Neumonitis por hipersensibilidad: a propósito de un caso

La neumonitis por hipersensibilidad o alveolitis alérgica extrínseca se produce como consecuencia de la inhalación repetida de determinadas sustancias orgánicas o antígenos que, en individuos susceptibles, puede producir una inflamación de las paredes alveolares, bronquiolo e intersticio con alteración del intercambio gaseoso. Su presentación clínica puede ser muy diversa según la edad del paciente. Los niños de mayor edad suelen debutar con cuadros de disnea de esfuerzo que progresa hasta aparecer en reposo, hipoxemia y estertores crepitantes en la auscultación. No hay ninguna prueba definitiva que diagnostique por sí misma la enfermedad, pero los hallazgos analíticos, pruebas de imagen y función pulmonar apoyan la sospecha clínica. La medida más importante de tratamiento es evitar la exposición al antígeno responsable. En algunas ocasiones puede ser útil la utilización de corticoides orales. A continuación, presentamos el caso de un menor con datos clínicos de neumonitis por hipersensibilidad secundaria a la exposición a aves con hipertensión pulmonar secundaria. (provisto por Infomedic International)

Hypersensitivity pneumonitis or extrinsic allergic alveolitis occurs because of the repeated inhalation of certain organic substances or antigens which, in susceptible individuals, can cause inflammation of the alveolar, bronchiole and interstice with gas exchange disorder.  Its clinical presentation can be very diverse according to the age of the patient. Older children usually present as dyspnea with exercises that progress to appear at rest, hypoxemia, and rattles on auscultation. There is no definitive test that by itself diagnoses the disease, but laboratory findings, imaging tests, and pulmonary function support clinical suspicion. The most important measure of treatment is to avoid exposure to the responsibility antigen. Sometimes, the use of oral corticosteroids may be useful. Below, we present the case of a minor with clinical data of hypersensitivity pneumonitis secondary to exposure to birds with secondary pulmonary hypertension. (provided by Infomedic International)

Hematological and biochemical parameters of premature kids of goats subjected to different dexamethasone protocols / Parâmetros hematológicos e bioquímicos de cabritos prematuros de cabras submetidas a diferentes protocolos de dexametasona

Corticosteroid therapy has been used for ruminants to allow lung maturation and the birth of premature babies. However, when considering laboratory analyses of these animals, very little data is available regarding hematological and biochemical patterns, especially for premature goats, and the effects of corticotherapy on these parameters are unknown. In this context, the objective of this study was to evaluate the hematological and biochemical parameters during the first hours of life of premature kids from goats subjected to different dexamethasone protocols. For this, the goats were divided into four groups: group I, goats that received 20 mg of dexamethasone at 139 days of gestation; group II, 2 mg of dexamethasone from the 133rd to 136th day of gestation, 4 mg from the 137th to 139th, and 20 mg on the 140th; group III, 16 mg of dexamethasone from the 139th day, with repeated doses every 12 h until elective surgery; and group IV, goats that received 4, 8, 16, and 20 mg of dexamethasone at 137, 138, 139, and 140 days of gestation, respectively. Blood samples were obtained at birth (T0h) and after 1 (T1h), 12 (T12h), 24 (T24h), and 48 h (T48h) of life for hemogram and serum biochemistry assessment of urea, creatinine, total protein (PT), and gamma-glutamyltransferase (GGT). PT levels and GGT activity were lower at birth in all groups and rose after colostrum ingestion. The creatinine values for all the experimental groups did not differ between T0h and T1h; however, they decreased in the subsequent moments. Except for group I, urea concentrations were higher at T48h than at T1h. The red blood cell, hemoglobin, hematocrit, and mean corpuscular hemoglobin counts decreased over time. The total leukocyte count behaved differently in different experimental groups, and was influenced by the levels of dexamethasone, mainly due to the change in the counts of segmented neutrophils and lymphocytes. It was concluded that significant changes in the hematological and biochemical parameters occur in the first hours of life of premature kids, and that the treatment of goats with dexamethasone can affect these parameters in a dose-dependent manner.

A corticoterapia tem sido empregada em ruminantes com o objetivo de permitir a maturação pulmonar e o nascimento de filhotes prematuros. Entretanto, ao se considerar análises laboratoriais desses animais, pouquíssimos dados estão disponíveis quanto aos padrões hematológicos e bioquímicos, especialmente em caprinos prematuros, e tampouco se sabe sobre os efeitos da corticoterapia sobre parâmetros hematológicos e bioquímicos nesses animais. Nesse contexto, objetivou-se avaliar os parâmetros hematológicos e bioquímicos das primeiras horas de vida de cabritos prematuros provenientes de cabras que utilizaram diferentes protocolos de dexametasona. Para tal, as cabras foram divididas em três grupos: grupo I, cabras que receberam 20 mg de dexametasona aos 139 dias de gestação; grupo II, 2 mg de dexametasona do 133° ao 136° dia de gestação, 4 mg do 137° ao 139° e 20 mg no 140° dia; grupo III, 16 mg de dexametasona a partir do 139° dia, com doses repetidas a cada 12 horas até a cirurgia eletiva; e grupo IV, cabras que receberam 4, 8, 16 e 20 mg de dexametasona no 137°, 138°, 139° e 140° dias de gestação, respectivamente. As amostras sanguíneas dos cabritos foram obtidas ao nascimento (T0h), 1 (T1h), 12 (T12h), 24 (T24h) e 48 horas (T48h) de vida para avaliação do hemograma e bioquímica sérica de ureia, creatinina, proteína total (PT) e gamaglutamiltransferase (GGT). Os teores de PT e atividade de GGT foram menores ao nascimento em todos os grupos, elevando-se após ingestão do colostro. Os valores de creatinina em todos os grupos experimentais não diferiram entre T0h e T1H, entretanto, diminuíram nos momentos subsequentes. Com exceção do grupo I, as concentrações de ureia foram maiores no T48h em comparação com o T1h. Os valores de hemácias, hemoglobina, hematócrito e volume corpuscular médio diminuíram ao longo do tempo, enquanto a concentração de hemoglobina corpuscular média aumentou ao longo do tempo. A contagem leucocitária total se comportou de maneira distinta nos diferentes grupos experimentais, demonstrado ser influenciada pelos teores de dexametasona principalmente em decorrência da alteração nas contagens de neutrófilos segmentados e linfócitos. Conclui-se que alterações significativas dos parâmetros hematológicos e bioquímicos ocorrem nas primeiras horas de vida de cabritos prematuros e o tratamento de cabras com dexametasona também pode afetar tais parâmetros de forma dependente da dose.

