Neuro-Behçet's disease.

We are reporting a case of Bechçet's disease without vasculitis but with acute neutrophilic inflammation which involved the brain and other organs. The patient exhibited waxing and waning neurological deficits which were unresponsive to treatment. The neuroradiologic findings simulated those of multiple sclerosis. The neuropathological examination revealed an acute, focal, though disseminated encephalitis involving the frontal lobe, internal capsule, basal ganglia, cerebellum, and brainstem. The acute inflammation consisted of a neutrophilic and eosinophilic infiltration of the perivascular spaces and parenchyma without evidence of vasculitis, fibrinoid necrosis, or thrombosis. Cultures and special stains for microbial organisms were negative. Ultrastructural examination revealed no viral structures or other microorganisms. In situ hybridization for Epstein-Barr virus (EBV), herpes simplex virus type I (HSV-I), and cytomegalovirus (CMV) was negative. In this case, the lack of inflammation in the vessel walls points out that the necrotizing lesions in neuro-Bechçet's disease need not be the result of vasculitis and superimposed thrombosis, but may occur as a result of primary, acute neutrophilic inflammation.

Cholesteryl hemisuccinate treatment protects rodents from the toxic effects of acetaminophen, adriamycin, carbon tetrachloride, chloroform and galactosamine.

In addition to its use as a stabilizer/rigidifier of membranes, cholesteryl hemisuccinate, tris salt (CS) administration has also been shown to protect rats from the hepatotoxic effects of carbon tetrachloride (CCl4). To further our understanding of the mechanism of CS cytoprotection, we examined in rats and mice the protective abilities of CS and the non-hydrolyzable ether form of CS, gamma-cholesteryloxybutyric acid, tris salt (CSE) against acetaminophen-, adriamycin-, carbon tetrachloride-, chloroform- and galactosamine-induced toxicity. The results of these studies demonstrated that CS-mediated protection is not selective for a particular species, organ system or toxic chemical. A 24-h pretreatment of both rats and mice with a single dose of CS (100mg/kg, i.p.), resulted in significant protection against the hepatotoxic effects of CCl4, CHCl3, acetaminophen and galactosamine and against the lethal (and presumably cardiotoxic) effect of adriamycin administration. Maximal CS-mediated protection was observed in experimental animals pretreated 24 h prior to the toxic insult. These data suggest that CS intervenes in a critical cellular event that is an important common pathway to toxic cell death. The mechanism of CS protection does not appear to be dependent on the inhibition of chemical bioactivation to a toxic reactive intermediate (in light of the protection observed against galactosamine hepatotoxicity). However, based on the data presented, we can not exclude the possibility that CS administration inhibits chemical bioactivation. Our findings do suggest that CS-mediated protection is dependent on the action of the intact anionic CS molecule (non-hydrolyzable CSE was as protective as CS), whose mechanism has yet to be defined.

Neuro-Behçet's disease.

We are reporting a case of Behçet's disease without vasculitis, but with acute neutrophilic inflammation which involved the brain and other organs. The patient exhibited waxing and waning neurological deficits which were unresponsive to treatment. The neuroradiologic findings simulated those of multiple sclerosis. The neuropathological examination revealed an acute, focal, though disseminated encephalitis involving the frontal lobe, internal capsule, basal ganglia, cerebellum, and brain stem. The acute inflammation consisted of a neutrophilic and eosinophilic infiltration of the perivascular spaces and parenchyma without evidence of vasculitis, fibrinoid necrosis, or thrombosis. Cultures and special stains for microbial organisms were negative. Ultrastructural examination revealed no viral structures or other microorganisms. In situ hybridization for Epstein-Barr virus (EBV), herpes simplex virus type I (HSVI), and cytomegalovirus (CMV) was negative. In this case, the lack of inflammation in the vessel walls points out that the necrotizing lesions in neuro-Behçet's disease need not be the result of vasculitis and superimposed thrombosis, but may occur as a result of primary, acute neutrophilic inflammation.

Protection against carbon tetrachloride-induced hepatotoxicity by pretreating rats with the hemisuccinate esters of tocopherol and cholesterol.

Previous studies have demonstrated that alpha-tocopheryl hemisuccinate (TS) protects hepatocyte suspensions from chemical-induced toxicity. It has been suggested that TS cytoprotection is related to unique properties of the TS molecule or is dependent on the cellular release and activity of unesterified alpha-tocopherol (T). To test the unique cytoprotective nature of TS in vivo, the protective ability of T and tocopherol esters against carbon tetrachloride (CCl4)-induced hepatotoxicity in rats was examined. Hepatoprotection [determined by serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels and histopathology] was not observed after T (or tocopheryl acetate and tocopheryl nicotinate) administration, even though this treatment resulted in a fivefold elevation in hepatic T content. Only pretreatment with TS (100 mg/kg, intraperitoneally) resulted in partial hepatoprotection against CCl4 (2.9 g/kg, orally) toxicity. These findings suggest that hepatoprotection results not from the cellular accumulation of T but rather from the intact TS molecule. To test this hypothesis, the hepatoprotective capacity of cholesteryl hemisuccinate (CS), unesterified cholesterol, and cholesteryl acetate (CA) was examined against CCl4 toxicity. As observed with the tocopherol derivatives, pretreatment with unesterified cholesterol or CA demonstrated no protective ability. However, when rats were pretreated with CS (100 mg/kg), the hepatotoxic effects of CCl4 (elevated serum AST and ALT levels and centrilobular necrosis) were completely prevented. The prevention of CCl4-induced hepatotoxicity by CS and TS do not appear to result from an alteration in hepatic drug metabolism. These data clearly demonstrate that CS and TS are unique and powerful cytoprotective agents against CCl4 hepatotoxicity in vivo.(ABSTRACT TRUNCATED AT 250 WORDS)

Successful transplantation of 50 single unit pediatric kidneys ages 11 to 48 months into adult recipients.

