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INTRODUCTION: Patients with inborn errors of immunity (IEI) have a compromised or inappropriate immune response. Although they might be considered a high-risk group for severe SARS-CoV-2 infection, the reported impact of COVID-19 in these patients has been reassuring, while the differential susceptibility of distinct types of IEI remains unclear. OBJECTIVE: We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19. METHODS: In a retrospective study from March 2020 to February 2021, four centers in Mexico collected clinical, laboratory, and genetic data from pediatric and adult patients with known diagnoses of IEI who presented with COVID-19, based on compatible symptoms and positive SARS-CoV-2 testing or known household exposure. RESULTS: We report 31 patients with known IEI from Mexico who presented with SARS-CoV-2 infection. Seventy-four percent were male, 52% were pediatric, and 81% survived. Their ages ranged from 5 months to 56 years, with a median of 17 years. Sixty-five percent had predominant antibody deficiencies, 48% were hospitalized, and 26% required ICU. Pediatric patients had a higher hospital admission rate than adults. Inpatient mortality was 40%, and ICU mortality rate was 63%. Forty-eight percent developed pneumonia, while 36% had evidence of hyperinflammation (4 adults and 7 children). Predominant laboratory features were lymphopenia and thrombocytopenia, seen in 70 and 44% of patients, respectively. The serum D-dimer median value was 2.6 (0.5-20.6) µg/mL, and the median highest ferritin value was 1015 (32-10,303) ng/mL. Intravenous immunoglobulin was used in 80% of patients. Other treatments included macrolides (39%) and corticosteroids (29%). Six patients died from secondary infection or uncontrolled systemic inflammation. DISCUSSION: Although impaired immunity due to IEI may be a predisposing factor for severe COVID-19, most of our patients with IEI who acquired the SARS-CoV-2 infection developed a well-tolerated infection and survived, as have more than 80% of worldwide reported patients to date. An impaired immune or inflammatory response may be a predisposing factor for some and a protective factor for others. A systematic review of the literature could help identify those patients at risk of severe disease and complications. Healthcare-associated infections should be aggressively prevented.
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COVID-19/diagnóstico , Doenças da Imunodeficiência Primária/diagnóstico , SARS-CoV-2/fisiologia , Adolescente , Adulto , COVID-19/epidemiologia , COVID-19/mortalidade , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Doenças da Imunodeficiência Primária/epidemiologia , Doenças da Imunodeficiência Primária/mortalidade , Estudos Retrospectivos , Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Adulto JovemRESUMO
Multiple sclerosis is a demyelinating inflammatory disease that affects the central nervous system. Its etiology is the result of a complex interaction between genetic and environmental factors that trigger a deregulated immune response, with the resulting inflammation and neuronal/axonal degeneration. Neuroinflammation is triggered when peripheral leukocytes migrate to the central nervous system and release cytokines such as interleukins 1 and 6 (IL-1 and 6) and tumor necrosis factor (TNF), which act on dwelling cells. The innate immune system plays an important role in the onset and progression of the disease by identifying molecular patterns associated with pathogens and damage, which modulate effector and regulatory functions of the cells where they are expressed, in order to direct the specific immune response. Th17 cells favor the disruption of the blood-brain barrier, which enables the migration of leukocytes to the central nervous system and the triggering of the inflammatory cascade; the Th1 profile (IL-1, IL-6) collaborates to perpetuate it. B-cell function is to produce antibodies and cytokines (IL-6, IL-12 and TFN). Knowledge on multiple sclerosis pathophysiology will enable the development of new therapeutic options that impact on natural history of the disease and its prognosis.
La esclerosis múltiple es una enfermedad inflamatoria desmielinizante que afecta el sistema nervioso central. Su etiología es el resultado de una compleja interacción entre factores genéticos y ambientales que desencadenan una respuesta inmune desregulada, con la consiguiente inflamación y degeneración neuronal/axonal. La neuroinflamación se desencadena cuando los leucocitos periféricos migran al sistema nervioso central y liberan citocinas como interleucinas 1 y 6 (IL-1, IL-6) y factor de necrosis tumoral (TNF), que actúan sobre células residentes del mismo. El sistema inmune innato desempeña un papel importante en el inicio y progresión de la enfermedad, mediante la identificación de patrones moleculares asociados con patógenos y daño, que modulan las funciones efectoras y reguladoras de las células donde se expresan, para dirigir la respuesta inmune específica. Las células Th17 favorecen la disrupción de la barrera hematoencefálica, que permite la migración de leucocitos al sistema nervioso central y desencadena la cascada de la inflamación; el perfil Th1 (IL-1, IL-6) colabora para perpetuarla. La función de las células B es la producción de anticuerpos y citocinas (IL-6, IL-12 y TFN). Conocer la fisiopatología de la esclerosis múltiple permitirá desarrollar nuevas opciones terapéuticas que impacten en la historia natural de la enfermedad y su pronóstico.
