Resumen El síndrome de persona rígida es un cuadro neuro lógico infrecuente caracterizado por rigidez muscular de tronco y extremidades y espasmos musculares ga tillados por estímulos sensoriales o emocionales, que progresa hacia la postración. Cuenta con un mecanismo fisiopatogénico con base inmunológica, en el cual los autoanticuerpos, como el antiGAD65, cumplen un rol central. Asimismo, la detección de dichos anticuerpos corrobora el diagnóstico ante un paciente con cuadro clínico sugestivo. Un 4 a 6% de los casos tienen neoplasias subyacentes. El tratamiento se basa en el manejo sintomático, inmunomodulador y de la enfermedad de base en los casos paraneoplásicos. Reportamos un caso de síndrome de persona rígida clásico asociado a timoma y describimos las características principales de esta entidad.

Abstract Stiff-person syndrome is a rare neurological condi tion characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostra tion. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detec tion of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We re port a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

Resumen Las neoplasias epiteliales del timo (NET) son tumores infrecuentes. El manejo inicial de las mismas se basa en el análisis de la resecabilidad del tumor en los estudios por imágenes, siendo la resección completa el tratamiento estándar para cualquier estadio. El objetivo principal fue evaluar la supervivencia global y la supervivencia libre de enfermedad de los pacientes con diagnóstico de NET sometidos a tratamiento quirúrgico. El objetivo secundario fue comparar la supervivencia global de acuerdo al tipo de resección quirúrgica, el estadio de Masaoka-Koga y subtipo histológico. Estudio observacional descriptivo realizado en el Hospital Británico de Buenos Aires desde 2004 a 2020. Se incluyeron 42 pacientes operados de tumores en mediastino anterior con diagnóstico histopatológico definitivo de NET. Treinta y nueve eran timomas y 3 carcinomas tímicos. La cirugía realizada en todos los pacientes fue una timectomia total. La mediana de seguimiento fue de 63.5 meses (RIQ 32-97.5). La supervivencia global estimada a los cinco y diez años fue del 87% (95% IC, 0.69-0.95) y 78% (95% IC, 0.5-0.92), respectivamente. La supervivencia libre de enfermedad estimada a cinco y diez años fue del 90% (95% IC, 0.74-0.96). Los pacientes a los que se le realizó una resección completa y que tenían estadios tem pranos de Masaoka-Koga presentaron una supervivencia global superior en comparación con las resecciones incompletas y estadios avanzados (p = 0.0097 y p = 0.0028, respectivamente). Al contar con un bajo número de pacientes en el grupo de carcinomas tímicos, no pudimos sacar conclusiones en cuanto a supervivencia entre éstos y los timomas.

Abstract Thymic epithelial tumors are a group of rare neoplasms of the mediastinum. When resectable, complete resection is considered the gold standard for any stage. The primary endpoint was to evaluate overall survival and disease-free survival of patients with thymus epithelial tumors who underwent surgical treatment. The secondary endpoint was to evaluate and compare the overall survival according to: resection type (complete vs. incomplete), Masaoka-Koga stage and tumor histology according to WHO classification. This is a descriptive observational study from January 2004 to December 2020, in which 42 patients with a postoperative histopathological diagnosis of thymic epithelial tumours were included. Thirty-nine were thymomas (92.9%) and 3 were thymic carcinomas (7.1%). In all patients a total thymectomy was performed. The median follow-up was 63.5 months (IQR 32-97.5). The estimated overall survival at five and ten years was 87% (95% CI, 0.69-0.95) and 78% (95% CI, 0.5-0.92), respectively. Estimated disease-free survival at five and ten years was 90% (95% CI, 0.74-0.96). Patients who underwent complete resection and with early Masaoka-Koga stages had superior overall survival compared to incomplete resections and advanced Masaoka-Koga stages (p = 0.0097 and p = 0.0028, respectively). We found no differences in terms of survival between histological subtypes due to a low number of patients in the thymic carcinoma group.

