Faculty Bibliography

CT was compared to fiberoptic bronchoscopy in a large series of patients to study the value of CT for visualizing bronchial disease. CT scans were available for review in 64 cases in which focal airway disease was identified with fiberoptic bronchoscopy and in 38 patients in whom the airways appeared normal at bronchoscopy. CT was positive in 59 of 64 cases in which lesions were detected endoscopically. If the results are analyzed according to the extent of involvement of individual bronchi, CT successfully identified 88 (90%) of 98 lesions. CT correctly excluded disease in 35 (92%) of 38 cases that were subsequently verified to be normal by fiberoptic bronchoscopy. In no case was the diagnosis of malignancy missed by CT. While extremely accurate in detecting focal lesions, CT was inaccurate in predicting whether a given abnormality was endobronchial, submucosal, or extrinsic (peribronchial). In three cases CT failed to detect submucosal extension into the left mainstream bronchus, which has important implications concerning the value of CT in staging bronchial malignancy. It is concluded that CT is helpful when bronchoscopy is contraindicated or refused. CT may also be used in selected cases when there is low clinical suspicion of endobronchial disease and as a complementary procedure to fiberoptic bronchoscopy for outlining the exact location of major mediastinal and hilar vessels, lymph nodes, and tumor in relation to adjacent airways

There is little consistency in drug treatment of children with juvenile rheumatoid arthritis, in part because there are few controlled studies of the use of nonsteroidal anti-inflammatory drugs (NSAIDs) in this disease. To determine the safety and efficacy of piroxicam in children with juvenile rheumatoid arthritis, 26 patients ranging in age from three to 25 years were randomly assigned to treatment with piroxicam or naproxen. The number of painful and swollen joints decreased significantly (p less than 0.05) in the piroxicam group. The overall assessment of the investigators was that 67% of the patients in the piroxicam group showed clinical improvement, in contrast to 38% of the naproxen-treated patients. Side effects in the piroxicam group were mild and transient, and no patient was removed from the study because of side effects. The recurrence of a cutaneous rash necessitated the removal of one naproxen-treated patient from the study. Although the results should be interpreted with caution because of the small sample size, piroxicam appears to be more effective and better tolerated than naproxen in this study

The mechanisms of hypercapnia in eight patients with the 'Pickwickian' syndrome and obstructive sleep apnea (OSAS) were evaluated pretherapy and posttherapy (tracheostomy in seven patients and chronic nocturnal use of nasal CPAP in one). Four patients (correctors) became eucapnic within two weeks of therapy. Four others (noncorrectors) remained hypercapnic. Neither residual apneas, changes in pulmonary function, change in anatomic dead space, nor changes in ventilatory chemoresponsiveness differentiated the two groups, nor did the last three factors account for return to eucapnia in the correctors. The results indicated two separate mechanisms exist for chronic hypercapnia in OSAS: a critical balance between the ventilation during the time spent awake and hypoventilation due to apneas, a mechanism removed by treatment for obstructive apnea; and sustained hypoventilation independent of the apnea phenomenon and therefore not correctible. The subset of patients with the second mechanism appears to represent the true 'Pickwickian' syndrome and can be identified before therapy by measuring a low level of ventilation in the sustained awake state

We present the radiologic features of four patients proven to have Hermansky-Pudlak syndrome. All four patients had evidence of pulmonary involvement characterized by a progressive, diffuse, bilateral interstitial fibrosis. Extensive bullous changes were seen in one patient. Two patients with evidence of diffuse colitis exhibited an asymmetrical pattern of focal, superficial, and deep ulcerations similar to that of Crohn's disease. The association of these radiographic abnormalities with albinism, ocular abnormalities, bleeding diathesis, and Puerto Rican ancestry establishes the diagnosis

The Hermansky-Pudlak syndrome is a rare disease characterized by multisystemic involvement. Seven families with the disorder were identified in the Puerto Rican population of one municipal hospital, suggesting that the incidence in the Puerto Rican community is sufficient to warrant both dissemination of information about the disease and further investigation. The present study was an attempt to achieve both of these goals