[Long-term efficacy of aspirin desensitization in aspirin-exacerbated respiratory disease. Review of two clinical cases]. / Eficacia a largo plazo de la desensibilización a aspirina en enfermedad respiratoria exacerbada por aspirina. Revisión de 2 casos clínicos.

BACKGROUND: The aspirin exacerbated respiratory disease (AERD) shows a prevalence of 7% among asthmatics and increases to 14% in patients with difficult to control asthma. Treatment includes the use of inhibitors of leukotriene receptor (), intranasal steroids, polypectomy, asthma management according to the severity and avoid taking nonsteroidal anti-inflammatory drugs (NSAIDs). In some patients it is necessary desensitization protocol to it. CLINICAL CASES: Two patients diagnosed with respiratory disease exacerbated by aspirin, with poor asthma control and need for multiple polypectomies, despite optimal pharmacological management, carrying out protocol desensitization to aspirin (AAS) successful, now after 4 years of having carried out, they have adequate asthma control without need for polypectomies with a maintenance dose of aspirin 150 mg/day.

Antecedentes: la prevalencia de enfermedad respiratoria exacerbada por aspirina es de 7% en pacientes asmáticos y se incrementa, incluso, a 14% en pacientes con asma de difícil control. El tratamiento incluye la prescripción de inhibidores de los receptores de leucotrienos, esteroides intranasales, polipectomías, tratamiento del asma según su severidad y evitar los antiinflamatorios no esteroides. En algunos pacientes es necesario realizar el protocolo de desensibilización a la aspirina. Casos clínicos: se describen 2 mujeres con diagnóstico de enfermedad respiratoria exacerbada por la administración de aspirina, con escaso control de los cuadros de asma y a quienes fue necesario realizar múltiples polipectomías, a pesar del manejo farmacológico óptimo. Se llevó a cabo protocolo de desensibilización a aspirina (AAS); la respuesta fue positiva. Después de cuatro años, las pacientes presentan adecuado control del asma, con una dosis de mantenimiento de AAS de 150 mg/ día y no han requerido polipectomías.

[Use of intravenous immunoglobulin in pregnancy. Report of a patient with common variable immunodeficiency]. / Uso de inmunoglobulina intravenosa en el embarazo. Reporte de una paciente con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency is the most commonly-diagnosed primary immunodeficiency in adults; it is characterized by recurrent sinopulmonary and gastrointestinal infections, and increased incidence of malignancy and autoimmune processes. Many patients begin to have clinical manifestations during reproductive age. CASE REPORT: A 34-year-old woman with 12 weeks of gestation who was diagnosed with common variable immunodeficiency after recurrent episodes of rhinosinusitis, pharyngoamygdalitis, and pneumonia. 0.6 g/kg of IVIG was prescribed every 21 days during the second trimester; the patient only presented one episode of pharyngoamygdalitis, with adequate response to treatment with antibiotics. During the third trimester the dose was adjusted to every 14 days. The patient ended the pregnancy at term without complications, with a child without defects and with proper weight and size. CONCLUSIONS: The administration of immunoglobulin is the main treatment to control common variable immunodeficiency. While the recommended starting dose is 400-800 mg/kg intravenously every 3 to 4 weeks, there is no consensus on the dose to be used in pregnant women. The recommendation is to perform serum level controls before infusion to determine and adjust it.

