Pulmonary infiltrates and fever in patients with hematologic malignancy: assessment of transbronchial biopsy.

Pulmonary infiltrates associated with fever are frequently encountered in patients with acute leukemia or lymphoma; In this prospective series, we analyze 47 such episodes in 43 patients. Overall mortality was 45 per cent in patients with infiltrates and somewhat higher when they also had neutropenia (55 per cent) or acute leukemia (67 per cent). Pulmonary infiltrates could be categorized into three roentgenographic patterns: local consolidation (55 per cent); cavitary disease (13 per cent) and diffuse interstitial disease (32 percent). The exact etiology of the infiltrates could not be predicted by roentgenographic study. Microbiologic or histopathologic diagnosis was established during life in 57 per cent of the patients, with infection most commonly encountered. Twenty-one patients underwent lung biopsy procedures. Biopsy specimens were frequently diagnostic (n = 17) and often dictated therapeutic changes (n = 12). Transbronchial lung biopsy via the fiberoptic bronchoscope was utilized in 14 patients during the latter part of this study; diagnoses were obtained in nine patients. Morbidity was minimal with this procedure, and the need for thoracotomy was diminished when it was available.

Diffuse pulmonary hemorrhage and rapidly progressive renal failure. An uncommon presentation of Wegener's granulomatosis.

A 57 year old woman presented with rapidly progressive renal failure and diffuse pulmonary hemorrhage and life-threatening respiratory failure promptly developed; these conditions resolved after bilateral nephrectomy. Renal pathology revealed rapidly progressive glomerulonephritis and vasculitis with granular deposition of immunoglobulin on immunofluorescent staining. One year later, multiple nodular cavitating pulmonary infilrates developed, and lung biopsy was diagnostic of Wegener's granulomatosis. Therapy with cyclophosphamide resulted in resolution of the pulmonary lesions. Diffuse pulmonary hemorrhage and rapidly developing renal failure mimicking Goodpasture's syndrome was the initial manifestation of Wegener's granulomatosis in this patient.

Bilateral diaphragmatic paralysis with hypercapnic respiratory failure. A physiologic assessment.

Bilateral diaphragmatic paralysis was suspected in a patient presenting with hypercapnic respiratory failure who exhibited paradoxic (i.e., inward) abdominal movement on inspiration during tidal breathing in the supine posture; no paradoxic abdominal motion was observed at the bedside with the patient upright. Transdiaphragmatic pressure measurements established the diagnosis of diaphragmatic paralysis, although 20 cm H2O pressure developed across the diaphragm during the latter part of a forced expiration, presumably due to the development of passive tension in the diaphragm as it was stretched near residual volume. Analysis of the relative motion of the rib cage and abdomen during breathing by the use of magnetometers confirmed the presence of abdominal paradox throughout the breathing cycle when the patient was supine, and established that paradoxic motion of the abdomen also occurred when the patient was in the erect posture but only in the latter half of inspiration. Our findings confirm that the use of transdiaphragmatic pressure measurements and magnetometry will help to quantify diaphragmatic function, that passive tension develops in the paralyzed diaphragm near residual volume and should not be confused with active contraction, and that paradoxic motion of the abdomen may be masked from the clinician when the patient is erect.

Apical fibrobullous disease with rheumatoid arthritis.

We describe a patient who developed noninfectious apical fibrobullous disease 12 years after the diagnosis of seropositive, nodular, deforming rheumatoid arthritis. Fibrobullous disease of the pulmonary apices is a rare entity that is usually found in association with ankylosing spondylitis. Its appearance with rheumatoid arthritis has not been reported. Speculative factors which may predispose to apical fibrobullous disease, such as a stiff chest wall, impaired esophageal motiligy, and HL-A antigen B27, were not present in our patient.

Asthma: pathophysiology and clinical correlates.

Sufficient data has now accumulated that demonstrates that a careful analysis of the presenting signs and symptoms of a patient with acute asthma will permit clinically useful conclusions to be drawn regarding the magnitude and severity of the underlying airway obstruction and the expected response to therapy. If a patient with severe obstruction deviates from this expected course, objective measurements of mechanical function and gas exchange should be obtained. In our current state of knowledge, these measurements should then be used as the prime indices of therapeutic effectiveness and less reliance should be placed on traditional clinical approaches.

An alternate method for fiberoptic bronchoscopic examination of the intubated patient.

