Social support and its association with depression, gender and socioeconomic indicators in individuals with spinal cord injury in Iran.

STUDY DESIGN: Descriptive cross-sectional study. OBJECTIVES: To investigate the relationship between perceived social support and depression and to evaluate the role of family, friends and other caregivers in the perception of social support in Iranian individuals with spinal cord injury (SCI). SETTING: Brain and Spinal Cord Injury Research Center, Tehran University of Medical Sciences, Tehran, Iran. METHODS: Social support was evaluated using the Multidimensional Scale of Perceived Social Support questionnaire, which gauges perceptions of support from family, friends and 'important persons'. The presence and severity of depression were assessed with the Beck Depression Inventory (BDI-II-PERSIAN)-a 21-item multiple-choice questionnaire. RESULTS: A total of 140 individuals with SCI were enrolled in the study. The average age of the participants was 29.4±7.9 years; the mean duration of injury was 46.3±46.5 months and most patients were male (72%). Social support and all subscales of social support were numerically greater in males; however, this difference was not statistically significant. The subcategory of friends' support in men was 17.9±7.9 compared to 14.6±8.0 in women (P=0.04). The self-reported social support score (r=-0.387, P<0.001) and subscales of social support, including family (r=-0.174, P=0.045), friends (r=-0.356, P<0.001) and important persons (r=-0.373, P<0.001), were all negatively correlated with depression. CONCLUSION: Higher self-reported perception of social support appears to be associated with lower levels of depression in individuals with SCI. SCI care providers should consider the relationship between social support and depression in their continuing care.

IgG anti-IgA antibodies in paediatric antibody-deficient patients receiving intravenous immunoglobulin

BACKGROUND: Immunoglobulin replacement therapy is an effective route of management for both infections and non-infectious complications in predominantly antibody deficiency (PAD). Trace levels of IgA (ranged from 0.4 to 2500 mg/ml), which exist in all immunoglobulin products, could lead to an increased susceptibility for adverse reactions in PAD patients. Furthermore, the exact mechanism which stimulates the anti-IgA antibody production in PAD is still unknown. The aim of this study was to evaluate IgG anti-IgA antibodies in PAD patients receiving intravenous immunoglobulin (IVIg) and its predisposing factors. METHODS: Available patients with confirmed diagnosis of PAD, who underwent regular IVIg replacement therapy in our centre, were enrolled in the study. Control group included 24 healthy individuals as the negative control and eight symptomatic patients with IgA deficiency as the positive control groups. IgG anti-IgA antibodies level was measured by the ELISA method. RESULTS: A significant difference was observed between Anti-IgA level of common variable immunodeficiency (CVID) and other PAD groups (p = 0.02). Moreover, six CVID patients were seropositive for the IgG anti-IgA antibody, with higher susceptibility to the adverse reactions (p < 0.001). IgG anti-IgA level has a negative relationship with serum IgA level (r = −0.06) and IVIg treatment duration (r = −0.006). CONCLUSION: Our data suggested that there was a significant association between anti-IgA antibody presence and the adverse reactions, especially in CVID patients with higher susceptibility to produce this constitutional antibody

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Autoimmunity in patients with selective IgA deficiency.

BACKGROUND AND OBJECTIVE: Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deticiency. Patients with SIgAD have a greater risk of concomitant autoimmune disorders than healthy individuals. The exact mechanism underlying the relationship between autoimmunity and SIgAD is not fully understood. The aim of this study was to evaluate potential associations between autoimmunity and specific clinical or immunological findings in patients with SIgAD. METHODS: The study population comprised 57 symptomatic patients (65% males) with confirmed SIgAD who were referred to our center. Demographic data and history of autoimmunity were recorded both for patients and for their relatives. Comprehensive clinical and laboratory examinations were performed to investigate autoimmune complications in all the patients. RESULTS: Autoimmune disorders were documented in 17 cases (29.8%; 9 males and 8 females). The most common manifestations were thyroiditis, vitiligo, and hemolytic anemia (3 cases each). Ten patients (17.5%) had a family history of autoimmunity. Significant associations were detected between autoimmunity and increased duration of follow-up (P = .003), serum level of IgM (P = .01), regulatory T-cell count (P = .03), and class-switched memory B-cell count (P = .01). Four cases of autoimmune SIgAD (23.5%) progressed to common variable immunodeficiency during the follow-up period (P = .006). CONCLUSIONS: Autoimmune disorders, autoimmune cytopenia, and Ig subclass deficiency can lead to severe clinical manifestations in patients with SIgAD. Therefore, immunologists and pediatricians should be aware of these conditions.