Antecedentes: La inmunodeficiencia común variable es la inmunodeficiencia primaria más diagnosticada en los adultos; se caracteriza por infecciones sinopulmonares y gastrointestinales de repetición y mayor incidencia de procesos autoinmunes y malignidad. Numerosos pacientes inician con las manifestaciones clínicas durante la edad reproductiva. Caso clínico: Mujer de 34 años de edad con 12 semanas de gestación, en quien se diagnosticó inmunodeficiencia común variable después de cuadros recurrentes de rinosinusitis, faringoadmidalitis y neumonías. Durante el segundo trimestre se prescribió 0.6 g/kg de inmunoglobulina intravenosa cada 21 días; la paciente solo presentó un episodio de faringoamigdalitis, con adecuada respuesta al tratamiento con antibióticos. Durante el tercer trimestre se ajustó la dosis a cada 14 días. La paciente concluyó el embarazo a término sin complicaciones, con producto sin malformaciones y con peso y talla adecuados. Conclusiones: La administración de inmunoglobulina es el principal tratamiento para controlar la inmunodeficiencia común variable. Si bien la dosis inicial recomendada es de 400-800 mg/kg en forma intravenosa cada 3 a 4 semanas, no existe un consenso sobre la dosis que debe emplearse en la mujer que cursa con embarazo. La recomendación es realizar controles de niveles séricos antes de la infusión para determinarla y ajustarla.

[Desensitization to darunavir in a pediatric patient]. / Desensibilización a darunavir en un paciente pediátrico.

Treatment of HIV infection requires the combination of multiple antiretroviral drugs, known as highly active antiretroviral therapy (HAART); however, up to 84% of patients experience adverse drug effects that lead to discontinuation within first months of treatment. Skin manifestations are reported to 22% of patients. The severity of these is variable, such as erythema multiforme, rash, hives and severe skin reactions at less than 2%. Mild rashes, usually transient and self-limiting, while severe reactions require immediately remove the drug involved to prevent progression of the reaction. Only in those cases where the offending drug does not have another alternative and documented the reaction is mediated type I hypersensitivity mechanisms, can be performed desensitization protocol.

El tratamiento para infección por VIH requiere la combinación de múltiples fármacos antirretrovirales, conocida como Terapia Antirretroviral Altamente Activa (TARAA), sin embargo hasta el 84% de los pacientes presentan efectos adversos a los fármacos, que los llevan a suspender el tratamiento dentro de los primeros meses de tratamiento. Las manifestaciones cutáneas se reportan hasta el 22.1% de los pacientes. La severidad de éstas es variable, como eritema multiforme, exantema, urticaria y reacciones cutáneas severas en menos del 2%. Los exantemas leves, suelen ser transitorios y autolimitados, mientras que las reacciones severas requieren retirar el fármaco implicado inmediatamente para prevenir la progresión de la reacción. Solamente en aquellos casos que el fármaco responsable no cuente con otra alternativa y se documente que la reacción este mediada por mecanismos de hipersensibilidad tipo I, se puede realizar protocolo de desensibilización.

[Structural and functional heart diseases in adult patients with common variable immunodeficiency]. / Cardiopatías estructurales y funcionales en pacientes adultos con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency (CVID) is the primary immunodeficiency with the largest number of comorbidities in adulthood. It has been associated with bronchiectasis between 17%-76%, and these with the presence of cardiovascular complications such as pulmonary hypertension and heart failure. The new image methods of diagnosis, to assess the cardiovascular structural and functional conformation of adult patients with bronchiectasis, help to establish more efficient and timely diagnoses. OBJECTIVE: To define the presence of structural and functional heart disease in CVID patients by transthoracic echocardiography. MATERIAL AND METHOD: A cross-sectional study was done in a cohort of 26 adult patients diagnosed with CVID and replacement therapy with intravenous immunoglobulin (IVIG), belonging to the Immunodeficiency Clinic. All patients underwent transthoracic echocardiography and tissue ECO doopler; the results were evaluated by a echocardiographer physician. RESULTS: We evaluated 26 patients, of whom 10 patients were male, with a mean age of 35.7 ± 13.7 years. The results of thoracic echocardiography of the left cardiac cavities found the following functional changes: 17 patients presented with mitral insufficiency and only 2 had aortic insufficiency, none symptoms. Regarding the structural alterations of the right cavities: 8 adults with CVID had right cavities growth and 5 of them, hypermobile atrial septum was reported; respect to functional alterations, 24 patients had tricuspid insufficiency; in 20 it was mild and only in 3 is was moderate. Up to 12 had pulmonary valve insufficiency, and 8 had pulmonary arterial hypertension (PAH); of which, in 2 it was mild and in one it was moderate; and 4 patients had PSAP in high limit values. CONCLUSIONS: Patients with CVID, despite having a high incidence of bronchiectasis, had low incidence of PAH, but a significant number of patients had PSAP in high cutoff level, so, these patients should be monitored annually, because probably they will evolve to PAH in the future. Also, they have a high incidence of mild valvular regurgitation due to mild degenerative changes with valvular sclerosis, therefore, they also require regular monitoring.

