ABSTRACT
Two unrelated infants were diagnosed with and initially treated for hemophagocytic lymphohistiocytosis (HLH), but progressed to cholestasis and liver failure. Early onset lysosomal acid lipase deficiency (EO-LAL-D) was suspected due to lymphocytes with cytoplasmic vacuolation and/or adrenal calcifications and confirmed by enzymatic and genetic analysis. Enzyme replacement therapy with sebelipase alfa was implemented, but both children died, despite initial improvement. Since this inborn error of metabolism progresses rapidly in infants, early diagnosis is crucial, and appropriate treatment should be started as soon as possible. The authors suggest that the diagnosis of EO-LAL-D should be considered in infants with symptoms of HLH.
Subject(s)
Sterol Esterase/therapeutic use , Wolman Disease/drug therapy , Age of Onset , Female , Humans , Infant, Newborn , Lymphohistiocytosis, Hemophagocytic/etiology , Male , Wolman Disease/complications , Wolman DiseaseABSTRACT
Anaplastic large cell lymphoma (ALCL) is a relatively rare and highly malignant form of non-Hodgkin lymphoma (NHL) which accounts for 10-15% of these childhood lymphomas. Current treatment protocols for ALCL in children consist of a short course of high intensity polychemotherapy. Here we describe an 8-year-old female with relapsed ALCL who achieved good response with anti-CD25 monoclonal antibody daclizumab. Daclizumab appears to offer a safe treatment option, but further research needs to be conducted in order to define its role in children with ALCL who do not respond to intensive chemotherapy.
Subject(s)
Antibodies, Monoclonal/therapeutic use , Immunoglobulin G/therapeutic use , Lymphoma, Large-Cell, Anaplastic/drug therapy , Antibodies, Monoclonal, Humanized , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Child , Daclizumab , Female , Humans , Immunosuppressive Agents/therapeutic use , Salvage Therapy , Treatment OutcomeABSTRACT
En 80 pacientes quirúrgicos se analizaron los cambios en los hábitos fisiológicos y cuidados de enfermería. De ellos, el 50 por ciento fue sometido a régimen de cirugía mayor ambulatoria (CMA) y el otro 50 por ciento a régimen de ingreso. Ambos grupos presentaron características fisiológicas, anestésicas y quirúrgicas similares. Su distribución por tipo de cirugía fue: cataratas (n = 10), quiste sacro (n = 10), cirugía de la mano (n = 30), hallux valgus (n = 20) y hernias inguinales (n = 10).El tiempo medio hasta la primera ingesta fue de 1 h y 37 min en CMA y de 4 h y 10 min en los pacientes ingresados; la deambulación se inició 1 h y 37 m después de la intervención en CMA frente a 15 h en los pacientes ingresados; los catéteres intravenosos se mantuvieron un promedio de 3 h y 30 min en CMA frente a 15 h y 42 min en los pacientes ingresados, y las perfusiones intravenosas se mantuvieron 12 min en CMA y 3 h y 17 min en los ingresados. Concluimos que mediante el régimen de CMA se reduce significativamente el período de adaptación al medio y se minimiza la incomodidad de los pacientes debida a catéteres, perfusiones, ayuno e inmovilización, mejorando la calidad asistencial sin retrasos innecesarios (AU)
