Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0348321 (Haemophilus)
 15,372 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antibody deficiency syndromes can be quantitative or qualitative. The major categories of antibody deficiency syndrome are: (1) X-linked agammaglobulinemia, involving the maturation arrest in the development of the B cells; (2) transient agammaglobulinemia, which affects both sexes is often associated with defective T helper function for immunoglobulin production; (3) acquired agammaglobulinemia, a heterogeneous disorder caused by a primary B cell defect, absence of B cells, presence of B cells but with an activation defect, failure of helper factor production by T cells or increase in suppressor cells; (4) IgG2 and IgG3 subclass deficiencies, causing significant and recurrent infections and, with IgG2, a significant impairment of the ability to respond to carbohydrate antigens such as Haemophilus influenzae, pneumococcus and meningococcus; (5) qualitative antibody deficiency syndrome in the response to carbohydrate antigens, presenting as recurrent infection and involving the inability of the patient to respond to immunization with polysaccharide antigens such as Haemophilus influenzae type b; (6) antibody deficiency states associated with T cell dysfunction. Impairment of T cell function, which is required for B cell activation, presents often as antibody deficiency syndrome. In these cases, total gamma-globulin level is normal, but the quality of the antibody is very poor.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Antibody deficiency syndromes and novel immunodeficiencies. 304 59

In 1974, an 11-year-old white boy with the X-linked lymphoproliferative syndrome developed hyper-IgM after becoming infected with Epstein-Barr virus. However, he failed to develop normal immune responses against the virus. In December 1981, when red cell aplasia occurred, he was given packed erythrocytes and gammaglobulin. Nine weeks later, acute infectious mononucleosis developed. Concurrently, his T4/T8 helper/suppressor ratio decreased from 2.7 to 0.2, and IgM antibodies to Epstein-Barr virus appeared. Subsequently, circulating B cells became undetectable in his blood, and agammaglobulinemia appeared. Red cell aplasia abated transiently. This patient's course was complicated by Haemophilus influenzae and Mycobacterium tuberculosis pneumonias, and red cell aplasia and agammaglobulinemia have persisted. Epstein-Barr virus acting as a slow virus probably induced the red cell aplasia and agammaglobulinemia because of the aberrant immune responses to Epstein-Barr virus. Immunodeficient responses to Epstein-Barr virus should be sought in other patients with the diseases documented in our patient.
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PMID:Delayed onset of infectious mononucleosis associated with acquired agammaglobulinemia and red cell aplasia. 633 Dec 39

Haemophilus influenzae can be demonstrated as a saprophyte in more than two-thirds of children, and almost as frequently in adults. Noncapsulated strains are more frequent than capsulated type b strains which are found in 5% of the samples. Other capsulated strains are rare. Transmission is made easier with close contact (daycare nurseries, home). Colonization is the result of adherence to nasopharyngeal epithelial cells, although characterized adhesion factors cannot be demonstrated for all strains (pili, adhesins, secretory IgA1 protease). Systemic infection is the result of the invasion of pharyngeal epithelium, made easier by upper respiratory tract infection. There is a risk of meningitis for high level bacteremia (> or = 10(5) CFU/ml). Risk factors are: age (child < 5 years), alteration of reticuloendothelial system, agammaglobulinemia. Anti-Haemophilus type b vaccine prevents nearly all infections, and suppresses or sharply reduces colonization.
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PMID:[Haemophilus influenzae: colonization and infection]. 774 11

Agammaglobulinaemia is the most common of the primary immunodeficiencies. Three major types can be distinguished: X-linked agammaglobulinaemia, early-onset agammaglobulinaemia and late-onset agammaglobulinaemia. In X-linked agammaglobulinaemia, the molecular defect has been elucidated, and genetic counseling, prenatal diagnosis and carrier detection have become important issues. The pathogenesis of early- and late-onset agammaglobulinaemia is heterogeneous and usually not within the B-cell lineage. Patients with agammaglobulinaemia mainly suffer from infections caused by pneumococci or encapsulated Haemophilus influenzae located in the respiratory tract, paranasal sinuses, ears and meninges. Other prominent infections are Campylobacter jejuni bacteraemia and Giardia lamblia infection of the intestine. Among the more rare infections are those caused by Ureaplasma and Mycoplasma hominis. There is quite a number of non-infectious abnormalities which bother agammaglobulinaemic patients, especially those with late-onset agammaglobulinaemia. Of these, gastric carcinoma and intestinal lymphoma in late-onset agammaglobulinaemia and colorectal cancer in X-linked agammaglobulinaemia are the most dramatic. Life-threatening bacterial infections can largely be prevented by immunoglobulin substitution, even at relatively low dosages. However, insufficient immunoglobulin substitution is associated with recurrent airway infection and cumulative damage to the respiratory tract. for adequate substitution, efficacieous and safe intravenous immunoglobulin preparations are available. For selected patients (children, adults with poor venous access, and those experiencing side-effects on intravenous immunoglobulin), 16% immunoglobulin can be given by the subcutaneous route. With optimal substitution and--in the case of infection--adequate antimicrobial treatment, these patients have a good prognosis.
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PMID:Agammaglobulinaemia. 783 40

