Advances in the Diagnosis and Treatment of Primary Immunodeficiency Diseases

Buckley, Rebecca H.

doi:10.1001/archinte.1986.00360140207031

Cited by 10 publications

(1 citation statement)

References 20 publications

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“…Diagnostic considerations (Buckley 1986, Tangsinmankong et al 2001, Bonilla et al 2005 The medical history should be thorough but special attention should be given to types, frequency, and severity of infections; age of onset, presence of atopic disease, systemic transfusion reactions, and risk factors or evidence for secondary immunodeficiency. Family history should address infections or early deaths in other family members, as well as parental consanguinity.…”

Section: X-linked Lymphoproliferative Syndrome (Xlp)mentioning

confidence: 99%

Current Perspectives on Primary Immunodeficiency Diseases

Kumar

Teuber

Gershwin

2006

Journal of Immunology Research

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Since the original description of X-linked agammaglobulinemia in 1952, the number of independent primary immunodeficiency diseases (PIDs) has expanded to more than 100 entities. By definition, a PID is a genetically determined disorder resulting in enhanced susceptibility to infectious disease. Despite the heritable nature of these diseases, some PIDs are clinically manifested only after prerequisite environmental exposures but they often have associated malignant, allergic, or autoimmune manifestations. PIDs must be distinguished from secondary or acquired immunodeficiencies, which are far more common. In this review, we will place these immunodeficiencies in the context of both clinical and laboratory presentations as well as highlight the known genetic basis.

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Section: X-linked Lymphoproliferative Syndrome (Xlp)mentioning

confidence: 99%

Current Perspectives on Primary Immunodeficiency Diseases

Kumar

Teuber

Gershwin

2006

Journal of Immunology Research

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Clinical uses of intravenous immune globulin: Immunoglobulin replacement therapy and treatment of autoimmune cytopenias

Sorensen

Kallick

1988

J of Clinical Apheresis

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Intravenous immune globulin (IVIG) is indicated for IgG replacement in antibody deficiency syndromes and as immunoregulatory therapy in some autoimmune diseases. Two case histories illustrate both aspects of IVIG therapy. 1. Patient 1 is a 24-year-old male with agammaglobulinemia. He was successively treated with monthly IMIG, paternal plasma, and then over the last 5 years, IVIG. On IgG doses of 250 mg/kg q/4 weeks, IgG trough levels remained below 200 mg IgG/dl. IgG half-life was reduced. Although asymptomatic for prolonged periods of time, he eventually developed clinically evident inflammatory bowel disease. Optimal IVIG replacement therapy requires normal IgG half-life and adequate IgG trough levels. 2. Patient 2 is a 12-year-old girl with autoimmune neutropenia, recurrent skin infections, and ileitis unresponsive to antibiotics and to steroid therapy. IVIG at a dose of 3,000 mg/IgG/kg over 3 days significantly increased her neutrophil count. Subsequently, she has required 1,000 mg/kg of IVIG q/4 weeks for maintenance. Antineutrophil autoantibodies have persisted. There is synergism of IVIG with high doses of corticosteroids. The mechanism of action of IVIG seems to involve a blockage of the RES system. IVIG therapy is safe even at high doses for most patients. However, anaphylactic reactions have been observed in IgA-deficient patients with IgE anti-IgA antibodies. The full spectrum of therapeutic applications of IVIG is still being explored. For some patients self-infusion of IVIG at home is an appealing treatment alternative.

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