Perfil clínica y electromiográfico de las distrofias musculares en República Dominicana / Clinical and electromyographic profile of muscular dystrophies in the Dominican Republic

Introducción: Las distrofias musculares son trastornos miogénicos hereditarios caracterizados por una atrofia muscular progresiva y una debilidad de distribución y gravedad variable. La población de Republica Dominicana es fruto de una mezcla de etnias, haciéndola portadora de una herencia cromosómica y ADN diverso, siendo susceptibles a poder presentar cualquier desorden de carácter hereditario. Material y métodos: Con una muestra de 17 pacientes obtenidos entre septiembre 2019- marzo 2020, se realizó un estudio retrospectivo, descriptivo y transversal, en el cual se hizo una revisión de los expedientes de la clínica de miopatías en la consulta de neurología pediátrica del Hospital Infantil Doctor Robert Reid Cabral, para describir el perfil clínico de los pacientes con distrofia muscular y los hallazgos de electromiografía en los casos que la misma. Resultados: se encontró que la distribución de la edad correspondió a 5-9 años en un 53%, siendo el sexo masculino, el más frecuente. En el 70.59% presentaron antecedentes familiares de distrofia muscular. Los principales motivos de consulta fueron cansancio y caídas frecuentes. Conclusión: En los hallazgos de electromiografía, el porcentaje de pacientes que presentó esta prueba con alteraciones fue de 88.24% y sin alteraciones el 11.76%. Esto nos demuestra, la gran utilidad de dicho estudio en el diagnóstico de las distrofias musculares en países donde no se cuenta con estudio molecular, siendo una de las pruebas esenciales en el abordaje diagnóstico de los pacientes con sospecha clínica de dichas patologías.

Introduction: Muscular dystrophies are hereditary myogenic disorders characterized by progressive muscular atrophy and weakness of variable distribution and severity. The population of the Dominican Republic is the result of a mixture of ethnic groups, making it the bearer of a diverse chromosomal inheritance and DNA, being susceptible to presenting any hereditary disorder. Methods: With a sample of 17 patients obtained between September 2019-March 2020, a retrospective, descriptive and cross-sectional study, in which a review of the files of the myopathies clinic was made in the pediatric neurology consultation of the Children's Hospital Doctor Robert Reid Cabral, to describe the clinical profile of patients with muscular dystrophy and the electromyography findings in the cases with the same. Results: The age distribution corresponded to 5-9 years; 53%, being the masculines, the most frequent sex. In 70.59%, there was a family history of muscular dystrophy. The main reasons for consultation were fatigue and frequent falls. Conclusion: In the electromyography findings, the percentage of patients who presented this test with alterations was 88.24% and 11.76% without alterations. This result shows us the great utility of said study in the workup of muscular dystrophies in countries with no availabilities for molecular studies, being one of the essential tests in the diagnostic approach of patients with clinical suspicion of said pathologies.

Nefritis lúpica: experiencia con dosis reducidas de glucocorticoides en una unidad de enfermedades autoinmunes sistémicas / Lupus nephritis: low-dose glucocorticoids in a systemic autoimmune diseases unit / Nefritis lúpica: experiência con doses reduzidas de glicocorticoides em uma unidade de doenças autoinmunes sistémicas

Resumen: Introducción: las recomendaciones actuales del tratamiento de la nefritis lúpica (NL) apuntan a dosis de glucocorticoides más bajas para lograr el control de la enfermedad y evitar el daño acumulado. Objetivo: conocer y comparar la respuesta al tratamiento de pacientes con NL proliferativa en su etapa de inducción con dos pautas de tratamiento con prednisona (PDN): dosis iniciales reducidas 30 mg/d. Método: se compararon variables clínicas, analíticas y terapéuticas de pacientes con NL proliferativa categorizados en dos grupos según la dosis inicial de prednisona (PDNi) estándar o reducida. Resultados: se estudiaron 21 pacientes con NL proliferativa (n=12 PDNi reducida vs. n=9 PDNi estándar). No hubo diferencias significativas en las variables clínicas y analíticas. Se observó una diferencia estadísticamente significativa en el número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) y en la dosis de prednisona acumulada a 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). No hubo diferencias significativas en la proporción de pacientes que alcanzaron la respuesta completa, en el tiempo hasta alcanzarla ni en los efectos adversos entre ambos grupos. Conclusiones: el esquema terapéutico del grupo PDNi <30 mg/d se asoció a una menor dosis acumulada de prednisona y una respuesta al tratamiento comparable, lo que hace presumir menor daño acumulado relacionado al uso de glucocorticoides.

Abstract: Introduction: current recommendations to treat lupus nephritis (LN) point to low-dose glucocorticoids to control the disease and avoid cumulativedamage. Objective: to learn about and compare the response of patients with proliferative LN who are treated following two prednisone therapy guidelines: reduced initial doses 30 mg/d during the induction stage. Method: clinical, analytical and therapeutic guidelines of patients with proliferative LN were compared and classified into two groups according to the standard or low-dose initial prednisone dose. Results: 21 patients with proliferative LN were studied (n=12 low-dose initial prednisonevs. n=9 standard initial prednisone). No significant differences were found between clinical and analytical variables, although a significantly different statistic difference was observed in the number of methylprednisone pulses (5 ± 2.95 initial prednisone 30 mg/d, p = 0.041) and in the prednisone dose accumulated in 6 months (12.8 mg ± 4.9 initial prednisone 30 mg/d, p =0.008). No significant differences were seen between both groups in the proportion of patients who achieved complete response, neither in terms of the time it took to achieve it or in the side effects. Conclusions: the treatment plan for the initial prednisone <30 mg/d was associated to a lower cumulative dose of response prednisone considering the comparable treatment, what suggests there being smaller cumulative harm as a consequence of the use of glucocorticoids.

