Adrenal insufficiency as a manifestation of disseminated non-Hodgkin's lymphoma.

OBJECTIVE: To analyze the clinical characteristics, laboratory features, and outcome in five patients who had biochemically proven adrenal insufficiency attributable to pathologically confirmed non-Hodgkin's lymphoma (NHL). MATERIAL AND METHODS: We retrospectively reviewed the medical records of all patients at Mayo Clinic Rochester during the period from 1976 to 1994 to identify those with both NHL, as listed in the surgical pathology tissue registry, and adrenal insufficiency. Histologically, the patients were classified on the basis of the working formulation and the revised European-American lymphoma classification. RESULTS: Three patients had diffuse large cell NHL, one patient had small noncleaved NHL, and one patient had cutaneous T-cell NHL. All five patients had stage IV disease. Adrenal insufficiency was confirmed by morning and evening determinations of serum cortisol levels and cosyntropin stimulation tests. All patients demonstrated loss of circadian rhythm. The median age of the patients was 77 years (range, 60 to 89). Three of the five patients died without treatment from 5 to 22 days after assessment. One patient died of a cerebrovascular accident. Despite initiation of chemotherapy, two patients died of progressive NHL at 7 weeks and 7 months. CONCLUSION: In our experience, biochemically proven adrenal insufficiency in patients with NHL is a manifestation of clinically advanced disease in elderly patients. A stepwise diagnostic approach is critical for the appropriate management of such patients.

Carcinoid tumors and sarcoidosis--does a link exist?

OBJECTIVE: To attempt to determine whether a relationship exists between carcinoid tumors and sarcoidosis. MATERIAL AND METHODS: We present a series of seven case reports and discuss hypotheses about possible disease associations. RESULTS: Certain malignant lesions have tended to occur in patients with sarcoidosis. Seven patients who were encountered at Mayo Clinic Rochester between 1950 and 1994 had both sarcoidosis and carcinoid tumors. These patients ranged in age from 31 to 66 years, and three of the patients had a history of benign thyroid disorders. Malignant tumors have been thought to be related to sarcoidosis in one of two ways: (1) immunologic abnormalities in sarcoidosis may promote the development of neoplasms or (2) malignant disease may promote the onset of sarcoidosis either by causing local sarcoid reactions that progress or by directly initiating the manifestations of systemic sarcoidosis. Because the chronology of events differed in our seven cases, various mechanisms of action may have a role in the manifestations of these two disease entities. Our cases emphasize the importance of avoiding the diagnosis of disseminated malignant disease in patients with cancer and associated hilar and mediastinal lymphadenopathy without biopsy confirmation of metastatic disease. CONCLUSION: Application of the knowledge gained about the mechanisms of disease in sarcoidosis will perhaps facilitate identification of the pathogenesis of carcinoid tumors and other neuroendocrine tumors.

The use of continuous i.v. sedation is associated with prolongation of mechanical ventilation.

STUDY OBJECTIVE: To determine whether the use of continuous i.v. sedation is associated with prolongation of the duration of mechanical ventilation. DESIGN: Prospective observational cohort study. SETTING: The medical ICU of Barnes-Jewish Hospital, a university-affiliated urban teaching hospital. PATIENTS: Two hundred forty-two consecutive ICU patients requiring mechanical ventilation. INTERVENTIONS: Patient surveillance and data collection. MEASUREMENTS AND RESULTS: The primary outcome measure was the duration of mechanical ventilation. Secondary outcome measures included ICU and hospital lengths of stay, hospital mortality, and acquired organ system derangements. A total of 93 (38.4%) mechanically ventilated patients received continuous i.v. sedation while 149 (61.6%) patients received either bolus administration of i.v. sedation (n=64) or no i.v. sedation (n=85) following intubation. The duration of mechanical ventilation was significantly longer for patients receiving continuous i.v. sedation compared with patients not receiving continuous i.v. sedation (185+/-190 h vs 55.6+/-75.6 h; p<0.001). Similarly, the lengths of intensive care (13.5+/-33.7 days vs 4.8+/-4.1 days; p<0.001) and hospitalization (21.0+/-25.1 days vs 12.8+/-14.1 days; p<0.001) were statistically longer among patients receiving continuous i.v. sedation. Multiple linear regression analysis, adjusting for age, gender, severity of illness, mortality, indication for mechanical ventilation, use of chemical paralysis, presence of a tracheostomy, and the number of acquired organ system derangements, found the adjusted duration of mechanical ventilation to be significantly longer for patients receiving continuous i.v. sedation compared with patients who did not receive continuous i.v. sedation (148 h [95% confidence interval: 121, 175 h] vs 78.7 h [95% confidence interval: 68.9, 88.6 h]; p<0.001). CONCLUSION: We conclude from these preliminary observational data that the use of continuous i.v. sedation may be associated with the prolongation of mechanical ventilation. This study suggests that strategies targeted at reducing the use of continuous i.v. sedation could shorten the duration of mechanical ventilation for some patients. Prospective randomized clinical trials, using well-designed sedation guidelines and protocols, are required to determine whether patient-specific outcomes (eg, duration of mechanical ventilation, patient comfort) can be improved compared with conventional sedation practices.

