Ana Luisa Rodríguez-Lozano

Intravenous Immunoglobulin Treatment for Macrophage Activation Syndrome Complicating Chronic Granulomatous Disease

ObjectivesChronic granulomatous disease is a rare phagocyte disorder characterized by an increased susceptibility to infections and inflammatory complications. We describe two patients with chronic granulomatous disease (CGD) complicated by macrophage activation syndrome (MAS) (secondary hemophagocytic lymphohistiocytosis) treated with intravenous immunoglobulin (IVIG).MethodsA report of two cases of CGD complicated by MAS who were successfully treated with IVIG was made, and a comparison was made with ten other cases reported in the literature.ResultsMAS is a severe potentially fatal complication of CGD. Most cases are associated with Burkholderia cepacia and leishmaniasis infection. The treatment of these patients varies between centers, and one example is the use of the HLH-2004 protocol. IVIG could be an effective first line option for this complication in CGD patients.ConclusionsThe exaggerated inflammatory response characteristic of CGD patients could play a role in the development of this complication. IVIG appears to be a safe and effective first line treatment in these patients.

Treatment of Kimura Disease With Intravenous Immunoglobulin

Kimura disease is an uncommon chronic inflammatory condition of unknown etiology and is characterized by painless subcutaneous nodules, usually affecting the head and neck, eosinophilia, and markedly elevated immunoglobulin E levels. Several reports have described the main modalities of treatment; both corticosteroids and surgery have provided good results, but occasionally corticosteroids cannot be tapered as the disease flares up. We report here the case of an 8-year-old boy diagnosed with Kimura disease who was successfully treated with 1 dose of intravenous immunoglobulin as a steroid-sparing agent.

Agreement Among Musculoskeletal Pediatric Specialists in the Assessment of Radiographic Joint Damage in Juvenile Idiopathic Arthritis

To evaluate agreement among musculoskeletal pediatric specialists in assessing radiographic joint damage in juvenile idiopathic arthritis (JIA).

Repetición del tratamiento con inmunoglobulina intravenosa (IGIV) en el síndrome de Guillain- Barré refractario en niños

Guillain-Barre syndrome (GBS) is an acute symmetrical pa- ralyzing disease due to a demyelinating polyrradiculoneuropathy, often induced by a preceding infection 1. The main modalities for the treatment of GBS include plasmapheresis and intrave- nous immune globulin. Reports of the use of IVIG in children with GBS are limited: 1 g/kg for two days or 400 mg/kg for five days. While these studies in children are not definitive because of design limitations, their results are consistent with the larger randomized trials in adults 2,3.

Gammaglobulina. Aspectos históricos

Actualmente, el acceso y administracion de la gammaglobulina no es una situacion extraordinaria; sin embargo, el desarrollo de la molecula, como ahora la conocemos, ha sido un proceso largo y complicado. Desde el descubrimiento de las antitoxinas, por von Behring y Kitasato, y la primera evidencia de los anticuerpos, por Ehrlich, la gammaglobulina ha seguido un camino complicado, con el fin de proveer delicado con el fin de proveer a los pacientes de un producto efectivo y seguro. Un paso importante en la historia fue el descubrimiento de Bruton, cuando administro gammaglobulina a un nino de ocho anos, con lo que disminuyeron el numero y la gravedad de las infecciones. Curiosamente, en otro nino de 12 anos, con purpura trombocitopenica idiopatica e hipogammaglobulinemia secundaria a los inmunosupresores, se observo un aumento dramatico de la cuenta plaquetaria despues de la administracion de gammaglobulina, lo que dio como resultado la administracion de esta tambien en episodios autoinmunitarios desde 1980. Actualmente, en el mercado existen multiples presentaciones: intravenosas, al 5, 10 y 20%, y subcutanea, al 16.5%, lo que ha mejorado significativamente el tiempo que los ninos pasan en el hospital y, algunos de ellos, pueden beneficiarse de su administracion subcutanea en casa