Tratamiento con inmunoglobulina G subcutánea en enfermos con inmunodeficiencias primarias: resultados preliminares del estudio multicentrico cubano / Treatment with subcutaneous immunoglobulin G in patients with primary immunodeficiencies: preliminary results of cuban multicenter study

Introducción : Estudios previos muestran que la infusión de inmunoglobulina G (IgG) subcutánea (SC) presenta una eficacia similar a la IgG endovenosa (EV)para prevenir las infecciones en enfermos con inmunodeficiencias primarias (IDP),predominantemente de anticuerpos con deficiencias de IgG y que este tratamiento es seguro y bien tolerado. Objetivo : Evaluar la seguridad, efectividad y tolerancia del tratamiento con IgG SC en un grupo de pacientes con IDP con deficiencias de IgG demostrada, previamente tratados con IgG EV. Métodos : Se realizó un estudio multicéntrico de remplazo en la administración de IgG EV (Intacglobin, de producción nacional) a IgG SC (Gammanorm, Octhapharma) en pacientes con IDP con deficiencia de IgG. Se incluyeron 6 enfermos; 3 niños y 3 adultos, procedentes de diferentes instituciones del país. La dosis de IgG SC fue similar a la dosis global mensual previa de IgG EV, administrada en 4 dosis divididas con valor promedio de 108 mg/kg (rango entre 100-200) semanal, durante 36 semanas. Resultados : En los enfermos que no recibieron tratamiento con IgG EV y presentaban valores muy disminuidos, los niveles de IgG sérica alcanzaron valores normales para la edad. En el resto de los pacientes, que llevaban tratamiento con IgGEV en la semana anterior al estudio, los niveles de IgG sérica se incrementaron o se mantuvieron superiores a 7 g/L dentro del rango normal. En todos los enfermos disminuyó la frecuencia y gravedad de las infecciones;durante el período de infusión de IgG SC,la tasa de infección disminuyó de 1.7 infecciones/sujeto/año a 0.5 y las infecciones fueron leves con buena respuesta al tratamiento. Todos los enfermos mantienen la administración de IgG SC domiciliaria con una buena respuesta. Conclusiones: El tratamiento de remplazo es bien tolerado y constituye una alternativa terapéutica efectiva para los enfermos con IDP(AU)

Intravenous immunoglobulin therapy in a selective IgG3 deficient patient with recurrent respiratory infections and asthma attacks

The IgG subclass deficiency is defined as a significant decrease in the serum concentrations of one or more subclasses of IgG in a patient whose total IgG concentration is normal. IgG subclass deficiency can predispose to recurrent sinopulmonary infections. A 29-year-old female patient with a 4-year history of bronchial asthma presented with cough, sputum, dyspnea, and recurrent respiratory infections. She had frequently been treated with antibiotics and systemic steroids for recurrent respiratory infections and acute asthma exacerbations. Chest X-ray and computed tomography showed pectus excavatum and bronchial wall thickening without lung parenchymal abnormalities. On immunological evaluation, she was found to have a low serum IgG3, with normal total IgG concentration. Under diagnosis of selective IgG3 deficiency, she was started on monthly infusions of intravenous immunoglobulin (IVIG) therapy. The frequency and severity of respiratory infections and acute asthma exacerbations were markedly decreased during 3 years of IVIG therapy. Our case report suggests that a patient who has underlying selective IgG3 deficiency and asthma may benefit from IVIG therapy as this can significantly reduce the incidence and severity of recurrent respiratory infections and acute asthma exacerbations.

A Case of Prolonged Hypogammaglobulinemia after Rituximab-Containing Chemotherapy in a Patient with Lymphoma / 대한내과학회지

Rituximab, an anti-CD20 monoclonal antibody, is an effective target agent against the B lymphocytes in B-cell lymphoid malignancies and various lymphoproliferative diseases. Moreover, the toxicity of rituximab is less severe than that of conventional cytotoxic agents, which has promoted the widespread application of rituximab in the treatment of B-cell lymphoma. However, depletion of B lymphocytes by rituximab, which leads to secondary hypogammaglobulinemia, can cause deterioration of humoral immunity. Although immune reconstitution after hematopoietic stem cell transplantation is known to prevent prolonged hypogammaglobulinemia, very few cases of long-standing hypogammaglobulinemia have been reported. We report herein a case of prolonged hypogammaglobulinemia after rituximab-containing chemotherapy and splenectomy in a patient with non-Hodgkin's lymphoma and discuss the clinical significance and pathogenetic mechanism of this phenomenon with a literature review.

