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Biomedical subjects

N G Hepper

Publications and source records attributed to N G Hepper.

11 recordsLinked to original sources

Intrathoracic lymphangiomatosis mimicking lymphangioleiomyomatosis in a young woman.

In this report, we describe a case of lymphangiomatosis that mimicked lymphangioleiomyomatosis in a 20-year-old woman. Lung biopsy specimens showed proliferation of anastomosing lymphatic channels in the visceral pleura and dilated peribronchiolar and septal lymphatic channels. During 8 years of follow-up, this process behaved like lymphangioleiomyomatosis with reticulonodular infiltrates and worsening obstructive and restrictive changes evident on pulmonary function tests.

Adult

Silo-filler's disease.

Between 1955 and 1987, 17 patients were examined at the Mayo Clinic shortly after exposure to silo gas. All exposures had occurred in conventional top-unloading silos. Acute lung injury occurred in 11 patients, 1 of whom died; early diffuse alveolar damage with hyaline membranes and hemorrhagic pulmonary edema and acute edema of the airways were found at autopsy. In one patient, hypoxemia and transient obstruction of the airways developed, but no pulmonary infiltrates were noted. One patient had symptoms for 5 weeks and diffuse confluent pulmonary infiltrates; many years later, chronic obstructive pulmonary disease developed (he had, however, been a heavy smoker before exposure). Bronchiolitis obliterans was not observed in the other patients, probably because of less severe exposure or early corticosteroid therapy. Prophylactic corticosteroid therapy is advised for workers who have been exposed to silo gas. The management of patients with established acute lung injury is reviewed. Previously unreported patterns of exposure to silo gas in conventional silos are described, and recommendations for avoiding exposure are suggested.

Acute Disease

Incidence of spontaneous pneumothorax in Olmsted County, Minnesota: 1950 to 1974.

Between 1950 and 1974, 318 cases of initial pneumothorax were diagnosed among the residents of Olmsted County, Minn. Seventy-five cases were due to trauma, and 102 were iatrogenic. One hundred forty-one cases were spontaneous in onset, of which 77 were primary and 64 secondary to a specific underlying pulmonary disease. The age-adjusted incidence of primary spontaneous pneumothorax was 7.4/100,000/yr for males and 1.2/100,000/yr for females. For secondary spontaneous pneumothorax, the incidence was 6.3 and 2.0/100,000/yr for males and females, respectively. The ratio of male-to-female incidence was 6.2:1 for primary and 3.2:1 for secondary spontaneous pneumothorax. The differences between the incidence rates for primary and secondary pneumothorax by sex or in total were not significantly different.

Female

Pulmonary tumorlets associated with Cushing's syndrome.

Tumorlets of the lung generally have been considered benign nests of cells, usually found with bronchiectasis or fibrosis. We saw a patient with Cushing's syndrome whose tumorlets ultimately became roentgenographically visible as multiple small nodules throughout both lungs and metastasized to the mediastinum. The plasma corticotropin concentration was increased, as were the concentrations of corticotropin-like peptides in the tumor tissue. This experience lends support to the concept that some tumorlets arise from Kulchitsky's cells in the epithelium of peripheral bronchioles, as do carcinoid tumors, and have the same peptide-producing potential. The possibility of such tumors should be considered in patients with Cushing's syndrome who have no initial roentgenographic abnormality.

Adult

Desmoid tumors of the chest wall.

Twenty-six cases of desmoid tumors of the wall of the chest were reviewed. These are rare lesions, which nonetheless should be considered in the differential diagnosis of all tumors of the chest wall. Although the lesion is most often palpable, several of the tumors were detectable only by means of an x-ray film of the thorax. Definitive diagnosis could not be made clinically but was easily established by pathologic examination of widely excised tumor. Like all desmoid tumors, these were locally aggressive in their growth but nonmetastatic and generally carried a good prognosis. Ideally, treatment consisted of wide local excision, but if there was encroachment of the tumor on vital structures, excision with suboptimal tumor-free margins was required. Even in the latter instance, tumefaction could be well controlled for long periods. Recurrences after an initial attempted removal were common, but most of these responded to reexcision.

Adolescent

Pleural fluid complement, complement conversion, and immune complexes in immunologic and nonimmunologic diseases.

Forty-four pleural fluids and 41 blood specimens from patients with various diseases were examined for concentration of whole complement, C4, C3, conversion products of C3 and C3PA, and immune complexes. C3 conversion was found in all eight pleural fluids from patients with rheumatoid arthritis, five of seven with lupus erythematosus, two of six with congestive heart failure, and nine of 23 with malignant diseases. Conversion of C3PA correlated closely with C3 conversion and both were significantly inversely related to whole complement, C4, and C3. Concentration of immune complexes was highest in patients with rheumatoid arthritis. Pleural fluid immune complex concentrations correlated positively with conversion of C3 and C3PA. These findings suggest that the reduced levels of pleural fluid complement in rheumatoid arthritis and lupus erythematosus may be secondary to complement conversion by immune complexes.

Antigen-Antibody Complex

Host factors in chronic obstructive pulmonary disease in an upper Midwest rural community. Design, case selection, and clinical characteristics in a matched-pair study.

A series of 111 index subjects with chronic obstructive pulmonary disease (COPD) who had forced expiratory volume in 1 second (FEV1) of 70% or less of that predicted were matched on the basis of age, sex, occupation, and smoking history with control subjects who had an FEV1 of 85% or more of that predicted. Index and control subjects with seasonal or reversible airway disease were excluded. Men outnumbered women by a ratio of 4.5 to 1. Thirty-five percent of the women and 2% of the men were nonsmokers (0 pack-years). There were three PiZ phenotypes in the index group (two nonsmokers) and none in the controls. PiMZ phenotypes in the index group outnumbered those in the controls by 8 to 5. Host factors that might be important in these closely matched pairs were sought by history, physical examination, and a large battery of laboratory tests. A standard respiratory questionnaire revealed the anticipated significantly higher frequency of cough, phlegm, noisy respiration, and all grades of dyspnea in index subjects. Previous lower respiratory tract infections also were more frequent in index subjects than in controls. There were no detectable differences between groups in the frequency of upper airway infections, nasal polyps, sinus surgery, or reported allergy to any substance. If the British Medical Research Council's definition of chronic bronchitis were applied to our study, about two-thirds of our index subjects and almost one-third of our controls would be considered to have chronic bronchitis. Pack-years of smoking were not significantly associated with the amount and duration of cough and expectoration in male or female index subjects or controls. Significant differences between index and control groups on physical examination included the audible forced expiratory flow time over the trachea, the estimated maximal midexpiratory flow, breath sounds, rales, and total excursion of the hemidiaphragms. An endocrine questionnaire and measurement of blood sex hormones did not give any clues as to the propensity of males to develop COPD. Women with airway obstruction similar to that of men had histories of significantly fewer pack-years than did the men, and there was a much larger proportion of women who never smoked. Further studies, specifically on genetic and immunologic characteristics, are under way to identify potential host factors.

Bronchitis