Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block.

BACKGROUND: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. OBJECTIVE: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. METHODS: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. RESULTS: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. CONCLUSIONS: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

ANTECEDENTES: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. OBJETIVO: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. MÉTODOS: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. RESULTADOS: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. CONCLUSIONES: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Impact of Prior Inotuzumab Ozogamicin Treatment on Brexucabtagene Autoleucel outcomes in Adults with B-cell ALL.

The effect of prior inotuzumab ozogamicin (InO) treatment on brexucabtagene autoleucel (brexu-cel) outcomes remains unclear in adults with acute lymphoblastic leukemia (ALL), particularly the influence off previous InO response and the timing of administration. We conducted a retrospective multicenter analysis of 189 patients with relapsed/refractory (r/r) ALL treated with brexu-cel. Over half of the patients received InO before brexu-cel (InO-exposed). InO-exposed patients were more heavily pretreated (p= 0.02) and frequently had active marrow disease pre-apheresis (p= 0.03). Response rate and toxicity profile following brexu-cel were comparable for InO-exposed and InO-naïve; however, consolidation therapy post brexu-cel response was utilized at a higher rate in InO-naïve patients (p= 0.005). With a median follow up of 11.4 months, InO-exposed patients had inferior progression-free survival (PFS) (p=0.013) and overall survival (OS) (p=0.006) in univariate analyses; however, prior InO exposure did not influence PFS (HR 1.20, 95%CI, 0.71-2.03) in multivariate models. When InO-exposed patients were stratified according to prior InO response, InO responders had superior PFS (p=0.002) and OS (p<0.0001) relative to InO-refractory. The timing of administering InO did not affect brexu-cel outcomes, with comparable PFS (p=0.51) and OS (p=0.86) for patients receiving InO as bridging therapy or pre-apheresis. In conclusion, while InO exposure was associated with inferior survival outcomes following brexu-cel in unadjusted analyses, these associations were no longer significant in multivariate analyses, suggesting it is unlikely that InO negatively impacts brexu-cel efficacy. Our data instead imply that InO-exposed recipients of brexu-cel tend to be higher-risk patients with intrinsic adverse leukemia biology.

Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block / Prevalencia de lupus neonatal en un pequeño grupo de madres portadoras de anticuerpos y frecuencia de bloqueo auriculoventricular completo

Abstract Background: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. Objective: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. Methods: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. Results: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. Conclusions: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

Resumen Antecedentes: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. Objetivo: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. Métodos: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. Resultados: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. Conclusiones: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Prevalence of Chagas heart disease in dilated cardiomyopathy / Prevalencia de cardiopatía chagásica en la miocardiopatía dilatada

Abstract Objectives: The main objective is to determine the prevalence of American trypanosomiasis in patients with dilated cardiomyopathy in a tertiary hospital in western Mexico. Methods: From January 1991 to February 2016, 387 consecutive patients with a confirmed diagnosis of dilated cardiomyopathy were included in the study. Cases with ventricular dilatation secondary to ischemic heart disease, valvular heart disease, hypertension, lung disease, pericardial disease, or congenital heart disease were excluded from the study. Diagnosis was made detecting antibodies against Trypanosoma cruzi with two different methods or parasite in blood. Results: Were included 387 patients with dilated cardiomyopathy, Chagas cardiomyopathy was confirmed in 6.9%, two patients in the acute phase (in one, suspected transfusion transmission was detected). Most patients were born in rural areas. About 96.2% showed congestive heart failure, only one patient with apical left ventricular aneurysm manifested palpitations. About 66% with right bundle branch block, left anterior fascicular block, or the association of both, in 14.8%, non-sustained ventricular tachycardia was found. Conclusions: Chagas cardiomyopathy is common in México, mainly in people who were born or lived during childhood in rural areas. It is a common cause of heart failure. Chagas’ heart disease should be suspected in patients receiving a blood transfusion, even without another epidemiological history.

