Asunto(s)
Bronquiolitis Obliterante , Glucocorticoides , Metilprednisolona , Humanos , Bronquiolitis Obliterante/tratamiento farmacológico , Bronquiolitis Obliterante/etiología , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Niño , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Quimioterapia por Pulso , Administración Intravenosa , Resultado del TratamientoRESUMEN
RESUMO A encefalomielite aguda disseminada é uma doença rara, aguda, inflamatória e desmielinizante do sistema nervoso central, presumivelmente associada, em mais de três quartos dos casos, a infecções (virais, bacterianas ou inespecíficas) e imunizações ou sem qualquer antecedente indentificável. Habitualmente, apresenta um curso monofásico com início de sintomas inespecíficos na fase prodrómica, podendo evoluir com alterações neurológicas multifocais e até à perda total da acuidade visual. Descrevemos o caso de um menino de 9 anos com quadro inicial de edema de papila causado por encefalomielite aguda disseminada devido a Bartonella henselae. Apesar da gravidade da doença, o diagnóstico e o tratamento precoce proporcionaram bons desfechos.
ABSTRACT Acute disseminated encephalomyelitis is a rare, acute, inflammatory, and demyelinating disease of the central nervous system. Presumably associated in more than three quarters of cases by infections (viral, bacterial, or nonspecific) and immunizations or without any identifiable antecedent. It usually presents a monophasic course with onset of nonspecific symptoms in the prodromal phase and may evolve with multifocal neurological changes and even visual acuity loss. We describe a case of a 9-year-old boy with an initial picture of papillary edema caused by acute disseminated encephalomyelitis due to Bartonella henselae. Despite the severity of the disease, early diagnosis and treatment provided good outcomes.
Asunto(s)
Humanos , Masculino , Niño , Enfermedad por Rasguño de Gato/complicaciones , Encefalomielitis Aguda Diseminada/etiología , Metilprednisolona/administración & dosificación , Imagen por Resonancia Magnética , Enfermedad por Rasguño de Gato/diagnóstico , Enfermedad por Rasguño de Gato/tratamiento farmacológico , Agudeza Visual , Doxiciclina/administración & dosificación , Bartonella henselae , Encefalomielitis Aguda Diseminada/diagnóstico , Encefalomielitis Aguda Diseminada/tratamiento farmacológico , Microscopía con Lámpara de Hendidura , Fondo de Ojo , CefaleaRESUMEN
Durante la pandemia por COVID-19 se observaron diversas reacciones adversas a fármacos. Esto pudo haber estado relacionado con una mayor susceptibilidad inmunológica de los pacientes con SARS-CoV-2 a presentar este tipo de cuadros, así como también con la exposición a múltiples medicamentos utilizados en su tratamiento. Comunicamos el caso de un paciente con una infección respiratoria grave por COVID-19, que presentó 2 reacciones adversas graves a fármacos en un período corto de tiempo. (AU)
During the COVID-19 pandemic, various adverse drug reactions were observed. This could have been related to a greater immunological susceptibility of patients with SARS-CoV-2 to present this type of symptoms, as well as exposure to multiple drugs used in their treatment. We report the case of a patient with a severe respiratory infection due to COVID-19, who presented 2 serious adverse drug reactions associated with paracetamol in a short period of time. (AU)
Asunto(s)
Humanos , Masculino , Adulto , Síndrome de Stevens-Johnson/diagnóstico , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/diagnóstico , Exantema/diagnóstico , Pustulosis Exantematosa Generalizada Aguda/diagnóstico , COVID-19/complicaciones , Tratamiento Farmacológico de COVID-19/efectos adversos , Grupo de Atención al Paciente , gammaglobulinas/administración & dosificación , Metilprednisolona/administración & dosificación , Incidencia , Factores de Riesgo , Síndrome de Stevens-Johnson/tratamiento farmacológico , Resultado del Tratamiento , Ciclosporina/efectos adversos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/tratamiento farmacológico , Exantema/tratamiento farmacológico , Pustulosis Exantematosa Generalizada Aguda/tratamiento farmacológico , Acetaminofén/efectos adversosRESUMEN
En Uruguay, la pandemia por SARS-CoV-2 ha generado menos afectación en pacientes de la edad pediátrica, aumentando el número de casos positivos en este grupo etario de forma proporcional al aumento de la circulación del virus. La forma de presentación es generalmente asintomática o con síntomas respiratorios leves a moderados. El síndrome inflamatorio multisistémico postinfección por SARS-CoV-2 (SIM-C) ha sido descrito como una de las principales complicaciones postinfección. Se describe el primer caso de un paciente con SIM-C en la ciudad de Paysandú, Uruguay. Se trata de un escolar de 6 años que cursó una infección por SARS-CoV-2 un mes previo. Se presenta con un cuadro febril de 4 días de evolución asociado a lesiones de piel e inyección conjuntival y odinofagia, con parámetros inflamatorios elevados y afectación cardiológica. Se traslada a CTI local con buena evolución posterior. El alto índice de sospecha de SIM-C mejora el diagnóstico y en consecuencia la morbimortalidad de la enfermedad.