Although people with bacterial meningitis lack adequate protective antibody against the invading pathogen, most do not have an underlying immunodeficiency. Certain comorbid conditions increase the risk for development of bacterial sepsis and meningitis. In addition, certain congenital complement deficiencies, defects of antibody production, or asplenia may be first recognized by the occurrence of bacterial meningitis, particularly when it occurs in infants or young children. Deficiencies of the terminal components of complement (C5-C9) or properdin have been associated with recurrent or invasive neisserial infections, and asplenia, agammaglobulinemia, and deficiencies of the early components of complement (e.g., C1-C3) are associated with risks of infections caused by Streptococcus pneumoniae, Haemophilus influenzae, and meningococci. The presence of congenital or acquired immunodeficiencies should be considered in persons who present with bacterial meningitis on the basis of the etiology, clinical epidemiology, and presence of other risk factors.
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PMID:Indications for the immunological evaluation of patients with meningitis. 1252 51

Agammaglobulinemia is the most common primary immunodeficiency, with an incidence of approximately 1 in 250,000 males in the United States. These patients are at risk for frequent recurrent infections, which may become fatal if untreated. Patients have increased susceptibility to encapsulated pyogenic bacteria. Haemophilus influenzae is second only to Streptococcus pneumoniae as the bacteria most frequently implicated in infections in these patients. We present a case involving an adolescent boy with X-linked agammaglobulinemia and H influenzae cervical adenopathy, confirmed twice by culture. We correlate the clinical, microbiologic, and histologic findings. Owing to the severity of infections in this population, surgical intervention is more common than in the immunocompetent population. This description may help the pathologist in considering a differential diagnosis when examining a diagnostic lymph node biopsy in these patients.
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PMID:Haemophilus influenzae lymphadenopathy in a patient with agammaglobulinemia: clinical-histologic-microbiologic correlation and review of the literature. 1562 87

Primary immune deficiencies (PIDs) are characterized by functional and/or quantitative abnormalities of one or more immune system components. Several bone and joint abnormalities can occur in patients with PID, with arthritis being the most common. Joint manifestations, of which arthritis is the most common, occur chiefly in humoral PIDs (agammaglobulinemia, common variable immunodeficiency, hyper-IgM syndromes, and IgA deficiency) and occasionally in other PIDs (chronic granulomatous disease and Wiskott-Aldrich syndrome). Monoarthritis or oligoarthritis is the usual pattern, although polyarthritis may occur, occasionally with nodules suggesting rheumatoid arthritis. Arthritis in patients with PID is usually infectious in nature, the most common causative organism being Mycoplasma, followed by Staphylococcus, Streptococcus, and Haemophilus. These bacteria can induce not only synovial infections, but also aseptic arthritogenic inflammatory responses. Arthritis having no demonstrable relation to chronic infection has been reported also and ascribed to dysimmunity-driven mechanisms that exhibit a number of specific features. Bone lesions are far less common and usually due to infections complicating humoral PID. Distinctive bone manifestations occur in a number of rare PIDs (e.g., hyper-IgE syndrome and Di George syndrome) and in syndromes characterized by spondyloepiphyseal dysplasia. Familiarity with PID syndromes both enhances the diagnostic capabilities of physicians and provides insight into the pathophysiology of bone and joint abnormalities associated with immune dysfunction. In children and occasionally in adults, a combination of bone and/or joint manifestations and hypogammaglobulinemia may indicate PID. When there is no evidence of lymphoproliferative disease, infection, or iatrogenic complications, investigations for PID should be obtained. PID-related arthritis is a unique model for studying the pathogenesis of presumably postinfectious arthritis and of inflammatory joint diseases including rheumatoid arthritis.
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PMID:Bone and joint disease associated with primary immune deficiencies. 1637 4