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Biomedical subjects

I K Rosner

Publications and source records attributed to I K Rosner.

8 recordsLinked to original sources

Pulmonary function abnormalities in thalassemia major and the role of iron overload.

To determine the predominant abnormality of pulmonary function in patients with thalassemia major (TM), we evaluated 29 patients with TM who were receiving hypertransfusion therapy and chelation with desferrioxamine (DFO), and who ranged in age from 6 to 40 yr (mean 19.8 +/- 8.5 yr). A reduction in the total lung capacity (TLC) was the most striking abnormality, found in 21 of 29 patients (79%). Fourteen of these patients (67%) had a moderate or severe reduction in TLC. Expiratory flow rates, FEV1, and FEF25-75 were decreased below predicted values in 48 and 17% of the patients, respectively, but no patients had pure obstructive disease. Significant hypoxemia (oxygen saturation of less than 95%) was observed in only one patient. There was a significant inverse correlation between TLC and patient age (p < 0.003), transfusional iron burden (p < 0.003) and DFO ratio (p < 0.024). Restrictive disease is the predominant abnormality of pulmonary function in TM, with a mixed restrictive-obstructive pattern in a small number of patients. The restrictive disease becomes more severe with increasing age, and the degree and duration of iron overload appear to be important in its pathogenesis. The role of DFO therapy in preventing the pulmonary complications associated with TM requires further study.

Adolescent↗

Pulmonary function testing in children with cardiac disease.

These cases illustrate the clinical importance of pulmonary function testing in children. The first case demonstrated a significant restrictive defect in a child with pulmonic stenosis and scoliosis. Although this child had no respiratory symptoms, it was important to document the degree of functional impairment to plan for her postoperative course. The second case demonstrated the way in which pulmonary function tests can lead to a specific diagnosis and provide important information about response to therapy. This patient had distressing symptoms which limited her ability to participate in sports. Her chest x-ray revealed no significant abnormality and she had no auscultatory finding. Pulmonary function tests defined both the nature and severity of her problems and provided objective information about her response to therapy. In the third case, one might have anticipated decreases in flow rates and lung volumes because of neuromuscular weakness. That was not evident on testing and the patient had an uneventful postoperative recovery. Pulmonary function testing provides invaluable information about the nature and severity of functional impairment in children with known or suspected pulmonary disease. New equipment and individuals specifically trained to work with children now permit the evaluation of even young children. Pulmonary function testing can expand our knowledge of disease processes and improve our therapeutic strategies.

Adolescent↗

Complement-dependent histaminase release from human granulocytes.

The role of particle-bound complement proteins in the induction of noncytotoxic enzyme release from human granulocytes was investigated with the use of sera genetically deficient in complement and highly purified complement components. Release of histaminase, one of two important histamine catabolizing enzymes, and beta-glucuronidase from polymorphonuclear leukocytes was solely dependent on particle-bound C3b (the larger cleavage product of the third component of complement) when fluid-phase complement was excluded. The extent of enzyme release was a function of particle-bound C3b input, was reduced by exposing the particles to C3b inactivator, and was blocked by fluid-phase C3b. Phagocytosis of the C3b-coated particles was not required for enzyme release from neutrophils. In contrast, phagocytosis of "opsonized" particles was required for noncytotoxic release of histaminase and arylsulfatase from eosinophils; other proteins, as well as C3b, were able to opsonize particles for induction of enzyme release from eosinophils. These studies suggest a dual role for complement (particularly C3) in modulating vascular permeability phenomena, i.e., release of vasoactive mediators by the action of C3a and C5a, and release of the corresponding enzymes that inactivate the mediators by C3b.

Amine Oxidase (Copper-Containing)↗