Resumo: Introdução: as recomendações atuais para o tratamento da nefrite lúpica (NL) estão orientadas a doses de glicocorticoides mais baixas para controlar a enfermidade e evitar o dano acumulado. Objetivo: conhecer e comparar a resposta ao tratamento de pacientes com NL proliferativa na etapa de indução com duas pautas de tratamento com prednisona (PDN): doses iniciais reduzidas 30 mg/d. Método: foram comparadas variáveis clínicas, analíticas e terapêuticas de pacientes com NL proliferativa divididos em dois grupos segundo a dose inicial de prednisona (PDNi) padrão ou reduzida. Resultados: 21 pacientes com NL proliferativa (n=12 PDNi reduzida vs. n=9 PDNi estândar) foram estudados. Não foram observadas diferenças significativas nas variáveis clínicas e analíticas. Observou-se uma diferença estatisticamente significativa no número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) e nas doses de prednisona acumulada aos 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). Não foram observadas diferenças significativas na proporção de pacientes que alcançaram a resposta completa, no tempo até alcançá-la nem nos efeitos adversos entre ambos grupos. Conclusões: o esquema terapêutico do grupo PDNi <30 mg/d foi associado a uma menor dose acumulada de prednisona em resposta ao tratamento comparável, o que sugere menos dano cumulativo relacionado ao uso de glicocorticoides.

Effects of salbutamol, montelukast and prednisone on orthodontic tooth movement in rats / Efeitos do salbutamol, montelucaste e prednisona no movimento dentário ortodôntico em ratos

Objective: To evaluate the effect of salbutamol, montelukast, and prednisone on orthodontic tooth movement in rats. Material and Methods: In vivo experimental preclinical study. The sample consisted of 48 rats randomly distributed in four study groups. Group A was given saline solution; to group B, salbutamol 4 mg/Kg; to group C, montelukast 2.5 mg/Kg and to group D, prednisone 2.5 mg/Kg. All were fitted with orthodontic devices and the medications were administered intraperitoneally every 12 hours for 5 days. The clinical evaluation (variation in the interincisal distance) was performed at one, three, five, and seven days and the histopathological analysis (cell count) at five and seven days. Results: In the clinical evaluation of the variation in the interincisal distance, a significant difference was found in all the evaluations (p <0.05). It was found that the salbutamol group presented higher variation values in the interincisal distance on all the days evaluated. In the histopathological analysis at five and seven days, it was found that the osteoblast and osteocyte count was significantly higher in the salbutamol group compared to the other groups (p <0.05). However, in the subgroup analysis, it was found that there was no significant difference in the osteoblast and osteocyte count between the prednisone, montelukast, and control group (p> 0.05). Conclusion: The administration of salbutamol increased the magnitude of orthodontic tooth movement; nonetheless, the administration of montelukast and prednisone did not modify the magnitude of orthodontic tooth movement in rats. (AU)

Objetivo: Avaliar o efeito do salbutamol, montelucaste e prednisona no movimento dentário ortodôntico em ratos. Material e métodos: Estudo pré-clínico experimental in vivo. A amostra foi composta por 48 ratos distribuídos aleatoriamente em quatro grupos de estudo. O grupo A recebeu solução salina; para o grupo B, salbutamol 4 mg/kg; ao grupo C, montelucaste 2,5 mg/kg e ao grupo D, prednisona 2,5 mg/kg. Todos foram equipados com dispositivos ortodônticos e os medicamentos foram administrados por via intraperitoneal a cada 12 horas por 5 dias. A avaliação clínica (variação da distância interincisal) foi realizada em um, três, cinco e sete dias e a análise histopatológica (contagem de células) em cinco e sete dias. Resultados: Na avaliação clínica da variação da distância interincisal, houve diferença significativa em todas as avaliações (p <0,05). Verificou-se que o grupo salbutamol apresentou maiores valores de variação na distância interincisal em todos os dias avaliados. Na análise histopatológica aos cinco e sete dias, verificou-se que a contagem de osteoblastos e osteócitos foi significativamente maior no grupo salbutamol em comparação aos demais grupos (p<0,05). No entanto, na análise de subgrupos, verificou-se que não houve diferença significativa na contagem de osteoblastos e osteócitos entre os grupos prednisona, montelucaste e controle (p>0,05). Conclusão: A administração de salbutamol aumentou a magnitude do movimento dentário ortodôntico; no entanto, a administração de montelucaste e prednisona não modificou a magnitude do movimento dos dentes ortodônticos em ratos. (AU)

Paracoccidiodomicosis suprarrenal / Adrenal paracoccidioidomycosis

La insuficiencia suprarrenal primaria es un defecto en la producción de glucocorticoides, mineralocorticoides y andrógenos sexuales. Los pacientes afectados por esta condición se caracterizan por concentraciones bajas de cortisol y deficiencia de aldosterona con hiponatremia e hiperpotasemia concomitantes. La etiología más común es el desarrollo de anticuerpos contra la enzima 21 hidroxilasa. Otra causa importante de la insuficiencia suprarrenal primaria son las enfermedades infecciosas, en especial en los países de bajos ingresos. Entre las causas infecciosas que se han descrito se encuentran: Mycobacterium tuberculosis, el complejo de Mycobacterium avium, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, citomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. y Paracoccidioides brasiliensis. En este artículo se presenta la imagen de la tomografía de un paciente que presentó falla suprarrenal, con masas en las glándulas suprarrenales, cuya biopsia permitió establecer el diagnóstico final de paracoccidioidomicosis.

Primary adrenal insufficiency is a defect in glucocorticoid, mineralocorticoid and sexual androgens production. Patients with this disorder have low cortisol levels and aldosterone deficiency with concomitant hyponatremia and hyperkalemia. The most common etiology of this disease is the production of antibodies against the enzyme 21 hydroxylase. Another common cause, particularly in low income countries, are infectious diseases. Several micro-organisms have been reported as a causal agent in adrenal insufficiency including Mycobacterium tuberculosis, Mycobacterium avium complex, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, cytomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. and Paracoccidioides brasiliensis. In this article, we present the computerized tomography and the adrenal biopsy of a patient with adrenal insufficiency. The final diagnosis was paracoccidioidomycosis.