Corticoterapia sistémica en el período post-agudo de la neumonía por COVID-19 con tórpida evolución clínico-radiológica / Systemic corticosteroid therapy in the post-acute period of COVID-19 pneumonia with torpid clinical and radiological evolution

La neumonía por COVID-19 genera tanto daños inmediatos por efecto viral como alejados por desregulación inmunológica inflamatoria. La corticoterapia sistémica ha demostrado ser beneficiosa en la primer parte del proceso, pero aún se desconoce con certeza su utilidad en el daño post agudo. El número de pacientes afectados hace imperativo encontrar terapéuticas que reduzcan las potenciales secuelas pulmonares. Se incluyeron en esta serie de casos 18 pacientes internados en instituciones médicas privadas polivalentes de la Ciudad de Buenos Aires: 15 de sexo masculino y 3 femenino y edad 58,4 ± 13,6 años. Antecedentes comórbidos más frecuentes: HTA (4 pacientes), obesidad (6 pacientes) y tabaquismo (4 pacientes). Cinco pacientes no presentaban ningún antecedente médico. Todos los incluidos presentaron disnea, desaturación de oxígeno y alteraciones tomográficas persistentes o en progresión, posteriores a los 14 días de su infección. Todos recibieron dexametasona acorde a las normativas vigentes. Posteriormente, dada la mala evolución, fueron tratados con corticoesteroides orales y/o endovenosos aplicando el tratamiento utilizado para neumonía organizada (OP) secundaria. En 6 de los pacientes se realizó biopsia transbronquial describiéndose patrón OP en 3 de ellos. Cuatro semanas después de iniciado dicho tratamiento todos los pacientes presentaron mejoría clínica expresada por menor disnea, ningún paciente mantuvo el requerimiento de oxígeno y todas las tomografías de tórax mostraron clara disminución de la afectación parenquimatosa pulmonar. Los corticoides sistémicos administrados en el periodo post agudo de la COVID-19 tienen un efecto beneficioso clínico y radiológico.

Corticoterapia con intención de rescate en pacientes con hepatitis autoinmune severa: ¿el trasplante hepático es evitable?

Resumen: Introducción: La hepatitis autoinmune es una enfermedad cuya presentación clínica inicial puede manifestarse de diversas formas, siendo su presentación aguda con aumento de los niveles de bilirrubina, caída de la función biosintética y necrosis masiva/submasiva o cirrosis en la histología, marcadores de severidad de la misma. La decisión de iniciar tratamiento con corticoides en este escenario resulta un desafío en la práctica clínica. Los objetivos del presente trabajo fueron evaluar el grado de respuesta al tratamiento, los predictores de respuesta, y la supervivencia global y libre de trasplante en pacientes cuyo debut clínico fue de forma aguda y severa. Resultados: Fueron incluidos 33 pacientes con bilirrubina total mayor a 2.5 mg/dl, sin tratamiento previo (naive), que cumplían criterios diagnósticos según el grupo internacional de hepatitis autoinmune. El 97% eran mujeres con una mediana de edad de 52 años, el 58% se encontraba en etapa de cirrosis con un MELD promedio de 24, y el 15% presentaba una necrosis masiva/submasiva en la muestra de biopsia hepática. En 27 casos se inició corticoterapia. El 66.7 % evolucionó con remisión completa, 14.8 % con remisión parcial, y en 18.5% hubo falla al tratamiento corticoideo. La colesterolemia basal y la presencia de encefalopatía hepática fueron predictores de no respuesta al tratamiento corticoideo, mientras que el MELD pre-tratamiento y la colesterolemia basal fueron las variables estadísticamente significativas asociadas a falla en el rescate del trasplante hepático. En los pacientes tratados con corticoides, la supervivencia global y libre de trasplante a 5 años en función del MELD (<25 vs ≥ 25) fue del 90% vs 60% respectivamente. Conclusión: Las altas tasas supervivencia logradas bajo tratamiento médico que fueron evidenciadas en esta serie reafirman la necesidad de priorizar el uso de corticoides en pacientes con hepatitis autoinmune aguda y severa.

Abstract: Introduction: Autoimmune hepatitis can initially manifest itself in various clinical ways. Its acute presentation with increased levels of bilirubin, fall of biosynthetic function and massive/submassive necrosis or cirrhosis in histology, is a marker of severity of it. The decision to start corticosteroid treatment in this scenario is a challenge in clinical practice. The objectives of this work were to assess the degree of response to treatment, response predictors, and overall and transplant-free survival in patients whose clinical debut was acute and severe. Results: 33 patients with total bilirubin greater than 2.5 mg/dL, without prior (naive) treatment, who met diagnostic criteria according to the international autoimmune hepatitis group, were included. 97% were women with a median age of 52, 58% were in the cirrhosis stage with an average MELD of 24, and 15% had massive/submassive necrosis in the liver biopsy sample. In 27 cases treatment with cortiocosteroids was initiated, 66.7% evolved with complete remission, 14.8% with partial remission, and in 18.5% there was failure of corticosteroid treatment. Basal cholesterol and the presence of hepatic encephalopathy were predictors of non-response to corticosteroid treatment, while pre-treatment MELD and basal cholesterol were the statistically significant variables associated with liver transplant rescue failure. In patients treated with corticosteroids, overall and transplant-free survival at 5 years based on MELD (<25 vs ≥ 25) was 90% vs 60% respectively. Conclusion: The high survival rates achieved under medical treatment that were demonstrated in this series reaffirm the need to prioritize the use of corticosteroids in patients with acute and severe autoimmune hepatitis.

Resumo: Introdução: A hepatite autoimune é uma doença cuja apresentação clínica inicial pode se manifestar de várias formas, sendo sua apresentação aguda com níveis elevados de bilirrubina, descida na função biossintética e necrose maciça/submassiva ou cirrose em histologia, marcadores de gravidade dela. A decisão de iniciar o tratamento corticosteroide nesse cenário é um desafio na prática clínica. Os objetivos deste trabalho foram avaliar o grau de resposta ao tratamento, preditores de resposta e sobrevida geral e livre de transplantes em pacientes cuja estréia clínica foi aguda e grave. Resultados: Foram incluídos 33 pacientes com bilirrubina total superior a 2,5 mg/dL, sem tratamento prévio (ingênuo), que atendiam aos critérios diagnósticos segundo o grupo internacional de hepatite autoimune. 97% eram mulheres com idade mediana de 52 anos, 58% estavam em estágio de cirrose com um MELD médio de 24, e 15% tinham necrose maciça/submassiva na amostra de biópsia hepática. Em 27 casos foi iniciado o tratamento médico e, em 6 casos, o transplante de fígado foi alcançado sem terapia prévia. 66,7% evoluíram com remissão completa, 14,8% com remissão parcial e, em 18,5%, houve falha no tratamento corticosteroide. O colesterol basal e a presença de encefalopatia hepática foram preditores de não resposta ao tratamento corticosteroide, enquanto o meld pré-tratamento e o colesterol basal foram as variáveis estatisticamente significativas associadas à falha de resgate do transplante de fígado. Em pacientes tratados com corticosteroides, a sobrevida geral e livre de transplante aos 5 anos com base no MELD (<25 vs ≥ 25) foi de 90% vs 60%, respectivamente. Conclusão: As altas taxas de sobrevivência alcançadas sob tratamento médico demonstrado nesta série reafirmam a necessidade de priorizar o uso de corticosteroides em pacientes com hepatite autoimune aguda e grave.

