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PubMedDate not supplied

Evaluation and management of B and T cell abnormalities.

Congenital deficiencies that involve the lymphocyte system are complex and represent abnormalities in genetic control or gestational development. Only the more common prototypes of these diseases will be discussed. Diseases of the B cell system presented include X-linked agammaglobulinemia, selective IgA deficiency, IgG subclass deficiency, and common variable immunodeficiency. T cell defects discussed are Di-George syndrome and severe combined immunodeficiency. Also mentioned briefly are HIV infection and the increasing problem of AIDS. Diagnostic evaluation and general principles of treatment of these diseases are considered.

Agammaglobulinemia↗
PubMed1978

Levamisole: lack of immunopotentiation in a controlled trial.

The immunopotentiating effect of levamisole was assessed in a double-blind trial in two comparable groups of patients with Down's syndrome, with which the immunodeficiency and susceptibility to infection are known to be associated. One group was given levamisole continuously and at the same dose for 16 weeks, and the other group was given placebo tablets. A checklist was designed to record the type, frequency, and duration of all infections which occurred in the patients, and delayed type hypersensitivity responses to various antigens were measured. There were no differences between the two groups in body weight, number of intercurrent infections, duration of illness, nor was there any change in degree of depression or cutaneous delayed-type hypersensitivity. These findings, taken with claims of success in some patients and failure in others, suggest that levamisole is not a general immunopotentiating agent, although it may have a specific site (or sites) of action on the immune system. If levamisole is to be used more selectively, this action needs to be characterized.

Adult↗
PubMedDate not supplied

Clinical and immunological spectrum of partial DiGeorge syndrome.

We present four cases of DiGeorge syndrome diagnosed at our center. Onset occurred during the neonatal period and was associated with severe congenital heart disease. In case 1, the patient had heart disease and absence of thymus. Total T-lymphocytes were 34%; total T4-lymphocytes were 27%. Stimulation test with phytohemagglutinin (PHA), concanavalin A (conA) and pokeweed mitogen were negative. Microdeletion was found in the chromosome 22q11 region. The second case involved heart disease, microstomia, round and rotated ears and branchial cyst. Total T-lymphocytes were 38% and total T4-lymphocytes 27%. Thymus was absent. Microdeletion in the chromosome 22q11 region. Case 3 showed heart disease, renal malformation, absence of thymus and parathyroid gland. The patient died 5 days postsurgery. Microdeletion was seen at chromosome 22q11. In the fourth case there was heart disease, microretrognathia, hypertelorism, short neck, absence of thymus and parathyroid glands. Total T-lymphocytes were 22%, total T4-lymphocytes 15%, and total T lymphocytes for pokeweed mitogen were negative. Microdeletion was found at chromosome 22q11. At the age of 13 days the patient died. The cases were recorded during a 2-year period, between 1997 and 1998. The prevalence of DiGeorge syndrome in the number of admissions for congenital heart disease among the neonates at our hospital was 3.14%. Presentation in the form of repeated infections is rare, since most cases of DiGeorge syndrome are partial, and functional cellular immunity is preserved.

Adult↗
PubMedDate not supplied

Systemic conditions associated with periodontitis in childhood and adolescence. A review of diagnostic possibilities.

The term periodontitis is used to describe a group of multifactorial diseases that result in the progressive destruction of the structures that support the teeth within the jaws, the so-called attachment apparatus, which includes the periodontal ligament, cementum and alveolar bone. If left untreated, this process can ultimately lead to tooth loss. The pathogenesis of these diseases involves the initial colonization of the gingival microenvironment of a susceptible host by pathogenic bacteria found in dental plaque. Subsequently, much of the tissue destruction characteristic of periodontitis is a , by-product, of the host response directed against these organisms. One of the major factors that appear to modulate disease severity is systemic health. Severe periodontitis in young individuals can therefore be a manifestation of an underlying systemic disease. In this review we will discuss the most important systemic diseases that should be considered in a differential diagnosis when evaluating a pediatric patient presenting with periodontitis.

Adolescent↗
PubMed1991

[The reticuloendothelial system in immunodeficiency].

The concept of the mononuclear phagocyte system (MPS) as advanced by van Furth et al. does not embrace other cell types which by virtue of their accessory cell function are inextricably linked to the immune response. Therefore, a modified functional definition of reticuloendothelial system (RES) encompassing the non-lymphatic effector and accessory cells of the immune response, i.e. monocytes/-macrophages, follicular and interdigitating reticulum cells as well as endothelial cells is proposed. In addition to their morphologic and functional characteristics, their behaviour in the setting of human immunodeficiency virus (HIV) infection is discussed. As examples serve the role of RES cells as target cells and reservoirs for HIV and their dysfunctions with regard to different HIV-associated conditions, e.g. tuberculosis and Kaposi's sarcoma.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Nocardia asteroides infection in horses: a review.

From 1965 to 1983, Nocardia asteroides infection was diagnosed in 16 horses at the Veterinary Medical Teaching Hospital, University of California, Davis. In 2 of the cases, the infection was traumatic in origin and local in extent; the horses recovered without relevant antimicrobial therapy. Fourteen horses had pulmonary or disseminated infections that ended fatally. All 14 had various degrees of immunosuppression. Of these, 8 were Arabian foals with combined immunodeficiency disease and 3 were aged horses with hyperadrenocorticism secondary to ACTH-secreting pituitary tumors. Of the other 3, one had lymphosarcoma, another, hepatic disease presumed to be of toxic origin, and the third, a mixed disseminated bacterial infection.

Animals↗