Summary: In Uruguay, the SARS-CoV-2 pandemic has affected the pediatric population less and the number of positive cases in this age group has increased proportionally to the rise of the virus circulation. The presentation is generally asymptomatic or with mild to moderate respiratory symptoms. Post-Infection Multisystem Inflammatory Syndrome by SARS-CoV-2 (MIS-C) has been described as one of the main post-infection complications. We describe the first case of a patient with MIS-C in the city of Paysandú, Uruguay. It is a 6-year-old schoolboy who had had a SARS-CoV-2 infection a month earlier. He showed a 4-day history of fever associated with skin lesions and conjunctival injection and odynophagia, with high inflammatory parameters and cardiac involvement. He was transferred to a local ICU and had a good subsequent evolution. The high index of suspicion of MIS-C improves the diagnosis and consequently the morbidity and mortality rates of the disease.
No Uruguai, a pandemia de SARS-CoV-2 gerou menos afetação em pacientes pediátricos, e o número de casos positivos nessa faixa etária aumentou proporcionalmente ao aumento da circulação do vírus. A forma de apresentação é geralmente assintomática ou com sintomas respiratórios leves a moderados. A Síndrome Inflamatória Multissistêmica Pós-Infecção por SARS-CoV-2 (MIS-C) tem sido descrita como uma das principais complicações pós-infecção. Descreve-se o primeiro caso de paciente com MIS-C na cidade de Paysandú, Uruguai. Ele é um estudante de 6 anos de idade que tinha tido uma infecção por SARS-CoV-2 um mês antes. Apresentou história de febre de 4 dias associada a lesões cutâneas e hiperemia conjuntival e odinofagia, com parâmetros inflamatórios elevados e envolvimento cardiológico. Foi transferido para uma UTI local com boa evolução posterior. O alto índice de suspeita de MIS-C melhora o diagnóstico e, consequentemente, a morbimortalidade da doença.
Asunto(s)
Humanos , Masculino , Niño , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , COVID-19/complicaciones , Metilprednisolona/administración & dosificación , Prednisona/uso terapéutico , Inmunoglobulinas Intravenosas/administración & dosificación , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Factores Inmunológicos/administración & dosificación , Antiinflamatorios/uso terapéuticoRESUMEN
En abril de 2020, durante el pico de la pandemia COVID-19 producida por el coronavirus emergente SARS-CoV-2, en el Reino Unido se comunicaron casos de shock hiperinflamatorio de características similares a la enfermedad de Kawasaki y el síndrome de shock tóxico en un grupo de ocho niños. El Royal College of Pediatrics and Child Health lo denominó síndrome inflamatorio multisistémico pediátrico temporalmente asociado con COVID-19 (SIM-C). Actualmente, el SIM-C es una enfermedad infrecuente, solapada con otras entidades, que requiere una alta sospecha clínica para identificarlo oportunamente. El síndrome inflamatorio multisistémico temporal asociado con SARS-CoV-2 pediátrico (PIMST) es una nueva entidad clínica con un amplio espectro de presentación postexposición al virus, inmunomediado con hiperinflamación y activación de una tormenta de citoquinas. Ocurre típicamente entre la segunda y cuarta semana de evolución. Se describen marcadores de inflamación característicamente elevados, como son la ferritina, proteína C reactiva (PCR), velocidad de eritrosedimentación (VES), lactato deshidrogenasa y dímero-D, asociados a neutropenia, linfopenia y anemia. La Organización Mundial de la Salud (OMS) define: caso a menores de 19 años con fiebre ≥3 días, marcadores inflamatorios elevados, evidencia de infección por SARS-CoV-2 y ninguna otra etiología microbiana; con afectación de al menos dos sistemas: dermatológico (rash, conjuntivitis no exudativa, inflamación mucocutánea), hemodinámico (hipotensión, shock), cardíaco (disfunción de miocardio, pericardio, valvular o coronario), hematológico (coagulopatía), digestivo (vómitos, diarrea, dolor abdominal). Considerando la gravedad de esta nueva entidad, es necesario el reconocimiento oportuno y referencia temprana para atención especiaizada y tratamiento oportuno.
Summary: In April 2020, during the peak of the COVID-19 pandemic caused by the emerging coronavirus SARS-CoV-2, 8 children reported cases of hyperinflammatory toxic shock with characteristics similar to Kawasaki disease and syndrome in the United Kingdom. The Royal College of Pediatrics and Child Health has called it pediatric Multisystem Inflammatory Syndrome (MIS) temporally associated with COVID-19. Currently, MIS-C is a rare disease, overlapping with other conditions, which requires a high clinical suspicion for its timely identification. Pediatric SARS-CoV-2-associated temporary multisystem inflammatory syndrome (TMIS-C) is a new clinical entity with a broad spectrum of presentation after exposure to the virus, immune-mediated with hyperinflammation and activation of a cytokine storm. It typically occurs between the 2nd to 4th week of evolution. Characteristically elevated markers of inflammation are described, such as ferritin, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), lactate dehydrogenase and D-dimer, associated with neutropenia, lymphopenia and anemia. The World Health Organization (WHO) defines it as: a case under 19 years of age with fever ≥ 3 days, elevated inflammatory markers, evidence of SARS-CoV-2 infection and no other microbial etiology; with involvement of at least 2 systems: dermatological (rash, non-exudative conjunctivitis, mucocutaneous inflammation), hemodynamic (hypotension, shock), cardiac (myocardial, pericardial, valvular, or coronary dysfunction), hematologic (coagulopathy), digestive (vomiting, diarrhea, abdominal pain) Considering the seriousness of this new entity, timely recognition and early referral for specialized care and timely treatment are key.