Incidencia de infecciones en pacientes con artritis reumatoide en tratamiento corticoideo e inmunosupresor / Incidence of infections in patients with rheumatoid arthritis in corticosteroid and immunosuppressive treatment

Introducción La artritis reumatoide (AR) es una enfermedad inflamatoria autoinmune, que requiere tratamiento farmacológico por tiempo muy prolongado. Se prescribe para su tratamiento fármacos con capacidad antiinflamatoria y potencial efecto inmunosupresor. Con estos fármacos los pacientes presentan menor intensidad inflamatoria, pero a la vez existe el riesgo de procesos infecciosos, siendo éstos, una importante causa de morbilidad y mortalidad. Objetivo Analizar la incidencia de infección durante el tratamiento corticoideo vs inmunosupresor en la terapia crónica en pacientes con artritis reumatoide. Material y métodos Estudio de tipo analítico, observacional, retrospectivo, con selección por intención, no controlado, de cohorte, con estudio de riesgo, incidencia y asociación cruzada, realizado en el Servicio de Reumatología del Hospital de Clínicas de La Paz, Bolivia; en pacientes con diagnóstico de AR entre las gestiones 2010 - 2020, tanto en consultorio externo y sala de internación. Se seleccionaron pacientes con adultos, ambos sexos, con edad mayor a 18 años, con diagnóstico de artritis reumatoide según clasificación ACR 2010, clinimetría CDAI en grado de actividad, con indicación de tratamiento asociado (corticoide + metotrexato o incluso adición de cloroquina / hidroxicloroquina) de acuerdo a guías ACR/EULAR, que realice control regular (bimensual). Resultados La incidencia de procesos infecciosos asociada al uso de prednisona con dosis >7,5mg/día, tras 6 meses de tratamiento es de 19,39% y de 18,88% a los 12 meses. La incidencia de procesos infecciosos asociada al uso de metotrexate (dosis >12,5mg/semana), es de 12,76% a 6 meses de tratamiento y de 13,27% a 12 meses. Conclusiones En pacientes adultos de ambos sexos, sin factores de riesgo asociados, la corticoterapia prolongada (Prednisona en dosis mayor a 7.5mg/día durante más de 6 meses de tratamiento) produce el desarrollo de procesos infecciosos con mayor frecuencia y gravedad que la terapia con metotrexate incluso a dosis intermedia-alta (MTX >12.5mg / semana).

Introduction Rheumatoid arthritis is an autoimmune inflammatory disease, which requires pharmacological treatment for a very long time. It is prescribed for its treatment drugs with anti-inflammatory capacity and potential immunosuppressive effect. With these drugs patients present lower inflammatory intensity, but at the same time there is a risk of infectious processes, these being an important cause of morbidity and mortality. Objective To analyze the risk of infection with corticosteroid vs. immunosuppressive treatment in chronic therapy in patients with rheumtaoid arthritis. Material and methods Analytical, observational, retrospective study, with selection by intention, uncontrolled, cohort, with study of risk, incidence and cross association, carried out in the Rheumatology Service of the Hospital de Clínicas, La Paz, Bolivia, in patients who were diagnosed with RA between the 2010 - 2020 management, both in the outpatient clinic and the hospitalization room. Patients were selected with adults, both sexes, aged over 18 years, with a diagnosis of rheumatoid arthritis according to ACR 2010 classification, CDAI clinimetry in degree of activity, with indication of associated treatment (corticoid + methoroxate or even addition of chloroquine / hydroxychloroquine) according to ACR/EULAR guidelines, which perform regular control (bi monthly). Results: The incidence of infectious processes associated with the use of prednisone with doses >7.5mg/day, after 6 months of treatment is 19.39% and 18.88% at 12 months. The incidence of infectious processes associated with the use of metotrexate (dose >12.5mg/week), is 12.76% at 6 months of treatment and 13.27% at 12 months. Conclusions In adult patients of both sexes, without associated risk factors, prolonged corticotherapy (Prednisone in doses greater than 7.5mg/day for more than 6 months of treatment) produces the development of infectious processes with greater frequency and severity than therapy with metotrexate even at intermediate-high dose (MTX >12.5mg / week).

Comparative Effect between Infiltration of Platelet-rich Plasma and the Use of Corticosteroids in the Treatment of Knee Osteoarthritis: A Prospective and Randomized Clinical Trial / Efeito comparativo entre a infiltração de plasma rico em plaquetas e o uso de corticosteroides no tratamento de osteoartrite do joelho: Estudo clínico prospectivo e randomizado

Abstract Objectives This study aimed to analyze the efficacy of platelet-rich plasma obtained from the peripheral, autologous blood of the patients in pain complaints reduction and functional improvement of knee osteoarthritis compared with the standard treatment with injectable corticosteroid, such as triamcinolone. Methods The patients were followed-up clinically at the preinfiltrative visit, with quantitative evaluation using the Knee Society Score (KSS), the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) score, and the Kellgren and Lawrence scales. In addition, they were reevaluated with the same scales after 1 month and 6 months of intervention with 2.5 mL of triamcinolone acetate or 5 mL of platelet-rich plasma. The study was performed on 50 patients with knee osteoarthritis treated at the Medical Specialty Center and randomly divided into equivalent samples for each therapy. Results The present study verified the reduction of pain scores, such as the WOMAC score, and elevations of functional scales, such as the KSS, evidenced in 180 days when using platelet-rich plasma, a therapy that uses the autologous blood of the patient and has fewer side effects. Conclusion Although both platelet-rich plasma and corticosteroid therapies have been shown to be effective in the reduction pain complaints and functional recovery, there was a statistically significant difference between them at 180 days. According to the results obtained, platelet-rich plasma presented longer-lasting effects within 180 days in the treatment of knee osteoarthritis.

