Overcoming limits: First ABO incompatible living donor paired kidney transplant in an hypersensitized pediatric recipient in Spain.

INTRODUCTION: HLA sensitization is a growing problem in children awaiting kidney transplantation. In some cases, finding an immunologically compatible donor entails contemplating the option of an ABO incompatible transplant or paired transplant. METHODS: Patient with genetic nephrotic syndrome and progressive chronic kidney disease, with a previous thrombosis of a first kidney transplant, resulting hypersensitized and remaining for a long-time on hemodialysis. Despite a desensitization strategy, family members were incompatible and deceased donation options must be ruled out due to the presentation of donor-specific antibodies (DSA). After 4 years, the possibility arises to perform a kidney paired transplant with a 62-year-old woman with an incompatible blood group. Although the current cytotoxicity- and cell-based crossmatches were negative, history of DSA were recorded. RESULTS: An intensive ABO and HLA desensitization protocol was performed in order to combat the isohemagglutinin antibodies and on the memory-HLA, based on rituximab, apheresis sessions, and immunoglobulins. Despite the donor being older in terms of pediatric transplantation, the donor-recipient weight difference, and immunological risk, the transplant was completed successfully. Maintenance of titration of up to 1/2 was confirmed after 3 weeks post-transplant (IgM and IgG). Kidney biopsy at 2 weeks and 6 months without signs of rejection. The patient is currently 12 months post-transplant and has not presented any signs of transplant rejection and has proper renal function. CONCLUSIONS: Kidney paired transplantation is an excellent solution for hypersensitized children, and ABO incompatibility can be considered to increase their options to find a good donor, without thereby obtaining worse results.

AZATAX: Acetazolamide safety and efficacy in cerebellar syndrome in PMM2 congenital disorder of glycosylation (PMM2-CDG).

OBJECTIVE: Phosphomannomutase deficiency (PMM2 congenital disorder of glycosylation [PMM2-CDG]) causes cerebellar syndrome and strokelike episodes (SLEs). SLEs are also described in patients with gain-of-function mutations in the CaV2.1 channel, for which acetazolamide therapy is suggested. Impairment in N-glycosylation of CaV2.1 promotes gain-of-function effects and may participate in cerebellar syndrome in PMM2-CDG. AZATAX was designed to establish whether acetazolamide is safe and improves cerebellar syndrome in PMM2-CDG. METHODS: A clinical trial included PMM2-CDG patients, with a 6-month first-phase single acetazolamide therapy group, followed by a randomized 5-week withdrawal phase. Safety was assessed. The primary outcome measure was improvement in the International Cooperative Ataxia Rating Scale (ICARS). Other measures were the Nijmegen Pediatric CDG Rating Scale (NPCRS), a syllable repetition test (PATA test), and cognitive scores. RESULTS: Twenty-four patients (mean age = 12.3 ± 4.5 years) were included, showing no serious adverse events. Thirteen patients required dose adjustment due to low bicarbonate or asthenia. There were improvements on ICARS (34.9 ± 23.2 vs 40.7 ± 24.8, effect size = 1.48, 95% confidence interval [CI] = 4.0-7.6, p < 0.001), detected at 6 weeks in 18 patients among the 20 responders, on NPCRS (95% CI = 0.3-1.6, p = 0.013) and on the PATA test (95% CI = 0.5-3.0, p = 0.006). Acetazolamide improved prothrombin time, factor X, and antithrombin. Clinical severity, epilepsy, and lipodystrophy predicted greater response. The randomized withdrawal phase showed ICARS worsening in the withdrawal group (effect size = 1.46, 95% CI = 2.65-7.52, p = 0.001). INTERPRETATION: AZATAX is the first clinical trial of PMM2-CDG. Acetazolamide is well tolerated and effective for motor cerebellar syndrome. Its ability to prevent SLEs and its long-term effects on kidney function should be addressed in future studies. Ann Neurol 2019;85:740-751.

Innovative Method for Coating of Natural Corrosion Inhibitor Based on Artemisia vulgaris.

In this work, the production of a novel methodology for the application of natural corrosion inhibitors on steel, using an autoclave is presented. Tests were carried out using Artemisia vulgaris. The inhibitor was produced with a simple soxhlet extraction process using 15 g of Artemisia vulgaris and 260 mL of Ether. Once the inhibitor was produced, the steel was immersed in it, to form a coating that protects the material against corrosion. Thermogravimetry analyzes (TGA) were performed on the inhibitor, to determine the degradation temperature; it was observed that, at 321 °C, the loss of organic mass begins. After applying the inhibitor to the steel, the Fourier Transform Infrared Spectroscopy (FTIR) technique was used to determine the vibrational bands and the difference between the spectra for the steels before and after the coating was applied. For the evaluation of the method efficiency, Electrochemical Impedance Spectroscopy (EIS) and polarization resistance tests were performed, where Nyquist diagrams and Tafel curves were obtained, for steels with and without treatment. In this case, an increase of 93% in the corrosion resistance, and an 88% decrease in the corrosion rate were observed, proving that this methodology can be used to protect steel against corrosion and extend the steel's useful life.

Realización de un procedimiento con anestesia total intravenosa ordenada por computador, utilizando un programa diseñado en la Universidad de Antioquia / Performing a procedure with total intravenous anesthesia ordered by computer, using a program designed at the University of Antioquia

Una infusión controlada por concentración objetivo, es un sistema guiado por ordenador que permite administrar fármacos de tal manera que se alcance y mantenga una concentración definida en un compartimento tisular, generalmente el plasma o el cerebro, basado en un modelo farmacocinético y farmacodinámico. En este artículo se presenta el caso de una paciente a la que se le aplicó anestesia total intravenosa controlada por concentración objetivo en sitio efectivo, utilizando un programa desarrollado conjuntamente por el Departamento de Anestesiología y el programa de Bioingeniería de la Universidad de Antioquia. Luego de haber sido validado con otros programas similares como el Stanpump®, se procedió a realizar una prueba clínica. Los resultados fueron satisfactorios y se alcanzaron profundidades de anestesia adecuadas para el procedimiento quirúrgico, acordes con las concentraciones en sitio efectivo reportadas a nivel mundial. Además, el comportamiento hemodinámico del paciente fue estable y su recuperación fue satisfactoria.

An infusión controlled by a target concentratiuon is a system guided by a computer that allows the administration of pharmachologycal drugs in Duch way that a defined concentration is reached and maintained in a tissue compartment; generally, plasma or the brain, based on a pharmachodynamic and pharmacokinetic model. In this article, the case of a patient to whom total intravenous anesthesia was applied, controlled by target concentration in effective site, using a program developed together by the anesthesiology department and the bioengineering program of the Universidad de Antioquia is presented. After being validated with other similar programs like the Standpump, a clinical test was made. The results were satisfactory and the adequate anesthesia depths were reached for the surgical procedure, in accordance with the concentrations in the effective site reported worldwide. Additionally, the homodynamic behavior of the patient was stable and his recovery was satisfactory as well.