ABSTRACT Introduction: Retinopathy of Prematurity (ROP) is one of the main preventable causes of blindness in childhood. The Vision Foundation implemented the Prevention, Detection and Treatment Program in 2015. Objective: To describe the implementation results and the outcomes achieved by a ROP program regarding its accessibility, effectiveness and quality of care. Materials and Methods: This was a descriptive and ambispective study of consecutive cases of premature newborns with criteria for evaluation, of a population evaluated in the year 2009 and from 2015 to 2019. Results: The participating neonatal units increased from 3 to 7; covering Asunción, the Central, Caaguazú and Alto Paraná Departments. From the records, it was found that program coverage increased from 36% in 2009 to 97% in 2019. Since the beginning of the program, during the first year the proportion of the number of evaluations increased between 28 and 218%; the global results from July 2015 to December 2019 were: total number of patients with criteria: 2397 patients; total number of patients evaluated: 2,080 (88%), number of patients with ROP: 416/2080 patients (20 %), number of patients with ROP requiring treatment: 76/416 patients (18.3%), overall proportion of severe ROP with treatment: 76/2080 patients (3.4%). Conclusions: The program, using a multidisciplinary team and the innovation of telemedicine, managed to increase the proportion of coverage and the reduction of serious cases that require treatment.

RESUMEN Introducción: La Retinopatía del Prematuro (ROP) es una de las principales causas de ceguera prevenibles en la infancia. La Fundación Visión implementa; el Programa de Prevención, Detección y Tratamiento en el año 2015. Objetivos: Describir los resultados de implementación y los resultados alcanzados por un programa de ROP en su accesibilidad, efectividad y calidad de atención. Materiales y Métodos: Estudio descriptivo, ambispectivo, de casos consecutivos de recién nacidos prematuros con criterios de evaluación; de una población evaluada en el año 2009 y del 2015 al 2019. Resultados: Las unidades neonatales incluidas aumentaron de 3 a 7; cubriendo Asunción, área Central, Caaguazú y Alto Paraná. De los registros se constato que la cobertura aumento de 36% en el año 2009 a 97% en el 2019. Desde que se instaló el programa; en el primer año la proporción del número de evaluaciones aumento entre el 28 y el 216%; y los resultados globales de julio 2015 a diciembre 2019 fueron: número totales de pacientes con criterio: 2397 pacientes; número totales de pacientes evaluados: 2080 (86,8%), número de pacientes con ROP: 416/2080 pacientes (20%), número de pacientes con ROP que requirieron tratamiento: 76/416 pacientes (18,2%), proporción global de ROP grave con tratamiento: 76/2080 pacientes (3,4%). Conclusiones: El programa a través de un equipo multidisplinario y la inovación con telemedicina logró aumentar la proporción de cobertura y la disminución de los casos graves que requirieron tratamiento.

Introduction: the use of insecticide-treated materials is one of the strategies currently recommended by the World Health Organization (WHO) for the prevention and control of vector-borne diseases. Even though insecticide-impregnated materials have not been used in Cuba, several pyrethroid insecticide formulations have been in use since 1986 for the control of various vector species. Objective: determine the effectiveness of pyrethroid insecticide beta-cypermethrin, formulated as Sipertrin 5 SC, for the control of Aedes aegypti. Methods: effectiveness of Sipertrin 5 SC was determined by three procedures: residual treatment of resting sites-perifocal, impregnation of curtains, and combination of the two, applying the methodologies recommended by WHO, all of which was carried out in households from Condado Norte Council, 20th Anniversary health area, in the municipality of Santa Clara, Villa Clara. Bioassays were conducted to determine the effectiveness of this pyrethroid on various surfaces, following the methodology recommended by WHO. Results: the greatest residual effect was obtained with the combined treatment (resting sites, perifocal treatment and curtain impregnation), with an effectiveness of up to 5 months. In households undergoing resting site and perifocal treatment, residuality was 4 months. Effectiveness of the product was highest on plastic and concrete surfaces, with a mortality rate of 95 to 100 % up to three months. On metal surfaces mortality was 85%. Conclusions: Sipertrin 5 SC is a candidate to be considered for possible inclusion in Aedes aegypti control programs in Cuba. Its use will contribute to the sustainability of the strategies implemented.