Resumo Objetivo Analisar a eficácia do uso de plasma rico em plaquetas, obtido do sangue periférico e autólogo dos pacientes, na redução das queixas álgicas e melhoria funcional dos pacientes portadores de osteoartrite de joelhos, em comparação com o tratamento padrão com injeção de corticosteroides de depósito, como a triancilonola. Métodos Os pacientes foram acompanhados clinicamente na consulta pré-infiltrativa, com avaliação quantitativa através das escalas Knee Society Score (KSS), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), e Kellgren and Lawrence. Além disso, os pacientes foram reavaliados com as mesmas escalas após 1 mês e 6 meses de intervenção com 2,5 mL de acetato de triancinolona ou 5 mL de plasma rico em plaquetas. O estudo foi feito em 50 pacientes portadores de osteoartrite de joelhos atendidos no Centro de Especialidades Médicas, divididos em amostras equivalentes randomizadas para cada terapia. Resultados No presente estudo, verificaram-se redução dos valores em escalas álgicas, como a WOMAC, e elevação das pontuações em escalas funcionais, como a KSS, evidenciadas em 180 dias após o uso de plasma rico em plaquetas, uma terapia feita a partir do sangue autólogo do paciente e com menos efeitos colaterais. Conclusão Embora ambas as terapias com plasma rico em plaquetas e corticosteroides tenham se mostrado eficazes na redução das queixas álgicas e na recuperação funcional, houve diferença significativa entre as terapias aos 180 dias. De acordo com os resultados obtidos, no tratamento da osteoartrite do joelho, o plasma rico em plaquetas demonstrou efeitos mais duradouros em 180 dias.

Doença de Behçet em adolescente: relato de caso / Behçet's disease in a adolescent: case report

OBJETIVO: Relatar o caso de uma paciente adolescente com diagnóstico de Doença de Behçet e correspondente revisão da literatura. DESCRIÇÃO DO CASO: Paciente de 17 anos apresentando severa mialgia generalizada e com queixa de febre intermitente há 10 dias, com edema de lábios, úlceras e aftas orais. Perda de peso por dificuldade de deglutição pelas dores das lesões orais, incapacidade de deambulação por piora progressiva da mialgia e dores articulares e febre elevada contínua. Apresentou melhora com corticoterapia e recebeu alta com prednisona oral, hidroxicloroquina, cálcio e vitamina D. COMENTÁRIOS: É importante direcionar o tratamento em tempo que evite um desfecho desfavorável por conta de complicações e manifestações mais graves da doença, por meio do diagnóstico precoce associado ao tratamento efetivo e individualizado.

OBJECTIVE: To report the case of an adolescent patient diagnosed with Behçet's Disease and a corresponding literature review. CASE DESCRIPTION: A 17-year-old patient with severe generalized myalgia and complaining of intermittent fever for 10 days, with edema of the lips, ulcers and oral thrush. Weight loss due to difficulty in swallowing due to pain from oral lesions, inability to walk due to progressive worsening of myalgia and joint pain and continuous high fever. She improved with corticosteroid therapy and was discharged with oral prednisone, hydroxychloroquine, calcium and vitamin D. COMMENTS: It is important to direct treatment in time to avoid an unfavorable outcome due to complications and more serious manifestations of the disease, through early diagnosis associated with effective and individualized treatment

Síndrome nefrótico congénito. Reporte de caso y revisión del enfoque diagnóstico / Congenital nephrotic syndrome. Case report and review of its diagnostic work up

Resumen El síndrome nefrótico se define como la unión de proteinuria masiva, hipoalbuminemia e hiperlipidemia, que pueden asociarse a edemas e hipercoagulabilidad. Se origina de una anormalidad de la barrera de filtración glomerular con una fuga masiva de proteína y los efectos secundarios consecuentes. En sus formas primarias, ocurre con una incidencia de 1-3 por cada 100.000 niños menores de 16 años. La forma congénita es una variante poco frecuente del síndrome nefrótico, la cual se presenta en el nacimiento o dentro de los tres primeros meses de vida, y suele ser resistente a la corticoterapia. Se debe evaluar primero la existencia de infecciones congénitas y luego buscar las enfermedades monogénicas más comunes, finalmente se puede recurrir a la secuenciación de nueva generación para buscar mutaciones en los demás genes candidatos. Se presenta el caso de una niña con síndrome nefrótico congénito de difícil control, enfatizando en el proceso diagnóstico y el manejo de soporte. Se resalta la importancia de la asesoría genética a la familia en todos los casos.

Abstract A nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminemia and hyperlipidemia, which may be associated with edema and hypercoagulability. It originates from an abnormality of the glomerular filtration barrier with a massive protein leak and the consequent side effects. In its primary forms, it occurs with an incidence of 1 - 3 per 100,000 children under 16 years of age. The congenital form is a rare variant of the nephrotic syndrome, which occurs at birth or within the first three months of life and is usually resistant to corticosteroid therapy. Congenital infections and most common related monogenic diseases should be tested. Finally, new generation sequencing must be used to search for mutations in other candidate genes. We present the case of a girl with congenital nephrotic syndrome difficult to control, emphasizing the diagnostic process and support management. The importance of genetic counseling to the family in all cases is highlighted.

Efeitos da corticoterapia materna nos valores hemogasométricos de cordeiros nascidos a termo e prematuros / Effects of maternal blood gas values corticotherapy of Lambs born at term and premature

The aim of the study was to evaluate blood gas parameters of full-term and premature lambs from birth to 48 hours of life. Four experimental groups were formed: NDG (normal delivery group - lambs vaginally delivered, n=15, average of 146-day gestation); NDEXG (normal delivery with dexamethasone group - lambs vaginally delivered whose mothers received 16 mg of dexamethasone at 141 days of gestation, n=8, average of 143-day gestation); PRE (premature lambs born by cesarean section at 138 days of gestation, n=10) and PREDEX (premature lambs born by cesarean section at 138 days gestation, whose mothers received 16mg of dexamethasone two days before, n=9). Immediately after birth, lambs from all groups showed respiratory acidosis (low pH and high pCO2 ), most obviously in premature animals. The concentration of HCO3 - was lower between 15 and 60 minutes of life, especially in PRE and PREDEX groups with subsequent increase in M24h. The values of base excess were lower in premature lambs, which showed abdominal breathing, severe dyspnea and lots of lung fluid. The stabilization of acid-base balance occurred in all animals during the first 24 hours of life. Dexamethasone had a positive effect on the clinical condition of the premature lambs, resulting in adequate ventilation and tissue perfusion, which guaranteed higher survival rate.(AU)