No mês de abril de 2020, durante o pico da pandemia de COVID-19 causada pelo emergente coronavírus SARS-CoV-2, 8 casos de crianças com choque hiperinflamatório com características semelhantes à doença e síndrome de Kawasaki foram relatados no Reino Unido. O Royal College of Pediatrics and Child Health nomeou-o como síndrome inflamatória multissistêmica pediátrica (MIS) temporariamente associada ao COVID-19. Atualmente, o SIM-C é uma doença rara, sobrepondo-se a outras entidades, o que requer alta suspeição clínica para sua identificação oportuna. A síndrome inflamatória multissistêmica temporária associada ao SARS-CoV-2 pediátrico (SIMT) é uma nova entidade clínica com amplo espectro de apresentação após exposição ao vírus, imunomediada com hiperinflamação e ativação de uma tempestade de citocinas. Geralmente ocorre entre a 2ª a 4ª semana de evolução. São descritos marcadores de inflamação caracteristicamente elevados, como ferritina, proteína C reativa (PCR), velocidade de hemossedimentação (VHS), lactato desidrogenase e D-dímero, associados a neutropenia, linfopenia e anemia. A Organização Mundial da Saúde (OMS) a define como: caso de menor de 19 anos com febre ≥ 3 dias, marcadores inflamatórios elevados, evidência de infecção por SARS-CoV-2 e nenhuma outra etiologia microbiana; com envolvimento de pelo menos 2 sistemas: dermatológico (erupção cutânea, conjuntivite não exsudativa, inflamação mucocutânea), hemodinâmica (hipotensão, choque), cardíaca (disfunção miocárdica, pericárdica, valvar ou coronariana), hematológica (coagulopatia), digestiva (vômitos, diarreia, dor abdominal) Considerando a gravidade dessa nova entidade, é necessário o reconhecimento oportuno e encaminhamento precoce para atendimento especializado e tratamento oportuno.
Asunto(s)
Humanos , Niño , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , COVID-19/complicaciones , Cardiomiopatías/etiología , Inmunoglobulinas/administración & dosificación , Metilprednisolona/administración & dosificación , Antiinflamatorios no Esteroideos/administración & dosificación , Aspirina/administración & dosificación , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Diagnóstico Diferencial , Factores Inmunológicos/administración & dosificación , Antiinflamatorios/administración & dosificaciónAsunto(s)
Lepra/tratamiento farmacológico , Metilprednisolona/administración & dosificación , Neuritis/tratamiento farmacológico , Quimioterapia por Pulso , Adolescente , Adulto , Antiinflamatorios/administración & dosificación , Niño , Femenino , Humanos , Lepra/complicaciones , Masculino , Persona de Mediana Edad , Neuritis/microbiología , Examen Neurológico , Nervios Periféricos/diagnóstico por imagen , Estudios Retrospectivos , Ultrasonografía , Adulto JovenRESUMEN
El diagnóstico diferencial entre la enfermedad de injerto contra huésped aguda grave (estadio IV) y la necrólisis epidérmica tóxica pude resultar difícil en el contexto de un paciente trasplantado, ya que ambas tienen presentaciones clínicas similares. Sin embargo, la distinción entre ellas es fundamental porque ocasionan una gran morbimortalidad, y su manejo y pronóstico difieren. Algunas pequeñas diferencias clínicas e histopatológicas son de gran ayuda para el diagnóstico diferencial y el dermatólogo deberá reconocerlas para tomar una conducta correcta y oportuna. Se comunica el caso de un paciente que presentó ampollas y epidermólisis después del trasplante de células hematopoyéticas y en el que se planteó la dificultad diagnóstica para diferenciar entre ambas afecciones.
The differental diagnosis between severe graft-versus-host disease (stage IV) and toxic epidermal necrolysis can be difficult in the context of a transplant patient, since both conditions have similar clinical presentations. However, the distinction between these two entities is critical because they produce great morbidity and mortality and their management and prognosis differ. Some small clinical and histopathological differences are of great help for the differential diagnosis, and the dermatologist must recognize them in order to take a correct and timely conduct. We present the case of a patient who developed blisters and epidermolysis after hematopoietic cell transplantation, and in whom the diagnostic difficulty to differentiate between the two entities was raised.
Asunto(s)
Humanos , Masculino , Adulto , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedad Injerto contra Huésped/diagnóstico , Metilprednisolona/administración & dosificación , Ciclosporina/administración & dosificación , Enfermedad Injerto contra Huésped/patología , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Suero AntilinfocíticoRESUMEN
In French Guiana, a French overseas region partly located in the Amazon, "Africanized" bees, a hybrid species of Brazilian bees known as "killer bees," have been observed since 1975. Since then, several cases requiring long hospitalization times have been described, allowing for a better understanding of the physiopathological mechanisms of this particular envenomation. Here, we report on a series of 10 cases of patients simultaneously attacked by hundreds of killer bees and immediately treated by a prehospital medical team already on site. Between 75 and 650 stingers were removed per victim. The reference treatment for anaphylaxis using intramuscular injection of epinephrine, vascular filling, and oxygen therapy was administered to all patients without delay. A clinical description was provided, and biological tests were performed immediately after the envenomation. We therefore observe the existence of a two-phase, medically well-controlled systemic toxic reaction. Thus, all our patients left the hospital after 44 hours of monitoring with no complications or sequelae, despite levels of intoxication described as potentially fatal elsewhere in the literature.