Resumen Objetivo: El objetivo principal del estudio es conocer la prevalencia de tripanosomiasis americana en pacientes con cardiomiopatía dilatada, en un hospital de concentración en el occidente de México. Métodos: Desde enero de 1991 a febrero de 2016 se incluyeron 387 pacientes consecutivos con diagnóstico de cardiomiopatía dilatada, se excluyeron los casos con dilatación ventricular secundaria a cardiopatía isquémica, valvulopatías, hipertensión arterial sistémica, enfermedad pulmonar, enfermedad pericárdica o cardiopatías congénitas. El diagnóstico se realizó mediante la detección de anticuerpos anti-tripanosoma cruzi con 2 métodos positivos diferentes o con la detección del parásito en sangre. Resultados: Se incluyeron 387 paciente con cardiomiopatía dilatada, en el 6.9% se confirmó cardiopatía chagásica; dos pacientes en fase aguda (uno con sospecha de transmisión transfusional). La mayoría de los pacientes provenían de zonas rurales. El 96.2% de los casos presentó insuficiencia cardiaca congestiva, un paciente con aneurisma apical del ventrículo izquierdo solo manifestó palpitaciones. El 66% presentó bloqueo de la rama derecha del haz de His, hemibloqueo anterior izquierdo o la asociación de ambos, en el 14.8% se encontró taquicardia ventricular no sostenida. Conclusiones: La cardiopatía chagásica es frecuente en nuestro medio, principalmente en personas que nacieron o vivieron durante la infancia en áreas rurales. Es causa común de insuficiencia cardiaca. La cardiomiopatía chagásica debe sospecharse en pacientes que reciben transfusión sanguínea, incluso sin otros antecedentes epidemiológicos

A1­A2 Fenestration Tube Technique for Clipping a Ruptured Anterior Communicating Artery Aneurysm

A fenestration tube is a clipping reconstruction technique that allows the preservation of critical vessels in aneurysm surgery. A patient with a ruptured anterior communicating artery (ACoA) aneurysm with a posterior projection was admitted to our neurosurgery unit. A right dominant A1 with rotation of the A2 fork was observed on preoperative computed tomography angiography (CTA). During surgery, we observed that the recurrent artery of Heubner branched off the A2 just distal to the neck of the aneurysm. Successful clipping was achieved by building an "A1­A2 fenestration tube," with preservation of the recurrent artery and of the ACoA perforators. Surgical nuances and the advantages of fenestration tubes are discussed.

Burden of genital warts in Ecuador: an observational study in the practice of physicians / Carga das verrugas genitais no Equador: estudo observacional sobre a clínica médica

Introduction: Human papillomavirus types 6 and 11 cause 90% of genital warts. Although the epidemiology of cervical cancer and the distribution of human papillomavirus genotypes have been investigated in Ecuador, little is known about the occurrence of genital warts. Objective: To estimate the incidence and prevalence of genital warts among patients routinely presenting at the practice of physicians, describe the demographics of genital warts cases and highlight the physician specialties that treat genital warts, including patterns of consultation and referral in Ecuador. Methods: Participants were a convenience sample of physicians who treat and/or diagnose genital warts in their practices. Physicians completed a daily log, recording the demographics and diagnosis of genital warts in all patients aged 18 to 60 years seen over 10 days in their practices. Physicians then completed a survey recording their practice characteristics and referral patterns of genital warts. Results: A sample of 105 physicians of different specialties participated in the study. Among 12,133 patients, the prevalence of genital warts was 5.5%, and the incidence, 3.7%. Prevalence was 6.9% in men, peaking at 12.6% in those aged from 25 to 29 years old. Prevalence was 5.1% for females, peaking at 6.5% in those aged from 30 to 34 years old. Most women were seen in direct consultations (75%) rather than by referral ones (24%). Most physicians (72%) treated females with genital warts, except for primary care physicians, who referred most cases (88%). Conclusion: Cases of genital warts are frequently encountered by physicians in Ecuador and are typically treated by specialists rather than primary care physicians.

Introdução: Os tipos 6 e 11 do papilomavírus humano causam 90% das verrugas genitais. Embora a epidemiologia do câncer do colo do útero e a distribuição dos genótipos do papilomavírus humano tenham sido investigadas no Equador, pouco se sabe sobre a ocorrência das verrugas genitais. Objetivo: Estimar a incidência e a prevalência das verrugas genitais em pacientes atendidos rotineiramente na clínica médica, descrever os dados demográficos dos casos de verrugas genitais e determinar as especialidades médicas que tratam as verrugas genitais, incluindo os padrões de consulta e encaminhamento no Equador. Métodos: Foi realizada uma amostragem por conveniência com médicos que tratavam e/ou diagnosticam verrugas genitais em sua clínica médica. Os médicos registraram as suas atividades cotidianas em um diário, anotando dados demográficos e diagnóstico de verrugas genitais de todos os pacientes com idade entre 18 e 60 anos atendidos durante 10 dias em suas clínicas. Posteriormente, os médicos responderam a uma pesquisa sobre as características da conduta tomada e os padrões de encaminhamento médico das verrugas genitais. Resultados: Uma amostra de 105 médicos de diferentes especialidades participou do estudo. Entre 12.133 pacientes, a prevalência de verrugas genitais foi de 5,5% e a incidência foi de 3,7%. A prevalência foi de 6,9% no sexo masculino, atingindo o valor máximo de 12,6% na faixa etária de 25 a 29 anos. A prevalência foi de 5,1% para o sexo feminino, alcançando 6,5% entre 30 e 34 anos. Na maior parte dos casos, as mulheres foram tratadas predominantemente pelo médico que realizou o primeiro atendimento (75%) e não por meio de encaminhamento a outra especialidade (24%). A maioria dos médicos (72%) tratou mulheres com verrugas genitais, exceto os médicos de atenção primária, que em geral encaminharam os casos (88%). Conclusão: Casos de verrugas genitais são frequentemente diagnosticados por médicos no Equador e são mais frequentemente tratados por especialistas do que por médicos de atenção primária.