Síndrome hipereosinofílico con eritrodermia / Hypereosinophilic syndrome with erythrodermia

Varón de 77 años de edad, con antecedentes de insuficiencia cardiaca y fibrilación auricular recibiendo warfarina, hipotiroidismo, diabetes mellitus e hiperuricemia, con historia de un año de lesiones dérmicas eritematosas y descamativas en el tronco, pruriginosas y con moderada eosinofilia recurrente. Después de descartarse causas alérgicas, parasitarias, autoinmunes y neoplásicas, se hizo el diagnóstico de síndrome hipereosinofílico idiopático. Recibió tratamiento con prednisona e hidroxiurea, consiguiéndose una remisión completa de la eosinofilia y mejoría progresiva de las lesiones dérmicas. (AU)

A 77-year-old male, with a history of heart failure and atrial fibrillation receiving warfarin, hypothyroidism, diabetes mellitus and hyperuricaemia, with a one-year history of erythematous and desquamative skin lesions in the trunk, pruritic and moderate recurrent eosinophilia. After allergic, parasitic, autoimmune and neoplastic causes were ruled out, the diagnosis of idiopathic hypereosinophilic syndrome was done. He was treated with prednisone and hydroxyurea, resulting in complete remission of eosinophilia and progressive improvement of dermal lesions. (AU)

Pitiriasis rubra pilaris atípica en pediatría. A propósito de un caso / Atypical pityriasis rubra pilaris in pediatrics. A case report

Resumen Pitiriasis rubra pilaris es una dermatosis eritematoescamosa infrecuente, de etiología desconocida producida por una alteración en la queratinización de la epidermis. Presenta una distribución bimodal con mayor incidencia en la primera y sexta década de vida. Posee una clínica heterogénea clasificada en 6 subtipos según Griffiths, de acuerdo a su presentación clínica y pronóstico. Sus principales hallazgos son pápulas hiperqueratósicas foliculares, queratodermia palmoplantar y placas eritematoesmamosas rojo-anaranjadas que pueden progresar a eritrodermia, con islas de piel sana. En niños las manifestaciones clínicas más frecuentes son la III y IV de la clasificación de Griffiths, según distintos estudios. La histología no es específica pero apoya el diagnóstico. Existen múltiples opciones terapéuticas según la extensión y severidad del cuadro. Presentamos el caso de un preescolar de 5 años de edad con diagnóstico de PRP atípica asociado a eritema extenso, con buena respuesta a corticoides sistémicos y posteriormente a retinoides tópicos.

Summary Pityriasis rubra pilaris (PRP) is an unusual erythematous squamous dermatosis of unknown etiology, caused by an alteration of keratinization in the epidermis. This disease presents a bimodal distribution, being its incidence greater in the first and sixth decade of life. It has a heterogeneous clinical manifestation, and, according to Griffiths, has been classified into 6 subtypes, based on clinical features and prognosis. The typical manifestations of this disease are follicular hyperkeratotic papules, palmoplantar keratoderma and orange-red scaly plaques that can progress to erythroderma, with islands of sparing. According to different studies, the most frequent clinical manifestations in children are type III and IV according to Griffiths classification. Histology is not specific but supports diagnosis. There are multiple therapeutic options, depending on the extension and severity of the disorder. This review presents the case of a 5-year- old child case with a diagnosis of atypical PRP associated with extensive erythema, his response to treatment of systemic corticosteroids and later to topical retinoids being good.

Distrofia muscular de Duchenne: reportes de caso / Duchenne Muscular Dystrophy: Case Reports

La distrofia muscular de Duchenne es una enfermedad muscular grave ligada al cromosoma X que afecta el gen que codifica la distrofina, proteína fundamental para el mantenimiento de la fibra muscular. Se caracteriza por debilidad muscular de inicio en la infancia que sigue un curso progresivo. Sin intervención alguna, los pacientes pierden la marcha antes de la adolescencia y el fallecimiento ocurre en la segunda década de la vida por complicaciones respiratorias o problemas cardiacos. Actualmente no existe tratamiento curativo, pero la terapia con corticoides y el manejo multidisciplinario y ortopédico modifican la historia natural de esta miopatía. En este artículo se presentan dos casos clínicos de niños que presentaron dificultad para realizar actividades físicas vigorosas. Se les diagnosticó distrofia muscular de Duchenne confirmada por creatina-fosfocinasa y electromiografía, con mejoría del cuadro clínico, gracias al tratamiento instaurado.

The Duchenne muscular dystrophy is a bound serious muscular illness to the X-linked chromosome that affects to the gene that codes the dystrophin, protein important for the maintenance of the muscle fiber. It is characterized by muscle weakness of beginning in the childhood that follows a progressive course. Without intervention some, the patients lose the march before the younger and the death happens in the second decade of life for breathing complications or heart problems. At the moment it doesn't exist healing treatment, but the therapy with corticosteroids and the handling several disciplines and orthopedic they modify the natural history of this muscle disorders. In this article, we present two clinical cases of children who had difficulty prefoming vigorous physical actives. Diagnosis of Duchenne muscular dystrophy confirmed by creatin phosphokinase and electromyography with improvement of the clinical picture thanks to the treatment provided.

Sarcoma de Kaposi por dosis altas de esteroides / Kaposi´s sarcoma due to high dose of steroids

Se describe el caso clínico de una paciente de 42 años de edad, quien necesitó tratamiento reiterado con prednisona y a partir de entonces comenzó a presentar lesiones cutáneas en ambas piernas. Luego de ser valorada por especialistas en reumatología y dermatología fue remitida al Servicio de Angiología, donde se le realizó biopsia (con el uso de la coloración de hematoxilina-eosina), cuyos resultados fueron compatibles con un sarcoma de Kaposi, atribuible al suministro de esteroides.