Antecedentes: la inmunodeficiencia común variable es la inmunodeficiencia primaria con mayor cantidad de comorbilidades en la vida adulta. Se ha asociado con bronquiectasias en 17 a 76%, y éstas, con complicaciones cardiovasculares, como hipertensión arterial pulmonar e insuficiencia cardiaca. Los nuevos métodos diagnósticos de imagen permiten evaluar la conformación estructural y funcional cardiovascular de los pacientes adultos con bronquiectasias y, de esta manera, establecer diagnósticos más eficientes y oportunos. Objetivo: determinar las cardiopatías estructurales y funcionales en pacientes con inmunodeficiencia común variable mediante ecocardiografía transtorácica. Material y método: estudio transversal, descriptivo, efectuado en una cohorte de 26 pacientes adultos con diagnóstico de inmunodeficiencia común variable y tratamiento sustitutivo con inmunoglobulina intravenosa (IgIV), pertenecientes a la Clínica de Inmunodeficiencias. A todos los pacientes se les realizó ecocardiografía transtorácica con ecocardiograma doppler y tisular; los resultados fueron evaluados por un médico cardiólogo ecocardiografista. Resultados: evaluamos a 26 pacientes; de ellos, 10 fueron del género masculino, con media de edad de 35.7 ± 13.7 años. Los resultados de la ecocargiografia torácica en las cavidades cardiacas izquierdas reportaron las siguientes alteraciones funcionales: 17 de 26 pacientes tuvieron insuficiencia mitral y sólo 2 tuvieron insuficiencia aórtica; ninguno de ellos con síntomas. Respecto de las alteraciones estructurales de las cavidades derechas: 8 pacientes adultos con inmunodeficiencia común variable tuvieron crecimiento de las cavidades derechas y en 5 pacientes se encontró tabique interauricular delgado e hipermóvil; respecto de las alteraciones funcionales, 24 pacientes tuvieron insuficiencia tricuspídea, en 21 de ellos fue leve y sólo en 3 fue moderada. Además, 12 pacientes tuvieron insuficiencia de la válvula pulmonar y 6 pacientes tuvieron hipertensión arterial pulmonar; de ellos, en 2 sujetos fue leve y en 2, moderada y 4 pacientes tuvieron presión sistólica de la arteria pulmonar con valores límites altos. Conclusiones: los pacientes con inmunodeficiencia común variable, a pesar de tener alta incidencia de bronquiectasias, tienen baja incidencia de hipertensión arterial pulmonar; pero un número importante de pacientes tiene presión sistólica de la arteria pulmonar con valor límite alto, por lo que estos pacientes deben tener un seguimiento anual, debido a que probablemente evolucionarán a hipertensión arterial pulmonar. Además, tienen alta incidencia de insuficiencias valvulares leves debido a cambios degenerativos con esclerosis valvular, por lo que también requieren vigilancia periódica.