Asunto(s)
Abejas/clasificación , Mordeduras y Picaduras de Insectos/epidemiología , Acetaminofén/administración & dosificación , Acetaminofén/uso terapéutico , Adulto , Analgésicos no Narcóticos/administración & dosificación , Analgésicos no Narcóticos/uso terapéutico , Animales , Antiinfecciosos Locales/administración & dosificación , Antiinfecciosos Locales/uso terapéutico , Antiinflamatorios/administración & dosificación , Antiinflamatorios/uso terapéutico , Clorfeniramina/administración & dosificación , Clorfeniramina/uso terapéutico , Epinefrina/uso terapéutico , Guyana Francesa/epidemiología , Antagonistas de los Receptores Histamínicos H1/administración & dosificación , Antagonistas de los Receptores Histamínicos H1/uso terapéutico , Humanos , Mordeduras y Picaduras de Insectos/tratamiento farmacológico , Masculino , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Persona de Mediana Edad , Oxígeno/uso terapéutico , Prednisolona/administración & dosificación , Prednisolona/uso terapéutico , Solución Salina , Simpatomiméticos/administración & dosificación , Simpatomiméticos/uso terapéutico , Adulto JovenRESUMEN
El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)
Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Leucemia Mieloide Aguda/patología , Piodermia Gangrenosa/diagnóstico , Síndromes Paraneoplásicos/patología , Respiración Artificial , Azacitidina/uso terapéutico , Síndromes Mielodisplásicos/patología , Aciclovir/administración & dosificación , Metilprednisolona/administración & dosificación , Vancomicina/administración & dosificación , Cardiotónicos/uso terapéutico , Ceftazidima/administración & dosificación , Anfotericina B/administración & dosificación , Imipenem/administración & dosificación , Síndrome de Sweet/etiología , Piodermia Gangrenosa/etiología , Piodermia Gangrenosa/patología , Piodermia Gangrenosa/tratamiento farmacológico , Corticoesteroides/uso terapéutico , Meropenem/administración & dosificaciónRESUMEN
INTRODUCCIÓN: La encefalopatía necrotizante aguda (ENA) es una patología rara, caracterizada por compromiso de conciencia y presencia de múltiples lesiones encefálicas simétricas localizadas principalmente en tá lamo. Se asocia a alta letalidad e importantes secuelas. OBJETIVO: Describir el caso de un paciente escolar con ENA asociada a influenza-A con evolución favorable. CASO CLÍNICO: Paciente de 6 años de edad, con historia de 3 días de evolución de síntomas respiratorios altos asociados a fiebre (39 °C). Veinticuatro horas previo a la consulta destacaba compromiso de conciencia cualicuantitativo. Se realizó punción lumbar con proteinorraquia leve. En resonancia magnética (RM) se identificó focos de restricción a la difusión bilaterales de distribución simétrica, talámicos, en cuerpos mamila res, periacueductales, de tegmento pontino, hipocampales y en ambas cápsulas externas, asociado a componente hemorrágico y edema vasogénico, sugerente de ENA. Recibió tratamiento empírico con metilprednisolona y oseltamivir. Posteriormente, se recibió resultado positivo para virus influenza- AH1. Dado diagnóstico, se decidió administrar inmunoglobulina, evolucionando lento pero favora blemente. Al alta levemente bradipsíquico, con disminución de agudeza visual, lenguaje espontáneo y marcha con apoyo. A los 6 meses de seguimiento presentaba lenguaje y marcha normales, persis tiendo alteración visual a derecha. CONCLUSIÓN: Nuestro paciente presentó una ENA cuyo diagnóstico y manejo oportunos se asociaron a una favorable evolución neurológica en el largo plazo. Si bien la ENA es una patología infrecuente, la morbimortalidad asociada es altísima, por lo que resulta de gran importancia tener un alto grado de sospecha, a fin de solicitar estudio imagenológico dirigido, buscar causas infecciosas relacionadas e iniciar un manejo oportuno.
INTRODUCTION: Acute necrotizing encephalopathy of childhood (ANEC) is a rare disease characterized by alteration of consciousness and multiple symmetric brain lesions mainly involving the thalamus. It presents a high mortality rate and severe sequelae. OBJECTIVE: To describe a school-age patient with influenza A-related ANEC with favorable evolution. CLINICAL CASE: Six-year-old boy with 3 days history of upper respiratory symptoms and fever (39 °C). One day previous to admission, he presented altered state of consciousness. A lumbar puncture was performed, showing a mild increase of protein level in CSF. MRI showed bilateral foci of symmetric restricted signal in the thalamus, mammillary bodies, periaqueductal gray, ventral tegmentum, hippocampus, and in both external capsules, which was compatible with ANEC. The patient received empirical treatment with methylprednisolone and oseltamivir. Subsequently, a positive result was received for influenza. Considering diagnosis and severity of illness, it was decided to administer immunoglobulin. The patient got better slowly but favorably. At discharge, he still was mildly bradypsychic with decreased visual acuity, spontaneous speech and walking with assistance. At 6 months of follow-up, the patient presented normal speech and gait, with persistent visual impairment in the right eye. CONCLUSIONS: Our patient presented ANEC, whose timely diagnosis and management were associated with a favorable neurological evolution in the long term. Although ANEC is an infrequent pathology, it has very high morbidity and mortality rates, so it is very important to have a high degree of suspicion in order to request a targeted imaging study, search for related infectious causes, and start proper treatment.