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Citocinas/imunologia , Inflamação/fisiopatologia , Esclerose Múltipla/fisiopatologia , Animais , Barreira Hematoencefálica/metabolismo , Movimento Celular/fisiologia , Progressão da Doença , Humanos , Imunidade Inata/imunologia , Inflamação/imunologia , Leucócitos/metabolismo , Esclerose Múltipla/imunologia , Prognóstico , Células Th17/imunologiaRESUMO
Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by B cell dysfunction and decreased serum immunoglobulin. CVID patients are classified by the absence or presence of memory B cells. In addition, T cell defects have been demonstrated in only a proportion of CVID patients. The aim of this study was to evaluate the function of CD4(+) T cells from CVID patients and its association with memory B cells. Patients were classified according to their Freiburg groups: group Ia and Ib, with decreased switched memory B cells (<0.4 of PBL), and group II, with normal B cell subsets. Their T cell function was evaluated after stimulation. We observed normal and even increased CD4(+) T cell proliferation in group Ia (p=0.0277). The proliferation positively correlated with the clinical severity score (r=0.4796). We observed lower levels of IL-17A and IL-10 in group Ia (p=0.0177, 0.0109) and Ib (p=0.0009, 0.0084) patients. Group Ib patients also had low levels of IL-13 and IL-9 (p=0.0169, 0.010). Group II patients had similar cytokine production to that of the controls. BAFFR expression was reduced in groups Ia (p=0.0001) and Ib (p=0.0002) and showed an inverse correlation with the severity score (p=0.0262; r=0.5371). ICOS expression was reduced in group Ia (p=0.0364), and PD-1 was increased in group Ib (p=0.0432) patients. This study shows a selective impairment in cytokine production in group Ia patients, which was more extensive than in group Ib patients. The impairment was associated with BAFFR expression in B cells, with ICOS and PD-1 in T cells and, remarkably, with the absence of memory B cells and with the disease severity. Our results suggest that the evaluation of cytokine expression by T cells in combination with the study of B cell memory could be important for understand the pathogenesis of CVID patients.
Assuntos
Receptor do Fator Ativador de Células B/imunologia , Linfócitos B/imunologia , Imunodeficiência de Variável Comum/imunologia , Memória Imunológica , Proteína Coestimuladora de Linfócitos T Induzíveis/imunologia , Receptor de Morte Celular Programada 1/imunologia , Linfócitos T/imunologia , Adulto , Idoso , Receptor do Fator Ativador de Células B/genética , Linfócitos B/patologia , Antígeno B7-H1/genética , Antígeno B7-H1/imunologia , Antígeno CTLA-4/genética , Antígeno CTLA-4/imunologia , Proliferação de Células , Imunodeficiência de Variável Comum/genética , Imunodeficiência de Variável Comum/patologia , Estudos Transversais , Feminino , Regulação da Expressão Gênica , Humanos , Proteína Coestimuladora de Linfócitos T Induzíveis/genética , Interleucina-10/genética , Interleucina-10/imunologia , Interleucina-13/genética , Interleucina-13/imunologia , Interleucina-17/genética , Interleucina-17/imunologia , Interleucina-9/genética , Interleucina-9/imunologia , Ativação Linfocitária , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Cultura Primária de Células , Receptor de Morte Celular Programada 1/genética , Índice de Gravidade de Doença , Transdução de Sinais , Linfócitos T/patologiaRESUMO
Background: The SARS-CoV-2 disease, called COVID-19, emerged in China has acquired pandemic dimensions. According to the WHO situational report of March 15, 2021, the global fatality rate is 2.2%; in Mexico, around 194 944 deaths have been confirmed by COVID-19. Studies in China identified that patients with severe COVID-19, when compared with those who had non-severe COVID-19, presented more severe neurological manifestations. Objective: To determine the frequency of neurological symptoms and manifestations in patients with severe COVID-19 in a tertiary care center. Material and methods: A cross-sectional, observational and analytical study was carried out at the Hospital de Especialidades del Centro Médico Nacional Siglo XXI, in patients hospitalized with severe COVID-19. Results: 183 cases were analyzed, of which 130 were men (71%). The median age was 55 years (IQR: 44-65). The neurological symptoms were: headache, anosmia and dysgeusia. Neurological manifestations occurred in 27 patients (16%), the most frequent was ischemic-type cerebrovascular disease (CVD) in 12 (44%), in patients older than 76.5 years vs. 54 years (p = 0.000), with history of cardiovascular disease. Conclusions: The most frequent neurological symptoms were headache, anosmia and dysgeusia. The most frequent neurological manifestation was ischemic CVD that appeared in older patients with severe COVID-19 with a history of cardiovascular disease.
Introducción: la enfermedad por SARS-CoV-2 denominada COVID-19 originada en China adquirió dimensiones pandémicas. De acuerdo con el reporte situacional de la OMS al 15 de marzo de 2021, la tasa de letalidad global es del 2.2%; en México se han confirmado alrededor de 194 944 defunciones por COVID-19. Estudios en China identificaron que los pacientes con COVID-19 severo, al compararlos con aquellos que cursaron con COVID-19 no severo, presentaron manifestaciones neurológicas más graves. Objetivo: determinar la frecuencia de síntomas y manifestaciones neurológicas en pacientes con COVID-19 severo en un centro de tercer nivel de atención. Material y métodos: estudio transversal, observacional y analítico, llevado a cabo en el Hospital de Especialidades del Centro Médico Nacional Siglo XXI, en pacientes hospitalizados con COVID-19 severo. Resultados: se analizaron 183 casos, de los cuales 130 eran hombres (71%). La mediana de edad fue de 55 años (RIC: 44-65). Los síntomas neurológicos fueron: cefalea, anosmia y disgeusia. Las manifestaciones neurológicas se presentaron en 27 pacientes, la más frecuente fue la enfermedad vascular cerebral tipo isquémica (EVC) en 12 pacientes (44%) en pacientes con mayor edad, 76.5 frente a 54 años (p = 0.000), y con antecedente de enfermedad cardiovascular. Conclusiones: los síntomas neurológicos más frecuentes fueron cefalea, anosmia y disgeusia. La manifestación neurológica más frecuente fue la EVC isquémica que se presentó en pacientes con COVID-19 severo de mayor edad y con antecedente de enfermedad cardiovascular.