Mortality and morbidity in patients with X-linked agammaglobulinaemia

BACKGROUND: X-linked agammaglobulinaemia (XLA) is a genetic disorder characterised by a defect in the generation of mature B cells, lack of antibodies production, and susceptibility to recurrent bacterial infections. Understanding of the risk factors responsible for morbidity and mortality in these patients can help in a better management of this disorder. However, there is a lack of specific studies in the literature regarding the morbidity and mortality of XLA patients. This study is designed to evaluate morbidities and mortality and survival rates in Iranian patients with XLA diagnosis during the past 20 years. METHODS: We have registered the clinical data of the XLA patients and followed them up until 2010. At the time of diagnosis, a four-page questionnaire including complete medical information was filled out for all patients. Follow-up information was gathered either by reviewing the patients' hospital records or regularly visiting the patients. RESULTS: Among 41 patients, 26.8% died during the follow up period. All of the complications before the initiation of treatment such as pneumonia, otitis media and diarrhoea were reduced after immunoglobulin replacement, except sinusitis and conjunctivitis. There were significant associations between some immunological and clinical characteristics such as lymphocyte subsets, consanguinity marriage and mortality. CONCLUSION: Despite recent advances in the treatment of XLA, these patients still suffer from severe complications. Associations between poor prognosis and clinical and some immunological characteristics of the patients may help physicians to select poor prognoses patients at higher risk of mortality to develop prevention strategies for them

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IgG anti-IgA antibodies in paediatric antibody-deficient patients receiving intravenous immunoglobulin.

BACKGROUND: Immunoglobulin replacement therapy is an effective route of management for both infections and non-infectious complications in predominantly antibody deficiency (PAD). Trace levels of IgA (ranged from 0.4 to 2500 mg/ml), which exist in all immunoglobulin products, could lead to an increased susceptibility for adverse reactions in PAD patients. Furthermore, the exact mechanism which stimulates the anti-IgA antibody production in PAD is still unknown. The aim of this study was to evaluate IgG anti-IgA antibodies in PAD patients receiving intravenous immunoglobulin (IVIg) and its predisposing factors. METHODS: Available patients with confirmed diagnosis of PAD, who underwent regular IVIg replacement therapy in our centre, were enrolled in the study. Control group included 24 healthy individuals as the negative control and eight symptomatic patients with IgA deficiency as the positive control groups. IgG anti-IgA antibodies level was measured by the ELISA method. RESULTS: A significant difference was observed between Anti-IgA level of common variable immunodeficiency (CVID) and other PAD groups (p=0.02). Moreover, six CVID patients were seropositive for the IgG anti-IgA antibody, with higher susceptibility to the adverse reactions (p<0.001). IgG anti-IgA level has a negative relationship with serum IgA level (r=-0.06) and IVIg treatment duration (r=-0.006). CONCLUSION: Our data suggested that there was a significant association between anti-IgA antibody presence and the adverse reactions, especially in CVID patients with higher susceptibility to produce this constitutional antibody.

Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment.

BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.

Mortality and morbidity in patients with X-linked agammaglobulinaemia.

BACKGROUND: X-linked agammaglobulinaemia (XLA) is a genetic disorder characterised by a defect in the generation of mature B cells, lack of antibodies production, and susceptibility to recurrent bacterial infections. Understanding of the risk factors responsible for morbidity and mortality in these patients can help in a better management of this disorder. However, there is a lack of specific studies in the literature regarding the morbidity and mortality of XLA patients. This study is designed to evaluate morbidities and mortality and survival rates in Iranian patients with XLA diagnosis during the past 20 years. METHODS: We have registered the clinical data of the XLA patients and followed them up until 2010. At the time of diagnosis, a four-page questionnaire including complete medical information was filled out for all patients. Follow-up information was gathered either by reviewing the patients' hospital records or regularly visiting the patients. RESULTS: Among 41 patients, 26.8% died during the follow up period. All of the complications before the initiation of treatment such as pneumonia, otitis media and diarrhoea were reduced after immunoglobulin replacement, except sinusitis and conjunctivitis. There were significant associations between some immunological and clinical characteristics such as lymphocyte subsets, consanguinity marriage and mortality. CONCLUSION: Despite recent advances in the treatment of XLA, these patients still suffer from severe complications. Associations between poor prognosis and clinical and some immunological characteristics of the patients may help physicians to select poor prognoses patients at higher risk of mortality to develop prevention strategies for them.

Autoimmunity in patients with selective IgA deficiency

Background and Objective: Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency. Patients with SIgAD have a greater risk of concomitant autoimmune disorders than healthy individuals. The exact mechanism underlying the relationship between autoimmunity and SIgAD is not fully understood. The aim of this study was to evaluate potential associations between autoimmunity and specific clinical or immunological findings in patients with SIgAD. Methods: The study population comprised 57 symptomatic patients (65% males) with confirmed SIgAD who were referred to our center. Demographic data and history of autoimmunity were recorded both for patients and for their relatives. Comprehensive clinical and laboratory examinations were performed to investigate autoimmune complications in all the patients. Results: Autoimmune disorders were documented in 17 cases (29.8%; 9 males and 8 females). The most common manifestations were thyroiditis, vitiligo, and hemolytic anemia (3 cases each). Ten patients (17.5%) had a family history of autoimmunity. Significant associations were detected between autoimmunity and increased duration of follow-up (P=.003), serum level of IgM (P=.01), regulatory T-cell count (P=.03), and class-switched memory B-cell count (P=.01). Four cases of autoimmune SIgAD (23.5%) progressed to common variable immunodeficiency during the follow-up period (P=.006). Conclusions: Autoimmune disorders, autoimmune cytopenia, and Ig subclass deficiency can lead to severe clinical manifestations in patients with SIgAD. Therefore, immunologists and pediatricians should be aware of these conditions (AU)