Prevalence of Primary Immunodeficiency in Korea

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.

Prevalence of serological markers for celiac disease (IgA and IgG class antigliadin antibodies and IgA class antiendomysium antibodies) in patients with autoimmune rheumatologic diseases in Belo Horizonte, MG, Brazil / Pesquisa de anticorpos antigliadina (classes IgA e IgG) e anticorpos antiendomísio classe IgA, em pacientes com doenças reumatológicas autoimunes em Belo Horizonte, Brasil

CONTEXT: Patients with autoimmune rheumatologic conditions and celiac disease tend to have a variety of autoantibodies, many of which have no clear pathogenic role. The literature contains frequent reports of celiac disease being more prevalent in patients with rheumatologic diseases, although this remains controversial. OBJECTIVES: To investigate the prevalence of positive serum tests for celiac disease, particularly IgA and IgG antigliadin (AGA) antibodies and IgA antiendomysium antibodies (EmA) in patients with autoimmune rheumatologic diseases. A second aim was to correlate positive serum tests with prednisone and immunosuppressant medication. METHODS: A total of 190 adults and pediatric patients with a variety of autoimmune rheumatologic diseases (systemic lupus erythematosus, rheumatoid arthritis, juvenile rheumatoid arthritis and spondyloarthrophathies) were evaluated and tested for IgA and IgG antigliadin-antibodies and IgA antiendomysium antibodies. Patients with positive serum tests underwent endoscopic duodenal biopsies for pathology studies. RESULTS: There were four positive sera (2.1 percent) for AGA IgA, all of which tested negative for AGA IgG and EmA. Three sera (1.6 percent) tested positive for AGA IgG; all were negative for AGA IgA and EmA. The EmA test at a 1:2.5 serum dilution tested positive in 94 patients (49.5 percent); at a 1:5 serum dilution it was positive in 41 patients (21.6 percent). Eleven subjects tested positive for EmA at 1:40 dilution; and all of these tested negative for IgA tissue antitransglutaminase (tTG) antibodies. Nine of the 11 EmA-positive patients and all 7 patients with positive antigliadin antibodies tests underwent duodenal endoscopic biopsies, and no significant changes were demonstrated in their duodenal mucosa. A positive EmA was associated with elevated optical density AGA IgA readings; however, there was no relationship between positive EmA and AGA IgG optical density readings. Prednisone and immunosuppressant use were unrelated to AGA IgA optical density readings or AGA IgG readings. These drugs were associated with fewer positive EmA tests. CONCLUSIONS: Positive AGAA, AGAG or EmA results are probably nonspecific for the presence of celiac disease among autoimmune rheumatologic disease patients. The intake of prednisone and immunosuprressant drugs seems to reduce the prevalence of IgA EmA, but it does not interfere with antigliadin antibodies tests.Further studies are required to estimate more accurately the prevalence of this disease in rheumatologic patients.

