Evaluation the FLAIR Sensitivity and DWI Post-inject in Comparison with Delayed Enhancement T1w for Better Detection of Active MS Lesions.

BACKGROUND: Multiple sclerosis (MS) is a chronic, typically progressive and most common autoimmune disease which damaged the central nervous system. According to the reports in 2008, this disorder has affected 2 and 2.5 million people globally. While the reason is not clear, proposed causes for this include immunologic, environmental, infectious and genetic factors, and sexuality. MS can cause many symptoms, including blurred vision, loss of balance, poor coordination, slurred speech, tremors, numbness, extreme fatigue, problems with memory and concentration, paralysis, blindness, and more. There are four distinguished illness fields in MS: relapsing-remitting MS (RRMS), primary-progressive MS (PPMS), secondary-progressive MS (SPMS), and progressive-relapsing. MRI is a great tool to identify the asymptomatic distribution of lesions in space and time. MATERIALS AND METHODS: 32 patients with MS plaques were evaluated by FLAIR and DWI pre- and post-Gadolinium injection compared with 15minutes delay T1w SE. RESULTS: FLAIR post-inject had significantly better detection of the number and signal intensity of active MS lesions. DWI and ADC images detected active plaques different from non-active lesions without contrast. CONCLUSION: The result of this study showed that FLAIR post-inject had the highest sensitivity in detection of active MS lesions due to the CSF signal suppression in FLAIR, thus offering enough TR time recovery in active enhanced plaques.

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.

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)