Pneumocystis carinii pneumonia in HIV infected patients from South Africa.

OBJECTIVE: To compare the spectrum of pulmonary disease in human immunodeficiency virus positive patients of African and European origin, with particular reference to Pneumocystis carinii pneumonia. DESIGN: Descriptive study with retrospective record review. SETTING: Tertiary care, university teaching hospitals in Johannesburg, South Africa. SUBJECTS: Sixty seven HIV-infected patients and 36 HIV-negative renal transplant patients (who served as controls) whose sputa were negative for tuberculosis and Pneumocystis carinii, and who had fibreoptic bronchoscopic evaluation for pulmonary disease between January 1985 and August 1992. MEASUREMENTS: Histological and cytological evaluation of pulmonary diseases. RESULTS: In the HIV infected group Pneumocystis carinii pneumonia was the most frequent disease in patients of both African and European origin but occurred less commonly in Africans than in Europeans (27.3% and 58.8%, respectively; p = 0.01). There was no statistically significant difference between patients of African and European origin in the frequency of any other diagnosis. In the renal transplant group the most frequent diagnosis was non-specific interstitial pneumonitis (Africans 33.3%, Europeans 37.0%), followed by Pneumocystis carinii pneumonia (Africans 22.2%, European 14.8%). There was no difference between HIV-infected and renal transplant patients in the frequency of any diagnosis. CONCLUSION: Pneumocystis carinii pneumonia should be considered in the diagnosis of HIV-infected African patients with pulmonary disease whose sputum smears for tuberculosis are negative.

Rheumatic fever revisited. Keep this diagnosis on your list of suspects.

The symptoms of recurrent rheumatic fever in adults can be very different from those in children, and the diagnosis is easy to overlook. Nonetheless, rheumatic fever is still a problem in many parts of the world, as illustrated by this case. Dr Capizzi and associates remind us to keep our options open.

Instructive case report. A 26-year-old Indian woman with seizures and multiple intracranial mass lesions.

We describe the case of a 26-year-old Indian woman who presented to our institution with seizures and papilledema. Her diagnosis was originally thought to be neurocysticercosis, but later confirmed to be intracranial tuberculoma. Antituberculous therapy with isoniazid, rifampin, pyrazinamide and ethambutol was initiated. Improvement in the patient's neurological symptoms with diminution in size of the intracerebral lesions was observed. This case illustrates the difficulty in distinguishing intracranial tuberculoma from neurocysticercosis. Radiological features that distinguish these two diseases are described. Both diseases must be considered in regions endemic for tuberculosis and cysticercosis.

Hereditary interstitial nephritis without basement membrane changes.

Hereditary interstitial nephritides are a heterogeneous group of disorders comprising medullary cystic disease, several varieties of Alport's syndrome and also one familial disorder with a distinct clinical syndrome and without characteristic ultrastructural glomerular basement membrane changes. Our family consisted of 11 members, 5 of which presented with renal dysfunction of varying degrees. Clinically, the affected siblings presented with long-standing hypertension, minimal proteinuria and no hematuria. All known causes of a secondary diffuse interstitial nephritis, Alport's syndrome and medullary cystic disease have been excluded. An HLA association is suggested between the affected and unaffected members of the family. Renal biopsy subsequently showed the typical features of a chronic interstitial nephritis without basement membrane changes.