The case report of a 42 years patient is described who needed repeated treatment with prednisone and from that time on she began to present cutaneous lesions in both legs. After being evaluated by reumatology and dermatology specialists, she was referred to the Angiology Service, where a biopsy was taken (with the use of the hematoxylin-eosine stain) which results were compatible with a Kaposi´s sarcoma, attributable to steroids supply.

Actualización de hepatitis autoinmune en pediatría: Reporte de un caso clínico y revisión de la literatura / Update on autoimmune hepatitis in pediatrics: Report of a clinical case and review of literature

La hepatitis autoinmune (HAI) es un proceso inflamatorio crónico y progresivo del hígado, de etiología desconocida. Se caracteriza por presentar niveles elevados de aminotransferasas e inmunoglobulina G (IgG), autoanticuerpos séricos y actividad necroinflamatoria en la histología; en ausencia de una patología conocida que pueda afectar al hígado. Predomina en el sexo femenino. Se describen dos tipos de acuerdo a los autoanticuerpos encontrados. El tratamiento se basa en la inmunosupresión, con el objetivo de evitar la progresión a cirrosis y falla hepática. Los pacientes no respondedores, o que debutan con falla hepática aguda pueden requerir de trasplante hepático. El objetivo es realizar una revisión del tema a partir de un caso clínico. Se presenta a una adolescente de 14 años, derivada para estudio por probable patología autoinmune. El diagnóstico inicial fue de probable HAI, que se presentó como una falla hepática grave-fulminante. Posteriormente al descartar otras etiologías (infecciosas y metabólicas), presentar autoanticuerpos antinucleares (ANA) positivos y evidenciar cirrosis en la punción biópsica hepática, se confirmó el diagnóstico de cirrosis autoinmune. Se inició tratamiento con prednisona y azatioprina, con buena respuesta clínica y de laboratorio. El diagnóstico oportuno de HAI y el inicio del tratamiento en forma temprana evitan en la mayoría de los casos la progresión de la enfermedad y el requerimiento de trasplante hepático.

Autoimmune hepatitis is a chronic and progressive inflammatory process of the liver, of unknown etiology. It is characterized by presenting increased levels of aminotransferases and immunoglobulin G (IgG), serum antibodies and histologic necro-inflamatory activity, with unknown pathology that may affect the liver. It is more frequent in women, Two types of autoimmune hepatitis are described, according to the antibodies found. Treatment is based on immunosuppression, with the objective of avoiding progression to cirrhosis and liver failure. Patients who do not respond or who present with severe liver failure may require liver transplant. The objective of the study is to review the topic based on a clinical case. The study presents the case of a 14 year old adolescent who is referred to be examined for possible autoimmune pathology. Initial diagnosis was probable autoimmune hepatitis, which presented with acute liver failure. Subsequently, when other etiologies were discarded (infectious and metabollic), positive antinuclear autoantibodies were present and liver hepatic biopsy evidenced cirrhosis, autoimmune cirrhosis was confirmed. Treatment was initiated with prednisone and azathioprine, being the clinical and lab response good. In most cases, timely diagnosis of autoimmune hepatitis and early initiation of treatment avoid progression of the disease and the need for a liver transplant.

Retroperitoneal fibrosis: case series of five patients and review of the literature / Fibrose retroperitoneal: série de cinco casos e revisão da literatura

ABSTRACT Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature.

RESUMO Periaortite crônica (PC) é um termo genérico usado para descrever um grupo de condições nosologicamente ligadas que incluem a fibrose idiopática retroperitoneal (doença de Ormond), o aneurisma da aorta abdominal inflamatório e a fibrose retroperitoneal perianeurismática. O termo fibrose retroperitoneal engloba uma gama de doenças que se caracterizam pela presença de um tecido fibroinflamatório que geralmente envolve a aorta abdominal e as artérias ilíacas, se estende ao retroperitôneo e envolve estruturas ureterais vizinhas. A fibrose retroperitoneal geralmente é idiopática, mas pode também ser secundária ao uso de determinados fármacos, doenças malignas, infecções e cirurgia. Este estudo descreve o seguimento por cinco anos (2006-2011) de cinco pacientes internados em nosso hospital que apresentavam sintomas e achados laboratoriais, de imagem e patológicos compatíveis com a fibrose retroperitoneal. Revisou-se a evolução clínica dos pacientes, que foi comparada com os achados da literatura.

Síndrome de Tolosa-Hunt / Tolosa-Hunt syndrome

RESUMO A Síndrome de Tolosa Hunt é uma doença rara, cuja etiopatogenia é desconhecida. Apresenta-se como uma oftalmoplegia dolorosa de um ou mais nervos cranianos oculomotores, que regride espontaneamente e responde bem ao tratamento com corticoides. O presente estudo trata-se de um relato de caso de um paciente que apresentou seguidos casos de oftalmoplegias dolorosas, envolvendo o nervo oculomotor e o abducente sendo tratado com corticoesteroides obteve uma resposta dramática. Objetiva-se ainda descrever as características fisiopatológicas, clínicas, o diagnóstico diferencial, visto que é um diagnóstico de exclusão, e medidas terapêuticas instituídas de acordo com o International Headache Society 2004 (ISH-2004) através da apresentação do caso clínico conduzido com as normas do estudo supracitado.

