.4% (20 patients). The success per cents in each group were: in intranodal tachycardias 100%; we failed in the two patients with ventricular tachycardias; in accessory pathways 76.1% and 50% in atrial tachycardia. There were no recurrences of arrhythmia in a mean chase period of 24 months (12-46). Major complications were only observed in one patient who developed a Wallenberg syndrome after ablation. CONCLUSIONS: Radiofrequency catheter ablation appears to be a safe and effective method to treat arrhythmias in children and adolescents, which in most cases can supersede surgery. Alow incidence of complications is reported, although long term damage on endocardial structures remains yet to be determined.
Adolescent↗