ABSTRACT
Juvenile dermatomyositis (JDM) is a chronic multisystemic disease. It is believed to be of autoimmune etiology and is characterized by the presence of vasculitis affecting striated muscle and skin. Clinical description consists of general symptoms (anorexia, weight loss, asthenia, fever), typical manifestations (muscular and cutaneous) and possible systemic alterations. We report the case of a 5-year-old boy diagnosed with JDM who presented difficulty in walking, inability to completely extend the right lower extremity and calcinosis 12 months after the development of incorrectly evaluated cutaneous alterations. We aim to highlight the importance of early diagnosis and treatment of this illness. Since the introduction of corticosteroid therapy, prognosis has improved but functional outcome still depends on the presence of calcinosis and muscular contractures.
Subject(s)
Dermatomyositis/diagnosis , Calcinosis/complications , Child, Preschool , Dermatomyositis/complications , Humans , MaleABSTRACT
La dermatomiositis juvenil es una enfermedad crónica y multisistémica, de supuesta etiología autoinmunitaria, caracterizada por la presencia de vasculitis en la piel y el músculo estriado. La sintomatología se caracteriza por la presencia de síntomas generales (anorexia, pérdida ponderal, astenia, fiebre), manifestaciones típicas (musculares y cutáneas) y posible afectación sistémica. Se aporta el caso de un niño de 5 años diagnosticado de dermatomiositis juvenil que presentaba dificultad para la deambulación, incapacidad para la extensión completa de la extremidad inferior derecha y calcinosis 12 meses después del inicio de unas manifestaciones cutáneas incorrectamente valoradas. Nuestro objetivo es resaltar la importancia del diagnóstico precoz de esta entidad y la necesidad de instaurar tratamiento lo antes posible. El pronóstico ha mejorado desde la introducción de los corticoides, pero el resultado funcional sigue estando determinado por la presencia de calcinosis y contracturas musculares
Juvenile dermatomyositis (JDM) is a chronic multisystemic disease. It is believed to be of autoimmune etiology and is characterized by the presence of vasculitis affecting striated muscle and skin. Clinical description consists of general symptoms (anorexia, weight loss, asthenia, fever), typical manifestations (muscular and cutaneous) and possible systemic alterations. We report the case of a 5-year-old boy diagnosed with JDM who presented difficulty in walking, inability to completely extend the right lower extremity and calcinosis 12 months after the development of incorrectly evaluated cutaneous alterations. We aim to highlight the importance of early diagnosis and treatment of this illness. Since the introduction of corticosteroid therapy, prognosis has improved but functional outcome still depends on the presence of calcinosis and muscular contractures
Subject(s)
Male , Child, Preschool , Humans , Dermatomyositis/diagnosis , Calcinosis/complications , Dermatomyositis/complicationsABSTRACT
OBJECTIVE: To analyze whether erythropoietin treatment increases hemoglobin and decreases transfusion requirements in critically ill children. PATIENTS AND METHODS: We performed an observational, prospective study of 23 critically ill children aged between 1 month and 6 years. Recombinant human eritropoietin (rHuEPO) was administered at a dosage of 150-750 U/kg/week over 3 days. Hemogram, reticulocyte, iron metabolism, serum ferritin and transferrin were measured before treatment started and weekly thereafter. RESULTS: After erythropoietin treatment, hematocrit, hemoglobin and red blood cells progressively increased, with a maximal response in the sixth week. At the end of treatment, hemoglobin increased 1.68 g/dl, hematocrit by 5 % and erythrocytes 600,000/ml/mm3. Transfusion requirements decreased from 59 transfusions at baseline to 12 in the first week of treatment and none from the sixth week. No treatment-related adverse effects were observed. CONCLUSION: Erythropoietin can be an effective treatment for anemia in some critically ill children, decreasing the number of transfusions and increasing hemoglobin.
Subject(s)
Anemia/drug therapy , Erythropoietin/therapeutic use , Child , Child, Preschool , Critical Illness , Female , Humans , Infant , Male , Prospective StudiesABSTRACT
Objetivo: Analizar si el tratamiento con eritropoyetina aumenta la hemoglobina (Hb) y disminuye las necesidades transfusionales en niños críticamente enfermos. Pacientes y métodos: Estudio observacional prospectivo, en 23 niños enfermos en estado crítico de edades entre un mes y 6 años. Se administró una dosis de eritropoyetina humana recombinante (r-HuEPO) entre 150-750 U/kg/semana distribuida en 3 días. Antes del inicio del tratamiento y semanalmente se realizaron los siguientes controles: hemograma, reticulocitos, sideremia, ferritina y transferrina. Resultados: Tras el tratamiento con eritropoyetina se observó un aumento progresivo de la serie roja (Hb, hematocrito, hematíes), y la mayor respuesta se encontró a la sexta semana. La Hb al final del tratamiento aumentó un 1,68 g/dl; el hematocrito, un 5 por ciento, y los hematíes, 600.000/mm3. La necesidad de transfusiones disminuyó de 59 antes del inicio del tratamiento con eritropoyetina a 12 en la primera semana de tratamiento; ninguna se realizó a partir de la sexta semana. No se observaron efectos secundarios al tratamiento. Conclusión: La eritropoyetina puede ser un tratamiento eficaz de la anemia en algunos niños enfermos en estado crítico, disminuyendo el número de transfusiones y aumentando las cifras de Hb (AU)
