Treatment with intravenous gammaglobulin in pediatric patients with primary vasculitis

Nowadays intravenous immunoglobulins have been used in differ- ent vasculitis with various results for each condition, being more recommended for its use in Kawasaki disease and ANCA-associated vasculitis. However, there is still no solid evidence to support its use in the entire group of these diseases. We present a review of the existing literature related to the use of intravenous immunoglobulin for the treatment of primary vasculitis in childhood

Human inmmunoglobulin in primary immunodeficiencies

Human immunoglobulin replacement therapy has become a corner- stone in the treatment of patients with primary immunodeficiencies (PID). Currently indicated as first-line therapy for predominantly antibody deficiencies, severe combined immunodeficiencies, and some well-defined syndromes with immunodeficiencies, it is also indicated as adjunct therapy in many other PID. Although considered a high-cost medication, elegant studies had showed that patients correctly treated with human immunoglobulin replacement therapy result in lower costs derived from their health- attention. Major benefits of immunoglobulin replacement therapy include but are not restricted to: protection against infectious processes, organ damage progression-arrest, immune modulation and quality-of-life improvement. Two modalities of treatment are currently used, intravenous and subcutaneous, each has clear advantages and disadvantages when compared to the other, which are presented in this article. The correct use of human immunoglobulin for the treatment of patients with PID translates in better medical-practices improving survival and quality of life of affected patients

Manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en un hospital pediátrico de tercer nivel en la Ciudad de México

Resumen: Objetivo: Describir las manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en pacientes atendidos en un hospital de tercer nivel de la Ciudad de México, México. Métodos: Estudio retrospectivo, descriptivo en pacientes con diagnóstico de enfermedad de Kawasaki de agosto de 1995 a diciembre del 2016 en el Instituto Nacional de Pediatría, México. Se estudio la demografía de los pacientes, características clínicas, tratamiento empleado y desarrollo de complicaciones cardiacas en la etapa aguda de la enfermedad. Resultados: Se estudiaron 508 casos de enfermedad de Kawasaki. La edad media al diagnóstico fue de 37.64 ± 35.56 meses. Predominio de pacientes masculinos del 65.4%, con una relación masculino/femenino de 1.88:1. La mayoría de los casos (79.2%) tuvo una presentación completa. La gammaglobulina intravenosa fue administrada en 92.4% de los casos.Veintiocho pacientes (5.5%) desarrollaron arritmias, se presentaron cambios en el segmento ST en 29 pacientes (5.6%) y 5 pacientes desarrollaron isquemia miocárdica.En el ecocardiograma inicial, 51 pacientes (9.9%) presentaron datos de miocarditis, 72 pacientes (14%) datos de pericarditis y 77 casos tuvieron derrame pericárdico (15%). Se detectaron alteraciones en las arterias coronarias en 169 casos (32.9%). Cuatro pacientes fallecieron en la etapa aguda de la enfermedad por complicaciones cardiacas de la enfermedad de Kawasaki. Conclusiones: En México cada vez existen más casos de enfermedad de Kawasaki con un alto porcentaje de manifestaciones cardiacas al diagnóstico. Se requiere de un mayor conocimiento de la enfermedad en México, para poder establecer cuál es la evolución cardiológica de los pacientes en el país. Abstract: Objectives: To describe the cardiac manifestations in the acute phase of patients with Kawasaki disease treated in a third level Children's hospital in Mexico City, Mexico. Methods: A cross-sectional study was conducted in patients with a diagnosis of Kawasaki disease treated in this hospital from August 1995 to December 2016. Information included patient demographics, clinical features, treatment used, electrocardiographic findings, extra-coronary echocardiographic findings, and the development of coronary artery aneurysms in the acute phase of the disease. Results: The study included 508 cases of Kawasaki disease, with a mean age at diagnosis of 37.64 ± 35.56 months (range from 2 to 200 months). Almost two-thirds (65.4%) of the patients were male, with a male/female ratio of 1.88:1. Complete Kawasaki disease was diagnosed in 79.2% of cases. Almost all cases (92.4%) received intravenous immunoglobulin.Twenty-eight patients (5.5%) developed arrhythmias, ST changes developed in 29 patients (5.6%), and 5 patients presented with ischaemic changes.In the initial echocardiographic evaluation, 51 patients (9.9%) were diagnosed with myocarditis, 72 patients (14.0%) with pericarditis and 77 cases (15.0%) developed pericardial effusion. Coronary artery anomalies were detected in 169 cases (32.9%). 32 cases were diagnosed as giant coronary aneurysms. Four patients died from cardiac complications in the acute phase of the disease. Conclusions: There has been an increase in the diagnosis of Kawasaki disease in Mexico. They presented with more cardiac complications than reported in literature. An increased knowledge of Kawasaki disease is required in Mexico in order to establish the cardiac outcomes of this group of patients. Palabras clave: Enfermedad de Kawasaki, Complicaciones cardiacas, Aneurismas coronarios, México, Keywords: Kawasaki disease, Cardiac complications, Coronary artery aneurysms, Mexic