ABSTRACT Tolosa Hunt syndrome is a rare disease, whose etiology is unknown. It presents as a painful ophthalmoplegia of one or more oculomotor cranial nerves, which resolves spontaneously and responds well to treatment with corticosteroids. This study is a case report of a patient who had followed painful oftalmoplegias cases involving the oculomotor and abdcens nerves being treated with corticosteroids, obtaining a dramatic response. Another goal is to describe the pathophysiological, clinical, differential diagnosis, since it is a diagnosis of exclusion, and the therapeutic measures adopted according to the International Headache Society 2004 (ISH-2004) by presenting the case study conducted with the standards the study cited above.

Pioderma gangrenoso. Presentación de un caso / Gangrenous pyoderma. Case presentation

El pioderma gangrenoso es una enfermedad de etiología desconocida, con variedad de manifestaciones clínicas especialmente cutáneas, de difícil diagnóstico, de evolución crónica, con exacerbaciones y remisiones frecuentes, en la cual no existe un tratamiento de elección efectivo, por lo que su respuesta a la terapia es muy variable. Se presentó el caso de una mujer, mestiza, de 64 años de edad, con antecedentes patológicos personales de gastritis, hipertensión arterial y cardiopatía isquémica, con un año de evolución de una lesión ulcerada en primer artejo de pie derecho, con fracasos terapéuticos anteriores al diagnóstico. Se le realizaron estudios hematológicos e histopatológico, confirmándose un pioderma gangrenoso, que respondió favorablemente a la terapia con prednisona, dapsona y oxigenación hiperbárica.

The gangrenous pyoderma is a disease of unknown etiology, with various clinical manifestations, especially cutaneous ones, of difficult diagnosis, chronic evolution and frequent exacerbations and remissions. There is not an effective elective treatment, so the answer to therapy is very variable. We presented the case of a female patient, mestiza, aged 64 years, with personal pathological antecedents of gastritis, arterial hypertension and ischemic cardiopathy, showing a year of evolution ulcerous lesion on the first joint of the right foot with therapeutic failures before the diagnosis. We ordered hematologic and histopathologic studies confirming a gangrenous pyoderma that favorably answered to the therapy with prednisone, dapsone and hyperbaric oxygenation.

Eficiencia del tratamiento con esteroides para la oftalmopatía tiroidea: Revisión narrativa con rigor metodológico

La Oftalmopatía Tiroidea (OT) es la manifestación extra-tiroidea más común y más importante de la enfermedad tiroidea autoinmune. El uso de esteroides en administración oral o endovenosa (EV) es una de las estrategias para el tratamiento. En esta revisión se evalúa el efecto de la administración de esteroides por vía EV comparativamente con esteroides administrados por vía oral en el tratamiento de la OT. Para ello, se identificaron ensayos clínicos disponibles en la literatura desde enero de 1980 hasta noviembre de 2013. Los estudios fueron seleccionados de forma independiente por los revisores. Se evaluaron 4 ensayos clínicos. Todos los pacientes que recibieron algún tipo de esteroide, sin importar la forma de administración presentaron mejoría con respecto a su condición antes del inicio del tratamiento. Tres de los estudios mostraron diferencias estadísticamente significativas en la mayoría de los parámetros evaluados en favor de los que recibieron el esteroide por vía EV. En general, los esteroides son bien tolerados, pero los efectos adversos son más frecuentes en los pacientes que reciben la terapia esteroidea por vía oral (p˂0,0001). Tres de los estudios utilizaron un mismo esquema y dosis para la administración de metilprednisolona EV. Para la administración oral cada estudio presentó un esquema diferente. Se concluye que la evaluación de los pacientes que tienen indicación para el uso de esteroides para el manejo de la OT debe ser integral y multidisciplinaria. Los que mayor beneficio tienen son los que se presentan con OT clasificada como moderada o severa, y con un puntaje en escala de actividad >4. Los pacientes con OT que reciben tratamiento con metilprednisolona EV, comparados con los que reciben tratamiento con prednisona o metilprednisolona oral, tienen un mayor porcentaje de mejoría de la OT; además, ésta mejoría se observa de forma más temprana, con menor tasa de eventos adversos.

Thyroid-associated ophthalmopathy (TO) is the most common extra thyroid manifestation and the most important of autoimmune thyroid disease. The use of oral or intravenous steroids administration is one of the strategies for treating. This review evaluates the effect of intravenous steroid administration versus orally administered in the treatment of TO. Clinical trials were identified in the literature from January 1980 to November 2013. Studies were selected independently by the reviewers. We found four clinical trials comparing the use of intravenous versus oral steroid in the management of TO. All patients receiving steroid regardless of method of administration have improvement with respect to their condition before the start of treatment. Three studies show statistically significant differences in the improvement of the parameters evaluated in favor of receiving the steroid intravenously. Steroids are generally well tolerated, but adverse effects are more frequent in patients receiving oral therapy (p˂0,0001). Three of the studies used the same doses and schedule for administration of intravenous methylprednisolone. For oral administration each study presents a different scheme. We conclude that the evaluation of patients who receive steroids for the management of thyroid ophthalmopathy should be comprehensive and multidisciplinary. Those who have most benefit are those who present with moderate or severe classified as OT, and with a scale score of activity >4. TO patients receiving intravenous methylprednisolone therapy, compared to those treated with oral prednisone or methylprednisolone have a greater percentage of improvement in the TO, and this improvement was also observed earlier and with fewer adverse events.