Fiberoptic bronchoscopy can be performed easily on patients requiring mechanical ventilation through endotracheal or tracheostomy tubes greater than 8 mm inside diameter. In patients with smaller tubes the procedure can still be performed by passing the bronchoscope transnasally and then through the glottis alongside the endotracheal tube.

Transbronchial lung biopsy in the compromised host.

Thirty-eight immunocompromised patients underwent transbronchial lung biopsy via the fiberoptic bronchoscope as part of a diagnostic evaluation for fever and roentgenographic evidence of a new pulmonary infiltrate. Diagnostic information was obtained from lung biopsy in 29 patients (76%), with infection accounting for ten cases and a nonspecific interstitial pneumonitis in 13 patients. Concomitant bronchial brushings were diagnostic in only three patients (all with infections). Diffuse roentgenographic infiltrates were expecially amenable to bronchoscopic lung biopsy diagnosis (84%), while in localized infiltrates, there was only a 43% diagnostic yield. Although thrombocytopenia and hypoxemia were common in these patients, morbidty was low (four patients had pneumothoraces with no noteworthy bleeding) and there were no deaths resulting from this procedure. Prebiopsy platelet transfusions were used in five patients with severe thrombocytopenia (platelet cound, less than 50,000/cu mm). The diagnostic efficiency and low morbidity associated with transbronchial lung biopsy indicate that this procedure can safely play a role in the evaluation of pneumonia in the compromised host.

Obstructive sleep apnea in family members.

Two sons and their father had severe hypersomnolence and obstructive sleep apnea. A third son, although asymptomatic, was shown to have upper-airway obstruction during sleep. Electromyographic recordings of genioglossus activity in the two symptomatic sons revealed loss of tonic activity in early stages of sleep at times when sleep apnea occurred. The asymptomatic son showed loss of tonic activity during rapid-eye-movement sleep, the sleep period when upper-airway obstruction occurred. Two sudden deaths occurred in this family. A 30-year-old brother died at home while asleep, and a child of the asymptomatic brother died at the age of four months from presumed sudden-infant-death syndrome. Obstructive sleep apnea may have a familial basis; the tongue may be involved in the genesis of upper-airway obstruction during sleep.

Carbon monoxide poisoning in fire victims: a reappraisal of prognosis.

In victims of carbon monoxide (CO) poisoning, metabolic acidosis has been considered a clinical finding of ominous prognostic importance. In previous studies, such patients either died or suffered serious neurologic sequelae, resulting in the recommendation by some authors that these patients require more complicated therapy, including hyperbaric oxygen and hypothermia. This report documents the full neurologic recovery of three patients with severe metabolic acidosis and CO poisoning. Our experience indicates that acidosis may not be as important a prognostic factor as previously thought, and that randomized prospective trials are needed in such patients before more complicated therapy becomes accepted medical practice.

Restrictive ventilatory defect in a patient with primary pulmonary hypertension.

A patient with the classic features of primary pulmonary hypertension developed a severe restrictive ventilatory defect that worsened during the clinical course. Histologic examination of the lung showed vascular changes consistent with primary pulmonary hypertension and no evidence of pulmonary parenchymal fibrosis. We suggest that severe progressive pulmonary hypertension caused a restrictive ventilatory defect in this patient.

Vocal cord closure. A cause of upper airway obstruction during controlled ventilation.

Studies of vocal cord function were undertaken in a quadriplegic patient requiring ventilatory assistance, and in 2 normal subjects during controlled ventilation in a tank-type respirator. When the patient and the normal subjects relaxed and made no conscious effort to assist the respirator, the vocal cords were observed to close during inspiration and a large pressure gradient (12 to 19 cm H2O) developed across the cords. When the subjects made a slight inspiratory effort ("assist" mode), the cords opened widely during inspiration. There were large increases in flow and tidal volume in the "assist" mode compared with passive ventilation. Measurements of transdiaphragmatic pressure and esophageal pressure showed that these variables did not increase with the slight assist. Thus, increase in ventilation during the "assist" mode appeared to be due to alleviation of inspiratory obstruction at the level of the vocal cords. The same phenomenon was observed in the patient during phrenic nerve pacing. A pacemaker was designed to be triggered by the electromyographic impulse from an accessory muscle of respiration. In this manner, vocal cord opening could be coordinated with the mechanical assist given by the phrenic nerve pacer.