Assuntos
COVID-19 , Doenças do Sistema Nervoso , Idoso , Estudos Transversais , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/epidemiologia , Doenças do Sistema Nervoso/etiologia , SARS-CoV-2 , Centros de Atenção TerciáriaRESUMO
BACKGROUND: Multiple questionnaires demonstrated the effect of the disease on the Quality of Life (QL), especially on allergies. Asthma and allergic rhinitis contributes to the reduction of QL. One of the accepted instruments for the evaluation of QL in chronic diseases is the questionnaire SF-36 (SF-36). Our objective was to validate the SF-36 in Mexican asthmatic with allergic rhinitis adult patients. METHODS: Fifty asthmatic patients with allergic rhinitis participated. They were placed according to the severity of their asthma (GINA 2004) and of allergic rhinitis (ARIA). The SF-36 was applied. The results were analyzed by descriptive statistics and validation by internal consistency through the Cronbach's test. RESULTS: It was observed that greater deterioration of QL in asthmatic patients with allergic rhinitis is directly related with the severity of their disease. Greater deterioration was observed on physical nature and of the perception of health. The aspect less affected was the social function and mental health. The Cronbach's test showed a coefficient of global reliability of 0.9314. CONCLUSIONS: The results obtained for the validation of SF-36, through the Cronbach's test in the population studied allows their use for later studies.
Assuntos
Asma/diagnóstico , Qualidade de Vida , Rinite Alérgica Perene/diagnóstico , Inquéritos e Questionários , Adulto , Asma/complicações , Estudos Transversais , Feminino , Humanos , Masculino , México , Rinite Alérgica Perene/complicaçõesRESUMO
BACKGROUND: X-linked agammaglobulinemia (XLA) is characterized by the absence of immunoglobulin and B cells. Patients suffer from recurrent bacterial infections from early childhood, and require lifelong immunoglobulin replacement therapy. Mutations in BTK (Bruton's Tyrosine Kinase) are associated with this phenotype. Some patients that present XLA do not show typical clinical symptoms, resulting in delayed diagnosis due to the lack of a severe phenotype. This study presents a report of five XLA patients from four different families and attempts to determine a relationship between delayed diagnosis and the occurrence of BTK mutations. METHODS: Samples from patients with antibody deficiency were analyzed to determine BTK expression, immunophenotyping and mutation analysis. Clinical and laboratory data was analyzed and presented for each patient. RESULTS: Most patients presented here showed atypical clinical and laboratory data for XLA, including normal IgM, IgG, or IgA levels. Most patients expressed detectable BTK protein. Sequencing of BTK showed that these patients harbored missense mutations in the pleckstrin homology and Src-homology-2 domains. When it was compared to public databases, BTK sequencing exhibited a new change, along with three other previously reported changes. CONCLUSIONS: Delayed diagnosis and atypical manifestations in XLA might be related to mutation type and BTK expression.
Assuntos
Agamaglobulinemia/diagnóstico , Linfócitos B/imunologia , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Infecções/diagnóstico , Mutação de Sentido Incorreto/genética , Domínios de Homologia à Plecstrina/genética , Proteínas Tirosina Quinases/genética , Domínios de Homologia de src/genética , Adolescente , Adulto , Tirosina Quinase da Agamaglobulinemia , Criança , Pré-Escolar , Análise Mutacional de DNA , Diagnóstico Tardio , Humanos , Imunoglobulinas/sangue , Imunoglobulinas/deficiência , Imunofenotipagem , Fenótipo , Adulto JovemRESUMO
Background: Bronchiectasis are permanent dilatations of the bronchi. Its prevalence in patients with variable common immunodeficiency (CVID) is high, however there is little information regarding the type and location of the same; therefore the objective of this study is to know the type and location of bronchiectasis in a cohort of adult patients with CVID. Methods: It has been made a transversal, observational and descriptive study that included 32 adult patients with diagnosis of CVID according to the criteria of the European Society of Immunodeficiencies (ESID). All patients underwent high resolution pulmonary computed tomography (HRCT), which were interpreted by an expert radiologist. The frequency, type and location of bronchiectasis were reported using descriptive statistics. Results: Thirty-two adult patients, ten men (31.25%) and 22 women (68.7%), were included. 40.6% had bronchiectasis. 23% had a lobe involvement, 15.3% two lobes, 46.1% 3 lobes and 15.3% complete involvement of the parenchyma. The types of bronchiectasis were distributed as follows: tubular 38.4%, varicose 23% and cystic and tubular combinations 15.3%, cystic and varicose 15.3% and cystic, tubular and varicose 7.6%. Conclusions: Our results show that 40% of adult patients with CVID have BQs, usually affecting three pulmonary lobes, located mainly in the right and middle lower lobe; The tubular type, is the most common. Their timely diagnosis and treatment can improve survival and reduce costs for patients and health care.
Introducción: Las bronquiectasias (BQs) son dilataciones permanentes de los bronquios. Su prevalencia en pacientes con inmunodeficiencia común variable (IDCV) es alta, sin embargo existe escasa información respecto al tipo y localización de las mismas. El objetivo de este estudio es conocer el tipo y localización de las bronquiectasias en una cohorte de pacientes adultos portadores de IDCV. Métodos: Estudio transversal, observacional y descriptivo que incluyó a 32 pacientes adultos con diagnóstico de IDCV de acuerdo con los criterios de la Sociedad Europea de Inmunodeficiencias. A todos se les realizó tomografía de alta resolución pulmonar, las cuales fueron interpretadas por un médico radiólogo experto. Se reportó la frecuencia, tipo y localización de las bronquiectasias mediante estadística descriptiva.Resultados: se incluyeron 32 pacientes adultos, diez hombres y 22 mujeres. El 40.6% presentaron bronquiectasias. El 23% tenía afección en un lóbulo, el 15.3% dos lóbulos, 46.1% 3 lóbulos y el 15.3% afectación completa del parénquima; distribuidos de la siguiente manera: tubulares 38.4%, varicosas 23% y las combinaciones quísticas y tubulares 15.3%, quísticas y varicosas 15.3% y quísticas, tubulares y varicosas 7.6%. Conclusión: Nuestros resultados muestran que el 40% de los pacientes adultos con IDCV tienen BQs, suelen afectar tres lóbulos pulmonares, el tipo más común fue el tubular. Su diagnóstico y tratamiento oportuno puede mejorar la supervivencia y reducir los costos para el paciente y las instituciones de salud.
