Intravenous immunoglobulin (IVIg) therapy may be considered medically necessary for the following indications.  Immunodeficiency States—patients with Primary Immunodeficiencies, including o Congenital agammaglobulinemia o Hypogammaglobulinemia o Common variable immunodeficiency o Severe combined immunodeficiency (SCID) o Wiskott-Aldrich syndrome o X-linked agammaglobulinemia (Bruton agammaglob...

X-linked agammaglobulinemia (XLA), or Bruton’s agammaglobulinemia, – is a primary immunodeficiency, caused by defects in the BTK gene encoding tyrosine kinase. The lead to arrest of B-lymphocyte development and, as result, agammaglobulinemia. disease manifests with recurrent infections starting infancy. gold standard XLA treatment intravenous subcutaneous immunoglobulin substitution proved effe...

conclusions according to the results of these work-ups, xla was diagnosed for the cases. introduction x-linked agammaglobulinemia (xla) is one of the primary humoral immunodeficiencies. it usually presents symptoms of recurrent infections, but in some unusual cases it may present rheumatologic manifestations. case presentation the current paper presents the cases of two boys with arthritis trea...

Bacterial lipopolysaccharides (LPS) derived from a variety of organisms effectively induced C consumption in humans, bovines, and porcines with developmental agammaglobulinemia; birds with experimental agammaglobulinemia; and humans with agammaglobulinemia syndromes. This interaction proceeded even in precolostral piglet sera which contained less than 2.5 x 10(-6) mg/ml gamma globulin, and led ...

Addition of tetanus toxoid to sensitized lymphocytes from normal subjects and patients with "secondary" acquired agammaglobulinemia resulted in an increased incorporation of tritiated uridine into RNA and this increase was sustained for 48 to 72 hours in vitro. In contrast, the quantity of H(3)-uridine incorporated into the RNA of lymphocytes from patients with "primary" acquired agammaglobulin...

Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to ...

As a new disease entity is studied it becomes apparent that certain clinical and laboratory features are particularly helpful to the clinician in the recognition of the disorder. A consideration of the recent literature indicates that recurrent pulmonary disease has assumed this role in the disease syndrome associated with agammaglobulinemia. Recurrent pulmonary infections including bronchitis,...

PCR: polymerase chain reaction TMP-SMX: trimethoprim-sulfamethoxazole XLA: X-linked agammaglobulinemia INTRODUCTION Helicobacter cinaedi is an unusual cause of cellulitis in immunocompromised patients. The organism is fastidious, and blood cultures are often negative, making the diagnosis challenging, especially in those without systemic signs. We report a case of chronic H cinaedi cellulitis i...