Introducción: el uso de materiales tratados con insecticidas es una de las estrategias actualmente recomendada por la Organización Mundial de la Salud (OMS), para la prevención y control de enfermedades trasmitidas por vectores. En Cuba, aunque no se han utilizado materiales impregnados con insecticidas, desde 1986 se utilizan distintos formulados de insecticidas piretroides para el control de diferentes especies de vectores. Objetivo: determinar la efectividad del insecticida piretroide beta cipermetrina, en su formulación Sipertrin 5 SC para el control de Aedes aegypti. Métodos: se determinó la efectividad del Sipertrin 5 SC, mediante tres procesos: tratamiento residual en los sitios de reposo-perifocal, impregnación de cortinas, y la combinación de los dos anteriores, a través de metodologías recomendadas por la OMS, en viviendas del Consejo Condado Norte del Área de Salud XX Aniversario, del municipio Santa Clara, Villa Clara. Se realizaron bioensayos para determinar la efectividad de este piretroide en diferentes superficies, siguiendo la metodología recomendada por la OMS. Resultados: el tratamiento combinado (sitios de reposo, perifocal e impregnación de cortinas) fue el de mayor efecto residual, con una efectividad de hasta 5 meses. En las viviendas con tratamiento en los sitios de reposo y perifocal la residualidad fue de 4 meses. La mayor efectividad del producto resultó en las superficies de plástico y concreto con una mortalidad de 95 a 100 % hasta los 3 meses, mientras que en la de metal fue de 85 %. Conclusiones: el Sipertrin 5 SC es un candidato a analizar para su posible inclusión en los programas de control de Aedes aegypti en Cuba, contribuirá a la sostenibilidad de las estrategias que se implementen.

OBJETIVO: Comparar pacientes submetidos à ultrafiltração convencional (UFC), sem e com modificação técnica que permite aproveitamento do sangue residual do circuito de tubos e do oxigenador. MÉTODO: No período de março de 2002 a janeiro de 2005, 301 pacientes submetidos à correção de cardiopatias congênitas com circulação extracorpórea (CEC) foram analisados, retrospectivamente, e divididos em dois grupos: A - constituído de 130 pacientes submetidos à UFC clássica e grupo B - constituído de 171 pacientes submetidos à UFC com modificação técnica para aproveitamento do sangue residual. Foram comparadas variáveis demográficas, diagnóstico, tratamento cirúrgico, dados do período intra-operatório e pós- operatório, necessidade e volume de transfusões, exames laboratoriais e permanência hospitalar. RESULTADOS: Não houve diferença no valor inicial de hematócrito antes da CEC (p = 0,06), mas no grupo B, os valores após a ultrafiltração foram maiores (p < 0,0001). O grupo B foi mais transfundido nas primeiras 48 horas do pós-operatório (p < 0,0001). Não houve diferença no tempo de ventilação mecânica (p = 0,34), mas o tempo de uso de drogas vasoativas (p < 0,0001), tempo de uso de antibióticos (p = 0,0006), tempo de internação na unidade de terapia intensiva (p < 0,0001) e o tempo total de internação no pós-operatório (p < 0,0001) foram maiores no grupo B. CONCLUSÕES: A UFC com a modificação técnica não apresentou resultados superiores aos da UFC clássica, pois, apesar de elevar o hematócrito após a circulação extracorpórea, proporcionou maior sangramento no pós-operatório com maior necessidade de transfusão de hemoderivados e prolongamento do tempo de permanência hospitalar.

OBJECTIVE: To compare patients submitted to the conventional ultrafiltration (CUF) with and without a technical modification that allows use of residual blood from the circuit tubes and oxygenator. METHOD: From March 2002 to January 2005, 301 patients undergoing to congenital heart defects surgery with cardiopulmonary bypass (CPB) were retrospectively analyzed and divided in two groups: Group A - 130 submitted to CUF and Group B, 171 patients submitted to CUF with a technical modification that uses residual blood. Demographic data, diagnosis, surgical treatment, intra-operative and postoperative data, the need and amount of blood transfusions, laboratorial results and length of hospital stay were compared between the groups. RESULTS: There was no differences in the initial hematocrit before CPB (p = 0.06), but in the Group B, the values after ultrafiltration were higher (p <0.0001). Group B patients received more transfusions in the first 48 hours of the postoperative period (p <0.0001). There was no signicant difference in the time of mechanical ventilation (p = 0.34), but the inotropic support (p <0.0001), antibiotic therapy (p = 0.0006), length of stay in the intensive care unit (p <0.0001) and length of hospital stay (p <0.0001) were greater for Group B. CONCLUSIONS: CUF with the technical modification was not better than conventional CUF, because in spite of elevating the hematocrit after the CBP, it caused greater postoperative bleeding with a greater need of blood transfusions and longer hospitalar stays.