Asunto(s)
Humanos , Masculino , Niño , Metilprednisolona/administración & dosificación , Leucoencefalitis Hemorrágica Aguda/diagnóstico , Gripe Humana/complicaciones , Oseltamivir/administración & dosificación , Antivirales/administración & dosificación , Virus de la Influenza A/aislamiento & purificación , Imagen por Resonancia Magnética , Estudios de Seguimiento , Leucoencefalitis Hemorrágica Aguda/tratamiento farmacológico , Leucoencefalitis Hemorrágica Aguda/virología , Gripe Humana/diagnóstico , Gripe Humana/tratamiento farmacológico , Glucocorticoides/administración & dosificaciónRESUMEN
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by a hyperinflammatory state and persistent macrophage activation, resulting in reactive phagocytosis of the hematopoietic elements. In children, it is usually a hereditary disorder, while in adults it is usually acquired secondary to viral infections, collagenoses, or tumors. Although accounting for 10% of hematologic malignancies, HLH is rarely associated with multiple myeloma (MM) and other plasmacytic dyscrasias. PATIENT CONCERNS: A 64-year-old Brazilian man seeked medical care with a 3-month history of intermittent fever, weight loss, night sweats, and progressive anemic symptoms. DIAGNOSIS: Total blood count showed severe bicytopenia (normocytic-normochromic anemia and thrombocytopenia), biochemical exams showed elevation of creatinine, as well as monoclonal peak in serum protein electrophoresis, high IgA dosage, and serum immunofixation with IgA kappa paraprotein. Bone marrow biopsy showed 30% of monoclonal and phenotypically anomalous plasmocytes, confirming the diagnosis of MM. Diagnosis of HLH was established by the presence of clinical and laboratory criteria: fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia, elevation of triglycerides, and several figures of erythrophagocytosis in bone marrow aspirate. INTERVENTIONS: The patient experienced pulse therapy with methylprednisolone for hemophagocytic lymphohistiocytosis, followed by initial therapy for multiple myeloma with cyclophosphamide and dexamethasone. OUTCOMES: Once the diagnosis of MM and secondary hemophagocytic syndrome was established, the patient had a rapid clinical deterioration despite the established therapeutic measures, evolving with cardiovascular failure, acute liver failure, acute disseminated intravascular coagulation, worsening renal dysfunction requiring dialysis support, respiratory dysfunction, and lowering of consciousness, characterizing rapid multiple organ dysfunction, ultimately leading to the death of the patient. INNOVATION: Here, we aimed to describe the sixth reported case of HLH associated with MM, according to cases cataloged in the PubMed database, and the first case evaluated by 18-fluordeoxyglucose positron emission tomography (18-FDG-PETCT). CONCLUSION: Our case report seeks to provide support for a better clinical and laboratory characterization of this rare paraneoplastic entity associated with MM, and aims to call the attention of hematologists and intensivists to this condition that falls within the scope of the differential diagnosis of rapid onset multiple organ failure in patients with plasmacytic neoplasms.
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Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/etiología , Mieloma Múltiple/complicaciones , Mieloma Múltiple/tratamiento farmacológico , Anemia/sangre , Anemia/etiología , Antineoplásicos Alquilantes/administración & dosificación , Antineoplásicos Alquilantes/uso terapéutico , Médula Ósea/patología , Brasil/epidemiología , Ciclofosfamida/administración & dosificación , Ciclofosfamida/uso terapéutico , Dexametasona/administración & dosificación , Dexametasona/uso terapéutico , Quimioterapia Combinada , Resultado Fatal , Fiebre/diagnóstico , Fiebre/etiología , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Humanos , Linfohistiocitosis Hemofagocítica/sangre , Linfohistiocitosis Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Persona de Mediana Edad , Mieloma Múltiple/metabolismo , Insuficiencia Multiorgánica/complicaciones , Paraproteinemias/sangre , Células Plasmáticas/patología , Esplenomegalia/diagnóstico , Esplenomegalia/etiología , Trombocitopenia/sangre , Trombocitopenia/etiología , Pérdida de PesoRESUMEN
The emergence of novel Coronavirus 2019 and the subsequent pandemic are presenting a challenge to neurologists managing patients with multiple sclerosis (MS). The clinical management has dramatically altered and it was necessary to change and/or adapt it to the new situation. Regarding relapses management, the use of intravenous corticosteroids and hospitalization during MS relapses increase the risk of viral exposure. OBJECTIVE: To review the efficacy and safety of high dose oral corticosteroids in acute relapses treatment compared to intravenous corticosteroids. METHODS: Descriptive review of the utility of high dose oral corticosteroids for MS relapses treatment was performed. We searched the literature available on PubMed and Scientific Electronic Library Online (Scielo). We focused on different trials comparing the use of high dose intravenous vs oral corticosteroids. RESULTS: Five studies were selected. One hundred and eighty two patients receiving treatment with high dose oral corticosteroids were included. The most frequent schedule was oral methylprednisolone 1000 mg (over three days). There were no significant differences between both routes of corticosteroids administration. CONCLUSION: Neurologists should be aware of the current evidence on the similar efficacy of both oral and intravenous corticosteroids for MS relapses. Using oral steroids during the pandemic would be a safe option for patients.