O objetivo do estudo foi avaliar as variáveis hemogasométricas de cordeiros nascidos a termo e prematuros do nascimento às 48 horas de vida. Foram constituídos quatros grupos experimentais: PN (cordeiros nascidos de parto normal, n=15, média de 146 dias de gestação); PNDEX (cordeiros nascidos de parto normal, cujas mães receberam 16 mg de dexametasona aos 141 de gestação, n=8, média de 143 dias de gestação); PRE (cordeiros prematuros nascidos de cesarianas aos 138 dias de gestação, n=10) e PREDEX (cordeiros prematuros nascidos de cesarianas aos 138 dias de gestação, cujas mães receberam 16 mg de dexametasona dois dias antes do parto, n=9). Imediatamente após o nascimento, os cordeiros de todos os grupos apresentaram quadro de acidose respiratória (pH baixo e pCO2 elevada), com maior ênfase nos animais prematuros. A concentração de HCO3 - diminuiu entre 15 e 60 minutos de vida, principalmente nos grupos PRE e PREDEX, com posterior aumento no M24h. Os valores de diferença de base foram menores nos cordeiros prematuros, os quais apresentaram respiração abdominal, intensa dispneia e grande quantidade de líquido pulmonar. A estabilização do equilíbrio ácidobase ocorreu em todos os animais ao longo das primeiras 24 horas de vida. A dexametasona teve influência positiva sobre a condição clínica dos animais prematuros, resultando em adequada ventilação e perfusão tecidual, o que garantiu maior taxa de sobrevivência.(AU)

Efeitos da corticoterapia materna nos valores hemogasométricos de cordeiros nascidos a termo e prematuros / Effects of maternal blood gas values corticotherapy of Lambs born at term and premature

O objetivo do estudo foi avaliar as variáveis hemogasométricas de cordeiros nascidos a termo e prematuros do nascimento às 48 horas de vida. Foram constituídos quatros grupos experimentais: PN (cordeiros nascidos de parto normal, n=15, média de 146 dias de gestação); PNDEX (cordeiros nascidos de parto normal, cujas mães receberam 16 mg de dexametasona aos 141 de gestação, n=8, média de 143 dias de gestação); PRE (cordeiros prematuros nascidos de cesarianas aos 138 dias de gestação, n=10) e PREDEX (cordeiros prematuros nascidos de cesarianas aos 138 dias de gestação, cujas mães receberam 16 mg de dexametasona dois dias antes do parto, n=9). Imediatamente após o nascimento, os cordeiros de todos os grupos apresentaram quadro de acidose respiratória (pH baixo e pCO2 elevada), com maior ênfase nos animais prematuros. A concentração de HCO3 - diminuiu entre 15 e 60 minutos de vida, principalmente nos grupos PRE e PREDEX, com posterior aumento no M24h. Os valores de diferença de base foram menores nos cordeiros prematuros, os quais apresentaram respiração abdominal, intensa dispneia e grande quantidade de líquido pulmonar. A estabilização do equilíbrio ácidobase ocorreu em todos os animais ao longo das primeiras 24 horas de vida. A dexametasona teve influência positiva sobre a condição clínica dos animais prematuros, resultando em adequada ventilação e perfusão tecidual, o que garantiu maior taxa de sobrevivência.(AU)

The aim of the study was to evaluate blood gas parameters of full-term and premature lambs from birth to 48 hours of life. Four experimental groups were formed: NDG (normal delivery group - lambs vaginally delivered, n=15, average of 146-day gestation); NDEXG (normal delivery with dexamethasone group - lambs vaginally delivered whose mothers received 16 mg of dexamethasone at 141 days of gestation, n=8, average of 143-day gestation); PRE (premature lambs born by cesarean section at 138 days of gestation, n=10) and PREDEX (premature lambs born by cesarean section at 138 days gestation, whose mothers received 16mg of dexamethasone two days before, n=9). Immediately after birth, lambs from all groups showed respiratory acidosis (low pH and high pCO2), most obviously in premature animals. The concentration of HCO3 - was lower between 15 and 60 minutes of life, especially in PRE and PREDEX groups with subsequent increase in M24h. The values of base excess were lower in premature lambs, which showed abdominal breathing, severe dyspnea and lots of lung fluid. The stabilization of acid-base balance occurred in all animals during the first 24 hours of life. Dexamethasone had a positive effect on the clinical condition of the premature lambs, resulting in adequate ventilation and tissue perfusion, which guaranteed higher survival rate.(AU)

Sarcoidose ocular: a nossa realidade nos últimos seis anos / Ocular sarcoidosis: our reality for the past six years

RESUMO Introdução: Na sarcoidose, as manifestações oculares são comuns e podem constituir a manifestação inicial da doença ou mesmo a única. O objetivo deste trabalho foi analisar os parâmetros demográficos, manifestações clínicas, terapêutica e principais complicações oculares em doentes com sarcoidose ocular. Métodos: Estudo descritivo e retrospectivo que incluiu doentes com o diagnóstico de sarcoidose ocular, observados nas consultas de Inflamação Ocular e de Doenças Auto-Imunes do Hospital Prof. Doutor Fernando Fonseca, no período entre 2009 e 2015. Resultados: Foram identificados 11 doentes com o diagnóstico de sarcoidose ocular, com predomínio do sexo feminino (54,5%) e caucasianos. A média da idade ao diagnóstico foi de 45±14 anos. A sarcoidose manifestou-se de forma exclusivamente ocular em 36% dos casos. O envolvimento ocular foi a manifestação inicial em 90,9% dos casos. Identificaram-se 9 casos de uveíte, 1 de esclerite anterior nodular e 1 de queratite intersticial. O tratamento com corticoterapia tópica foi realizado em 100% dos casos, sendo o tratamento único em apenas 1 doente. Nos restantes, foi necessário associar corticoterapia oral. Em 4 desses doentes, pela gravidade da doença e atingimento binocular, utilizou-se também corticoterapia pulsada endovenosa. O tratamento adjuvante imunossupressor mais frequentemente utilizado foi o metotrexato (45%). Um doente necessitou de terapia biológica com infliximabe para controle da doença. Conclusão: A manifestação ocular mais comum foi a uveíte, com predomínio da panuveíte. O tratamento mais utilizado e com maior taxa de controle da doença foi a corticoterapia sistêmica em associação com o metotrexato.