CONTEXTO: Tanto os pacientes com doenças reumatológicas autoimunes quanto os com doença celíaca costumam apresentar vários tipos de autoanticorpos, muitos deles ainda sem papel definido na etiopatogênese dessas afecções. Apesar de tratar-se de assunto controverso, é bastante citada na literatura a maior prevalência da doença celíaca em diversos grupos de pacientes reumatológicos. OBJETIVO: Investigar a prevalência de marcadores sorológicos positivos para doença celíaca: anticorpos antigliadina (AGA) classes IgA e IgG (AGAA e AGAG) e anticorpos antiendomísio classe IgA (EmA), em pacientes com doenças reumatológicas autoimunes. Procurou-se também avaliar a correlação entre a positividade dos testes sorológicos com o uso de prednisona e de medicamentos imunossupressores. MÉTODOS: Foram avaliados 190 pacientes adultos e pediátricos com doenças reumatólogicas variadas (lúpus eritematoso sistêmico, artrite reumatóide, artrite reumatóide juvenil e espondiloartropatias. Em todos foram realizadas pesquisas de AGAA e AGAG e de EmA, encaminhando-se os casos positivos para biopsias endoscópica duodenal e estudos histológicos. RESULTADOS: Houve quatro soros positivos (2,1 por cento) para AGAA, todos com resultados negativos para AGAG e EmA. Três soros (1,6 por cento) tiveram resultados positivos para AGAG, todos com resultados negativos para AGAA e EmA. Na pesquisa de EmA, a diluição do soro em 1:2,5 mostrou resultados positivos em 94 pacientes (49,5 por cento) e na diluição de 1:5, em 41 (21,6 por cento). Em 11 indivíduos obteve-se resultado positivo para EmA na diluição 1:40 e todos eles tiveram resultado negativo para a pesquisa de anticorpos antitransglutaminase tecidual IgA (tTg). Nove dos 11 pacientes positivos para EmA e todos os 7 pacientes com anticorpos antigliadina positivos foram submetidos a biopsia duodenal endoscópica, não se constatando alterações significativas da mucosa duodenal em nenhum deles. Todos os soros positivos para EmA apresentaram resultados negativos para a pesquisa de anticorpos antitransglutaminase tecidual classe IgA (tTG). A positividade para EmA associou-se a leituras de densidade óptica mais altas para AGAA. O mesmo não foi observado para AGAG. O uso de prednisona e de imunossupressores não se relacionou às leituras de densidade óptica dos AGAA, tampouco dos AGAG. O uso dessas medicações se relacionou, contudo, a menor positividade para EmA. CONCLUSÃO: Resultados positivos para AGAA, AGAG ou EmA demonstraram-se inespecíficos para a presença de doença celíaca em pacientes com doenças reumatológicas autoimune. O uso de prednisona e drogas imunossupressoras parece diminuir a prevalência de anticorpos antiendomísio IgA, mas não de anticorpos antigliadina. Mais estudos são necessários para se avaliar com maior precisão a prevalência da doença celíaca em pacientes reumatológicos.

Down-Syndrome associated with MBL-deficiency, IgG-deficiency, vasculitis and mutated prothrombin / Síndrome de Down asociado a deficiencia de lectina de unión a manosa, deficiencia de IgG, vasculitis y mutación de la protrombina: Informe de un caso

The association of Down syndrome with mannose-binding lectin (MBL)-deficiency, recurrent infections and vasculitis has not been reponed. We repon a 30 year-old female with Down-syndrome associated with MBL-deficiency with the genotype LXA/HYD, IgG-deficiency, recurrent uro-genital infections, cutaneous vasculitis, G20.210A prothrombin mutation, deep venous thrombosis, and pulmonary embolism. MBL-deficiency in combination with IgG deficiency might have favored the development of recurrent uro-genital infections. Immunodeficiency might be also involved in the pathogenesis of cutaneous vasculitis. Deep venous thrombosis and pulmonary embolism were attributed to the genetically determined prothrombotic state and intake of oral contraceptives.

La asociación de síndrome de Down con deficiencia de lectina de unión a manosa, infecciones recurrentes y vasculitis no ha sido informada. Presentamos una mujer de 30 años de edad con síndrome de Down asociado a deficiencia de lectina de unión a mañosa, con el genotipo LXA/HYD, deficiencia de IgG, infecciones urogenitales recurrentes, vasculitis cutánea, mutación de protrombina G20.210A, trombosis venosa profunda y embolia pulmonar. La deficiencia de lectina de unión a manosa combinada con la deficiencia de IgG puede haber favorecido las infecciones urogenitales recurrentes. La inmunodeficiencia puede también tener relación con la patogenia de la vasculitis cutánea. La trombosis venosa profunda y la embolia pulmonar pueden deberse al estado protrombótico derivado de la mutación de protrombina y el uso de contraceptivos orales.