Fundamento y objetivo: La deficiencia selectiva de IgA (SIGAD) es la inmunodeficiencia primaria de anticuerpos más frecuente. Se conoce que los pacientes con SIGAD tienen un mayor riesgo de padecer trastornos autoinmunes asociados, en comparación con la población normal. Sin embargo, no se encuentra aún esclarecido el mecanismo exacto de la relación entre la autoinmunidad y el SIGAD. El objetivo de este estudio ha sido el evaluar las asociaciones entre la autoinmunidad y los hallazgos clínicos o inmunológicos en los pacientes con SIGAD. Métodos: Han sido estudiados cincuenta y siete pacientes sintomáticos (65% varones), con diagnóstico confirmado de SIGAD. Se registraron sus datos demográficos y los antecedentes, personales y familiares, de enfermedades autoinmunes, y se realizaron múltiples exámenes clínicos y de laboratorio. Resultados: Se documentaron enfermedades autoinmunes en 17 casos (29,8%; 9 hombres y 8 mujeres), siendo la tiroiditis, el vitíligo y la anemia hemolítica, las manifestaciones autoinmunes más comunes, con 3 casos para cada trastorno. Diez pacientes (17,5%) contaban con antecedentes familiares de autoinmunidad. Se encontraron asociaciones significativas con el desarrollo de enfermedades autoinmunes en estos pacientes con SIGAD: un prolongado período de seguimiento (p=0,003), el nivel sérico de IgM (p=0,01), la cuantificación de las linfocitos T reguladores (p=0,03) y el cambio de isotipo de los linfocitos B de memoria (p=0,01). Cuatro casos de SIGAD, con enfermedad autoinmune asociada (23,5%), evolucionaron hacia una inmunodeficiencia variable común, durante el período de seguimiento (p=0,006). Conclusiones: Los pacientes con SIGAD pueden desarrollar enfermedades autoinmunes que en ocasiones se manifiestan con formas clínicas graves y deben ser objeto de estudio y de seguimiento por parte del inmunólogo y del pediatra (AU)

Primary antibody deficiency in a tertiary referral hospital: a 30-year experiment

Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. Objectives: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. Materials and Methods: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children’s Medical Center, Tehran, Iran. Results: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. Conclusions: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively (AU)

Antecedentes: Las inmunodeficiencias humorales primarias (PAD) es el grupo más frecuente de inmunodeficiencias primarias (IDP), y engloba un amplio espectro de características clínicas, que van desde los pacientes con infecciones graves y recurrentes a los casos asintomáticos. Objetivos: El presente estudio se realizó para evaluar y comparar los datos demográficos y clínicos de los tipos más comunes de PAD. Materiales y Métodos: Se revisaron retrospectivamente, las historias clínicas de todos los pacientes con PAD con un diagnóstico confirmado de: inmunodeficiencia variable común (CVID), síndrome de hiper IgM (HIgM), deficiencia selectiva de IgA (SIgAD),y de agammaglobulinemia ligada al cromosoma X (XLA), que fueron diagnosticados durante los últimos 30 años, en el Centro Médico de Niños, Teherán, Irán. Resultados: Se incluyeron en este estudio un total de 280 casos de PAD, englobando 125 pacientes con CVID, 32 HIgM, 63 SIgAD, y 60 pacientes con XLA. La mediana (rango) de edad al inicio de la enfermedad en la CVID, HIgM, SIgAD y XLA fue: 2 (0-46), 0,91 (0-9), 1 (0-26) y 1 (0-10) años, respectivamente. Las infecciones gastrointestinales fueron más frecuentes en los pacientes con CVID, mientras que las infecciones del sistema nervioso central lo fueron en la XLA. Las complicaciones autoinmunes fueron más prevalentes en los pacientes con HIgM, los tumores malignos en las CVID y las enfermedades alérgicas en las SIgAD. La tasa de mortalidad de CVID, HIgM y XLA fue 27,2%, 28,1% y 25%, respectivamente. No hubo mortalidad en el grupo de pacientes con SIgAD. Conclusiones: Los pacientes con SIgAD tuvieron el mejor pronóstico. Aunque todos los pacientes con PAD deben ser controlados estrechamente para evitar las complicaciones infecciosas, se debe prestar especial atención a la aparición de enfermedades malignas y autoinmunes en los pacientes con CVID y HIgM, respectivamente (AU)