ABSTRACT Purpose: In sarcoidosis, ocular manifestations are common and can be the initial or even the only clinical manifestation. The aim of this study was to analyze the demographic parameters, clinical manifestations, treatment and the major ocular complications in patients with ocular sarcoidosis. Methods: We conducted a descriptive and retrospective study that included patients with the diagnosis of ocular sarcoidosis, followed by inflammatory ophthalmology and immune-mediated disease consults at the Prof. Doutor Fernando Fonseca Hospital, between 2009 and 2015. Results: Eleven patients with the diagnosis of ocular sarcoidosis were identified, with a predominance of females (54,5%) and Caucasians. The average age at diagnosis was 45 ± 14 years. Sarcoidosis was exclusively ocular in 36%. The first manifestation of sarcoidosis was eye disease in 90.9 % of cases. Nine cases of uveitis, one of nodular scleritis and one of interstitial keratitis were observed. Topical corticoid treatment was applied in 100% of cases, with only one achieving remission of the disease. Oral corticoid treatment was necessary in 10 cases, four of which needed a high dose methylprednisolone induction. Methotrexate was the adjunctive immunosuppressive treatment of choice in 45% of cases. There was one refractory case for conventional immunosuppressive therapy, having achieved remission with biologic agent infliximab. Conclusion: Uveitis was the commonest ocular manifestation, and there was a predominance of panuveitis. Systemic corticoid and methotrexate were the most used immunosuppressive treatments for maintaining the controlled stated of the disease.

Sordera súbita: a la luz de la evidencia / Sudden deafness: in light of the evidence

La sordera súbita es una condición de hipoacusia neurosensorial aguda usualmente unilateral, de instalación en un período menor o igual a 72 horas, que en la mayoría de los casos es idiopática; sin embargo, causas infecciosas, autoinmunes y vasculares, se han propuesto como los principales mecanismos involucrados. La sordera súbita es considerada una urgencia otorrinolaringológica, sin embargo el tratamiento de ésta continúa siendo un tema controvertido.

Sudden deafness is usually a condition of acute unilateral sensorineural hearing loss, installation less than or equal to 72 hours period, which in most cases are idiopathic; however, infectious, autoimmune and vascular causes, have been proposed as key mechanisms involved. Sudden deafness is considered an ENT urgency, however this treatment remains controversial.

Parasitosis intestinal en pacientes tratados con corticoides

Se presenta el reporte del examen coproparasitológico de 23 pacientes de con enfermedades reumatológicas y tratamiento inmunosupresor del Servicio de Reumatología del Hospital de Clínicas (Asunción, Paraguay) en el año 2013. Se detectaron 16 pacientes parasitados, siendo en orden de frecuencia: Entamoeba coli (41%), Giardia lambia (23%), Strongyloides stercoralis (14%), el resto 1 caso de Hymenolepis nana y Ascaris lumbricoides.

The report of the coproparisotologic exam of 23 patients, with rheumatic diseases and receiving immunosuppresive therapy from the Rheumatology Service of the Hospital de Clínicas (Asunción, Paraguay) in 2013, is presented. Sixteen patients with parasites were detected with this frequency order: Entamoeba coli (41%), Giardia lambia (23%), Strongyloides stercoralis (14%). The remaining was 1 case of Hymenolepis nana and Ascaris lumbricoides.

Manuseio do eritema nodoso hansênico em pacientes pediátricos / Handling erythema nodosum leprosum in pediatric patients

Os autores relatam o caso de uma paciente de 14 anos, do sexo feminino, com eritema nodoso hansênico, em tratamento com predinisona em altas doses, de difícil controle há dois anos, que iniciou o quadro concomitante à introdução de poliquimioterapia para tratamento de hanseníase multibacilar. O manuseio do eritema nodoso hansênico em pacientes pediátricos é um grande desafio na área médica. O único tratamento formalmente indicado é a prednisona, já que a talidomida está contraindicada em pacientes menores de 12 anos e mulheres em idade reprodutiva. É sabida a grande quantidade de efeitos colaterais deletérios da corticoterapia crônica em crianças; além disso, ainda há pacientes refratários ao tratamento. Portanto, fica evidente a necessidade de discussão da terapêutica atual, sendo de suma importância o incentivo à pesquisa de novas drogas para o combate a essa moléstia...

Furunculose eosinofílica canina / Canine eosinophilic furunculosis

Background: The eosinophilic furunculosis is an uncommon skin disease that affects young dogs aged between two and five years. Sex predilections are not noted. Most reported cases have been in large breeds with abundant access to the outdoors like public gardens and parks. The exact pathogenesis remains unknown but the pattern of lesions development suggests involvement reaction to insect bites and arthropods. The time between contact with the agent and the appearance of lesions is short, often less than 24 h. The skin lesions appear suddenly in the face, usually on the dorsal muzzle and/ or periocular region, pinnae and lips. More rarely on the trunk, chest and legs and it is characterized by predominantly hemorrhagic ulcers with edema. Pustules, nodules and plaques rapidly fistulate and drain serosanguinous exudates. Pruritus is variable, but may be severe. Severely affected dogs may be febrile, lethargic and anoretic. Peripheral blood eosinophilia is seen in the majority of cases. Clinical differential diagnosis include demodicosis, dermatophytosis, nasal deep bacterial folliculitis and furunculosis, pemphigus foliaceus, pemphigus erythematosus and drug reactions. None of these diseases share the fulminant rapid onset of eosinophilic furunculosis. The diagnosis is based on history, clinical signs, cytology and skin biopsies. The treatment involves oral steroids until complete remission of lesions and the antibiotic therapy is indicated in cases of associated bacterial infection. Case: The patient was a 4-year-old, male Pit Bull dog attended in a small animal clinic in Niteroi-Rio de Janeiro, which presented ulcerated, exudative lesions on the dorsal muzzle and right leg. After sedation, clinical examination was performed and an exudate was collected from the ulcerated skin lesion for cytopathological analysis .The slide containing the lesion impression was stained by a quick panoptic method. In order to collect samples, the dog was sedated with a combination of ketamine hydrochloride and acepromazine and a skin fragment was collected from the nasal bridge lesion with a 6 mm punch after local anesthesia with 2% lidocaine hydrochloride. The specimen was fixed in 10% buffered formalin and sent for histopathological analysis. The cytopathological exam revealed a marked eosinophilic inflammation. Histopathological examination revealed ulcerated skin. The epidermis was moderately acanthotic with mild espongiosis and the dermis was characterized by intense eosinophilic folliculocentric inflammations. An extensive folicular rupture, eosinophilic mural foliculitis were presented and PAS staining did not identify fungal structures. Oral prednisone (2 mg/Kg) at 24h intervals was prescribed until complete remission of the lesions. After fifteen days of glucocorticoids therapy, involution of the skin lesions was observed by physical examination and was also reported by the owner. Discussion: The eosinophilic furunculosis is an acute, severe predominantly facial disease of outdoor dogs, which occurrence is rare. The diagnosis and treatment of this disease are frequently neglected because they are not included in the differential diagnosis of diverse cutaneous infections. In view of the scarcity of reports and to alert veterinarians that the disease should be included in the differential diagnosis with other bacterial diseases, this report described a case of canine eosinophilic furunculosis.