Evaluation of humoral immune function in patients with bronchiectasis

Bronchiectasis is a chronic debilitating condition characterized by abnormal dilated thickwalled bronchi. To investigate humoral immune function in bronchiectatic patients, this study was performed. Forty patients with established diagnosis of bronchiectasis, who were referred from two tertiary care pulmonology centers in Tehran, were investigated in this study. Immunoglobulin isotypes concentrations and IgG-subclasses were measured by nephelometry and enzymelinked immunosorbent assay [ELISA] methods, respectively. All patients received unconjugated pneumococcal vaccine, and blood samples were taken before and 21 days after vaccination. Specific antibodies against whole pneumococcal antigens were measured using the ELISA method. Fifteen [37.5%] out of 40 patients were diagnosed to have defects in antibody mediated immunity including 5 [12.5%] patients with immunoglobulin class deficiency [2 with common variable immunodeficiency and 3 with IgA deficiency], 3 [7.5%] with IgG subclass deficiency and 7 [17.5%] patients had Specific antibody deficiency [SAD] against polysaccharide antigen despite normal levels of serum immunoglobulins and IgG subclasses. Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals

Importância da imunoglobulina G subclasse 2 e de anticorpos específicos na otite média aguda recorrente na infância: uma revisão sistemática / Importance of immunoglobulin G subclass 2 and especific antibodies in recurrent acute otitis media in childhood: a systematic review

Descrever a importância imunológica da IgG2 e de anticorpos específicos nas otites médias agudas de repetição na infância, através de revisão sistemática de trabalhos de literatura. Foi realizada uma busca eletrônica nas bases de dados MEDLINE, LILACS e Cochrane, no período de 1980 a 2005, no Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro...

Relate the immunological status of IgG2 and specific antibodies in recurrent acute otitis media in childhood, throut academic review of medical papers. An eletronic research was performed on MEDLINE, LILACS, and Cochrane databases, in a period between 1980 and 2005 at Clementino Fraga Filho University Hospital from Rio de Janeiro Federal University...

Chronic diarrhea and abnormal serum immunoglobulin levels: a case report.

A 15-year-old Thai boy with multiple episodes of chronic diarrhea caused by giardiasis with hypogammaglobulin M and IgG4 subclass deficiency (but normal antibody response to rabies vaccine) is reported. Immune status follow-up is necessary for a definite diagnosis and proper management.

Chronic rhinosinusitis and recurrent nasal polyps in two children with IgG subclass deficiency and review of the literature.

Chronic rhinosinusitis (CRS) is a chronic inflammatory disorder of mucosa of the nose and the paranasal sinuses. Two major forms of CRS can be differentiated; CRS with nasal polyps (CRSwNP) and CRS without nasal polyps (CRSsNP). The pathophysiology and etiology of nasal polyps (NPs) are partly understood. IgG subclass deficiency was shown to be associated with an increased susceptibility to infections. However the association between NPs and IgG subclass deficiency has never been reported. OBJECTIVES: To report two cases of recalcitrant CRS and recurrent NPs with IgG subclass deficiency. CASE REPORT: Two children (6 and 8 year-old boys) were referred to the Pediatric Allergy/Immunology Clinic, Siriraj Hospital due to a prolonged history of CRS and recurrent NPs. Both of them were treated with aggressive medical (topical and systemic corticosteroids, antibiotics, leukotriene antagonist, nasal irrigation) as well as surgical therapy, without significant improvement. Immunologic investigation in both patients showed that IgG, IgA, and IgM level were normal. IgG subclasses level in patient No. 1 were IgG1 1,235 (280-1120) mg/dl (79%), IgG2 235 (30-630) mg/dl (23.5%), IgG3 27.3 (40-250) mg/dl (1.74%), and IgG4 92.4 (11-620) mg/dl (5.9%). IgG subclasses level in patient No. 2 were IgG1 1,139 (280-1120) mg/dl (82.5%), IgG2 170 (30-630) mg/dl (12.3%), IgG3 5.6 (40-250) mg/dl (0.4%), IgG4 65.7 (11-620) mg/dl (4.8%). The diagnosis of CRS and recurrent NPs with IgG3 subclass deficiency in the first patient and IgG2/IgG3 subclass deficiency in the second patient were made. Patient No. 1 was given monthly IVIG therapy for the total of 7 courses and medications were gradually tapered. Currently, the patient is doing well after the cessation of IVIG therapy for 3 months. Patient No. 2 denied the IVIG treatment and was lost to follow up. CONCLUSION: We reported two cases of recalcitrant CRS and recurrent NPs in children. Immunologic work up revealed IgG subclass deficiency. The treatment with monthly IVIG improved CRS and NPs in treated patient which brought up the possibility of association between NPs and IgG subclass deficiency. Further study on the direct role of IVIG in NPs will be needed in the future.