Effects of prednisone on eosinophilic bronchitis in asthma: a systematic review and meta-analysis, / Efeitos da prednisona na bronquite eosinofílica na asma: revisão sistemática e meta-análise

OBJECTIVE: To evaluate the effect size of oral corticosteroid treatment on eosinophilic bronchitis in asthma, through systematic review and meta-analysis. METHODS: We systematically reviewed articles in the Medline, Cochrane Controlled Trials Register, EMBASE, and LILACS databases. We selected studies meeting the following criteria: comparing at least two groups or time points (prednisone vs. control, prednisone vs. another drug, or pre- vs. post-treatment with prednisone); and evaluating parameters before and after prednisone use, including values for sputum eosinophils, sputum eosinophil cationic protein (ECP), and sputum IL-5-with or without values for post-bronchodilator FEV1-with corresponding 95% CIs or with sufficient data for calculation. The independent variables were the use, dose, and duration of prednisone treatment. The outcomes evaluated were sputum eosinophils, IL-5, and ECP, as well as post-bronchodilator FEV1. RESULTS: The pooled analysis of the pre- vs. post-treatment data revealed a significant mean reduction in sputum eosinophils (↓8.18%; 95% CI: 7.69-8.67; p < 0.001), sputum IL-5 (↓83.64 pg/mL; 95% CI: 52.45-114.83; p < 0.001), and sputum ECP (↓267.60 µg/L; 95% CI: 244.57-290.63; p < 0.0001), as well as a significant mean increase in post-bronchodilator FEV1 (↑8.09%; 95% CI: 5.35-10.83; p < 0.001). CONCLUSIONS: In patients with moderate-to-severe eosinophilic bronchitis, treatment with prednisone caused a significant reduction in sputum eosinophil counts, as well as in the sputum levels of IL-5 and ECP. This reduction in the inflammatory response was accompanied by a significant increase in post-bronchodilator FEV1. .

OBJETIVO: Avaliar o tamanho do efeito do tratamento com prednisona oral na bronquite eosinofílica na asma por meio de revisão sistemática e meta-análise. MÉTODOS: Revisão sistemática de artigos nas bases de dados do Medline, Cochrane Controlled Trials Register, EMBASE e LILACS. Foram selecionados estudos que preencheram os seguintes critérios: comparar ao menos dois grupos ou dois momentos (prednisona vs. controle, prednisona vs. outra droga ou pré vs. pós-tratamento com prednisona) e avaliar parâmetros antes e depois do uso de prednisona, incluindo eosinófilos, proteína catiônica eosinofílica (PCE) e IL-5 no escarro - com ou sem valores de VEF1 pós-broncodilatador - com os IC95% correspondentes ou com dados suficientes para calculá-los. As variáveis independentes foram uso e dose de prednisona e duração do tratamento. Os desfechos avaliados foram eosinófilos, IL-5 e PCE no escarro, bem como VEF1 pós-broncodilatador. RESULTADOS: A análise conjunta dos dados de pré e pós-tratamento revelou uma redução significativa nas médias de eosinófilos no escarro (↓8,18%; IC95%: 7,69-8,67; p < 0,001), IL-5 no escarro (↓83,64 pg/mL; IC95%: 52,45-114,83; p < 0,001), PCE no escarro (↓267,60 μg/L; IC95%: 244,57-290,93; p < 0,001), assim como um aumento significativo na média de VEF1 pós-broncodilatador (↑8,09%; IC95%: 5,35-10,83; p < 0,001). CONCLUSÕES: Em pacientes com bronquite eosinofílica de moderada a grave, o tratamento com prednisona determinou uma redução significativa nos níveis de eosinófilos no escarro, assim como nos níveis de IL-5 e PCE no escarro. Essa redução na resposta inflamatória foi acompanhada de um aumento significativo do VEF1 pós-broncodilatador. .

Síndrome DRESS com manifestação renal grave: relato de caso / DRESS syndrome with several renal involvement: case report

Reação a fármacos com eosinofilia e sintomas sistêmicos, também chamada de síndrome DRESS, é uma reação adversa grave a fármacos, idiossincrática e com envolvimento de múltiplos órgãos. Os critérios diagnósticos incluem dermatose induzida por fármaco, anormalidades hematológicas e comprometimento sistêmico. A síndrome pode levar a altas taxas de mortalidades e não identificada precocemente. O objetivo deste estudo foi descrever o caso de um paciente que desenvolveu a síndrome DRESS depois do uso de antibiótico para tratamento de úlcera em membro inferior. Paciente do sexo masculino, negro, 70 anos, apresentou síndrome DRESS após o uso de ampicilina +sulbactam para quadro de úlcera venosa infectada em membro inferior direito. O caso compreendia eritrodermia com lesões erosadas e acometimento mucoso, caracterizando eritema multiforme major, eosinofilia >1.500cel./dL e lesão renal aguda. Foi tratado com prednisona oral (1mg/kg/dia), com remissão das lesões cutâneas, melhora da função renal e redução da leucocitose e eosinofilia. Relatou-se um caso clássico, cursando com síndrome DRESS e responsivo à corticoterapia oral. O tratamento com corticoide permanece controverso, devendo-se avaliar orisco-benefício em cada caso.(AU)

Drug reaction with eosinophilia and systemic symptoms, also calledDRESS syndrome is a severe adverse reaction to medication,idiosyncratic with involvement of multiple organs. Diagnosticcriteria include: drug-induced dermatosis, hematologicalabnormalities and systemic involvement. The syndrome canlead to high mortality rates if not promptly recognized. The aimof this study was to describe the case of a patient who developedDRESS syndrome after antibiotic use for lower limb ulcer. Malepatient, black, 70 years, presented DRESS syndrome after useof ampicillin + sulbactam for infected venous ulcer in the rightlower limb. The case included erythroderma with injuries anderoded mucosal involvement, featuring erythema multiformemajor, eosinophilia greater than 1,500cel/dL and acute kidneyinjury. He was treated with oral prednisone (1mg/kg/day), withremission of skin lesions, improvement in renal function andreduced leukocytosis and eosinophilia. We reported a classiccase of DRESS syndrome coursing with acute kidney injury,responsive to oral steroids. The treatment with corticosteroidsremains controversial and need to evaluate the risk-benefit ratioin each case.(AU)

Efeitos colaterais do uso da ribavirina, prednisona e DMSO em cães naturalmente infectados pelo vírus da cinomose / The side effects of ribavirin, prednisone and DMSO in dogs naturally infected by canine distemper virus

