Astrovirus encephalitis in boy with X-linked
agammaglobulinemia.
A girl aged 65 months with
agammaglobulinemia was infected with iVDPV2 and developed AFP in February 2016.
indications: - replacement therapy in primary immunodeficiency syndromes, - congenital
agammaglobulinemia and hypogammaglobulinemia, - replacement therapy in myeloma or chronic lymphatic leukemia with severe secondary hypogammaglobulinaemia and recurrent infections.
Bruton's tyrosine kinase: From X-linked
agammaglobulinemia toward targeted therapy for B-cell malignancies.
X-linked
agammaglobulinemia (XLA) is a primary immunodeficiency with more than 600 mutations in Bruton tyrosine kinase (Bkt) gene which are responsible for early-onset
agammaglobulinemia and repeated infections.
Furthermore, patients with X-linked
agammaglobulinemia (XLA) and common variable immune deficiency (CVID) have been shown to suffer from sensorineural hearing loss (SNHL) (6, 7).
Enteropatia linfocitica Atrofia vellositaria Infeccion por Helicobacter pylori Esprue tropical Lesion por AINE Enteropatia autoinmune Linfoma intestinal Linfoma intestinal Parasitosis por Giardia lamblia Parasitosis por Giardia lamblia Enfermedad de Crohn Intolerancias alimentarias en ninos Enteropatia del sida (proteinas de la leche de vaca) Sobrecrecimiento bacteriano Enfermedad de injerto contra huesped Enteritis eosinofilica Isquemia cronica del intestino delgado Enfermedad de Whipple Deficit de IgA, especialmente si se asocia Hipo o
agammaglobulinemia a sobrecrecimiento bacteriano Amiloidosis Antagonistas de receptores de Linfangiectasia intestinal angiotensina II (olmesartan) Enteritis por radiacion Hipertiroidismo Gastroenteritis infecciosa Antiinflamatorios no esteroideos
In addition, autoimmunity findings may also be observed during the course of the disease in common variable immune deficiency (CVID), hyper IgM syndrome, X-linked
agammaglobulinemia, selective IgA deficiency, CKGD, severe combined immune deficiencies, WAS, and complement deficiencies.
Mycobacterial, fungal and other opportunistic infections force the clinician to rule out a large list of conditions associated with secondary immunodeficiency as infectious agents (HIV, Herpesvirus, HTLV), drugs (steroids, immunosuppressants, biologics, chemotherapy), metabolic diseases (diabetes, renal failure, cirrhosis), malignancies (leukemia, lymphomas and solid tumors) and environmental conditions (radiation, heavy metals) (2) but even adult patients can have late onset primary genetic immunodeficiency disorders as chronic granulomatous disease, X-linked
agammaglobulinemia, interleukin-12 receptor deficiency or interferongamma (IFN-?
The most common immune deficiency diseases causing recurrent pulmonary infections and bronchiectasis are common variable immune deficiency (CVID) and X-linked
agammaglobulinemia (XLA).
Ogden Bruton's observation of
agammaglobulinemia in a male child suffering from recurrent otitis media, lacking tonsillar tissue, led to the ground-breaking diagnosis of XLA in 1952.
Gastrointestinal pathology in patients with common variable immunodeficiency and X-linked
agammaglobulinemia.
agammaglobulinemia - a rare immunological disorder characterized by the virtual absence of gamma globulin in the blood and consequent susceptibility to infection