IgA deficiency: clinical correlates with IgG subclass and mannan-binding lectin deficiencies

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.

A case of IgG subclass deficiency with the initial presentation of transient hypogammaimmuno-globulinemia of infancy and a review of IgG subclass deficiencies.

Primary immunodeficiency diseases are not common in children. The possibility of an immunological defect should be considered in any individual with repeated infections. A definite diagnosis for immodeficiency is sometimes difficult to achieve because of overlapping clinical manifestations. Immunoglobulin subclass deficiency is an immunological deficiency disease with which, one or more IgG subclasses are deficient. T cell immunity is normal. Patients may develop recurrent bacterial and respiratory infections or could remain asymptomatic. OBJECTIVE: The authors report a case of immunoglobulin G subclass deficiency presenting initially as transient hypogammaglobulinemia of infancy. CASE REPORT: A 2 month-old boy presented to Siriraj Hospital with a history of chronic protracted diarrhea, disseminated scabies and sepsis. On presentation, he had generalized scaly and maculopapular rash with no palpable lymph nodes. CBC revealed WBC 22,100 cells/cm3 with PMN 42 per cent, lymphocytes 38 per cent, Eosinophils 4 per cent, Basophil 2 per cent and platelets 254,000/cm3. The immunoglobulin levels were as follows: IgG 181 mg/dl, IgA < 6.6 mg/dl, IgM 26.3 mg/dl. Lymphocyte enumerations revealed CD4 of 2,433 cells/cm3 (N 1,460-5,160); CD8 4,682 cells/cm3 (N 650-2,450); CD19 1,588 cell/cm3 (N 500-1,500); CD16 230 cell/cm3 (N 573 +/- 264). The initial diagnosis was X-linked agammaglobulinemia vs common variable immunodeficiency disease. His diarrhea and five courses of sepsis responded well to antibiotics administration and courses of intravenous immunoglobulin (IVIG) replacement. His through IgG became normal at 2 years of age (after 12 months of IVIG). IVIG was stopped and the diagnosis was changed to transient hypogammaglobulinemia of infancy (THI). Nevertheless, during his 4 month follow-up he developed recurrent sinopulmonary infections (i.e, otitis media and pneumonia). Repeated immunoglobulin profile showed IgG 1,200 mg/dl, IgA 135 mg/dl, IgM 26 mg/dl, IgG subclass were IgG, 1,030 mg/dl (N 280-830), IgG2 30 mg/dl (N 40-2,400), IgG3 22 mg/dl (N 6-130), IgG4 3 mg/dl (N 3-120). A diagnosis of IgG2 subclass deficiency presenting early as transient hypogammaglobulinemia of infancy was then made. Treatment with monthly IVIG was reinitiated and the patient is currently doing well. CONCLUSION: The authors present a case of IgG subclass deficiency presenting as transient hypogammaglbulinemia of infancy. Follow-up of the immune profile and clinical manifestation is necessary for a definite diagnosis.