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COVID-19 , Glucocorticoides/uso terapéutico , Metilprednisolona/uso terapéutico , Esclerosis Múltiple/tratamiento farmacológico , Corticoesteroides/administración & dosificación , Corticoesteroides/uso terapéutico , COVID-19/epidemiología , Glucocorticoides/administración & dosificación , Humanos , Inyecciones Intravenosas , Metilprednisolona/administración & dosificaciónRESUMEN
INTRODUCTION: Acute necrotizing encephalopathy of childhood (ANEC) is a rare disease characterized by alteration of consciousness and multiple symmetric brain lesions mainly involving the thalamus. It presents a high mortality rate and severe sequelae. OBJECTIVE: To describe a school-age patient with influenza A-related ANEC with favorable evolution. CLINICAL CASE: Six-year-old boy with 3 days history of upper respiratory symptoms and fever (39 °C). One day previous to admission, he presented altered state of consciousness. A lumbar puncture was performed, showing a mild increase of protein level in CSF. MRI showed bilateral foci of symmetric restricted signal in the thalamus, mammillary bodies, periaqueductal gray, ventral tegmentum, hippocampus, and in both external capsules, which was compatible with ANEC. The patient received empirical treatment with methylprednisolone and oseltamivir. Subsequently, a positive result was received for influenza. Considering diagnosis and severity of illness, it was decided to administer immunoglobulin. The patient got better slowly but favorably. At discharge, he still was mildly bradypsychic with decreased visual acuity, spontaneous speech and walking with assistance. At 6 months of follow-up, the patient presented normal speech and gait, with persistent visual impairment in the right eye. CONCLUSIONS: Our patient presented ANEC, whose timely diagnosis and management were associated with a favorable neurological evolution in the long term. Although ANEC is an infrequent pathology, it has very high morbidity and mortality rates, so it is very important to have a high degree of suspicion in order to request a targeted imaging study, search for related infectious causes, and start proper treatment.
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Gripe Humana/complicaciones , Leucoencefalitis Hemorrágica Aguda/diagnóstico , Metilprednisolona/administración & dosificación , Oseltamivir/administración & dosificación , Antivirales/administración & dosificación , Niño , Estudios de Seguimiento , Glucocorticoides/administración & dosificación , Humanos , Virus de la Influenza A/aislamiento & purificación , Gripe Humana/diagnóstico , Gripe Humana/tratamiento farmacológico , Leucoencefalitis Hemorrágica Aguda/tratamiento farmacológico , Leucoencefalitis Hemorrágica Aguda/virología , Imagen por Resonancia Magnética , MasculinoRESUMEN
Abstract Introduction: Glucocorticoids are considered the first-line therapy for sudden sensorineural hearing loss. But there is currently no consensus on administering them as a single dose versus multiple divided daily doses. Objective: We aim to evaluate the treatment outcome of sudden sensorineural hearing loss between a single-dose and multiple divided daily doses of steroid treatment. Methods: A total of 94 patients who were diagnosed and treated for sudden sensorineural hearing loss and followed up for more than three months were reviewed retrospectively. Patients were divided into single-dose and multiple divided-dose groups, based on their medication regimens. Hearing thresholds were repeatedly measured: on the initial visit and 1 week, 1 month, and 3 months after the initial treatment. Treatment outcomes were analyzed by comparing hearing recovery rates and post-treatment audiometric changes. Results: The hearing threshold was significantly reduced at three months post-treatment in both groups. The hearing recovery rate of the single-dose group was significantly higher than that of the multiple divided-dose groups. Audiometric changes showed no statistical difference either in pure tone threshold or speech discrimination. Conclusion: When oral steroids are indicated for sudden sensorineural hearing loss, both a single dose and multiple divided doses can be effective for treatment and have comparable results. However, the single-dose regimen seems to be more efficacious than the divided-dose regimen.
Resumo Introdução: Os glicocorticoides são considerados terapia de primeira linha para perda auditiva neurossensorial súbita. Contudo, atualmente não há consenso em como para administrá-los, se em dose única ou múltiplas doses diárias. Objetivo: Nosso objetivo é avaliar o resultado do tratamento da perda auditiva neurossensorial súbita com uma dose única ou várias doses diárias de tratamento com esteróides. Método: Um total de 94 pacientes que foram diagnosticados e tratados para perda auditiva neurossensorial súbita e acompanhados por mais de três meses pós-tratamento foram avalia-dos retrospectivamente. Os pacientes foram divididos em grupos de dose única diária e dose diária dividida em múltiplas tomadas, baseado em seu regime medicamentoso. Os limiares auditivos foram medidos repetidamente: na visita inicial e em 1 semana, 1 mês e 3 meses após o tratamento inicial. Os resultados do tratamento foram analisados comparando-se as taxas de recuperação da audição e as alterações audiométricas pós-tratamento. Resultados: O limiar auditivo foi significativamente reduzido aos três meses pós-tratamento em ambos os grupos. A taxa de recuperação auditiva no grupo de dose única foi significativamente maior do que no grupo de dose diária dividida em múltiplas tomadas. As alterações audiométricas não mostraram diferença estatística, tanto no limiar de tom puro quanto na discriminação da fala. Conclusão: Quando esteroides orais são indicados para perda auditiva neurossensorial súbita, tanto uma dose única quanto múltiplas doses podem ser eficazes para o tratamento e têm resultados comparáveis. No entanto, o regime de dose única diária parece ser mais eficaz do que o regime de dose diária dividida em múltiplas tomadas.