Canine eosinophilic furunculosis / Furunculose eosinofílica canina

Background: The eosinophilic furunculosis is an uncommon skin disease that affects young dogs aged between two and five years. Sex predilections are not noted. Most reported cases have been in large breeds with abundant access to the outdoors like public gardens and parks. The exact pathogenesis remains unknown but the pattern of lesions development suggests involvement reaction to insect bites and arthropods. The time between contact with the agent and the appearance of lesions is short, often less than 24 h. The skin lesions appear suddenly in the face, usually on the dorsal muzzle and/ or periocular region, pinnae and lips. More rarely on the trunk, chest and legs and it is characterized by predominantly hemorrhagic ulcers with edema. Pustules, nodules and plaques rapidly fistulate and drain serosanguinous exudates. Pruritus is variable, but may be severe. Severely affected dogs may be febrile, lethargic and anoretic. Peripheral blood eosinophilia is seen in the majority of cases. Clinical differential diagnosis include demodicosis, dermatophytosis, nasal deep bacterial folliculitis and furunculosis, pemphigus foliaceus, pemphigus erythematosus and drug reactions. None of these diseases share the fulminant rapid onset of eosinophilic furunculosis. The diagnosis is based on history, clinical signs, cytology and skin biopsies. The treatment involves oral steroids until complet

Background: The eosinophilic furunculosis is an uncommon skin disease that affects young dogs aged between two and five years. Sex predilections are not noted. Most reported cases have been in large breeds with abundant access to the outdoors like public gardens and parks. The exact pathogenesis remains unknown but the pattern of lesions development suggests involvement reaction to insect bites and arthropods. The time between contact with the agent and the appearance of lesions is short, often less than 24 h. The skin lesions appear suddenly in the face, usually on the dorsal muzzle and/ or periocular region, pinnae and lips. More rarely on the trunk, chest and legs and it is characterized by predominantly hemorrhagic ulcers with edema. Pustules, nodules and plaques rapidly fistulate and drain serosanguinous exudates. Pruritus is variable, but may be severe. Severely affected dogs may be febrile, lethargic and anoretic. Peripheral blood eosinophilia is seen in the majority of cases. Clinical differential diagnosis include demodicosis, dermatophytosis, nasal deep bacterial folliculitis and furunculosis, pemphigus foliaceus, pemphigus erythematosus and drug reactions. None of these diseases share the fulminant rapid onset of eosinophilic furunculosis. The diagnosis is based on history, clinical signs, cytology and skin biopsies. The treatment involves oral steroids until complet

Canine eosinophilic furunculosis / Furunculose eosinofílica canina

Background: The eosinophilic furunculosis is an uncommon skin disease that affects young dogs aged between two and five years. Sex predilections are not noted. Most reported cases have been in large breeds with abundant access to the outdoors like public gardens and parks. The exact pathogenesis remains unknown but the pattern of lesions development suggests involvement reaction to insect bites and arthropods. The time between contact with the agent and the appearance of lesions is short, often less than 24 h. The skin lesions appear suddenly in the face, usually on the dorsal muzzle and/ or periocular region, pinnae and lips. More rarely on the trunk, chest and legs and it is characterized by predominantly hemorrhagic ulcers with edema. Pustules, nodules and plaques rapidly fistulate and drain serosanguinous exudates. Pruritus is variable, but may be severe. Severely affected dogs may be febrile, lethargic and anoretic. Peripheral blood eosinophilia is seen in the majority of cases. Clinical differential diagnosis include demodicosis, dermatophytosis, nasal deep bacterial folliculitis and furunculosis, pemphigus foliaceus, pemphigus erythematosus and drug reactions. None of these diseases share the fulminant rapid onset of eosinophilic furunculosis. The diagnosis is based on history, clinical signs, cytology and skin biopsies. The treatment involves oral steroids until complet

Background: The eosinophilic furunculosis is an uncommon skin disease that affects young dogs aged between two and five years. Sex predilections are not noted. Most reported cases have been in large breeds with abundant access to the outdoors like public gardens and parks. The exact pathogenesis remains unknown but the pattern of lesions development suggests involvement reaction to insect bites and arthropods. The time between contact with the agent and the appearance of lesions is short, often less than 24 h. The skin lesions appear suddenly in the face, usually on the dorsal muzzle and/ or periocular region, pinnae and lips. More rarely on the trunk, chest and legs and it is characterized by predominantly hemorrhagic ulcers with edema. Pustules, nodules and plaques rapidly fistulate and drain serosanguinous exudates. Pruritus is variable, but may be severe. Severely affected dogs may be febrile, lethargic and anoretic. Peripheral blood eosinophilia is seen in the majority of cases. Clinical differential diagnosis include demodicosis, dermatophytosis, nasal deep bacterial folliculitis and furunculosis, pemphigus foliaceus, pemphigus erythematosus and drug reactions. None of these diseases share the fulminant rapid onset of eosinophilic furunculosis. The diagnosis is based on history, clinical signs, cytology and skin biopsies. The treatment involves oral steroids until complet

Duchenne muscular dystrophy: quality of life among 95 patients evaluated using the Life Satisfaction Index for Adolescents / Distrofia muscular de Duchenne: avaliação da qualidade de vida em 95 pacientes através do Life Satisfaction Index for Adolescents

The purpose of this study was to evaluate the quality of life (QoL) of patients with Duchenne muscular dystrophy (DMD) in different stages of the disease, by means of the Life Satisfaction Index for Adolescents (LSI-A). The practicality of this scale was also verified. The LSI-A was applied four times to 95 patients with DMD who were undergoing steroid therapy, at three-month intervals. The patients were divided into four groups according to age. The results from the four applications and the inter and intra-examiner concordance were treated statistically. Comparing the different age groups, patients with DMD did not lose QoL, even with disease progression. We concluded that, in spite of the progressive course of the disease, the QoL in patients with DMD does not get worse. The use of a scale that embraces a great diversity of circumstances in patients' lives, without considering clinical aspects excessively, is a good alternative for assessing the QoL of these patients.

