Two unrelated 16-year-old boys had mental retardation, cardiomegaly, and proximal myopathy. One also had hepatomegaly. Histochemistry and electronmicroscopy of muscle biopsies showed lysosomal glycogen storage resembling acid maltase deficiency. Biochemical studies of skeletal muscle showed increased content of glycogen of normal structure; acid alpha-glucosidase activity in both urine and muscle was normal. Other enzymes of glycogen metabolism were also normal. The cause of this apparently generalized glycogenosis with no demonstrable enzyme defect is unknown.

-IFN should not be used in infants under 1 year of age unless life-threatening conditions do not respond to any other form of treatment. If -IFN must be used, children should have monthly neurologic examinations. If a motor developmental disturbance is detected and -IFN therapy can be discontinued, it should be.

The purpose of this study was to evaluate the pharmacokinetic profile of intranasal lorazepam in comparison to currently established administration routes. Eleven healthy volunteers completed this randomized crossover study. On three occasions, each separated by a 1-week washout, subjects received a 2 mg dose of lorazepam via the intranasal, intravenous, or intramuscular route. Blood samples were collected serially from 0 to 36 hours. Noncompartmental methods were used to determine pharmacokinetic parameters. Lorazepam was well absorbed following intranasal administration with a mean (%CV) bioavailability of 77.7(11.1). Intranasal administration resulted in a faster absorption rate than intramuscular administration. Elimination profiles were comparable between all three routes. The concentration-time profile for intranasal delivery demonstrated evidence of a double peak in several subjects, suggesting partial oral absorption. Females were found to have significantly higher AUC values than males for all three delivery routes. Overall, this study demonstrated favorable pharmacokinetics of intranasal lorazepam in relation to standard administration methods. Intranasal delivery could provide an alternative, noninvasive delivery route for lorazepam.

Evidence of acute cellular with occult humoral rejection is identified in more than 40% of late AR episodes. Late acute humoral rejection may be associated with interstitial hemorrhage and plasma cells and contributes significantly to graft loss.

The abuse of drugs via an intranasal route is an increasingly prevalent pattern of behavior. In the past year, a number of patients received care at our institution for complications resulting from the previously unreported phenomenon of intranasal prescription narcotic abuse. This report describes the clinical manifestations of this form of drug abuse in 5 patients. Their symptoms consisted of nasal and/or facial pain, nasal obstruction, and chronic foul-smelling drainage. Common physical findings were nasal septal perforation; erosion of the lateral nasal walls, nasopharynx, and soft palate; and mucopurulent exudate on affected nasal surfaces. In addition, 2 of the 5 patients had invasive fungal rhinosinusitis, which appears to be a complication unique to intranasal narcotic abuse.