Assuntos
Bronquiectasia/etiologia , Imunodeficiência de Variável Comum/complicações , Adulto , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/epidemiologia , Bronquiectasia/patologia , Estudos Transversais , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
Introducción: la enfermedad por SARS-CoV-2 denominada COVID-19 originada en China adquirió dimensiones pandémicas. De acuerdo con el reporte situacional de la OMS al 15 de marzo de 2021, la tasa de letalidad global es del 2.2%; en México se han confirmado alrededor de 194 944 defunciones por COVID-19. Estudios en China identificaron que los pacientes con COVID-19 severo, al compararlos con aquellos que cursaron con COVID-19 no severo, presentaron manifestaciones neurológicas más graves. Objetivo: determinar la frecuencia de síntomas y manifestaciones neurológicas en pacientes con COVID-19 severo en un centro de tercer nivel de atención. Material y métodos: estudio transversal, observacional y analítico, llevado a cabo en el Hospital de Especialidades del Centro Médico Nacional Siglo XXI, en pacientes hospitalizados con COVID-19 severo. Resultados: se analizaron 183 casos, de los cuales 130 eran hombres (71%). La mediana de edad fue de 55 años (RIC: 44-65). Los síntomas neurológicos fueron: cefalea, anosmia y disgeusia. Las manifestaciones neurológicas se presentaron en 27 pacientes, la más frecuente fue la enfermedad vascular cerebral tipo isquémica (EVC) en 12 pacientes (44%) en pacientes con mayor edad, 76.5 frente a 54 años (p = 0.000), y con antecedente de enfermedad cardiovascular. Conclusiones: los síntomas neurológicos más frecuentes fueron cefalea, anosmia y disgeusia. La manifestación neurológica más frecuente fue la EVC isquémica que se presentó en pacientes con COVID-19 severo de mayor edad y con antecedente de enfermedad cardiovascular.
Background: The SARS-CoV-2 disease, called COVID-19, emerged in China has acquired pandemic dimensions. According to the WHO situational report of March 15, 2021, the global fatality rate is 2.2%; in Mexico, around 194 944 deaths have been confirmed by COVID-19. Studies in China identified that patients with severe COVID-19, when compared with those who had non-severe COVID-19, presented more severe neurological manifestations. Objective: To determine the frequency of neurological symptoms and manifestations in patients with severe COVID-19 in a tertiary care center. Material and methods: A cross-sectional, observational and analytical study was carried out at the Hospital de Especialidades del Centro Médico Nacional Siglo XXI, in patients hospitalized with severe COVID-19. Results: 183 cases were analyzed, of which 130 were men (71%). The median age was 55 years (IQR: 44-65). The neurological symptoms were: headache, anosmia and dysgeusia. Neurological manifestations occurred in 27 patients (16%), the most frequent was ischemic-type cerebrovascular disease (CVD) in 12 (44%), in patients older than 76.5 years vs. 54 years (p = 0.000), with history of cardiovascular disease. Conclusions: The most frequent neurological symptoms were headache, anosmia and dysgeusia. The most frequent neurological manifestation was ischemic CVD that appeared in older patients with severe COVID-19 with a history of cardiovascular disease.
Assuntos
Humanos , Masculino , Feminino , Atenção Terciária à Saúde , Transtornos Cerebrovasculares , COVID-19 , Manifestações Neurológicas , Atenção Terciária à Saúde , CefaleiaRESUMO
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.
