Elevated levels of serum mucin-associated antigen in adult patients with cystic fibrosis.

Previous studies using the CA 19-9 antibody have demonstrated that serum mucin levels in patients with cystic fibrosis (CF) are elevated and that the degree of elevation relates to the age of the patient and possibly to his or her clinical status. However, CA 19-9 only recognizes the mucin-associated blood group sialyl Le(a+) antigen, so mucin levels cannot be measured in patients without Lewis antigens. The present study used the 17B1 monoclonal antibody to measure serum mucin levels in normal subjects, and in patients with CF, patients with chronic obstructive pulmonary disease (COPD), and patients with lung transplants. Serum mucin levels were 25 ng/ml (+/- 1 SEM, n = 8) in normal subjects, 13,853 ng/ml (+/- 1,281, n = 25) in patients with CF, and 25.5 ng/ml (+/- 1.9, n = 17) in patients with COPD. Patients with CF who were sialyl Le(a-b-) also had elevated serum mucin levels (715 +/- 152, n = 2). Serum mucin levels of six lung transplant recipients with CF were elevated compared with those in normal subjects (4,621 +/- 765 ng/ml), but they were not different from serum mucin levels in six lung transplant recipients without CF (5,307 +/- 1.677 ng/ml). Preliminary characterization of the serum mucin antigen showed that: (1) in CF sera, the antigen is polydisperse and smaller than the antigen in normal sera; (2) the mucin antigen is distinct from ABO blood group antigens. Serum mucin levels may be a useful marker to follow a specific patient's response to therapy.

Jejunal distention with elemental feedings following duodenal injury.

A man whose second portion of the duodenum was destroyed by blunt trauma and who developed jejunal distention, abdominal pain, and clinical toxicity is described. There was no circulation in the wall of the distended segment of the jejunum by intraoperative Doppler evaluation. Circulation returned immediately to that segment of bowel when it was decompressed.

Infarction of the quadriceps muscle: a complication of diabetic vasculopathy.

Accelerated atherosclerosis is a common and recognized complication of diabetes mellitus. We have reported the fifth case of spontaneous infarction of a portion of the quadriceps muscle group in a patient with diabetes mellitus.

Mediastinal meningioma.

A case of meningioma appearing to arise from the stellate ganglion and associated with a unilateral Horner syndrome of long standing is presented. Clinical absence of central nervous system involvement and a normal computerized tomographic scan of the brain favor the neoplasm as being a primary extracranial meningioma. Light and electron microscopy showed characteristic structural features. A brief review of the literature is made, and theories concerning the origin of extracranial primary meningioma are presented.

Tracheobronchiomegaly: report of two cases with widely differing symptomatology.

Two cases of tracheobronchiomegaly are reported to call attention to this disease. Physicians must be mindful of this entity as another cause of recurring pneumonia which may be treatable. Various proposed etiologies for this syndrome are reviewed. The findings in and interpretation of our cases and those in the literature suggest that tracheobronchiomegaly is a congenital anomaly differing from others of the tracheobronchial tree because of its spectacular radiological appearance. The two following cases typify different presentations of this syndrome. One is largely asymptomatic; the other demonstrates super-infection of stagnant secretions, making the patient functionally bronchiectacic with progressive suppurative pulmonary destruction. The authors believe that aggressive management designed to mobilize airway secretions is indicated. There is usually no place for surgery in this disease beyond establishing the diagnosis.

Pleuroscopy and cautery control of intrathoracic hemorrhage with a flexible fiberoptic bronchoscope.

Intrathoracic hemorrhage from adhesions torn by a spontaneous pneumothorax is relatively uncommon. Continued hemorrhage from such adhesions after the evacuation of 1,500 ml of blood would usually require thoracotomy for control. Through a flexible fiberoptic bronchoscope, we confirmed the source of hemorrhage and cauterized the bleeding point. This case illustrates another application for pleuroscopic examination, further broadening the use of the flexible fiberoptic bronchoscope.

Endobronchial control of bronchopleural fistulae.

This report describes a proposed solution to the problem of high-flow bronchopleural fistulae in the adult respiratory distress syndrome. Animal studies and clinical application demonstrate the efficacy of this treatment.

Pathologic features and mechanisms of hypoxemia in adult respiratory distress syndrome.

In 45 consecutive patients referred for severe hypoxemia (Pao2 less than 100 mm Hg on positive end-expiratory pressure of 5 cm H2O and fraction of inspired O2 of 1.0), physiologic studies of gas exchange were correlated with pathologic features from 36 open lung biopsies and 15 autopsies. Three distinct groups were defined. Group 1 included 11 patients with the most severe hypoxia (Pao2, 47 +/- 12 mm Hg), minimal Pao2 response to a 10 cm H2O increase in positive end-expiratory pressure (+2.0 +/- 4.0 mm Hg), and a fixed shunt at all fractions of inspired O2. Pathologic study showed edema, exudation, and hemorrhage to the point of consolidation. In group 2 were 13 patients who had less severe hypoxia (Pao2, 60 +/- 17 mm Hg) and a moderate Pao2 response to a 10 cm H2O increase in positive end-expiratory pressure (+15 +/- 8 mm Hg), but whose maximal response was slowly achieved (30 min to several hours). Pathologic examination showed extensive fibrosis. The 21 patients in group 3 had the least hypoxia (66 +/- 15 mm Hg), and had a rapid and marked improvement in Pao2 with a 10 cm H2O increase in positive end-expiratory pressure (+68 +/- 59 mm Hg). Pathologic features were similar to but less severe than those in group 1. Venous admixture increased with decreasing inspired concentrations of O2, indicating diffusion or ventilation-perfusion abnormalities in groups 2 and 3. Prognosis was best for group 3, with 10 of 21 long-term survivors. Two of 11 group 1 patients survived, but only after prolonged periods of extracorporeal membrane oxygenation. Despite biopsy evidence of extensive fibrosis, 3 of 13 in group 2 survived with moderate to good pulmonary function, including 1 survivor who had had extracorporeal membrane oxygenation. Such combined physiologic and pathologic studies are useful (1) for optimal respiratory care, (2) for prognosis, (3) for development of indications for extracorporeal membrane oxygenation, and (4) for better understanding of the pathophysiology of adult respiratory distress syndrome.

Pulmonary pathology in acute respiratory insufficiency: lung biopsy as a diagnostic tool.

Forty-two patients underwent open-lung biopsy during the early phase of acute respiratory insufficiency. Correlation between the gross appearance of the lung at operation and the microscopic findings was good. Although only fair correlation was found between lung and tracheal cultures, the findings of two positive cultures in the lung only was of utmost importance. Biopsying multiple areas from the same operation showed identical pathology in 86 per cent of cases. The mortality rate of open-lung biopsy was zero; the morbidity rate was 4 per cent. The over-all survival rate of acute respiratory insufficiency (ARI) due to trauma was 39 per cent; that of pneumonia, 11 per cent. In 17 (33 percent) patients specific diagnoses and/or specific therapies were employed as a direct result of the biopsy or the thoracotomy. The incidence and prognostic implications of fibrosis and microthromboembolism are presented and discussed. Open-lung biopsy has been extremely safe and valuable in characterizing and managing ARI.

Complications associated with membrane lung support by venoarterial perfusion.

A series of patients supported with long-term venoarterial perfusion is presented. Gas exchange was achieved with a membrane oxygenator at flows usually in excess of 50% of the patient's baseline cardiac output. Perfusions were maintained for 7 to 12 days. Two major complications were encountered: thromboembolic myocardial infarction and liquefaction necrosis of the lung. Possible etiologies and suggestions for prevention are discussed.