Liver transplant with controlled donors after circulatory death with normothermic regional perfusion and brain dead donors: A multicenter cohort study of transfusion, one-year graft survival and mortality.

BACKGROUND: Controlled donation after circulatory death (cDCD) has expanded the donor pool for liver transplantation (LT). However, transfusion requirements and perioperative outcomes should be elucidated. The aim of this multicenter study was to assess red blood cell (RBC) transfusions, one-year graft and patient survival after LT after cDCD with normothermic regional perfusion (NRP) compared with donors after brain death (DBD). METHODS: 591 LT carried out in ten centers during 2019 were reviewed. Thromboelastometry was used to manage coagulation and blood product transfusion in all centers. Normothermic regional perfusion was the standard technique for organ recovery. RESULTS: 447 patients received DBD and 144 cDCD with NRP. Baseline MCF Extem was lower in the cDCD group There were no differences in the percentage of patients (63% vs. 61% p = 0.69), nor in the number of RBC units transfused (4.7 (0.2) vs 5.5 (0.4) in DBD vs cDCD, p = 0.11. Twenty-six patients (6%) died during admission for LT in the DBD group compared with 3 patients (2%) in the cDCD group (p = 0.15). To overcome the bias due to a worse coagulation profile in cDCD recipients, matched samples were compared. No differences in baseline laboratory data, or in intraoperative use of RBC or one-year outcome data were observed between DBD and cDCD recipients. CONCLUSIONS: cDCD with NRP is not associated with increased RBC transfusion. No differences in graft and patient survival between cDCD and DBD were found. Donors after controlled circulatory death with NRP can increasingly be utilized with safety, improving the imbalance between organ donors and the ever-growing demand.

Genetic diagnosis of epidermolysis bullosa: recommendations from an expert Spanish research group. / Diagnóstico genético de la epidermólisis bullosa: recomendaciones de un grupo español de expertos.

Epidermolysis bullosa (EB) is a rare genetic disease that causes mucocutaneous fragility. It comprises a clinically and genetically heterogeneous group of disorder characterized by spontaneous or contact/friction-induced blistering. EB is classified into 4 types-simplex, junctional, dystrophic, and Kindler syndrome-and 30 subtypes. The disease is caused by defects in proteins implicated in dermal-epidermal adhesion. At least 19 genes have been characterized and more than 1000 mutations identified, thus rendering diagnosis complex. Molecular diagnosis of EB is the last stage of a laborious process that starts with a detailed clinical history compilation and careful procurement of a skin fresh biopsy that includes an area where the epidermis detaches from the dermis. The detachment area makes it possible to establish the cleavage plane by antigen mapping and, in the best scenario, to identify a single candidate gene to search for pathogenic mutations. The results of the molecular diagnosis enable the physician to provide appropriate genetic counseling (inheritance pattern, risk of recurrence, and options for prenatal and preimplantation diagnosis) and implement subsequent preventive programs, as well as to establish a reasonable clinical prognosis facilitating access to specific therapy and rehabilitation. Lastly, molecular diagnosis is essential for the participation of patients in clinical trials, a critical issue given the current incurable status of EB. The present guidelines aim to disseminate the procedure for diagnosing EB in our laboratory and thus avoid suboptimal or incomplete clinical diagnoses. The recommendations we provide are the result of more than 10 years' experience in the molecular diagnosis of EB in Spain.

[Dual atrioventricular nodal conduction and arrhythmia with severe hemodynamic alterations during liver retransplantation]. / Arritmia por doble via intranodal con descompensación hemodinámica grave en un retrasplante hepático.

We report the case of a man who developed tachycardia caused by atrioventricular reentry related to dual nodal conduction during liver retransplantation. The hemodynamic alterations were severe. Arrhythmia and altered cardiac conduction are potential complications of liver transplantation. The development of tachyarrhythmias--atrial fibrillation as well as episodes of supraventricular and ventricular tachycardia and bradycardia--have been described. Such arrhythmias tend to occur particularly during reperfusion of the graft. Risk factors implicated are the severe ion imbalances, acid-base imbalance, and hypothermia that accompany the reperfusion of a new organ. A review of the possible pathogenic and etiological mechanisms that lead to arrhythmia in patients with end-stage liver disease is provided.

[Endoscopic autotransplantation of fat tissue in the treatment of urinary incontinence in the female]. / Autotransplantation endoscopique de tissu graisseux dans le traitement de l'incontinence urinaire chez la femme.

Urinary incontinence in women is a frequently encountered condition. We present an endoscopic technique for correction of this disorder, which may be used to treat vesico-ureteral reflux and also post-prostatectomy incontinence in men. The procedure, carried out under epidural anesthesia, consists of implanting cylinders of fatty tissue removed by liposuction from subcutaneous tissue, in the bladder neck using an endoscopic injection needle. Our series presently includes 12 patients suffering from incontinence without cystocele, followed up on average for 6 months. Although it is too early to make conclusions, the results are quite promising in terms of subsequent prognosis.