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PubMed1990

Impaired antibody response of C57BL/6 beige mutant mice to a thymus-independent type 2 antigen.

The murine equivalent of the human Chediak-Higashi Syndrome is the beige (bg) mutant in the C57BL/6 (B6) background. Besides the well-known lack of natural killer (NK) activity in bg-homozygous mice, functional abnormalities of T cells, macrophages and various granulocytes have been reported. With the exception of one study indicating a decreased in vitro response to lipopolysaccharide, there is no report concerning the B cell compartment of the beige mutant. The in vivo anti-trinitrophenyl antibody response to a TI-2 antigen (TNP-Ficoll) was found here to be significantly lower in B6 beige than in B6 wild mice, although both strains responded similarly to an analogous TD antigen (TNP-ovalbumin). Since the marginal zone macrophages of the spleen were previously shown to be essential for the initiation of antibody responses to TI-2 antigens, they might be another target of the beige mutation.

Animals↗
PubMed1984

Congenital cardiac anomalies associated with the DiGeorge syndrome: a neonatal experience.

The DiGeorge syndrome is a rare congenital abnormality of absent of hypoplastic thymus and parathyroid glands. Thirty neonates who had cardiac lesions and the DiGeorge syndrome are reviewed. The early mortality for 10 neonates undergoing palliative procedures was 80%. Seventy-five percent of the deaths were secondary to sepsis. Twenty neonates did not undergo palliative procedures. In this group, early mortality was 60% and late mortality was 65%. Sixty percent of the deaths in this group were associated with sepsis, with cardiac failure responsible for the remaining deaths. Survival in both groups has improved with appropriate treatment of the immunological and metabolic consequences of the DiGeorge syndrome.

Abnormalities, Multiple↗
PubMed1984

Familial third-fourth pharyngeal pouch syndrome with apparent autosomal dominant transmission.

A family is presented in which both siblings and their father had evidence of third-fourth pharyngeal pouch syndrome (DiGeorge syndrome). All three individuals had hypocalcemia and unusual facies. Both infants had truncus arteriosus. One infant had evidence of impaired cell-mediated immunity; the father had a relatively decreased number of T-lymphocytes. The syndrome is uncommon, most cases being isolated, and familial presentations are even rarer. Two recent reports described several affected individuals who also had partial deletions of chromosome 22. Chromosome banding studies in our family were normal. Thus our family demonstrates an autosomal dominant pattern of inheritance, although it cannot be proved that this is a single gene defect. We propose that inasmuch as the presentation of the syndrome is quite varied, thorough family investigation including high-resolution cytogenetic analysis is necessary. Familial cases may be more common and require genetic counseling.

Chromosome Aberrations↗
PubMed1997

Specificity and function of "natural" antibodies in immunodeficient subjects: clues to B cell lineage and development.

The origin of natural antibodies has long been a subject of controversy. Polyreactive natural antibodies recognize multiple ligands and are thought to arise from B1 B cells. Natural antibodies against carbohydrate antigens such as Gal alpha 1-3Gal or against blood groups A and B are thought to be "elicited" by gut bacteria, but their origin is uncertain. To explore the origin of naturally occurring anticarbohydrate antibodies, the specificity and function of the xenoreactive antibodies and isohemagglutinins were investigated in immunodeficient subjects. Subjects with defects in T cell-dependent antibody synthesis had normal levels of xenoreactive natural antibodies, most of which, like xenoreactive antibodies from normal individuals, were specific for Gal alpha 1-3Gal. On the other hand, some subjects with hyper-IgM syndrome who were able to synthesize abundant quantities of xenoreactive antibodies and polyreactive antibodies were devoid of anti-Gal alpha 1-3Gal antibodies. These results suggest that the lineages of B cells giving rise to anti-Gal alpha 1-3Gal antibodies and isohemagglutinins are distinct from B1 B cells or at least exist at a more "advanced" stage of development than those B1 B cells that give rise to polyreactive antibodies. The findings also suggest that B cells which synthesize anti-Gal alpha 1-3Gal antibodies and isohemagglutinins may be distinct from B2 B cells or exist at a more "primitive" stage of development than B2 B cells that synthesize elicited antibodies in normal individuals.