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Humanos , Masculino , Femenino , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Pérdida Auditiva Súbita/tratamiento farmacológico , Glucocorticoides/administración & dosificación , Pérdida Auditiva Sensorineural/tratamiento farmacológico , Audiometría de Tonos Puros , Esteroides/administración & dosificación , Metilprednisolona/administración & dosificación , Prednisolona/administración & dosificación , Administración Oral , Estudios Retrospectivos , Resultado del TratamientoAsunto(s)
Antirreumáticos/administración & dosificación , Dermatomiositis/diagnóstico , Enfermedades Pulmonares Intersticiales , Metilprednisolona/administración & dosificación , Parapsoriasis , Enfermedad de Still del Adulto , Adulto , Biopsia/métodos , Lavado Broncoalveolar/métodos , Diagnóstico Diferencial , Femenino , Humanos , Pruebas Inmunológicas/métodos , Inmunosupresores/administración & dosificación , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/etiología , Parapsoriasis/diagnóstico , Parapsoriasis/etiología , Pronóstico , Pruebas de Función Respiratoria/métodos , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/fisiopatología , Tomografía Computarizada por Rayos X/métodosRESUMEN
AIM: The aim of this study was to identify factors predictive of serious infections over time in patients with systemic lupus erythematosus (SLE). METHODS: A multi-ethnic, multi-national Latin American SLE cohort was studied. Serious infection was defined as one that required hospitalization, occurred during a hospitalization or led to death. Potential predictors included were sociodemographic factors, clinical manifestations (per organ involved, lymphopenia and leukopenia, independently) and previous infections at baseline. Disease activity (SLEDAI), damage (SLICC/ACR Damage Index), non-serious infections, glucocorticoids, antimalarials (users and non-users), and immunosuppressive drugs use; the last six variables were examined as time-dependent covariates. Cox regression models were used to evaluate the predictors of serious infections using a backward elimination procedure. Univariable and multivariable analyses were performed. RESULTS: Of the 1243 patients included, 1116 (89.8%) were female. The median (interquartile range) age at diagnosis and follow-up time were 27 (20-37) years and 47.8 (17.9-68.6) months, respectively. The incidence rate of serious infections was 3.8 cases per 100 person-years. Antimalarial use (hazard ratio: 0.69; 95% confidence interval (CI): 0.48-0.99; p = 0.0440) was protective, while doses of prednisone >15 and ≤60 mg/day (hazard ratio: 4.18; 95 %CI: 1.69-10.31; p = 0.0019) and >60 mg/day (hazard ratio: 4.71; 95% CI: 1.35-16.49; p = 0.0153), use of methylprednisolone pulses (hazard ratio: 1.53; 95% CI: 1.10-2.13; p = 0.0124), increase in disease activity (hazard ratio: 1.03; 95% CI: 1.01-1.04; p = 0.0016) and damage accrual (hazard ratio: 1.22; 95% CI: 1.11-1.34; p < 0.0001) were predictive factors of serious infections. CONCLUSIONS: Over time, prednisone doses higher than 15 mg/day, use of methylprednisolone pulses, increase in disease activity and damage accrual were predictive of infections, whereas antimalarial use was protective against them in SLE patients.
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Hospitalización/estadística & datos numéricos , Infecciones/epidemiología , Lupus Eritematoso Sistémico/tratamiento farmacológico , Adulto , Antimaláricos/administración & dosificación , Estudios de Cohortes , Relación Dosis-Respuesta a Droga , Femenino , Estudios de Seguimiento , Glucocorticoides/administración & dosificación , Humanos , Inmunosupresores/administración & dosificación , Infecciones/etiología , América Latina , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Metilprednisolona/administración & dosificación , Prednisona/administración & dosificación , Factores Protectores , Factores de Riesgo , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.
RESUMO A glomerulonefrite (GN) pauci-imune é mais comum em idosos em comparação com crianças, e a etiologia ainda não é completamente compreendida. A positividade do anticorpo citoplasmático antineutrófilo (ANCA) ocorre em 80% dos pacientes. Relatamos o caso de uma menina de 7 anos de idade que apresentou mal-estar e creatinina discretamente elevada, diagnosticada como glomerulonefrite rapidamente progressiva pauci-imune associada a ANCA com crescentes em 20 dos 25 glomérulos (80%). Destes 20 crescentes, 12 eram celulares, 4 fibrocelulares e 4 globalmente escleróticos. Ela não apresentava púrpura, artrite ou sintomas sistêmicos e respondeu bem ao tratamento imunossupressor inicial, apesar da histopatologia relativamente grave. A paciente recebeu três pulsos de metilprednisolona intravenosa (30 mg/kg em dias alternados) inicialmente e continuou com ciclofosfamida (2 mg/kg por dia) por via oral durante 3 meses com prednisona (1 mg/kg por dia). Em um mês, a remissão foi alcançada com creatinina sérica normal e a prednisona foi gradualmente reduzida. O caso desta criança com uma doença pediátrica relativamente rara enfatiza a importância do tratamento imunossupressor precoce e agressivo em pacientes com GN rapidamente progressiva renal associada à ANCA, mesmo com uma apresentação clínica leve. Como em nossa paciente, os achados clínicos e laboratoriais podem nem sempre refletir exatamente a gravidade da histopatologia renal e, assim, a biópsia renal é obrigatória nessas crianças para orientar a conduta clínica e auxiliar no prognóstico.