Antecedentes: los pacientes con inmunodeficiencia común variable tienen riesgo elevado de padecer enfermedades neoplásicas, con incidencia de 11 a 13%, especialmente durante la quinta y sexta décadas de la vida. Los linfomas de tipo celular B son los más frecuentes, además del linfoma no Hodgkin y tumores epiteliales (carcinomas de estómago, mama, vejiga y cuello uterino). Objetivo: conocer el tipo de enfermedades neoplásicas en una cohorte de pacientes adultos con inmunodeficiencia común variable. Material y método: estudio observacional, transversal y descriptivo, en el que se revisaron los expedientes con diagnóstico de inmunodeficiencia común variable de la Clínica de Inmunodeficiencias Primarias del servicio de Alergia e Inmunología Clínica del Hospital de Especialidades del Centro Médico Nacional Siglo XXI del IMSS. Resultados: se incluyeron 23 pacientes con diagnóstico de inmunodeficiencia común variable; de éstos, 13 (56%) eran mujeres, el promedio de edad fue de 36.7 años. Se encontraron neoplasias en cuatro pacientes de este grupo (dos mujeres y dos hombres), la prevalencia de cáncer fue de 17.3%. Los tipos de cáncer reportados fueron: linfoma Hodgkin de células B (1/23), carcinoma neuroendocrino de la cabeza del páncreas (1/23), leucemia mieloide crónica (1/23) y cáncer papilar de tiroides (1/23). En dos de los pacientes, el diagnóstico de cáncer fue previo al diagnóstico de la inmunodeficiencia primaria. El promedio de edad al momento del diagnóstico de cáncer fue de 27 años (límites: 19-34 años). Conclusión: la frecuencia de cáncer en nuestra población fue similar a lo reportado en la bibliografía. Sin embargo, los tipos de cáncer fueron distintos a lo publicado. Consideramos que es necesario realizar un protocolo de escrutinio en estos pacientes para diagnosticar de manera oportuna cualquier tipo de neoplasia, debido al mayor riesgo que tienen estos pacientes.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
BACKGROUND: Oral allergy syndrome (OAS) is the mildest form of an allergic reaction to foods. It is characterized by the presence of IgE mediated symptoms restricted to oral mucosa after intake of fresh fruits and vegetables. OAS diagnosis is based on suggestive clinical manifestations and can be confirmed with tests such as prick-to-prick skin test with the food implicated, which have a sensitivity > 90%, specificity 30-60%, positive predictive value (PPV) of 40% and negative predictive value (NPV) > 95%. OBJECTIVE: To know the prevalence of OAS in adult patients attending a third level hospital in southern Mexico City, and also describe the demographics of these patients and comorbidities more frequently associated. PATIENTS AND METHOD: We studied 44 patients (40 women and 4 men), with an average age of 33.4 years, with suggestive clinical features of OAS, evaluated with prick-by-prick skin tests with fresh food. RESULTS: There was a predominance of OAS in woman (91%) and a prevalence of asthma and allergic rhinitis of 54% among this population. We found that 60.4% of these patients had positive prick-by-prick skin tests, being the most frequently involved families of foods: rosaceae (60%), crustaceans (25%), musaceae (23%), actinidacea (21%), lauraceae (16%) and cucurbitaceae (16%). Only 5 patients presented adverse reactions with prick-by-prick skin tests, in 4 of the cases with grade 3 and grade 4 anaphylaxis, while testing with banana-watermelon, peach, papaya and peanut, respectively. CONCLUSIONS: Oral allergy syndrome affects more women than men, the most frequent comorbidities in patients with oral allergy syndrome are allergic rhinitis and asthma, thus, in patients with asthma and allergic rhinitis, sensitized to pollens we have to ask about symptoms suggestive of oral allergy syndrome.
ANTECEDENTES: el síndrome de alergia oral es la forma más leve de una reacción alérgica a alimentos. Se distingue por síntomas restringidos a la mucosa oral, que son mediados por la IgE y generados por la ingestión de frutas frescas y verduras. El diagnóstico de síndrome de alergia oral se establece con un cuadro clínico sugerente y se corrobora con diversos estudios, como las pruebas de Prick-to-Prick a alimentos, que poseen sensibilidad mayor a 90% y especificidad de 30 a 60%. OBJETIVO: conocer la prevalencia del síndrome de alergia oral en pacientes adultos atendidos en un hospital de tercer nivel del sur de la Ciudad de México, además de sus características demográficas y comorbilidades asociadas con más frecuencia. PACIENTES Y MÉTODO: estudio prospectivo efectuado con 44 pacientes, 40 mujeres y 4 hombres, con edad promedio de 33.4 años, quienes tuvieron datos clínicos sugerentes de síndrome de alergia oral, y a quienes se realizaron pruebas Prick-to-Prick a alimentos. RESULTADOS: hubo mayor predominio en mujeres (91%) y prevalencia de asma y rinitis alérgica de 54%. De los pacientes, 60.4% tuvo pruebas positivas Prick-to-Prick a alimentos de las siguientes familias: rosáceas (60%), crustáceos (25%), musáceas (23%), actinidáceas (21%), lauráceas (16%) y cucurbitáceas (16%). Sólo cinco pacientes tuvieron reacciones adversas con las pruebas Prick-to-Prick; en cuatro casos anafilaxia grados 3 y 4 al realizar pruebas con plátano-sandía, durazno, papaya y cacahuate, cada uno. CONCLUSIONES: el síndrome de alergia oral afecta más a mujeres que a hombres, las comorbilidades alérgicas más frecuentes en pacientes con síndrome de alergia oral son rinitis alérgica y asma, por tanto, en los pacientes con asma y rinitis alérgica sensibilizados a polénes debemos interrogar con respecto a los síntomas sugerentes de síndrome de alergia oral.
Assuntos
Hipersensibilidade Alimentar , Adolescente , Adulto , Feminino , Hipersensibilidade Alimentar/epidemiologia , Hipersensibilidade Alimentar/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Testes Cutâneos , Síndrome , Centros de Atenção Terciária , Adulto JovemRESUMO
BACKGROUND: Quality of life is a multidimensional concept that includes physical, emotional and social components associated with the disease. The use of tools to assess the Quality of Life Health Related (HRQOL) has increased in recent decades. Common variable immunodeficiency (CVID) is the most commonly diagnosed primary immunodeficiency. OBJECTIVE: To evaluate the quality of life in patients with CVID using the questionnaire SF -36. PATIENTS AND METHOD: A descriptive cross-sectional survey included 23 patients diagnosed with CVID, belonging to the Immunodeficiency Clinic Service of Allergology and Clinical Immunology in CMN Siglo XXI, IMSS. The questionnaire SF- 36 validated in Spanish was applied. STATISTICAL ANALYSIS: descriptive statistics with simple frequencies and percentages, inferential statistics: Fisher exact test and ANOVA to compare means. RESULTS: The study involved 23 patients, 14 women (60%) and 9 men (40%), mean age 38.6 ± 14.7 years. The highest score was obtained in 83% emotional role. Dimensions with further deterioration in both genders were: 54% general health, vitality 59% and physical performance 72%. No differences were found regarding gender. The only issue in which statistically significant differences were found in patients with more than 3 comorbidities was change in health status in the past year (p=0.007). Patients with severe comorbidities, such as haematologicaloncological (leukemias, lymphomas, neoplasms), and pulmonary (severe bronchiectasis) showed further deterioration in the aspects of physical performance 73% and 64% emotional role. 65% of patients reported an improvement in health status in 74% in the last year. CONCLUSIONS: Adult patients with CVID show deterioration in different dimensions, particularly in the areas of general health, vitality and physical performance. Patients with severe comorbidities such as leukemia, lymphomas, malignancies and severe bronchiectasis show further deterioration in some aspects of quality of life, especially in physical performance and emotional role. A higher number of comorbidities was significantly associated with a lower score in changing health. The questionnaire SF-36 is useful for evaluating the quality of life of our patients with CVID.
