ABSTRACT
Los anticuerpos anti endomisio IgA (EMA) están dirigidos hacia antígenos del tejido conectivo que rodea a las fibras del músculo liso. El objetivo de este trabajo fue evaluar la eficacia del cordón umbilical humano (CUH) como sustrato para detectar EMA mediante inmunofluorescencia indirecta y compararlo con una de las metodologías disponibles comercialmente, la cual utiliza como sustrato esófago de mono. Se obtuvieron 100 sueros de pacientes con diagnóstico de enfermedad celíaca y 50 sueros de pacientes clínicamente sanos con biopsia de mucosa intestinal normal, los cuales realizaron su consulta y atención en el Hospital Privado Centro Médico de Córdoba, en un periodo de tiempo comprendido entre los años 2006 y 2009. Los resultados obtenidos mostraron una "muy buena" concordancia entre ambos métodos. Se estimó para el método que utiliza CUH una sensibilidad y especificidad de 98% (93-99%) y 100% (93-100%) respectivamente con una eficacia del 99%. De acuerdo con lo anterior se concluye que utilizar CUH como sustrato para evaluar la presencia de EMA es confiable y efectivo para detectar pacientes con enfermedad celíaca no tratada.
The antiendomysium antibodies (EMA) are directed toward antigens of connective tissue that surrounds the smooth muscle fibers. The aim of this study was to evaluate the efficiency of human umbilical cord (HUC) as substrate to detect EMA by indirect immunofluorescence and to compare it with one of the commercially available methodologies which use monkey esophagus as substrate. Serum samples obtained from 100 patients with celiac disease diagnosis and 50 healthy controls with normal intestinal mucosa were evaluated. Patients were treated at the Hospital Privado Centro Médico de Córdoba over a period of time between 2006 and 2009. The results showed an "almost perfect" concordance between both methods. The calculated sensitivity and specificity for HUC was 98% (93-99%) and 100% (93-100%) respectively, with an efficiency of 99%. This results indicate that the use of HUC as substrate to evaluate the presence of EMA is reliable and effective for the detection of patients with untreated celiac disease.
Os anticorpos anti-endomísio IgA (EMA) são direcionados contra os antígenos do tecido conectivo que cercam as fibras do músculo liso. O objetivo deste trabalho foi avaliar a eficácia do cordão umbilical humano (CUH) como substrato para detectar EMA através da imunofluorescência indireta e compará-lo com uma das metodologias disponíveis comercialmente, a qual utiliza como substrato esôfago de macaco. Foram obtidos 100 soros de pacientes com diagnóstico de doença celíaca e 50 soros de pacientes clinicamente saudáveis com biópsia de mucosa intestinal normal, os quais realizaram sua consulta e atendimento no Hospital Privado Centro Médico de Córdoba, em um período de tempo compreendido entre os anos 2006 e 2009. Os resultados obtidos mostraram uma "ótima" concordância entre ambos os métodos. Foi calculada para o método que utiliza CUH uma sensibilidade e especificidade de 98% (93-99%) e 100% (93-100%) respectivamente com uma eficácia de 99%. De acordo com o acima exposto, se conclui que utilizar CUH como substrato para avaliar a presença de EMA é confiável e eficaz para detectar pacientes com doenças celíacas não tratadas.
Subject(s)
Humans , Celiac Disease/diagnosis , Immunoglobulin A , Umbilical Cord , Argentina , IgA Deficiency/diagnosis , Serology , Umbilical Cord/cytologyABSTRACT
Transient hypogammaglobulinemia of infancy (THI) is originally defined as a physiological maturation defect of immunoglobulin G (IgG) production that occurs at 3-6 months of age and lasts until 18 to 36 months of age. We report here on a 22-month-old child with THI and IgA deficiency, who had massive pneumococcal empyema. Her depressed IgG level returned to normal within 6 months, but IgA level was still low at 6 yr of age. Although THI is an age-dependent and self-limiting disorder, severe infection that includes an atypical presentation of an infection may occur in some patients and this requires evaluation with immunologic study.
Subject(s)
Female , Humans , Infant , Agammaglobulinemia/complications , Anti-Bacterial Agents/therapeutic use , Ceftriaxone/therapeutic use , Drug Resistance, Bacterial , Empyema, Pleural/diagnosis , IgA Deficiency/diagnosis , Immunoglobulin A/blood , Immunoglobulin G/blood , Staphylococcal Infections/diagnosis , Tomography, X-Ray ComputedABSTRACT
BACKGROUND AND OBJECTIVES: IgA deficient patients are at risk of severe anaphylactic reaction on being transfused blood and blood products, and its prevalence varies in different parts of the world. No data are available from India.We did a blood donor survey to look for prevalence of IgA deficiency in north India. METHODS: A sensitive enzyme linked immunosorbent assay developed in-house was used to detect IgA deficiency in a total of 3818 blood donors. Complete IgA deficiency was defined as value less than 5 mg/dl whereas partial IgA deficiency was defined as value between 5-30 mg/dl. RESULTS: Of the 3818 blood donors screened, 3640 (95.3%) were males with a mean age of 31.2 yr. No donor was found to have complete IgA deficiency; however, 257 (6.7%) had partial IgA deficiency. INTERPRETATION AND CONCLUSION: Our study shows that IgA deficiency is rare in north India.