O estudo tem o objetivo de identificar efeitos indesejáveis da ribavirina, prednisona e DMSO em cães naturalmente infectados com o vírus da cinomose. Foram utilizados 60 cães apresentando quadro neurológico da cinomose com evolução de 10 dias. Os animais foram internados e receberam tratamento de suporte; foram avaliados diariamente e realizados hemograma, dosagem bioquímica e exame de urina tipo I. Os grupos 1 e 2 foram tratados com ribavirina e sua associação com DMSO; os grupos 3 e 4 com DMSO e prednisona e o grupos 5 com ribavirina e prednisona e o grupo 6 com ribavirina, prednisona e DMSO. Os animais foram anestesiados para a colheita de líquor, medula óssea e sangue, antes do tratamento para diagnóstico através da RT-PCR. As amostras negativas foram analisadas pela técnica de hn-PCR. Todos os animais apresentaram resultado positivo em pelo menos uma das duas reações. O efeito adverso da ribavirina e a sua associação com a prednisona foi a anemia hemolítica, que foi confirmada pela observação de bilirrubina na urina apenas dos cães tratados com ribavirina.

The present study aims at the identification of undesirable effects of ribavirin, predinisone and DMSO in dogs naturally infected by canine distemper virus. The research analyzed 60 dogs with clinical neurological signs and 10 days of evolution. The animals were hospitalized for the appropriate support treatment; were daily observed, and complete blood cells count, biochemical analysis, and urine exam type I were conducted. Groups 1 and 2 were treated with ribavirin and its combination with DMSO; Groups 3 and 4 treated with prednisone and DMSO, Group 5 treated with ribavirin and prednisone, while Group 6 with ribavirin, prednisone and DMSO. Before the treatment, animals were anesthetized for the cerebrospinal fluid, bone marrow and blood samples collection for the diagnosis based on RT-PCR. The negative samples were analyzed using the hn-PCR technique. All the animals presented positive results in at least one of the 2 tests. The adverse result of ribavirin and its association with prednisone was characterized by haemolytic anemia, confirmed by the evaluation of bilirrubin occurrence only in the urine of dogs treated with ribavirin.

Psicosis, lupus y corticosteroides: a propósito de un caso / Psychosis, lupus and corticosteroids: A case report

Se presenta el caso de una mujer de 35 años con diagnóstico de lupus eritematoso sistémico en tratamiento con corticosteroides y otros medicamentos, que desarrolla síntomas psicóticos. Dicha situación lleva a establecer el diagnóstico diferencial entre psicosis lúpica, psicosis por corticosteroides, psicosis por una infección concomitanteo como consecuencia de un trastorno metabólico.

We present the case of a 35-years-old woman, with diagnosis of systemic lupus erythematosus in treatment with corticosteroids and other medications, who developed psychotic symptoms. This situation leads to a differential diagnosis beetween lupic psychosis, corticosteroid psychosis, psychosis induced by concomitant infection or as a consequence of a metabolic disorder.

Study of dermatoses in kidney transplant patients / Estudo das dermatoses em pacientes transplantados renais

BACKGROUND: The increasing in the number of kidney transplant recipients has favored, more frequently than before, the emergence of dermatoses and warranted their study through subsequent publications. OBJECTIVES: to evaluate the frequency of dermatoses in kidney transplant recipients. METHODS: kidney transplant recipients with suspected dermatoses between March 1st 2009 and June 30th 2010. RESULTS: 53 patients (28 males and 25 females), aged between 22 and 69 (mean age = 45 years) were evaluated. Most of them came from the cities of Ceilândia, Samambaia and São Sebastião/DF, and had already been transplanted for 5 to 10 years before (37.7%); 62.3% were recipients of living donors and 83% were prednisone-treated. The most prevalent dermatoses were of fungal (45.3%) and viral (39.6%) etiologies. Among the non-melanoma malignant neoplasms, the basal cell carcinoma prevailed (six cases), in spite of the low incidence. Concerning fungal dermatoses, 12 cases of onychomycosis, five of pityriasis versicolor and four of pityrosporum folliculitis were reported. For diagnosis, in most cases (64.2%), laboratory examinations (mycological and histopathological) were performed. CONCLUSION: cutaneous manifestations in kidney transplant recipients are generally secondary to immunosuppression. The infectious dermatoses, especially those of fungal origin, are frequently found in kidney transplant recipients and their occurrence increases progressively according to the time elapsed from the transplantation, which makes follow-up important. .

FUNDAMENTOS: o crescente aumento do número dos transplantados renais tem favorecido o aparecimento mais frequente das dermatoses e permitido o estudo em sucessivos trabalhos. OBJETIVOS: avaliar a frequência das dermatoses em pacientes transplantados renais. MÉTODOS: captação de pacientes transplantados renais durante o período de 1° de março de 2009 a 30 de junho de 2010 com suspeita de dermatoses. RESULTADOS : foram avaliados 53 pacientes (28 homens e 25 mulheres), entre 22 e 69 anos (com uma média de 45 anos), a maioria procedente de Ceilândia, Samambaia e São Sebastião/DF, entre 5 e 10 anos de transplante renal (37,7%), sendo 62,3% receptor de doador vivo e 83% em uso de prednisona. As dermatoses mais prevalentes foram as de etiologia fúngica (45,3%) e viral (39,6%). Das neoplasias malignas não-melanoma, apesar da baixa incidência, predominou o carcinoma basocelular (seis casos). Com relação s dermatoses de origem fúngica, ocorreram 12 casos de onicomicoses, cinco casos de pitiríase versicolor e quatro casos de foliculite pitirospórica. Para realização do diagnóstico, na maioria dos casos (64,2%), foi utilizado os exames laboratoriais ( micológicos e histopatológicos). CONCLUSÃO: as manifestações cutâneas em pacientes transplantados renais são geralmente secundárias imunossupressão. As dermatoses infecciosas, principalmente as de etiologia fúngica, são frequentes em pacientes transplantados renais, e sua ocorrência aumenta progressivamente, conforme o tempo transcorrido, a partir do transplante, sendo importante o acompanhamento. .