Adolescent↗
PubMed2002

Chest imaging in the immunocompromised child.

The immunodeficiency states in children may be sub-divided into two major groups; congenital (primary), and acquired (secondary). The spectrum of illness and imaging appearances are similar, regardless of the underlying cause of immunodeficiency. All immunodeficiency states are associated with an increased susceptibility to infection and neoplasia, with the lymphoproliferative disorders being the most frequent. However, the type of infections encountered and the risks of neoplasia are influenced by the underlying defect (whether predominantly humoral or cell mediated), the use of immunosuppressive drugs or radiotherapy as well as the length of immunosuppression.A working knowledge of the underlying likely immune defect is therefore important when interpreting imaging findings in children with immunodeficiency states. The typical imaging appearances of the common infectious, non-infectious and neoplastic complications of immunodeficiency are described.

AIDS-Related Opportunistic Infections↗
PubMedDate not supplied

Epidemiologic designs for the study of acquired immunodeficiency disease: options and obstacles.

Epidemiologic methods are designed to identify risk factors involved in production of a particular disease, even when the specific etiologic agent is unknown. However, a major problem currently presenting obstacles to research on the acquired immunodeficiency syndrome (AIDS) relates to classification; approaches are described that will permit reclassification when better laboratory techniques are developed or when more information is available from studies on natural history. Further descriptive studies will be valuable in the determination of whether extension of disease has occurred to new population groups or geographic areas. Case control studies can provide information on changes of known risk factors or can confirm and help to specify those factors already recognized. Because of the dynamic nature of AIDS and the probable existence of multiple risk factors, prospective cohort studies will be of greatest value, although they will take longer to complete. As understanding of the etiologic factors improves, preventive measures will become more efficient.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Otolaryngology problems in the immune compromised patient--an evolving natural history.

As the human immunodeficiency virus is being detected in increasing numbers of asymptomatic individuals at risk, newer earlier patterns of disease have become apparent--including cranial and cervical herpes zoster, oral hairy leukoplakia, and oral candidiasis--thus linking viral and other disease to the development of acquired immunodeficiency disease (AIDS). Many similarities between patients with AIDS and other immunosuppressed patients have emerged. As immunosuppressed patients survive longer, they begin to manifest cancers such as lymphomas and squamous cell cancers in addition to Kaposi's sarcoma. Otolaryngologists can learn to identify and treat otitis and sinusitis in the immunosuppressed patient, to identify predictive early signs such as oral hairy leukoplakia, herpes simplex virus, and oral candidiasis, and to diagnose and treat Kaposi's sarcomas of the head and neck, lymphomas, squamous cell cancers, and opportunistic infections as the immunodeficiency disease progresses.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Ossification centre of the hyoid bone in DiGeorge syndrome and tetralogy of Fallot.

The incidence of radiographic visibility of the ossification centre of the body of the hyoid bone in radiographs taken during the first month of life was analysed for 34 autopsied infants: 16 with DiGeorge syndrome (DGS), 14 with tetralogy of Fallot (TOF), four with interrupted aortic arch (IAA) and a further 13, surviving infants with non-DGS TOF or non-DGS IAA. The incidence of visible hyoid ossification centre (HOC) was 75.7% in a control series of infants with neither congenital heart disease (CHD) nor DGS. Autopsied patients with DGS, TOF without DGS, and IAA without DGS showed a significantly low incidence of visible HOC. Infants with TOF (and possibly those with IAA) who did not have DGS and who did not die during infancy showed a normal incidence of visible HOC in radiographs taken during the first post-natal month. Radiological visibility of the HOC in the first post-natal month appears useful in the diagnosis of DGS and forms of CHD often seen in association with DGS and in assessing prognosis of neonates with certain types of CHD.

Age Factors↗