52-Week Mid-term Efficacy of Tildrakizumab in Moderate-to-severe Psoriasis: A Real-life Multicenter Experience.

Tildrakizumab is an IL-23-inhibitor that has been approved to treat plaque psoriasis. However, few reports have become available on its efficacy profile in the real-world. Our objective was to study the mid-term efficacy of tildrakizumab in patients with moderate-to-severe psoriasis in the Spanish routine clinical practice setting. This was a retrospective multicenter study that included a total of 91 psoriatic patients on tildrakizumab. The mean Psoriasis Area and Severity Index (PASI) was 9.09 (SD, 5.30). The overall tildrakizumab survival rate was 93.47% for a mean treatment exposure of 30.18 weeks (SD, 16,57). No drug discontinuation was associated with drug tolerability, or adverse reactions. Absolute PASI ≤ 3 was reached by 91.3% and 96.5% of the patients on weeks 28 and 52, respectively. Response was not impacted by weight, age (> 65), metabolic syndrome, presence of arthritis, or previous number of biological therapies used. Based on our own experience tildrakizumab is an effective strategy to treat plaque psoriasis and difficult-to-treat-areas.

52-Week Mid-term Efficacy of Tildrakizumab in Moderate-to-severe Psoriasis: A Real-life Multicenter Experience.

Tildrakizumab is an IL-23-inhibitor that has been approved to treat plaque psoriasis. However, few reports have become available on its efficacy profile in the real-world. Our objective was to study the mid-term efficacy of tildrakizumab in patients with moderate-to-severe psoriasis in the Spanish routine clinical practice setting. This was a retrospective multicenter study that included a total of 91 psoriatic patients on tildrakizumab. The mean Psoriasis Area and Severity Index (PASI) was 9.09 (SD, 5.30). The overall tildrakizumab survival rate was 93.47% for a mean treatment exposure of 30.18 weeks (SD, 16.57). No drug discontinuation was associated with drug tolerability, or adverse reactions. Absolute PASI ≤3 was reached by 91.3% and 96.5% of the patients on weeks 28 and 52, respectively. Response was not impacted by weight, age (>65), metabolic syndrome, presence of arthritis, or previous number of biological therapies used. Based on our own experience tildrakizumab is an effective strategy to treat plaque psoriasis and difficult-to-treat-areas.

[Cryptogenic new-onset super-refractory status epilepticus following SARS-CoV-2 vaccination. A case report]. / Estado epiléptico superrefractario de nueva aparición criptógeno tras vacunación contra el SARS-CoV-2. A propósito de un caso.

INTRODUCTION: New-onset super-refractory status epilepticus (NOSRSE) is a neurological emergency characterised by the development of status epilepticus in a patient without epilepsy or any known prior neurological disease and with no clear structural, toxic or metabolic cause, which recurs after 24 hours of induced coma. The most common identifiable cause is inflammatory-autoimmune. Consequently, we present a case of NOSRSE related to SARS-CoV-2 vaccination as an opportunity to investigate the dysimmune origin of this pathology. CASE REPORT: We report the case of a 40-year-old male who presented at the emergency department with fever and headache with no clear source of infection. His personal history included bacterial meningitis in childhood without any sequelae and protein S deficiency without treatment at the time, as well as vaccination with ChAdOx1 nCoV-19 21 days earlier. He was initially diagnosed with a urinary tract infection and treated with cefuroxime. Two days later, he was taken back to the emergency department with confusional symptoms and tonic-clonic seizures. He did not respond to midazolam and finally required sedation and orotracheal intubation for refractory status epilepticus. While in hospital, he required a number of lines of antiepileptic drugs, ketamine, a ketogenic diet, immunotherapy and plasmapheresis in order to successfully limit NOSRSE. The aetiological study offered normal results for serology, antineuronal antibodies in serum and cerebrospinal fluid, transthoracic echocardiography, testicular ultrasound and computed tomographic angiography. Only the control MRI scan showed a diffuse and bilateral alteration of the right hemispheric cortex and thalamic pulvinar as the only finding. CONCLUSION: It is crucial to report suspected adverse reactions associated with SARS-CoV-2 vaccination, thereby allowing continued monitoring of the risk/benefit ratio of vaccination.