O objetivo deste estudo foi de quantificar a qualidade de vida (QV) em crianças com distrofia muscular de Duchenne (DMD) em diferentes idades através do uso do questionário Life Satisfaction Index for Adolescents (LSI-A). Foi também avaliada a praticidade do questionário. O LSI-A foi aplicado a 95 pacientes com distrofia muscular de Duchenne em corticoterapia, em diferentes idades, e por quatro vezes com intervalos de três meses. Os resultados concernentes às quatro avaliações e a concordância inter e intra-observador foram tratados estatisticamente. Comparando diferentes faixas etárias, mesmo ao longo da progressão da doença, não notamos perda da QV. Concluímos que por não valorizar excessivamente os aspectos clínicos e abranger uma diversidade de circunstâncias cotidianas, O LSI-A é útil na avaliação da QV das crianças com DMD, sendo também de fácil aplicação.

Factores obstétricos claves en los resultados neonatales y a los dos años de seguimiento en la prematuridad extrema / Influence of obstetrical factors in the neonatal outcome and after two-year follow-up in extreme preterm

Objetivo: Analizar la morbimortalidad en pretérminos extremos evaluando la influencia de factores obstétricos. Método: Estudio retrospectivo de 132 casos nacidos entre las semanas 23 y 27 en el Hospital La Paz, desde 2003 a 2005. Se establecieron tres grupos obstétricos: Amenaza de Parto Pretérmino, Rotura Prematura de Membranas y la asociación de ambas. Se evaluaron como variables obstétricas: tocolisis, corticoterapia, motivo de finalización de la gestación y vía de parto, y como variables en niños: mortalidad y morbilidad respiratoria, neurológica, visual y auditiva en neonatos y a los dos años. Resultados: Los casos con amenaza de parto pretérmino presentaron mayor displasia broncopulmonar y ductus arterioso persistente que los otros dos grupos obstétricos (p=0,03). Las pacientes con amenaza de parto pretérmino y tocolisis desarrollaron menos hemorragia intraventricular [36,4 por ciento (12/33)] e infarto periventricular (0 por ciento) que los casos sin tocolisis, en los que aparecieron en el 68,4 por ciento (13/19) y 31,6 por ciento(6/19), respectivamente (p=0,03 y p=0,001). Además en este subgrupo, los casos que recibieron corticoterapia desarrollaron menos infarto periventricular (0 por ciento) y parálisis cerebral a los 2 años [6,7 por ciento (2/30)], que los que no la recibieron, en los que apareció un 40 por ciento (6/15) de infarto y un 40 por ciento (4/10) de parálisis cerebral, respectivamente (p=0,0001 y p=0,02). La hemorragia intraventricular y la parálisis cerebral fueron más frecuentes en partos vaginales de casos con amenaza de parto pretérmino que en cesáreas [63,3 por ciento (19/30) y 26,1 por ciento (6/23) frente a 27,3 por ciento (6/22) y 0 por ciento; p=0,01 y 0,03]. Conclusión: La conducta obstétrica puede modificar el pronóstico neonatal y a los 2 años de seguimiento.

Objective: Analyse morbidity and mortality in extreme preterm at birth and at 2 year follow-up evaluating the influence of obstetrical factors. Methods: Retrospective study of 132 cases born between weeks 23 and 27 at La Paz Hospital from 2003 to 2005. Three obstetrical groups were established: Threat of Preterm Birth, Premature Rupture of Membranes and the combination of both. The following were evaluated as obstetrical variables: tocolysis, corticosteroid therapy and type of delivery. As variables in children: mortality and respiratory, neurological, visual and auditive morbidity in neonates and two years of age. Results: In the cases of threat of preterm birth a greater bronchopulmonary dysplasia and persistent ductus arteriosus appeared than in the other two obstetrical groups (p=0.03). Focusing on the threat of birth group, the cases with maternal tocolysis developed fewer neurological complications, intraventricular hemorrhage of 36.4 percent (12/33) and periventricular infarct of 0 percent, whereas the cases without tocolysis showed 68.4 percent (13/19) and 31.6 percent (6/19) respectively (p=0.03, p=0.001). Also in this subgroup, the cases that received corticosteroid therapy developed less periventricular infarct (0 percent) and cerebral palsy at age 2 [6.7 percent (2/30)] than the ones that did not receive it in which the percentages were 40 percent (6/15) and 40 percent (4/10) (p=0.0001 and p=0.02 respectively). Also, intraventricular hemorrhage and cerebral palsy were more frequent in vaginal delivery than in caesarean sections in this subgroup [63.3 percent (19/30) and 26.1 percent (6/23) against 27.3 percent (6/22) and 0 percent; p=0.01 and p=0.03)]. Conclusion: Obstetrical characteristics and behaviour can have a decisive impact in the neonatal outcome and after two-year follow-up.

Comparison of motor strength and function in patients with Duchenne muscular dystrophy with or without steroid therapy / Comparação da força e função motora em pacientes com distrofia muscular de Duchenne com ou sem corticoterapia

OBJECTIVE: To compare muscle strength (MS) and motor function in patients with Duchenne muscular dystrophy (DMD) receiving steroids for different times against the natural evolution of DMD described by Scott et al. METHOD: 90 patients with DMD (aged 5- 12 years), receiving steroids for one to seven years, were evaluated by Medical Research Council Scale (MRC) and Hammersmith motor ability score. The relation between MS and motor abilities measurement from our data and Scott's ones were ascertained statistically. RESULTS: The relation between patient's age and Hammersmith scores revealed decrease of 0.76 point per year for age against decrease of 2.23 points on Scott's study. The relation between MRC scale and patient's age showed decrease of 0.80 point per year of age against decrease of 3.65 points on Scott's study. CONCLUSION: In patients with DMD aged five to 12 years the progression of the disease is delayed by steroids and the motor function is less reduced than muscular strength.

OBJETIVO: Comparar força muscular e função motora de pacientes com distrofia muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença descrita por Scott et al. MÉTODO: Noventa pacientes, entre 5 e 12 anos de idade, em corticoterapia por um até sete anos, foram avaliados quanto à força muscular (FM) (escala MRC) e função motora (Hammersmith motor ability score). A relação entre idade, FM e função motora e a comparação com o estudo de Scott et al foram determinadas estatisticamente. RESULTADOS: a relação idade/escore Hammersmith diminuiu 0,76 pontos a cada ano de aumento da idade (2,23 pontos na história natural). A relação idade/MRC decresceu 0,80 pontos a cada ano de aumento da idade (3,65 pontos na história natural). CONCLUSÃO: Nos pacientes em corticoterapia, a progressão da doença é mais lenta que na evolução natural em todas as faixas etárias avaliadas, sendo a FM mais comprometida que a função motora.