Malignancies in adult patients with common variable immunodeficiency

Background: Common variable immunodeficiency (CVID) implies an increased risk of cancer, with an estimated incidence of 11-13%, particularly during the 5th and 6th decade of life. B cell-Hodgkin lymphomas are the more frequent cancer, followed by non-Hodgkin lymphoma and epithelial tumors (gastric, breast, bladder and cervix). Objective: To describe the types of cancers in a cohort of adult patients with CVID. Material and method: An observational, cross-sectional and descriptive study was made in which we reviewed the charts of patients with CVID attending the Primary Immunodeficiencies Clinic at Specialties Hospital Dr. Bernardo Sepulveda, Centro Medico Nacional Siglo XXI, Mexico City. Results: There were included 23 patients with CVID diagnosis, 13 women (56%) and 10 men (44%), with an average age of 36.7 years. Four patients developed malignancies (2 men and 2 women), with a prevalence of 17.3%. The types of cancers in this group of patients were: B cell-Hodgkin lymphoma (1/23), neuroendocrine carcinoma of the pancreas (1/23), myeloid chronic leukemia (1/23) and thyroid papillary carcinoma (1/23). In two of the subjects the diagnosis of cancer was established previous to CVID diagnosis. The average age of diagnosis of cancer was 27 years (19-34 years). Conclusions: In our patients we found different types of malignancies compared to previously described. We consider necessary a screening protocol for an early diagnosis of cancer in these patients. The frequency of cancer in our population was the same as reported in the literature

BCG: a vaccine with multiple faces

BCG has been recommended because of its efficacy against disseminated and meningeal tuberculosis. The BCG vaccine has other mechanisms of action besides tuberculosis protection, with immunomodulatory properties that are now being discovered. Reports have shown a significant protective effect against leprosy. Randomized controlled trials suggest that BCG vaccine has beneficial heterologous (nonspecific) effects on mortality in some developing countries. BCG immunotherapy is considered the gold standard adjuvant treatment for non-muscle-invasive bladder cancer. BCG vaccine has also been tested as treatment for diabetes and multiple sclerosis. Erythema of the BCG site is recognized as a clinical clue in Kawasaki disease. BCG administration in the immunodeficient patient is associated with local BCG disease (BCGitis) or disseminated BCG disease (BCGosis) with fatal consequences. BCG administration has been associated with the development of autoimmunity. We present a brief review of the diverse facets of the vaccine, with the discovery of its new modes of action providing new perspectives on this old, multifaceted and controversial vaccine

Neurological manifestations in atypical Kawasaki disease

Background: Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. Clinical cases: We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease. Treatment consisted of immunoglobulin and corticosteroids with good evolution. Conclusions: KD is a systemic vasculitis that can involve many territories. Atypical manifestations can mislead the clinician and delay diagnosis. Pediatricians and sub-specialists should be aware of these neurological manifestations in order to provide adequate and opportune treatment

Tumor germinal mixto con componentes de disgerminoma y coriocarcinoma de ovario en mujer adolescente con ataxiatelangiectasia

Antecedentes: la ataxia-telangiectasia es una enfermedad hereditaria con patrón de herencia autosómico recesivo. Se caracteriza por deterioro neurológico, telangiectasias e inmunodeficiencia. Su causa es una mutación en el gen ATM (ataxia telangiectasia mutated) localizado en el cromosoma 11q22.3-23.1, relacionado con la síntesis de una proteína con actividad fosfoinositol-3-cinasa que participa en la regulación del ciclo celular y en la reparación del ácido desoxirribonucleico; esto explica, en parte, la susceptibilidad al desarrollo de neoplasias. Su asociación con algunos tipos de cáncer se ha establecido previamente y la mayor incidencia es con leucemias y linfomas. Se han reportado siete niñas con ataxia-telangiectasia asociada con tumores sólidos germinales, específicamente con disgerminoma. Caso clínico: mujer adolescente de 12 años con diagnóstico de ataxiatelangiectasia desde los tres años. Tuvo infecciones repetidas de las vías respiratorias que requirieron múltiples hospitalizaciones de dos a tres veces por mes; recibió tratamiento con inmunoglobulina y profilaxis antimicrobiana. A los 12 años acudió al servicio de urgencias con abdomen agudo; fue operada y se halló una masa abdominopélvica que se extirpó. El diagnóstico fue de tumor germinal mixto con componente de disgerminoma y coriocarcinoma de ovario. Recibió un primer ciclo de quimioterapia con bleomicina (10 UI/m2/día, una dosis), ciclofosfamida (1 g/m2/día por cuatro días) y cisplatino (20 mg/m2/dosis por cinco días). Se suspendió la quimioterapia porque desarrolló choque séptico que puso en riesgo su vida. Por su enfermedad de base permaneció bajo vigilancia. Actualmente, 17 meses después, la paciente vive sin actividad tumoral.  Conclusión: existe asociación entre ataxia-telangiectasia, leucemia y linfomas. Se describe el caso clínico de una paciente con ataxia-telangiectasia y tumor germinal mixto con componente de disgerminoma y coriocarcinoma de ovario. Se propone establecer un tratamiento especial para estos pacientes inmunocomprometidos, con alto riesgo de cáncer pero, a la vez, de no tolerar los esquemas habituales