There is currently an imbalance between the need for cadaveric kidneys for transplantation and the supply. The medical criteria for accepting cadaveric donors are changing and organs that were originally thought to be unacceptable have functioned well. Previous reports have discussed the problems with transplanting pediatric allografts less than 4 years old into adult recipients, and the results have not been encouraging. From 1986 to 1991 a total of 50 kidneys ages 11 to 48 months were transplanted as single units into adult recipients (Group A). Ninety-one adult donor cadaveric transplants were used as controls (Group B). The cadaveric transplants were 2nd or 3rd transplants in 7 of the Group A and 12 of the Group B patients. Renal preservation, storage times, and demographics were the same. Prednisone, cyclosporine, and either Minnesota ALG or OKT3 were used for immunosuppression in both groups. Imuran was added in immunologically high-risk patients. The 1-year actuarial patient and allograft survivals for Group A versus Group B were 89.5% versus 94.2% (p=0.49) and 71.3% versus 87.8% (p=0.01), respectively. There was no difference in allograft or patient survival in kidneys from donors 11-24 months of age or 25-48 months (p=0.56). Renal growth, as measured by sonography, occurred while on cyclosporine A. Excretory and hormonal function as measured by creatinine and hematocrit both improved. Seventy percent of the Group A patients and 76% of the Group B patients were free from rejection in the first 2 months post transplantation (p=0.45).(ABSTRACT TRUNCATED AT 250 WORDS)

Systematic transrectal ultrasound guided prostate biopsy after negative digitally directed prostate biopsy.

The diagnostic accuracy of digitally directed prostate biopsies was evaluated by performing systematic ultrasound guided biopsies on 44 patients who had previously had a negative prostate biopsy by systematic digitally directed technique. All 44 patients still had palpable abnormalities. Core biopsies (2 to 4) were obtained from each lateral lobe of the prostate with 2 additional cores directed at hypoechoic lesions. Only 4 of the 44 ultrasound directed biopsies (9.1%) were positive for cancer. These results confirm the postulate that random systematic digitally directed biopsy is highly accurate in making the diagnosis of prostate cancer. They also suggest that performing biopsies in a systematic pattern is more important than the method used to guide the biopsy needle.

Technetium-99m-methylene diphosphonate (MDP) uptake in a sympathetic effusion: an index of malignancy and a review of the literature.

We report a patient with a sympathetic pleural effusion secondary to T-cell lymphoma that accumulated the bone imaging agent, Technetium-99m-methylene diphosphonate (99mTc-MDP). This case is significant in that malignant cells were not present on three cytologic examinations of the pleural fluid or multiple pleural biopsies. We also present a review of the published literature on pleural effusions that accumulate bone tracers. We conclude that pleural effusions that accumulate 99mTc-MDP should be considered malignant or secondary to a malignancy and further work-up is essential even if the cytologic exam of the pleural fluid is unremarkable.

Gallium localization in dissecting aortic aneurysm.

Gallium concentration was demonstrated in a dissecting aneurysm of the aortic arch, imaged approximately 2 weeks after dissection. Concentration of gallium was apparently due to the inflammatory reaction associated with the organizing intramural hematoma.

Cushing's syndrome due to ectopic ACTH production: cutaneous manifestations.

Ectopic production of adrenocorticotrophic hormone (ACTH) usually results in hypokalemic myopathy but is occasionally associated with florid Cushing's syndrome. We present a patient with Cushing's syndrome associated with a small-cell undifferentiated lung carcinoma. We describe the skin changes observed in this patient and review the cutaneous manifestation of Cushing's syndrome, and present current knowledge of the effects of glucocorticoids on the skin.

Different humidification systems for high-frequency jet ventilation.

This study examined the effect of several different high-frequency jet ventilation (HFJV) humidification techniques on tracheobronchial mucosa. Six groups (2 in each group) of mongrel dogs, chronically instrumented, were cared for in an intensive care-like setting for data acquisition. Four groups received HFJV with different humidification systems during 72 h of continuous ventilation. Two groups served as controls (conventional ventilation with and without humidity). Although there was significant damage to tracheal mucosa during ventilation without humidification, there were no significant pathologic differences in bronchial mucosa between humidified and nonhumidified groups. Several techniques of HFJV humidification produced no pathologic evidence of mucosal damage and could be clinically useful.

Pathologic findings in mucopolysaccharidosis type IIIB (Sanfilippo's sydnrome B).

The pathologic changes in a rare case of mucopolysaccharidosis (MPS) type IIIB or Sanfilippo's syndrome B (absence of alpha-N-acetylglucosaminidase) are presented, along with the biochemical findings. Comparisons were made with other reported cases of MPS III subtypes and related storage disorders in terms of clinical, light microscopic, electron microscopic, and chemical findings, and a correlation of the ultrastructural changes made with the severe neurological dysfunction noted in this disorder. At present, MPS III subtypes cannot be separated from one another by morphological means because the same expression and distribution of lesions may be encountered among differing subtypes.