Infectious complications of the primary immunodeficiencies.

The primary manifestation of the immunodeficiencies is undue susceptibility to infection. This means too many, too severe, too prolonged, too complicated and too unusual infections. Infections in immunodeficiency have a characteristic cause depending on the nature of the immune deficiency. Antibody deficiencies are associated with infections with gram-positive infections. Cellular immune deficiencies are associated with mycobacterial, protozoan, fungus, virus, and opportunistic bacterial infection. Phagocytic disorders are associated with staphylococcal, fungal, and gram-negative organisms. Complement disorders are associated by neisserial infections. Infections have also been implicated in the pathogenesis of some immunodeficiencies in some circumstances. These include human T lymphotropic virus type III (HTLV-III), rubella virus, cytomegalovirus, and Epstein-Barr virus. Several infectious syndromes in specific immunodeficiencies have been identified. Examples include enteric cytopathic human orphan (ECHO) virus encephalitis in agammaglobulinemia, and meningococcal meningitis in C6 deficiency. Infections can also be induced by live vaccines given in immunodeficiency (e.g., paralytic polio in agammaglobulinemia.) Unusual infectious syndromes will be illustrated including parainfluenza infection in severe combined and immunodeficiency, Legionella pneumonia in chronic granulomatous disease, and Cryptosporidium infection in hyper-IgM immunodeficiency.

Abnormal erythrocyte and lymphocyte chemiluminescence in chronic granulomatous disease: evidence for a generalized membrane defect.

Concanavalin A (soluble)- and/or opsonized zymosan (particulate)-stimulated luminol-dependent chemiluminescence was demonstrated in normal human lymphocytes, erythrocytes, and in the membrane (ghost) fractions of polymorphonuclear neutrophils and erythrocytes. By contrast, seven patients with chronic granulomatous disease (five boys with the X-linked type and two female variants) had diminished luminescent responses to con A and/or opsonized zymosan in their lymphocytes, erythrocytes, erythrocyte membranes, as well as neutrophil membranes, suggesting generalization of the deficiency in the neutrophil oxidase system to other cells.

Bronchodilator characteristics of nebulized metaproterenol sulfate, isoetharine, and atropine in chronic asthma.

A randomized double-blind comparison of aerosolized META, ISO, ATR, and PI solutions delivered by motorized mist nebulizer was conducted in subjects with known reversible airway obstruction. Vital signs and spirometric parameters were monitored sequentially during each of four 6 hr test periods. Abstention from all medication was required for at least 12 hr before each test period. Baseline percent predicted FEV1 and FEF25-75 among subjects for all test periods were statistically similar. Compared to the other agents tested META treatment (15 mg) resulted in significantly improved global response and higher mean percent increases in FEV1 and FEF25-75 over the entire test period as well as at individual measurement points up to 4 hr for FEV1 and 5 hr for FEF25-75. Peak FEV1 and FEF25-75 values with META treatment were noted at 30 min and persisted above baseline during the entire 6 hr. ATR (2 mg) treatment resulted in later onset of bronchodilatory effect and, as with ISO (125 micrograms), more rapid diminution in measured response. Administration of saline alone resulted in a net 9% increase in FEV1 and 11% in FEF25-75 over the entire test period. Clinically insignificant tremor was noted in 41% of META-treated subjects. Delivery of META by a motorized mist nebulizer provides safe, effective bronchodilation and META is superior to the other agents tested in terms of onset of action, peak reversal of airway obstruction, and duration of effect.

Treatment of laryngeal stenosis. A conservative new approach.

One of the most perplexing problems in laryngology is the treatment of laryngeal scar tissue. This can be supraglottic, glottic, or infraglottic. Two acceptable methods of treatment have been surgical and endoscopic. Endoscopic treatment consists of dilatation, excision of scar tissue with a laser, removal of scar tissue with insertion of a stent, and removal and scar tissue with injection of intralesional cortisone. Twelve cases are reported, treated with microscopic removal of scar tissue followed by voice rest and beclomethasone dipropionate (Vanceril) inhalations, a topical steroid. All twelve cases showed marked improvement measured by improved glottic opening and improvement of voice quality. Further evaluation of this method of treatment should be done as an auxiliary treatment to our present accepted regime of treatments in this troublesome condition.

Deficient activity of the alternative pathway of complement in beta thalassemia major.

Patients with thalassemia major suffer frequent and serious infections, especially after splenectomy. To explore the basis for this susceptibility, we examined activity of the complement system in sera from 24 patients. All sera had normal or increased activity of the classic complement pathway. However, six of the 24 (three with and three without splenectomy) had abnormal alternative pathway function, and mean alternative pathway activity was significantly decreased in both splenectomized and nonsplenectomized patients. Mean concentrations of C3, factor B, properdin, and immunoglobulins were normal. Defective alternative pathway function, especially in conjunction with asplenia, could contribute to the propensity to infection that exists in thalassemia.

Non-Hodgkins lymphoma of the infratemporal fossa: a case report.

Lymphomas are statistically the second most common malignant tumor of the head and neck, following squamous cell carcinoma. They may present in the cervical lymph nodes or in extranodal areas. This paper presents an unusual case of a non-Hodgkins lymphoma that presented in the infratemporal fossa of a 25-year-old woman. The anatomy of the infra-temporal fossa is reviewed, and evaluation and therapy of lesions in this area are discussed.