BACKGROUND Living with a chronic disease, such as primary antibody deficiency, will often have consequences for quality of life. Previous quality-of-life studies in primary antibody deficiency patients have been limited to different treatment methods. We wanted to study how adults with primary antibody deficiencies manage their conditions and to identify factors that are conducive to coping, go...

Immunoglobulins (IgG) as replacement therapy in primary antibody deficiencies can be given as intramuscular injections, or as intravenous or subcutaneous infusions. Our aims were to obtain information on the frequency of adverse systemic reactions during subcutaneous therapy, the occurrence and intensity of tissue reactions at the infusion sites, and serum IgG changes. Furthermore, we compared ...

The primary antibody deficiency syndromes are a rare group of immunodeficiencies where diagnostic delay remains common due to limited awareness of the existence and heterogeneity of their presenting features. Referral for specialist assessment leads to earlier diagnosis and appropriate therapy to prevent or limit structural organ and tissue damage. Greater education of healthcare professionals ...

BACKGROUND AND AIM Portal hypertension has been reported in association with acquired and primary immune deficiencies without a comprehensive description of associated spleno-portal axis abnormalities. Pathological mechanisms are poorly defined. METHODS Observational, single centre study with the aim of assessing the prevalence of spleno-portal axis abnormalities in an unselected cohort of 12...

types of primary immune deficiencies in human subjects. Often symptoms do not appear until the latter part of the first year of life, as passively acquired IgG from the mother decreases to below protective levels. As with the T-cell immune deficiencies, the spectrum of antibody deficiencies is broad, ranging from the most severe type of antibody deficiency with totally absent B cells and serum ...

common variable immunodeficiency (cvid) is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. here a 16-year-old boy is presented who was referred to our center with cough, dyspnea,cyanosis, and history of recurrent pneumonia. the diagnosis of cvid was made according to reduction all serum immunoglobulin levels...