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Humanos , Niño , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Glomerulonefritis/diagnóstico , Glomerulonefritis/sangre , Riñón/patología , Biopsia , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Prednisona/administración & dosificación , Prednisona/uso terapéutico , Estudios de Seguimiento , Resultado del Tratamiento , Creatinina/sangre , Ciclofosfamida/administración & dosificación , Ciclofosfamida/uso terapéutico , Glomerulonefritis/tratamiento farmacológico , Inmunosupresores/administración & dosificación , Inmunosupresores/uso terapéutico , Antiinflamatorios/administración & dosificación , Antiinflamatorios/uso terapéuticoRESUMEN
Glucocorticoids represent some of the most prescribed drugs that are widely used in the treatment of neuromuscular diseases, but their usage leads to side effects such as muscle atrophy. However, different synthetic glucocorticoids can lead to different muscle effects, depending upon its chemical formulation. Here, we intended to demonstrate the muscle histologic and molecular effects of administering different glucocorticoids in equivalency and different dosages. Methods: Seventy male Wistar rats distributed into seven groups received different glucocorticoids in equivalency for ten days or saline solution. The study groups were: Control group (CT) saline solution; dexamethasone (DX) 1.25 or 2.5 mg/kg/day; methylprednisolone (MP) 6.7 or 13.3mg/kg/day; and deflazacort (DC) 10 or 20 mg/kg/day. At the end of the study, the animals were euthanized, and the tibialis anterior and gastrocnemius muscles were collected for metachromatic ATPase (Cross-sectional area (CSA) measurement), Western blotting (protein expression of IGF-1 and Ras/Raf/MEK/ERK pathways) and RT-PCR (MYOSTATIN, MuRF-1, Atrogin-1, REDD-1, REDD-2, MYOD, MYOG and IRS1/2 genes expression) experiments. Results: Muscle atrophy occurred preferentially in type 2B fibers in all glucocorticoid treated groups. DC on 10 mg/kg/day was less harmful to type 2B fibers CSA than other doses and types of synthetic glucocorticoids. In type 1 fibers CSA, lower doses of DC and DX were more harmful than high doses. DX had a greater effect on the IGF-1 pathway than other glucocorticoids. MP more significantly affected P-ERK1/2 expression, muscle fiber switching (fast-to-slow), and expression of REDD1 and MyoD genes than other glucocorticoids. Compared to DX and MP, DC had less of an effect on the expression of atrogenes (MURF-1 and Atrogin-1) despite increased MYOSTATIN and decreased IRS-2 genes expression. Conclusions: Different glucocorticoids appears to cause muscle atrophy affecting secondarily different signaling mechanisms. MP is more likely to affect body/muscles mass, MEK/ERK pathway and fiber type transition, DX the IGF-1 pathway and IRS1/2 expression. DC had the smallest effect on muscle atrophic response possibly due a delayed timing on atrogenes response.
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Dexametasona/farmacología , Metilprednisolona/farmacología , Músculo Esquelético/efectos de los fármacos , Pregnenodionas/farmacología , Animales , Peso Corporal/efectos de los fármacos , Dexametasona/administración & dosificación , Regulación de la Expresión Génica/efectos de los fármacos , Glucocorticoides/administración & dosificación , Glucocorticoides/farmacología , Proteínas Sustrato del Receptor de Insulina/genética , Proteínas Sustrato del Receptor de Insulina/metabolismo , Factor I del Crecimiento Similar a la Insulina/genética , Factor I del Crecimiento Similar a la Insulina/metabolismo , Sistema de Señalización de MAP Quinasas/efectos de los fármacos , Masculino , Metilprednisolona/administración & dosificación , Desarrollo de Músculos/efectos de los fármacos , Desarrollo de Músculos/genética , Fibras Musculares Esqueléticas/efectos de los fármacos , Tamaño de los Órganos/efectos de los fármacos , Pregnenodionas/administración & dosificación , ARN Mensajero/genética , ARN Mensajero/metabolismo , Ratas WistarRESUMEN
Resumo Esclerite Posterior (EP) é uma inflamação do segmento escleral posterior, de etiologia inflamatória ou infecciosa, pouco diagnosticada, com evolução rápida, progressiva e irreversível com severo comprometimento visual, principalmente se o diagnóstico e o tratamento da (EP) não forem realizados a tempo hábil. A dor ocular, dor à movimentação ocular, cefaléia e embaçamento visual são os principais sinais e sintomas. Pode ser de causa Idiopática ou associada a doença sistêmica em até 45% dos caso. A Artrite Reumatoide é descrita como a associação mais comum. Causas infecciosas podem estar presente tais como: Herpes Simples, Herpes Zoster Oftálmico, Sífilis e Tuberculose. A bilateralidade pode ocorrer em até 35% dos casos sendo mais prevalente em mulheres a partir da quinta década de vida.
Abstract Posterior Scleritis (PS) is an inflammation of the posterior scleral segment, of low inflammatory or infectious etiology, with a rapid, progressive and irreversible evolution with severe visual impairment, especially if the diagnosis and treatment of (PS) are not performed in time skillful. Ocular pain, eye movement pain, headache and visual haze are the main signs and symptoms. It can be of idiopathic cause or associated with systemic disease in up to 45% of the cases. Rheumatoid arthritis is described as the most common association. Infectious causes may be present such as: Simple Herpes, Ophthalmic Herpes Zoster, Syphilis and Tuberculosis. Bilaterality can occur in up to 35% of cases being more prevalent in women from the fifth decade of life.