Reporte de caso: anestesia espinal multimodal en paciente pediátrico con vía aérea difícil / Case report: Multimodal spinal anesthesia in a pediatric patient with a difficult airway

Introducción: La anestesia espinal llevamás de 100 años en la práctica anestésica pediátrica. Actualmente viene aumentando su uso por ser eficaz, eficiente y segura. Se expone un caso exitoso en paciente con vía aérea difícil. Objetivo: Reportar un caso de anestesia espinal y sedación con remifentanilo, haciendo la revisión de la literatura incluyendo fármacos alfa 2 agonistas, para procedimientos locorregionales pediátricos. Métodos: Búsqueda bibliográfica relevante en las bases bibliográficas PubMed, MD consult y BIREME. Inicialmente se obtienen 306 artículos, seleccionando 23 considerados relevantes por los autores. Resultados: Se presenta el caso de un niño de un año de edad, con vía aérea difícil predicha por un hemangioma cavernoso en labio inferior, programado para corrección quirúrgica de pie chapín bilateral. Se administra anestesia espinal con bupivacaína hiperbárica 0,5% carbonatada 5mg y clonidina 30 1g(1,3 ml total), manteniendo sedoanalgesia 3-4/6 de Ramsay con remifentanilo 0,05-0,075 1g/kg/min, con oxígeno 50% por máscara facial y ventilación espontánea sin efectos adversos hemodinámicos o respiratorios. Conclusiones: La anestesia espinal es una alternativa ante una vía aérea difícil predicha. La clonidina (alfa 2 agonista) prolonga la duración del bloqueo sin complicaciones hemodinámicas o respiratorias. El remifentanilo para sedación en los procedimientos locorregionales pediátricos es de fácil titulación, con resultados predecibles.

Introduction: Spinal anesthesia has been part of the pediatric anesthesia practice for more than 100 years. Its use has been increasing in recent years because of its effective-ness.efficiency and safety. We report a successful case in a patient with a difficult airway. Objective: To report a case of spinal anesthesia and sedation with remifentanil, together with a review of the literature including alpha 2 agonists for locoregional procedures in pediatrics. Methods: Search of relevant references in PubMed, MD consult and BIREME. The search resulted in 306 articles, and 23 considered relevant by the authors were finally selected. Results: We present a case of a 1-year-old boy with an expected difficult airway because of the presence of a cavernous hemangioma of the lower lip, scheduled for surgical correction of bilateral club foot. Spinal anesthesia consisted of 0,5% hyperbaric carbonated bupiva-caine plus 30 g of clonidine (1.3 ml total), maintaining sedation-analgesia at 3^1/6 on the Ramsay scale with remifentanil 0,05-0,075 g/kg/min, 50% oxygen with facial mask, and spontaneous ventilation, with no hemodynamic or respiratory adverse effects. Conclusions: Spinal anesthesia is an option in cases of predicted difficult airway. Clonidine (alpha 2 agonist) prolongs blockade with no hemodynamic or respiratory complications. Remifentanil used for sedation in pediatric locoregional procedures is easy to titrate with-predictable results.

Regresión de la cardiopatía chagásica aguda en un lactante menor con sospecha de infección transfusional. Nueve años de seguimiento / Regression of acute chagasic cardiopathy in a nine months old infant with suspected transfusional infection. Nine years follow up

La enfermedad de Chagas o Tripanosomosis Americana fue descrita en México por Mazzotti en 1940. No ha sido documentada la transmisión a través de transfusión sanguínea. Presentamos el caso de una niña de 9 meses de edad con diagnóstico comprobado de cardiopatía chagásica aguda, el antecedente de transfusión sanguínea nos hace sospechar la transmisión por esta vía. Recibió manejo a base de Nifurtomox con los que se obtuvo negativización de la parasitología. En su seguimiento de nueve años se observa una regresión paulatina de la cardiopatía, tanto en estudios radiológicos como ecocardiográficos, hasta llegar a la normalidad. En su control durante este tiempo conserva la negatividad en los exámenes serológicos y parasitológicos, ha permanecido asintomática y su desarrollo y crecimiento son adecuados