ANTECEDENTES: la calidad de vida es un concepto multidimensional que incluye componentes físicos, emocionales y sociales asociados con la enfermedad. El uso de instrumentos para evaluar la calidad de vida relacionada con la salud ha aumentado en las últimas décadas. La inmunodeficiencia común variable es, por mucho, la inmunodeficiencia primaria más diagnosticada. OBJETIVO: evaluar la calidad de vida de pacientes con inmunodeficiencia común variable usando el cuestionario SF-36. PACIENTES Y MÉTODO: encuesta transversal descriptiva en la que participaron 23 pacientes con diagnóstico de inmunodeficiencia común variable, pertenecientes a la Clínica de Inmunodeficiencias del Servicio de Alergología e Inmunología Clínica del Centro Médico Nacional Siglo XXI del Instituto Mexicano del Seguro Social. Se aplicó el Cuestionario SF-36 validado al español. Análisis estadístico: estadística descriptiva con frecuencias simples y porcentajes, estadística inferencial: prueba exacta de Fisher y ANOVA para comparación de medias. RESULTADOS: participaron en el estudio 23 pacientes, 14 mujeres (60%) y 9 hombres (40%), con edad promedio de 38.6 ± 14.7 años. La puntuación más alta se obtuvo en el rol emocional con 83%. Las dimensiones con mayor deterioro en ambos géneros fueron: salud general 54%, vitalidad 59% y desempeño físico 72%. No se encontraron diferencias en relación con el género. El único tema en el que se encontró diferencia estadísticamente significativa en los pacientes con más de tres comorbilidades fue el cambio en el estado de salud en el último año (p = 0.007). Los pacientes con comorbilidades graves, como las hematológicas-oncológicas (leucemias, linfomas, neoplasias) y neumológicas (bronquiectasias severas) tuvieron mayor deterioro en los aspectos de desempeño físico (73%) y rol emocional (64%). El 65% de los pacientes refirió mejoría del estado de salud en 74% en el último año. CONCLUSIONES: los pacientes adultos con inmunodeficiencia común variable muestran deterioro en diferentes dimensiones, particularmente en las áreas de salud general, vitalidad y desempeño físico. Los pacientes con comorbilidades graves, como leucemias, linfomas, neoplasias y bronquiectasias severas, muestran mayor deterioro en algunos aspectos de su calidad de vida, especialmente en su desempeño físico y rol emocional. Un mayor número de comorbilidades se asoció significativamente con menor puntaje en el cambio de salud. El cuestionario SF-36 es útil para evaluar la calidad de vida de los pacientes con inmunodeficiencia común variable.
Assuntos
Imunodeficiência de Variável Comum , Qualidade de Vida , Inquéritos e Questionários , Adulto , Imunodeficiência de Variável Comum/diagnóstico , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Common variable immunodeficiency is a primary immunodeficiency, in which from 70 to 80 % of patients have tumors and 25 % of cases are associated with autoimmune diseases. Common variable immunodeficiency patients have a higher incidence of neoplasms, with a risk 12-18 times higher than the general population. There are few cases of common variable immunodeficiency patients with leukemia. CLINICAL CASE: Female of 36 years old, with left upper quadrant pain, early satiety, weight loss of 8 kg in three months and splenomegaly. The complete blood count showed: leukocytosis 206 000/mL, with 8 % blasts, platelets 530 000/mL and hemoglobin 8 mg/dL. Abdominal ultrasound: 19??12 cm splenomegaly. Karyotype BCR/ABL IS 64.20 %, 100 % Philadelphia chromosome. The diagnosis was of chronic myeloid leukemia. Given the presence of recurrent respiratory tract infection, frequent diarrheas and reduced concentrations of IgG, IgM and IgA, common variable immunodeficiency was diagnosed and human immunoglobulin was used successfully. CONCLUSIONS: The association between chronic myeloid leukemia and common variable immunodefficiency is unusual. Given the high frequency of hematological neoplasm in common variable immunodeficiency patients, we suggest that hematological patients with repeated infections and decreased concentrations of immunoglobulin be referred to an immunological evaluation.
INTRODUCCIÓN: la inmunodeficiencia común variable es la inmunodeficiencia primaria más sintomática, 70 a 80 % de los pacientes presentan neoplasias. Existen escasos informes de pacientes portadores de leucemia mieloide crónica e inmunodeficiencia común variable. CASO CLÍNICO: mujer de 36 años, quien inició su padecimiento con dolor en hipocondrio izquierdo, pérdida de peso de 8 kg en tres meses y esplenomegalia. Los resultados de la biometría hemática indicaron cuenta leucocitaria de 206 000/mL, 8 % de blastos, cuenta plaquetaria de 530 000/mL y hemoglobina de 8 g/dL. Con la ultrasonografía abdominal se identificó esplenomegalia de 19 ´ 12 cm. El cariotipo mostró el gen BCR/ABL 64.20 % IS y cromosoma Filadelfia 100 %. Se diagnosticó leucemia mieloide crónica. Por infecciones gastrointestinales y respiratorias frecuentes, así como por concentraciones reducidas de IgG, IgM e IgA, la paciente fue valorada por el servicio de alergia e inmunología clínica, donde se diagnosticó inmunodeficiencia común variable. CONCLUSIONES: la relación de la leucemia mieloide crónica con la inmunodeficiencia común variable es poco frecuente. Es importante que los pacientes portadores de leucemia mieloide crónica que cursen con infecciones de repetición, se realicen cuantificación de inmunoglobulinas séricas y, en caso de disminución de las mismas, sean referidos para su valoración y tratamiento.