Acquired Immunoglobulin Deficiency after Chemotherapy in Childhood Acute Leukemia Patients / 대한소아혈액종양학회지

PURPOSE: Although an increasing number of children with cancer survive as a result of more intense chemotherapy with advanced supportive care, they may be immunosuppressed because of the anticancer chemotherapy with radiotherapy and are susceptible to severe infections. We surveyed the pattern of immunoglobulin deficiency after chemotherapy in childhood acute leukemia with severe infections. METHODS: We reviewed the medical records and laboratory reports of 11 acute leukemia patients who developed immunoglobulin deficiency after chemotherapy at the Department of Pediatrics, Kyungpook National University Hospital in Daegu, Korea from January 1995 to September 2003. RESULTS: Among 11 acute leukemia patients, the median time interval from the diagnosis of leukemia to that of immunoglobulin deficiency was 21 (2~44) months. At the diagnosis of leukemia, 10 patients had normal levels of IgG, IgA, and IgM. At the diagnosis of immunoglobulin deficiency, 9 patients had low level of IgG (median, 339 mg/dL), IgA (median, 14.1 mg/dL), IgM (median, 24.4 mg/dL), and the other 2 patients had isolated IgG deficiency and isolated IgA deficiency, respectively. They were treated with antibiotics and high dose intravenous immunoglobulin G. CONCLUSION: Acquired immunoglobulin deficiency is one of the causes of frequent and serious infections which develops in childhood acute leukemia with chemotherapy. We suggest that periodic monitoring of immunoglobulin levels is important for early detection and treatment.

Síndrome de hiper IgE y deficiencia de IgG1 e IgM / Hyper-IgE syndrome associated with IgG1 and IgM deficiency

Introducción. El síndrome de hiperinmunoglobulina E (hiper IgE) es una inmunodeficiencia congénita autosómica dominante con penetrancia incompleta, caracterizada por dermatitis crónica grave, infecciones frecuentes, formación de abscesos, valores séricos de IgE elevados (>2000 UI/mL) y eosinofilia periférica. Se puede asociar con múltiples alteraciones de la respuesta inmune. Se presenta el caso de un paciente con la rara asociación no reportada de hiper IgE y deficiencia de IgG1. Caso clínico. Masculino de 6 años de edad con dermatosis crónica, infecciones de vías aéreas de repetición, presencia de abscesos fríos en miembros inferiores, con mala respuesta a antihistamínicos, queratolíticos y lubricantes. Por mala evolución clínica y sospecha de inmunodeficiencia fue enviado al Hospital Infantil de México Federico Gómez. A su ingreso presentaba dermatosis generalizada activa y sinusitis maxilar bilateral, manejándose con cefaclor, antihistamínicos, y cuidados de piel. Un mes después se complicó con varicela por lo que se hospitalizó, quedando con cicatrices queloides. Se le diagnosticó hiper IgE por evolución clínica y niveles de IgE de 11 000 UI/mL, asociado con deficiencia de IgG1 y niveles persistentemente subnormales de IgM. Se le inició tratamiento con trimetoprim y sulfametoxazol a 5 mg/kg/día durante 3 meses con buena respuesta clínica; posteriormente presentó remisión de su enfermedad. Conclusión. Se encontró el síndrome de hiper IgE asociado a una deficiencia de IgG1 que lo hace más susceptible a infecciones por diferentes patógenos y niveles subnormales de IgM.

Quiantification and characterization of protein loss in continuous ambulatory peritoneal dialysis / Cuantificación y caracterización de las pérdidas proteicas en diálisis peritoneal continua ambulatoria