Subject(s)
Adolescent , Adult , Anaphylaxis/etiology , Autoantibodies/blood , Blood Donors , Blood Transfusion/adverse effects , Enzyme-Linked Immunosorbent Assay , Female , Humans , IgA Deficiency/diagnosis , Immunoglobulin A/blood , India/epidemiology , Male , Middle AgedABSTRACT
A doença por depósito linear de imunoglobulina A (IgA) é um processo mucocutâneo crônico, raro e de origem auto-imune, caracterizado por depósitos lineares do anticorpo ao longo da membrana basal da pele e mucosas. O estudo da enfermidade é de grande importância, visto que a mesma é de complexo diagnóstico e tratamento, além de sua etiopatogenia não estar ainda definida. O presente artigo teve por objetivo revisar a literatura referente à doença da IgA linear, abordando suas características clínicas, diagnóstico diferencial e alternativas de tratamento
Subject(s)
Male , Female , Child , Adult , IgA Deficiency/diagnosis , IgA Deficiency/pathology , Autoimmune Diseases/diagnosis , Immunoglobulin A, Secretory/immunology , Immunoglobulin A, Secretory , Fluorescent Antibody Technique/methods , Basement Membrane/immunology , Basement Membrane/injuriesABSTRACT
OBJECTIVE: To characterize an IgA deficient population in terms of the incidence of IgG subclass and mannose-binding lectin (MBL) deficiencies and the type and severity of infections and other associated disorders. BACKGROUND: Selective IgA deficiency is probably the commonest of the primary immunodeficiency disorders and although it may lead to an increased risk for respiratory and gastrointestinal infections and associated to various autoimmune diseases, it may also be asymptomatic. Several studies have suggested the need of a concomitant defect in order for manifestation of its symptoms. METHODS: A total of 27 patients fulfilling the diagnostic criteria of selective IgA deficiency were evaluated for IgG subclass and MBL deficiencies after a thorough medical history, physical examination and pertinent evaluation for concomitant medical conditions. RESULTS: The overall incidence of IgG subclass deficiency found in the IgA deficient group was 18.5. MBL deficiency was found to be 3.7. These frequencies may have been influenced by the age group evaluated and the size of the population studied. Severe infections were more common in patients with combined deficiencies, either IgA and any of the IgG subclasses or IgA and MBL deficiency. Atopy was widely represented in the patients studied. CONCLUSIONS: The observed relationship between combined deficiencies Ig A, IgG subclasses and MBL and the increased representation of severe infections needs to be corroborated in a larger sample of patients with an inclusion of pediatric patients.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , IgA Deficiency/diagnosis , Mannose-Binding Lectin/deficiency , IgA Deficiency/blood , IgG Deficiency/blood , IgG Deficiency/diagnosis , Immunoglobulins/blood , Mannose-Binding Lectin/bloodABSTRACT
Objetivos. Caracterizar os principais dados clínicos que orientem a pesquisa da DIgA e descrever sua evoluçao clínica e complicaçoes durante o seguimento ambulatorial. Material e Métodos. Foram avaliados, retrospectivamente, 60 pacientes portadores de DIgA (níveis séricos de IgA < 5 mg/dL) e submetidos à avaliaçao Clínica e provas laboratoriais específicas. Resultados. As queixas principais foram os processos infecciosos (50 por cento), quadros alérgicos (34 por cento) e doenças auto-imunes (10 por cento). Os processos infecciosos recorrentes acometeram preferencialmente o trato respiratório, sendo asma e rinite as manifestaçoes alérgicas mais frequentes. à avaliaçao laboratorial, além da deficiência de IgA, apenas os níveis de IgG e IgM encontravam-se elevados em 50 por cento dos pacientes. Durante a evoluçao, houve melhora da sintomatologia clínica e alguns pacientes tornaram-se assintomáticos durante a idade escolar e puberdade. Conclusao: A DIgA tem espectro clínico variado devendo ser sempre pesquisada em pacientes com infecçoes de repetiçao, processos alérgicos ou doenças auto-imunes para orientaçao de profilaxia precoce dos processos infecciosos e doenças alérgicas.
Subject(s)
Female , Humans , Infant , Child , Child, Preschool , Adolescent , IgA Deficiency/diagnosis , Ambulatory Care , Immunoglobulin G/blood , Immunoglobulin M/blood , Retrospective Studies , Follow-Up Studies , IgA Deficiency/complicationsSubject(s)
Humans , Defense Mechanisms , Immunologic Deficiency Syndromes , Agammaglobulinemia/diagnosis , Ataxia Telangiectasia/diagnosis , Granulomatous Disease, Chronic/diagnosis , IgA Deficiency/diagnosis , IgG Deficiency/diagnosis , Wiskott-Aldrich Syndrome/diagnosis , Immunologic Deficiency Syndromes/classification , Immunologic Deficiency Syndromes/diagnosisABSTRACT
Se analiza una casuística de 6 pacientes con inmunodeficiencias primarias, estudiados entre 1981 y 1994 y que cumplen con los criterios establecidos por la OMS en 1991. Se plantean las siguientes conclusiones: 1. Las inmunodeficiencias primarias específicas más frecuentes fueron las predominantemente de anticuerpos 2. Las infecciones recurrentes fueron el pilar del diagnóstico clínico. Estas fueron causadas por bacterias encapsuladas y afectaron de preferencia la piel y los sistemas respiratorio y digestivo 3. A todo paciente con score de Haeney mayor o igual a 25, independiente de su edad y especialmente si no tiene causa reconocible de inmunodeficiencia secundaria, debería evaluarse inmunológicamente 4. En las inmunodeficiencias humorales los exámenes más útiles fueron la electroforesis de proteínas séricas y la cuantificación de inmunoglobulinas, mientras que en las celulares lo fue el Multitest