Assuntos
Imunodeficiência de Variável Comum/diagnóstico , Leucemia Mielogênica Crônica BCR-ABL Positiva/etiologia , Adulto , Imunodeficiência de Variável Comum/complicações , Feminino , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnósticoRESUMO
BACKGROUND: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency, affecting 1:25,000-75,000 people. It is characterized by the absence or decrease antibody production. Treatment for CVID consists on human immunoglobulin administration, and the intravenous route is the most common route for administration, at 400-800 mg/kg of weight every 3-4 weeks. Adverse effects associated with intravenous immunoglobulin (IVIg) use occur in 25% of all infusions, with severe adverse reactions presenting in less than 1% of all patients. Acute renal failure can occur as a severe adverse reaction, which presents 1-10 days after starting IVIg treatment. In our center we implemented an ambulatory scheme for IVIg administration, which allows its administration in an average of 3 hours, without severe adverse effects. OBJECTIVES: To describe adverse effects and to evaluate the frequency of renal failure secondary to ambulatory IVIg administration in patients with common variable immunodeficiency. MATERIAL AND METHOD: A descriptive and prospective study was done including adult patients con definitive diagnosis of common variable immunodeficiency, receiving IVIg at replacement dose every 3 weeks. All patients were evaluated with clinical exploration, somatometry, serum creatinine, albumin and urea determination, 24 hours creatinine clearance, glomerular filtration rate with CKD-EPI, and immediate renal function associated with accumulated IVIg. Results were analyzed with descriptive statistics. RESULTS: We determined adverse effects in 25 patients with common variable immunodeficiency (15 women and 10 men, average age 36.7 years), during a 10 months period (January-September 2013). During this period 284 IVIg infusions were administered using our scheme, frequency of adverse effects were 12.9%, with 5.2% of early adverse effects and 7.7% late adverse effects, all being mild to moderate, in some cases required analgesic and/or antihistamine administration, without having to stop the IVIg infusion. In the renal function study 19 patients were included (12 women and 7 men, average age 36 years, average weigh 58.74 kg and average height 1.60 m), evaluated from January 2009 to October 2013. Average serum creatinine was 0.76 ± 0.18 mg/dL, average serum urea was 28.6 ± 7.6 mg/dL, none patient presenting acute renal failure. Glomerular filtration rate was determined with CKD-EPI formula, and the average was 116 ± 34 mL/min/1.73 m2, finding chronic renal failure in 4 patients. Average 24 hours creatinine clearance was 98.64 ± 22 mL/min/1.73 m2, with chronic renal failure data in 6 patients. CONCLUSIONS: There were no severe adverse effects with this ambulatory IVIg scheme (anaphylaxis, acute renal failure). We did not find data of acute renal failure secondary to IVIg administration in this population, but we did find data of chronic renal failure secondary to IVIg administration through 24 hours creatinine clearance in 6 patients. No relation was found between accumulated IVIg dose in the last 5 years and decreased glomerular filtration rate. Another benefit worth of mentioning with this scheme is the reduction in costs for the health institution and to the patient.
Antecedentes: la inmunodeficiencia común variable es la inmunodeficiencia primaria sintomática más frecuente, afecta a 1 por cada 25,000 a 75,000 sujetos. Se distingue por la ausencia o disminución de anticuerpos. Su tratamiento consiste en el reemplazo de anticuerpos con inmunoglobulina humana y la vía de administración más frecuente es la intravenosa, a dosis de 400 a 800 mg/kg de peso/dosis cada tres a cuatro semanas. Los efectos adversos asociados con la administración de inmunoglobulina intravenosa (IgIV) ocurren incluso en 25% de todas las infusiones realizadas, las reacciones severas afectan a menos de 1% de los pacientes. Entre las reacciones adversas severas están la insuficiencia renal aguda, que sobreviene 1 a 10 días después del inicio de tratamiento con IgIV. En nuestro centro elaboramos e implementamos un esquema ambulatorio para la aplicación de IgIV que permite su administración en un promedio de 3 h, sin efectos adversos graves. Objetivos: describir los efectos adversos y evaluar la frecuencia de insuficiencia renal secundaria a la aplicación ambulatoria de IgIV en pacientes adultos con inmunodeficiencia común variable. Material y método: estudio descriptivo y prospectivo en el que participaron pacientes adultos con diagnóstico definitivo de inmunodeficiencia común variable, que recibían IgIV a dosis de sustitución cada tres semanas, a quienes se realizó exploración física, somatometría, determinación sérica de creatinina, albúmina y urea, depuración de creatinina en orina de 24 horas, cálculo de la tasa de filtración glomerular por la fórmula CKD-EPI y evaluación de la función renal inmediata, así como la asociada con la administración acumulada de IgIV a través del cálculo de la tasa de filtración glomerular. Los resultados se analizaron con estadística descriptiva para el reporte de los efectos en la función renal y la dosis acumulada de IgIV. Resultados: se determinó la frecuencia de reacciones adversas en 25 pacientes con diagnóstico de inmunodeficiencia común variable (15 mujeres y 10 varones, con edad promedio de 36.7 años), durante un periodo de 10 meses (enero a septiembre de 2013). En este periodo se aplicaron 284 infusiones de IgIV utilizando el esquema propuesto, la frecuencia de reacciones adversas fue de 12.9% del total de