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Clinical immunology is a medical specialty with a pathogenetic rather than organ-based focus. The main clinical areas covered are allergic disease, autoimmune disease, lymphoproliferative disease, transplantation and immunodeficiency. The role and training of the physician immunologist and the organ-based specialist with an interest are outlined.
Di-George's syndrome is a rare condition of congenital immunodeficiency. An infant presented as hypocalcemic tetany with multiple infections. Autopsy confirmed the hypoplasia of thymus and thyroid associated with other anomalies. A brief of the relevant literature is presented.
A sophisticated system of pulmonary host defense strives to maintain the functional integrity of the lung against the threats of infections, toxins, and malignancy. Congenital and acquired defects in the immune mechanisms of this host defense are associated with a variety of pulmonary disorders that include infections with usual or opportunistic organisms; inflammatory disorders; and malignancies. The age of the patient, associated abnormalities, family history, and the type of pulmonary and systemic diseases that are present provide clues to the specific underlying disorder. Laboratory tests including immunoglobulin levels, lymphocyte subset enumeration, and tests of lymphocyte function can help to confirm the clinical impression. Determination of the specific disorder allows the physician to anticipate possible complications, initiate appropriate prophylactic measures, and, in an increasing number of diseases, offer specific therapy.
Patients with Down's syndrome (DS) are predisposed to infectious diseases, particularly of the respiratory tract. Together with the occurrence of cardiac defects, this is one of the main determinants of their life expectancy. The aim of the present work was to study a series of immunological parameters that would allow us to evaluate the functional behaviour of the neutrophil polymorphonuclears of the patients with trisomy 21 and the possible relationship between this and the susceptibility of this kind of patient to such infections. The results obtained point a significant decrease (p less than 0.001) in the adhesiveness index and in the two indexes relating to the evaluation of random mobilities of cells ("leading front" and "lower face of filter"). Although the neutrophil chemotactic responsiveness of these patients is decreased with any of the attractants employed (casein and activated sera), the differences observed with respect to the control population are more patent upon analyzing the function on the "lower face of filter". Study of phagocytosis of Candida albicans and candidicidal activity of PMN reveals a normal behaviour in the presence of autologous (patient) and heterologous (control) serum. We propose that the alterations found in the PMN cell functions of DS patients could be due to intrinsic cellular defects and that this would help to explain why such patients tend to undergo repetitive infective processes.
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Peripheral blood T and non-T lymphoid cells were examined in 31 patients with a variety of primary immmunodeficiencies for their locomotor activity toward casein and endotoxin-activated serum. T cells from 1 and non-T cells from 2 of 7 patients with Bruton type agammaglobulinemia had poor locomotor activity in this system. Both T and non-T lymphoid cells from all patients with common variable immunodeficiency disease had normal locomotor response to casein and EAS. Heterogeneity in locomotor abnormaltiy of T and non-T cells toward casein and endotoxin-activated serum was observed in other immunodeficient patients. No direct correlation was observed between the proportion of cells in the several T cell subsets and the abnormalities of locomotion of T cells.
Tuberculosis is one of the opportunist infections which may complicate the course of many immunodepressive conditions generally those affecting cell-mediated immunity as the Koch bacillus is an organism which develops intracellularly provoking the activation of the cellular type of defence reaction. Recently, patients with malignant disease and immunosuppressive therapy have been joined by those with the acquired immunodepressive syndrome. It is important to understand that the frequency of tuberculosis in immunodepression is a reflection of the frequency of the disease in the general population. In addition, the diagnosis of atypical forms of tuberculosis can be difficult: the presenting signs are not specific and extrapulmonary forms are common. Therefore, tuberculosis should be suspected whenever immunodepressed patients develop unexplained symptoms. All practical means of confirming the diagnosis should be employed and the patient given a course of antituberculous drugs if there is any doubt as early treatment is an important prognostic factor.
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Di George syndrome is caused by anomalous development of the organs arising from the third and fourth pharyngeal pouches and results in congenital aplasia of the thymus, aplasia or hypoplasia of the parathyroid glands and cardiovascular malformations. Clinically, affected children show hypoparathyroidism and, because of depressed cell-mediated immunity, serious bacterial, viral and fungal infections. We present an infant, aged 6 weeks, with convulsions due to hypocalcemia, in which cell-mediated immunodeficiency was detected. Additionally diagnostic and therapeutic possibilities in DiGeorge syndrome are shown.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.