OBJETIVO: Comparar os pacientes onde a operação foi realizada com circulação extracorpórea (CEC) àqueles sem CEC, analisando suas características e verificando se houve ou não superioridade de alguma das técnicas empregadas. MÉTODO: Entre janeiro de 2002 e janeiro de 2004, 16 pacientes foram submetidos à operação de Glenn bidirecional (GB), todos com defeitos cardíacos complexos. Quatorze eram do sexo feminino, com idade mediana de 19 meses. Os pacientes foram divididos em dois grupos: A (GB com CEC) e B (GB sem CEC), sendo sete do grupo A e nove do grupo B. Foram analisadas comparativamente as variáveis: sexo, idade, pressão arterial pulmonar média (PAPM), operação realizada, tempo de CEC, pinçamento da aorta, derivação venoatrial, operações prévias, internação na unidade de terapia intensiva (UTI), hospitalização e complicações imediatas. RESULTADOS: A mediana da PAPM foi 13 mmHg. No grupo A, o tempo foi de CEC 91 ± 47 minutos (57-195 minutos) e o de isquemia miocárdica 25 ± 33 minutos (0-80 minutos). Destes, quatro necessitaram de procedimento intracardíaco ou ampliação de ramos pulmonares e três, auxílio da CEC como suporte ventilatório. No grupo B o tempo de desvio venoatrial foi de 21 ± 10 minutos (0-39 minutos). O tempo para extubação 9 ± 13 horas, mediana 3 horas (1-43 horas). A permanência UTI foi de 8 ± 12 dias, mediana 5 dias (1-50 dias). O período médio de internação hospitalar foi de 12 ± 12 dias, mediana 7 dias (0-50 dias). Cinco pacientes haviam sido submetidos à operação prévia. Dois (12,5%) faleceram, sendo um em cada grupo. Não houve complicações neurológicas, derrame pleural ou pericárdico. Todas as variáveis estudadas entre os grupos não apresentaram diferença estatística significativa. CONCLUSÃO: Apesar da amostra relativamente pequena, o presente estudo sugere que a operação de GB pode ser realizada com ou sem CEC com resultados semelhantes no que se refere à morbidade e mortalidade. Assim sendo, a operação sem CEC pode ser empregada com segurança quando os achados anatômicos forem apropriados e não houver hipóxia grave.

OBJECTIVE: To compare patients who underwent the Bidirectional Glenn Operation with and without cardiopulmonary bypass (CPB), analyzing the characteristics and confirming if there is superiority of either of the employed techniques. METHOD: Between January 2002 and January 2004, 16 patients with complex heart defects were submitted to this operative technique. The mean age of the patients was 19 months and 14 were female. The patients were divided into two groups: Group A with seven patients (using CPB) and group B with 9 patients (without the use of CPB). Gender, age, mean pulmonary artery pressure (MPAP), CPB time, aortic clamping time, venoatrial shunt, previous operations, time in intensive care unit (ICU), total hospitalization time and immediate complications were all compared between the two groups. RESULTS: The median MPAP was 13 mmHg. In group A the CPB time was 91 ± 47 minutes (57-195 minutes), myocardial ischemia was 25 ± 33 minutes (0-80 minutes). Of these four patients required intracardiac procedures or enlargement of the pulmonary branches and in three, CPB assistance as ventilatory support was needed. In group B the venoatrial shunt was 21 ± 10 minutes (0-39 minutes). The time to extubation was 9 ± 13 hours with a median of 3 hours (1-43 hours). The ICU stay was 8 ± 12 days with a median of 5 days (1-50 days). Hospitalization was 12 ± 12 days with a median of 7 days (0-50 days). Five patients had been submitted to surgeries previously. Two, one patient from each group, died (12.5%). No neurological complications, pleural or pericardial effusions were observed. No significant differences were evidenced between the two groups in respect to all the variables studied. CONCLUSION: In spite of the relatively small cohort, this study suggests that the bidirectional Glenn operation can be performed with or without CPB giving similar results in respect to morbidity and mortality. Thus, the operation without CPB can be safely employed when the anatomic findings are appropriate and there is no severe hypoxia.