las infusiones, de las que 5.2% fueron reacciones adversas tempranas y 7.7% reacciones adversas tardías, todas fueron reacciones leves a moderadas, que ameritaron en algunos casos la administración de analgésico, antihistamínico o ambos, sin llegar a requerirse la suspensión de la infusión de IgIV. En el estudio de la función renal se incluyeron 19 pacientes (12 mujeres y 7 hombres, edad promedio 36 años, peso promedio 58.74 kg y talla promedio 1.60 m) y en el lapso estudiado (enero de 2009 a octubre de 2013) la determinación de creatinina en promedio fue de 0.76 ± 0.18 mg/dL; la urea sérica promedio del grupo fue de 28.6 ± 7.6 mg/dL, ningún paciente tuvo datos de insuficiencia renal aguda. La tasa de filtración glomerular se determinó mediante la fórmula de CKD-EPI y fue, en promedio, de 116 ± 34 mL/min/1.73 m2, se encontraron datos de deterioro renal crónico en cuatro pacientes. La determinación promedio de la depuración de creatinina en orina de 24 horas fue de 98.64 ± 22 mL/min/1.73 m2 y se encontraron datos de deterioro crónico en la función renal en seis pacientes. Conclusiones: con este esquema ambulatorio de aplicación de IgIV no hubo reacciones adversas graves (anafilaxia, broncoespasmo, insuficiencia renal aguda). En esta población de pacientes no se encontró afección renal aguda secundaria a la aplicación de IgIV, pero mediante la depuración de creatinina de 24 horas sí se encontraron datos que sugirieron daño renal crónico en seis pacientes. No se encontró relación entre la dosis acumulada de IgIV aplicada en los últimos cinco años con el hallazgo de disminución de la tasa de filtración glomerular. Otro beneficio de este método de aplicación de IgIV es la reducción en los costos para la institución de salud y para el paciente, además de permitir a éste reintegrarse a sus actividades cotidianas laborales o escolares de manera rápida.
RESUMO
Allergy to wheat proteins is the most common type of occupational asthma caused by cereals. Its incidence based on international reports is about 1-2.4/1000 cases among workers per year. In Mexico there is an underdiagnosis of occupational asthma > 99%. During the period 2009-2012 we recorded 5 cases of occupational asthma, 40% of them caused by cereals. This is the case of a 38 year old male, baker for 24 years exposed to wheat flour. In his workplace he used to wear surgical mask and there are no exhaust fans. He initiated 10 years ago with sneezing, itching, hyaline rhinorrhea and nasal obstruction. The following year he presented mild to moderate dyspnea, chest tightness, cough and wheezing during working hours, which improved during his days off. Pulmonary function tests were confirmatory of asthma, with negative skin prick tests to aeroallergens. Specific bronchial challenge was performed with wheat flour resulting positive with a decrease in VEF1 greater than 20%. Pharmacological treatment was provided and was sent to occupational medicine service for evaluation. The case was qualified as an occupational disease with a permanent partial disability of 25%, receiving a financial compensation. He returned to work in the same company but in a different area without wheat flour exposure. The diagnosis of occupational asthma is mainly clinical, according to Brooks¥ criteria, and a bronchial positive challenge test will confirms the diagnosis.
Assuntos
Asma Ocupacional/etiologia , Farinha/toxicidade , Adulto , Asma Ocupacional/diagnóstico , Árvores de Decisões , Humanos , MasculinoRESUMO
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 (â 28.3 ± 25.18 vs. â 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.
Assuntos
Bronquiectasia/complicações , Imunodeficiência de Variável Comum/complicações , Qualidade de Vida , Adulto , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários , Adulto JovemRESUMO
BACKGROUND: Mites are the most common cause of respiratory allergy. Sensitization to house dust mites is estimated at 30%. Families Euroglyphus and Dermatophagoides, are the most important. Recently, storage mites, from the families Acaridae and Glyciphagidae, have become more important as a cause of allergic respiratory diseases. OBJECTIVE: The aim of this study was to identify sensitization to house dust and storage mites by skin tests in adult patients with allergic respiratory diseases. METHODS: This is a descriptive study in patients with asthma and/or allergic rhinitis, with the approval of the local research committee 2009-3601-46. Patients underwent skin prick tests, with seven standardized extracts of mites (IPI ASACR). The results were analyzed with descriptive statistics. RESULTS: A total of 150 patients, 109 women and 41 men, with an average age 31 years (±11) were studied. The more common allergic diseases in this group of patients were: asthma and rhinitis 72 and 26%, respectively, and asthma plus rhinitis 3.2%. Positive skin tests for Dermatophagoides pteronyssinus and Dermatophagoides farinae were 82.1% 71.5%, respectively, and for storage mites: A. siro 51.7%, Tyrophagus 47.4%, Glycyphagus 47.7%, B. tropicalis 39.7% and Lepidoglyphus 39.1%. Storage mites produced greater sensitization than house dust mites, 32% vs 10%. CONCLUSIONS: The results show that adult patients with respiratory allergy, in southern Mexico City, have a higher rate of sensitization to storage mites than the one reported in the literature.
Assuntos
Poeira/imunologia , Pyroglyphidae/imunologia , Hipersensibilidade Respiratória/imunologia , Adolescente , Adulto , Animais , Feminino , Humanos , Masculino , México , Pessoa de Meia-Idade , Hipersensibilidade Respiratória/diagnóstico , Testes Cutâneos , Saúde da População Urbana , Adulto JovemRESUMO
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.