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J Huml

Publications and source records attributed to J Huml.

3 recordsLinked to original sources

Tracheobronchial tumor cast formation and pneumothorax.

A 34-year-old white man was admitted to the hospital for progressive respiratory difficulty, recurrent left pneumothorax, and a diagnosis of aplastic left lung. Further evaluation, including bronchoscopy and CT scan, demonstrated a cast-forming tracheobronchial obstructing tumor. Surgical resection of the tumor through the left chest with left lower lobectomy resolved his symptomatology. Improved ventilation with an open airway led to expansion of the left upper lobe. The pathologic diagnosis remained controversial as to a definite designation and was primarily descriptive: spindle cell proliferation with myxoid and collagenized stromal benign tumor. However, the extent of this rubbery tumor cast and the ability to remove it intact illustrate the tenacity of the structure and the very unusual nature of a cast of the entire tracheobronchial system on the left side.

Adult↗

Hypogammaglobulinemia and anemia 18 years after thymoma resection.

Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in 7% to 13% of patients with adult-onset hypogammaglobulinemia. In 80% of cases, hypogammaglobulinemia is detected within 5 years of the identification of the thymoma. A 70-year-old man was found to have hypogammaglobulinemia 18 years after a thymoma had been resected. Cellular immunophenotyping revealed there were no detectable B cells, decreased CD4+ cells, and increased CD8+ cells. Both CD4+ and CD8+ subsets expressed increased populations of CD38+ DR+ cells and CD45RO+ cells. The CD8+ CD28+ population was markedly reduced. Inducible cytokine production by the patient's peripheral blood mononuclear cells revealed decreased IL-2, IL-10, and interferon-gamma production. These data suggest that patients with Good's syndrome have activated memory T cells that have dysregulated cytokine production.

Agammaglobulinemia↗

Office-based assessment of pulmonary function.

PFT is a valuable tool for evaluating the patient with respiratory complaints and for screening the high-risk population. It is no longer only a hospital-based test, but, with current technology, is an efficient, practical procedure that can be performed in an office at minimal cost to the patient. In addition, serial spirometry can be used to identify high-risk populations and gauge response to bronchodilator therapy. Spirometry should be performed routinely in all smokers older than 40.

Adult↗