[Orofacial clinical manifestations in adult patients with variable common immunodeficiency]. / Manifestaciones clínicas orofaciales en pacientes adultos con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency is the primary immunodeficiency (CVID) frequently found in adults. Its prevalence is estimated from 1:25,000 to 75,000 alive newborns; there are variations by ethnic groups, it is estimated about 50-70% in Caucasian patients. Oral cavity lesions are rarely found in adult patients with CVID, there are reports about lesions on pediatric patients mostly caused by infections. OBJECTIVE: To describe the orofacial lesions (oral, maxillofacial and neck area) affecting adults with CVID. MATERIAL AND METHOD: A transversal, prospective study was done in patients with CVID attended at Specialties Hospital, CMN SXXI, Mexico City. Patients where examined by the oral and maxillofacial surgeon and clinical findings were reported, then the descriptive analysis of the lesions was done. RESULTS: We evaluated 26 patients, 16 female and 10 males, average age of 38.6 years. In 18/26 patients we found oral lesions on 7 different types. The most frequent was minor salivary glands hiperplasia (19/26),petechiae (12/26) and herpetic ulcers (7/26). In head and neck, we found 4 different lesions, the most common was lymphadenopathy <2cm (4/26). CONCLUSIONS: The immunologic alterations associated to CVID favors the development of lesions mainly of infectious and probably autoinmune origin that affects the oral cavity and head and neck area.

Antecedentes: la inmunodeficiencia común variable es la inmunodeficienci primaria más común en adultos. Su prevalencia se estima en 1 por cada 25,000 a 75,000 recién nacidos vivos; existen variaciones por grupos étnicos, se estima en 50 a 70% en pacientes de raza caucásica. Las lesiones de la cavidad oral raramente se describen en pacientes adultos con inmunodeficiencia común variable, en niños con esta enfermedad existen informes de lesiones principalmente de origen infeccioso. Objetivo: describir las lesiones orofaciales (cavidad oral, macizo facial y cuello) en pacientes adultos con inmunodeficiencia común variable. Material y método: estudio transversal, prospectivo, efectuado en todos los adultos con inmunodeficiencia común variable adscritos a la Clínica de inmunodeficiencias primarias, del Hospital de Especialidades, Centro Médico Nacional Siglo XXI, que fueron examinados por un cirujano maxilofacial; se realizó el reporte de hallazgos en lista de cotejo y, posteriormente, el análisis descriptivo de las lesiones. Resultados: se incluyeron 26 pacientes, 16 mujeres y 10 hombres, con edad promedio de 38.6 años. En 18 de 26 pacientes estudiados se observaron lesiones orales, con siete lesiones diferentes y predominio en el sexo femenino 2:1. Las lesiones más frecuentes fueron: hiperplasia de glándulas salivales menores (19/26), petequias (12/26) y úlceras herpetiformes (7/26). En la cara y el cuello se observaron cuatro lesiones distintas, las adenopatías < 2 cm (4/26) fueron las más comunes. Conclusiones: las alteraciones inmunológicas asociadas con la inmunodeficiencia común variable favorecen la aparición de lesiones de origen infeccioso y probablemente autoinmunitario que afectan la cavidad oral, la cara y el cuello.

[Quality of life in adults with variable common immunodeficiency and bronchiectasis]. / Calidad de vida en adultos con inmunodeficiencias común variable y bronquiectasias.

BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia. Its prevalence is 1:10000 to 1:50000 with the highest incidence in children, 6 to 10 years, and in adults around age 29. The association of CVID and bronchiectasis has a prevalence of 17-90%. The quality of life in these patients can be assessed through instruments such as the Saint George Respiratory Questionnaire (SGRQ), which is designed for patients with chronic lung diseases and validated for the Mexican population. OBJECTIVE: [corrected] To evaluate the quality of life in adult patients with CVID and bronchiectasis using the SGRQ. METHODS: We applied CRSG to all the patients of the Immunodeficiencies Clinic from Hospital Especialidades CMN SXXI diagnosed with CVID and bronchiectasis for assessing quality of life which was evaluated in the three areas of application 1)Symptoms, 2)Activities and 3)Impact and the overall quality of life. We also analyzed its relationship with the severity of bronchiectasis confirmed by HRCT. RESULTS: 19 patients were included, (5 males, 14 women) in whom it was found affected the overall quality of life in 84.2%. Women showed higher scores for all areas of the SGRQ (♀ 28.3 ± 25.18 vs. ♂ 9.89 ± 8.14). The most affected area by both sexes was the Symptoms in 92% of women and 80% men. About the severity of bronchiectasis patients with mild bronquietasias presented scores 0-22.2 (x= 11.1 ± 15.6), those with moderate bronchiectasis 3.24-56.9 (x=25.5 ± 21.76) and severe with scores of 0-74.8 (x= 24.1 ± 28.5.). CONCLUSIONS: Patients with CVID and bronchiectasis have impaired quality of life, mainly associated with respiratory symptoms, with less involvement of and impact areas. Females were most affected in intensity to show higher scores.

