Uncommon conundrum in common variable immunodeficiency.
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Adrenal Cortex Hormones↗
Biomedical subjects
Robert Hostoffer
Publications and source records attributed to Robert Hostoffer.
Health-related quality of life of children with primary immunodeficiency disease: a comparison study.
BACKGROUND: Many symptoms of primary immunodeficiency (PI) disease can be successfully managed with intravenous immunoglobulin infusion. Although survival rates and prognosis have greatly improved, children with PI disease are still at risk for physical, social, and psychological problems owing to their chronic health condition. However, to our knowledge, there are no empirical data concerning health-related quality of life (HRQOL) in children with PI disease receiving intravenous immunoglobulin infusion. OBJECTIVE: To compare parental reports of HRQOL of children with PI disease receiving intravenous immunoglobulin infusion with children with juvenile idiopathic arthritis (JIA) and a healthy sample. METHODS: Demographic, illness, and HRQOL data were collected from parents of 4- to 18-year-old children with PI disease (n = 36), children with JIA (n = 36), and healthy children (n = 36). The HRQOL was evaluated using the Child Health Questionnaire-Parent Report version. RESULTS: Compared with children with JIA, children with PI disease were similar in many aspects of their HRQOL. However, parents of children with PI disease reported greater limitations in their personal time, poorer general health of their children, greater limitations in their children's physical functioning and family activities, and less bodily pain than children with JIA. In contrast, children with PI disease scored lower on most HRQOL domains compared with healthy children. CONCLUSION: Children with PI disease experience similar HRQOL to children with JIA and poorer HRQOL than healthy children, indicating potential areas to be addressed by future medical and psychosocial interventions.
Adolescent↗
Quality of life in common variable immunodeficiency requiring intravenous immunoglobulin therapy.
BACKGROUND: There are no studies of patients with primary immunodeficiency states receiving intravenous immunoglobulin (IVIG) therapy that assess health-related quality of life (HRQOL) using a well-standardized and reproducible method. OBJECTIVES: To determine the HRQOL of patients with common variable immunodeficiency (CVID) requiring IVIG therapy, to compare these patients with patients with diabetes mellitus (DM) and congestive heart failure (CHF), to determine the factors that affect HRQOL, and to develop normative data on the HRQOL of these CVID patients, which can be used to follow the effects of future therapies. METHODS: Fifty-eight adults with CVID receiving IVIG therapy completed the Medical Outcomes Study 36-Item Short-Form Health Survey to evaluate their HRQOL and were compared with DM and CHF patients. The impact of demographic, socioeconomic, and disease-related variables and comorbid conditions was examined in the CVID population. RESULTS: Patients with CVID had lower HRQOL scores in all dimensions compared with patients with DM and in 4 of 8 dimensions compared with patients with CHF. Increasing age and female sex were negatively associated with certain aspects of HRQOL. There were no significant effects from other socioeconomic or disease-related variables or comorbid conditions examined. CONCLUSIONS: Patients with CVID receiving IVIG therapy have a significantly worse HRQOL than patients with other chronic illnesses, indicating there is much room for improvement in future therapies for this primary immunodeficiency state. The effects of future therapies can be evaluated by comparison with the normative data developed in this study.
Adult↗
The use of infliximab in a patient with steroid-dependent Churg-Strauss syndrome.
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Letter↗
Simultaneous nonparotid cranial mucosa-associated lymphoid tissue lymphoma and common variable immunodeficiency.
Common variable immunodeficiency (CVID) is a condition characterized by low levels of immunoglobulin (Ig) G and either IgA or IgM in the presence of recurrent infections. This disorder is associated with an increased risk of malignancy. Mucosa-associated lymphoid tissue (MALT) lymphoma is a recently recognized form of non-Hodgkin's lymphoma that is not often present in the head. MALT lymphoma in patients with CVID is rare, and until now, it has not been reported in a cranial location outside of the parotid gland. We report the cases of 2 patients who had CVID and cranial MALT lymphoma outside of the parotid gland, and we describe their successful treatment with chemotherapy.
Antigens, CD20↗
Intrainfusion and postinfusion adverse events related to intravenous immunoglobulin therapy in immunodeficiency states.
The i.v. immunoglobulin (IVIG) therapy is one of the mainstays of treatment for humoral immunodeficiencies, but there is limited knowledge of the adverse reactions associated with this therapy, especially reactions occurring in the postinfusion period. The purpose of this prospective, observational, multicenter study was to identify and quantify the adverse reactions that can occur both during and after IVIG infusions (Gamimune N) in patients with humoral immunodeficiency. Patients with primary immunodeficiencies requiring IVIG therapy were followed over a 6-month period, and data regarding adverse events, particularly the time of onset, duration, and type of reaction associated with IVIG infusions wzas collected via direct observation and patient interviews. Data were obtained during and up to 72 hours after the completion of infusions. Sixty-five patients were recruited and received a total of 447 infusions over a 6-month period. Four hundred fifty-one adverse reactions were noted, with 17% of infusions associated with an intrainfusion reaction and 41% associated with a postinfusion reaction. Most postinfusion reactions occurred within 24 hours of the infusion and consisted mainly of headaches, fatigue, and nausea. Adverse reactions to Gamimune N infusions are common and occur primarily in the postinfusion period. Estimates of the rate of adverse reactions to Gamimune N infusions currently are underestimated because they do not account for postinfusion reactions. In addition, once recognized, effective treatment of postinfusion reactions may improve patient compliance and quality of life.
Adolescent↗