TITLE: Estado epiléptico superrefractario de nueva aparición criptógeno tras vacunación contra el SARS-CoV-2. A propósito de un caso.Introducción. El estado epiléptico superrefractario de nueva aparición (NOSRSE) es una emergencia neurológica caracterizada por el desarrollo de estado epiléptico en un paciente sin epilepsia ni enfermedad neurológica previa conocida y sin clara causa estructural, tóxica o metabólica, que recurre tras 24 horas del coma inducido. La causa identificable más frecuente es la inflamatoria-autoinmune. En consecuencia, planteamos un caso de NOSRSE relacionado con la vacunación para el SARS-CoV-2 como una oportunidad de indagar el origen disinmune de esta patología. Caso clínico. Varón de 40 años que acude al servicio de urgencias refiriendo fiebre y cefalea sin claro foco infeccioso. Entre sus antecedentes personales destacamos una meningitis bacteriana en la infancia sin secuelas y un déficit de proteína S sin tratamiento en ese momento, así como vacunación con ChAdOx1 nCoV-19 21 días antes. Fue inicialmente diagnosticado de infección del tracto urinario y tratado con cefuroxima. Dos días después, se le llevó de nuevo a urgencias con cuadro confusional y crisis tonicoclónicas, sin respuesta al midazolam, y requirió finalmente sedación e intubación orotraqueal por estado epiléptico refractario. Durante su ingreso requirió múltiples líneas de antiepilépticos, quetamina, dieta cetógena, inmunoterapia y plasmaféresis para conseguir limitar el NOSRSE. El estudio etiológico ofrecía normalidad de los resultados de serología, anticuerpos antineuronales en el suero y líquido cefalorraquídeo, ecocardiografía transtorácica, ecografía testicular y angiotomografía computarizada. Únicamente la resonancia magnética de control mostró una alteración difusa y bilateral de la corteza hemisférica y pulvinar talámica derecha como único hallazgo. Conclusión. Es crucial notificar las sospechas de reacciones adversas asociadas a la vacunación frente al SARS-CoV-2, permitiendo así una supervisión continuada de la relación riesgo/beneficio de ésta.

Congenital and Infantile Hemangiomas: Epidemiological, Clinical, and Treatment Characteristics Based on 3 Years' Experience at a Tertiary Care Hospital - A Retrospective Case Comparison and Review of the Literature. / Características clínico-epidemiológicas y consideraciones terapéuticas de los hemangiomas congénitos e infantiles de un hospital de tercer nivel durante un periodo de 3 años: estudio comparativo y revisión de la literatura.

Distinguishing between congenital and infantile hemangiomas is challenging, but essential for appropriate treatment. The immunohistochemical marker glucose transporter type 1 is helpful, but biopsies are uncommon in this setting. The aim of this retrospective study was to describe and compare epidemiological, clinical, and treatment characteristics of congenital and infantile hemangiomas diagnosed at a tertiary care hospital over 3 years. We studied 107 hemangiomas: 34 congenital hemangiomas (rapidly involuting, partially involuting, and noninvoluting), 70 infantile hemangiomas, and 3 hemangiomas pending classification. Superficial infantile hemangiomas of the head and neck were the most prevalent tumors. Congenital hemangiomas were most often located on the trunk. Studied risk factors were more common in patients with infantile hemangiomas. In this group of patients, treatment response was independent of sex, in vitro fertilization, lesion depth and location, and type of treatment.

Epidemiological Characteristics of the Largest Kidney Transplant Program in Mexico: Western National Medical Center, Mexican Institute of Social Security.

BACKGROUND: According to the National Transplant Center (CENATRA), in 2013, a total of 2707 transplantations were performed in Mexico; of them, 10% (270 transplantations) were done in our Tertiary Care Hospital (Western National Medical Center). This means that one in 10 transplant recipients undergoes transplantation at our medical center. The aim of our study was to describe the characteristics of and to compare changes in the kidney transplantation program over time. MATERIALS AND METHODS: This was a cross-sectional study. Data were collected from the hospital transplant registry from January 1994 to December 2014. RESULTS: During the study period, 3643 kidney transplantations were conducted; most were living donor 3236 (89%), and only 407 patients (11%) received a graft from a deceased donor. Of living donors, 2786 (87%) were related, and 450 (13%) were genetically unrelated. The average recipient age was 28 years, and the average age of the donor was 34 years. It was observed that siblings donated more frequently (51%), followed by parents (34%). Among unrelated donors, spouses donated the most (66%). In 80% of cases, the cause of end-stage renal disease (ESRD) was unknown (80%). The most frequent renal replacement therapy was peritoneal dialysis (54%), followed by hemodialysis (18%); only 5% of patients received preemptive kidney transplant. The most frequent immunosuppression scheme was tacrolimus, mycophenolate mofetil, and prednisone in 70% of patients. CONCLUSION: The Western National Medical Center is the largest kidney transplantation program in Mexico. The main activity is living donor transplantation. Recipients are relatively young persons with unknown etiology of ESRD.

Expanding the clinical spectrum of the 'HDAC8-phenotype' - implications for molecular diagnostics, counseling and risk prediction.

Cornelia de Lange syndrome (CdLS) is a clinically heterogeneous disorder characterized by typical facial dysmorphism, cognitive impairment and multiple congenital anomalies. Approximately 75% of patients carry a variant in one of the five cohesin-related genes NIPBL, SMC1A, SMC3, RAD21 and HDAC8. Herein we report on the clinical and molecular characterization of 11 patients carrying 10 distinct variants in HDAC8. Given the high number of variants identified so far, we advise sequencing of HDAC8 as an indispensable part of the routine molecular diagnostic for patients with CdLS or CdLS-overlapping features. The phenotype of our patients is very broad, whereas males tend to be more severely affected than females, who instead often present with less canonical CdLS features. The extensive clinical variability observed in the heterozygous females might be at least partially associated with a completely skewed X-inactivation, observed in seven out of eight female patients. Our cohort also includes two affected siblings whose unaffected mother was found to be mosaic for the causative mutation inherited to both affected children. This further supports the urgent need for an integration of highly sensitive sequencing technology to allow an appropriate molecular diagnostic, genetic counseling and risk prediction.