Púrpura Schõnlein Henoch con compromiso renal: reporte de un caso / Henoch Schõnlein purpura with renal involvement: case report

INTRODUCCIÓN: El Púrpura Schõnlein Henoch es una vasculitis sistémica que afecta principalmente a la piel, articulaciones, sistema gastrointestinal y renal. Es una de las vasculitis más comunes en la infancia. El compromiso renal se da en aproximadamente el40 por ciento de los casos, y en la mayoría se presenta con hematuria, sin embargo, también puede manifestarse como proteinuria, síndrome nefrótico y nefrítico. PRESENTACIÓN DEL CASO: Se presenta el caso de una paciente de 6 años que consulta en Servicio de Urgencia por dolor en el tarso del pie izquierdo de 4 días de evolución, sin antecedentes de trauma, acompañándose de dolor en rodilla izquierda con limitación a la movilización, y lesiones purpúricas en extremidades inferiores. Durante la hospitalización, la paciente presentó al inicio presiones arteriales altas y oliguria, manejándose con antihipertensivos, además del inicio del tratamiento con corticoides. Luego de la mejoría del compromiso articular y cutáneo, se evidenció compromiso renal con proteinuria, que al persistir se aumentó las dosis de corticoides, teniendo favorable respuesta al disminuir sus niveles. Al encontrarse en mejores condiciones, se decidió manejo ambulatorio manteniendo tratamiento corticoidal y antihipertensivo, controlándose en Nefrología y Reumatología Infantil. DISCUSIÓN: Se concluye de este trabajo que la corticoterapia y el manejo de la presión arterial fueron pilares fundamentales para el tratamiento en este caso, mejorando su evolución clínica y principalmente la disminución de la proteinuria con el uso de los corticoides, evitando un compromiso mayor a pesar de que no esté comprobado su beneficio en la literatura.

INTRODUCTION: The Henoch Schõnlein Purpura is a systemic vasculitis that mainly affects the skin, joints, gastrointestinal system and renal system. It is one of the most common vasculitis in childhood. Renal involvement occurs in approximately40 percent of cases, and in most cases it occurs with hematuria. However, it may also manifest as proteinuria, nephrotic and nephritic syndrome. CASE REPORT: We report the case of a 6-year old patient who consulting in the emergency service for 4 days with pain in left foot tarsal, without a history of trauma, accompanied by pain in left knee with limited mobilization, and purpuric lesions in lower extremities. During hospitalization, the patient presented at the beginning high blood pressure and oliguria, handling itself with antihypertensive, in addition to the initiation of treatment with corticosteroids. After improvement of cutaneus and articular involvement, renal involvement was evident with proteinuria, at persist was increased doses of corticosteroids, with favorable response to decrease levels. Being in better conditions was decided ambulatory management keeping antihypertensive and corticosteroid treatment, controlling in Nephrology and Child Rheumatology. DISCUSSION: It can be concluded from this study that the steroids and blood pressure management were fundamental pillars for the treatment in this case, improving their clinical course and mainly the proteinuria decreased with the use of corticosteroids, preventing a greater commitment although is not verified in the literature their advantage.

Fibrosis retroperitoneal primaria / Primary retroperitoneal fibrosis

La fibrosis retroperitoneal es una enfermedad infrecuente con una epidemiología de 1/1 000 000 personas/año, más frecuente en el hombre que en la mujer, en la edad media de la vida y en el 75 % de los casos, es de causa primaria o desconocida. Paciente de 43 años de edad, con antecedentes de salud, que acude al médico de la familia con malestar general y dolor lumbar asociado a alteraciones del sedimento urinario que se interpretó como infección urinaria. Se comenzó tratamiento antibiótico con ciprofloxacino, sin mejorìa. Llega al cuerpo de guardia donde es atendido por la guardia de nefrología que indica análisis complementarios de urgencia. Los resultados fueron: creatinina con valores de 1 024 µmol/L, acidosis metabólica con anión gap elevado y ultrasonido con riñones hidronefróticos. LLeva tratamiento con hemodiálisis en tres sesiones. En la discusión de caso complejo se valoró el diagnóstico de fibrosis retroperitoneal, que se corroborró por tomografía axial computarizada a las 72 h de su llegada al centro hospitalario. Se comenzó tratamiento con metilprednisolona por 3 días, seguido de prednisona y ciclofosfamida durante dos años, con estudios de química sanguínea, hemograma completo y estudios de orina en correspondencia con la respuesta al tratamiento. Se alcanza la remisión completa de la enfermedad sin recaídas.

Retroperitoneal fibrosis is a rare disease. Its epidemiology is a 1/1 million persons/year, It is more common in men than in women, ocuring in midle age, and its cause is primary or unknown in 75 % of cases. A 43 year-old patient, who had previous health history, referred to his family doctor not to feel well and with back pain associated with abnormalities of the urinary sediment, which was interpreted as urinary tract infection. Treatment started with ciprofloxacin antibiotic with no improvement. This patient was attended at nefrology guard, where emergency supplemental analysis were indicated. Results showed: creatinine values of 1024 micromol/L, high anion gap metabolic acidosis, and hydronephrotic kidney ultrasound. three hemodialysis sessions were indicated. At discussion of complex cases, a retroperitoneal fibrosis diagnosis was assessed, which was confirmed by CT scan at 72 hours of hospital arrival. Methylprednisolone therapy during three days was iniciated, followed by prednisone and cyclophosphamide for two years, with blood chemistry studies, complete blood count, and urine studies in correspondence with treatment response. complete remission of the disease without relapse was achieved.