Antecedentes/Objetivos. La pérdida de proteínas en el dializado ha sido involucrado en la etiología de la hipoalbuminemia y/o desnutrición en DPCA. No hay información acerca del transporte peritoneal de proteínas en pacientes en DPCA en México ni en Latinoamérica. Por lo tanto, el objetivo de este estudio fue cuantificar y caracterizar el transporte peritoneal de albúmina (Alb), IgG, IgA, e IgM en pacientes en DPCA. Además, algunos factores asociados a las pérdidas proteicas fueron investigados. Métodos. Treinta y siete pacientes en DPCA fueron seleccionados al azar y sometidos a una prueba de equilibrio peritoneal (PEP). Durante dicha PEP, se midieron las concentraciones de Alb, IgG, IgA, e IgM en el suero y el dializado. Resultados. La IgM no se detectó en el dializado por el método nefelométrico usado. Un claro patrón de incremento continuo y gradual en las pérdidas peritoneales de Alb, IgG e IgA se observó durante toda la PEP. Durante la PEP, los pacientes con los tipos de transporte peritoneal más rápidos tuvieron las concentraciones más bajas de Alb (pero no de inmunoglobulinas) y las pérdidas dialíticas más grandes de todas las proteínas evaluadas. El factor predictor más importante de las pérdidas de Alb, IgG, e IgA fue la tasa de transporte peritoneal, tanto en el análisis univariado como en el multivariado. Conclusiones. Las pérdidas peritoneales de Alb, IgG, e IgA en pacientes mexicanos en DPCA son principalmente dependientes de la tasa de transporte peritoneal y del tiempo de permanencia de la solución de diálisis en la cavidad peritoneal.

IgG2 immunodeficiency: association to pediatric patients with bacterial meningoencephalitis

An IgG subclass deficiency is often associated with bacterial infections. We studied four pediatric patients suffering from meningoencephalitis, two of them due to Streptococcus pneumoniae and two due to Haemophilus influenzae type b. Simultaneous diagnostic serum and cerebrospinal fluid samples were taken during income. The four subclasses of IgG and albumin were quantified in both biologic fluids by radial immunodiffusion. Very low levels of seric IgG2 with non detectable cerebrospinal fluid IgG2 were found in the patients. No intrathecal IgG subclass synthesis was found in two patients. One patient with S. pneumoniae had IgG3 intrathecal synthesis. Intrathecal IgG1, IgG3 and IgG4 synthesis was found in one patient suffering from H. influenzae according with reibergrams. Substitutive therapy with intravenous gammaglobulin was given to the patients as part of the treatment.

IgG subclasses in wheezing infants.

The wheezing infant is a common but difficult patient to approach diagnostically. The prevalence of IgG subclass antibody deficiency in wheezing infants is still controversial. We studied serum concentration of IgG subclasses in 38 wheezing infants (aged 6-24 months who had not received systemic steroids before investigation) and in 30 healthy age matched control (aged 6-24 months). The prevalence of one or more IgG subclass deficiency was 31.6% in wheezing infants and 26.7% in controls. There was no significant difference in prevalence of IgG subclass deficiency between patients and controls (p > 0.05). The mean concentration of IgG subclasses in patients were compared with controls. There was no significant difference in mean serum concentration of IgG1, G2 and G3 subclasses. But there was a trend towards higher concentrations of IgG4 in wheezing infants and this difference for IgG4 was significant (p < 0.01). However, IgG subclass deficiency was found in 25% and 36.4% of wheezing infants who had experienced from two to four and five or more wheezing episodes in two years, respectively (p > 0.05). These findings suggest that wheezing in infancy is not associated with IgG subclass deficiency and in wheezing infants low IgG subclass levels do not increase the frequency of wheezing.

Avaliaçäo imunológica em crianças com amigdalite aguda de repetiçäo / Humoral immunodeficiency in children with acute recurrent tonsillitis

Sessenta crianças com idade entre dois e doze anos - vinte com quadro de amigdalite aguda de repetiçäo e hipertrofia amigdaliana (Grupo I), vinte com amigdalite aguda de repetiçäo sem hipertrofia amigdaliana (Grupo II) e vinte com diagnóstico de hipertrofia amigdaliana sem amigdalite de repetiçäo (Grupo III) - foram submetidas à dosagem sérica de imunoglobulinas IgA, IgM e IgG e subclasses de IgG, para investigaçäo de imunodeficiência humoral. Os resultados evidenciaram apenas uma criança com diminuiçäo dos níveis séricos de IgA no Grupo I, sendo que as outras imunoglobulinas apresentaram-se normais em todos os grupos e faixas etárias