[Vitamin D deficiency in patients with common variable immunodeficiency, with autoimmune diseases and bronchiectasis]. / Deficiencia de vitamina D en pacientes con inmunodeficiencia común variable, con enfermedades autoinmunes y bronquiectasias.

BACKGROUND: Vitamin D regulates cellular activity, also it has effects on: innate and adaptive immunity, antimicrobial, anti-inflammatory and immunomodulatory functions. It has been suggested a possible association between vitamin D deficiency and increased risk and length of respiratory infections in patients with common variable immunodeficiency (CVID). METHODS: We conducted a descriptive study in patients with CVID. Vitamin D serum levels were measured in all patients. The results were analyzed using descriptive statistics by calculating measures of dispersion and central tendency. RESULTS: We studied 20 patients, 13 women and 7 males, with an age average of 37 ± 13.43 years. The mean concentration of vitamin D in patients was 15.43 ng/mL ± 7.23. Thirty percent of all patients had severe Vitamin D deficiency, 45% had mild to moderate deficiency and 25% had insufficiency. Autoimmune diseases were associated in 35% of CVID patients, 42.8% of them had severe vitamin D deficiency. In patients with bronchiectasis, 85% were found with vitamin D deficiency, and it was severe in 35.2%. 41% of patients with bronchiectasis were classified as severe on the Bhalla score of severity, 57% of these patients had severe deficiency and 14.2% mild-moderate deficiency. CONCLUSIONS: : We found deficiency of vitamin D in patients with CVID patients. Most of patients with CVID and bronchiectasis were detected with mild to severe vitamin D deficiency. Almost the half of patients with autoimmune diseases associated to CVID showed deficiency of vitamin D. We recommend to investigate the association of vitamin D deficiency on patients with immunodeficiencies.

[Rhinosinusitis and its impact on the quality of life in patients with common variable immunodeficiency]. / Rinosinusitis y su impacto en la calidad de vida en pacientes con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency (CVID) is characterized by a derangement in IgG, IgM and IgA antibody production in respiratory and gastrointestinal infections, caused mainly by encapsulated bacteria. Rhinosinusitis is related to both morbidity and quality of life impairment in patients with CVID. In this article we describe the prevalence of rhinosinusitis, its localization by CT scan and the perception of disability determined by the Rhinosinusitis Disability Index (RSDI) score in a group of CVID patients. OBJETIVE: To show the frequency of rhinosinusitis and its impact on quality of life in patients with CVID. METHODS: We included 14 CVID patients. Rhinosinusitis was diagnosed according to the criteria of the European Position Paper on Rhinosinusitis and Nasal Polyps (EP3OS 2007). Patients answered the RSDI questionnaire. An axial, coronal and saggital slices of a CT scan of the paranasal sinuses were performed to all patients. RESULTS: In our patientsí sample, ten of them were women. Average age was 34 years (+/-11). Eight patients (57%) had rhinosinusitis at sampling moment, 6 of them (75%) had a chronic evolution and 2 (25%) had chronic rhinosinusitis with periods of acuteness. Maxillar sinuses were the most affected, followed by the ethmoidal sinuses. Five patients were asymptomatic but had CT scan images compatible with rhinosinusitis. The most impaired RSDI domains were the physical and the functional ones. CONCLUSIONS: A Little over half of our group of patients with CVID presented rhinosinusitis, which was corroborated by MDCT. The majority of cases had a chronic evolution. The association between CVID and rhinosinusitis has a negative impact on the quality of life of patients.