Congenital adrenal hypoplasia and selective absence of pituitary luteinizing hormone: a new autosomal recessive syndrome

Congenital hypoplasia of the adrenal glands (CHA) is a rare condition, particularly in the absence of a central nervous system (CNS) anomaly. Two major types of CHA have been described in the setting of an apparently normal CNS and pituitary: a cytomegalic type usually with X-linked recessive inheritance and a miniature adult type that, when hereditary, is an autosomal recessive trait. Glycerol kinase deficiency (GKD) is an X-linked recessive trait, and it may be associated with CHA and adrenal insufficiency, presumably because of deletion of adjacent X-linked loci. We report on three sibling infants, one male and two females, with normal CNS and lethal CHA of the miniature adult type, selective absence of pituitary LH; two of the infants also had glycerol kinase (GK) activity that was decreased but not in the GKD range. Restriction fragment length polymorphism (RFLP) analysis of X chromosome markers located at Xp21-p22 was carried out on the maternal grandfather, both parents, two of three affected infants, and a living normal brother. The results excluded the X-linked type of this disorder associated with GKD in this family. Autosomal recessive inheritance is most likely.     

Comparison of ultrasonic cleaning schemes: a pilot study.

OBJECTIVE: Ultrasonic cleaning is an effective method for cleaning dental instruments prior to sterilisation. However, there are few studies that directly compare precleaning and ultrasonic cleaning solutions. This study evaluated the efficacy of different ultrasonic cleaning schemes. METHOD AND MATERIALS: Twenty representative dental instruments, five of which were soiled with a mixture of blood and hydroxyapatite, were used in a series of cleaning runs. Cleaning employed a presoaking agent, ultrasonic cleaning, or a combination of both. Two presoaking agents (Non-ionic Ultrasonic Cleaning Solution and ProEZ Foaming Enzymatic Spray) plus five ultrasonic cleaners (UltraDose, General Purpose Cleaner, Co-enzyme Concentrate, Enzol Enzymatic Detergent, and Non-ionic Ultrasonic Cleaning Solution) were compared, with tap water serving as a control. There were two cleaning times: seven and 15 minutes. After rinsing, the working ends of the instruments underwent scrubbing for 20 seconds using a dental polishing brush held in a haemostat. After scrubbing, the brush and instrument were placed in a tube containing sterile saline. Vortexing of the tube lasted 30 seconds. Testing for the post-cleaning presence of blood involved Hemastix dipsticks. These sticks measure minute amounts of blood in urine and can detect as few as 35 red blood cells per ml. Comparisons of colour change were made to a standard scale followed by assignment of numeric values. RESULTS: Tap water was the poorest cleaning solution, while UltraDose was the most effective. Blood removal improved when cleaning time was increased from seven to 15 minutes. The combined effect of a presoak immersion followed by ultrasonic cleaning was the most effective cleaning scheme overall. Cleaning by either ultrasound or presoaking only was less effective. Some instruments were more difficult to clean than others. CONCLUSION: Within the constraints of the small number of test runs performed, it was concluded that application of a presoak agent before ultrasonic cleaning produced the most effective instrument-cleaning regimen.     

Drug information course for pharmacy staff development.

An interactive course in drug information skills developed for pharmacists at a not-for-profit, tertiary-care hospital is described. Faculty members from the area school of pharmacy developed, taught, and evaluated the program. Before the course was developed, pharmacy staff members were asked to rate their drug information skills; the pharmacists' responses indicated their belief that they were not proficient enough in the skills needed in daily practice. The course content and format were refined after 11 pharmacists completed a pilot program. A handbook was developed that contained objectives, session outlines, and literature for each of the six topics chosen for the course. Although the handbook was the primary teaching aid, wall charts and computer demonstrations were also used. Sessions were structured for the needs of adult students by using a small-group discussion format that emphasized the practical relevance of the information and encouraged participants to share personal experiences. Each session was offered on two separate days to facilitate attendance. Those who completed the course received credit for 12 contact hours of continuing education. Of 16 pharmacists enrolled in the course, 11 completed it. An interactive course in drug information skills, developed to meet the needs of hospital pharmacists, was well accepted because it incorporated personal experiences, small-group activities, and flexible scheduling.     

Epidemiologic evidence for early onset of mental disorders and higher risk of drug abuse in young adults

Data from the National Institute of Mental Health (NIMH) Epidemiologic Catchment Area Program, an epidemiologic survey of five communities, showed that four major disorders commonly begin in late adolescence or young adulthood. The median age at onset for anxiety disorders is 15 years; for major depressive episode, 24 years; for drug abuse or dependence, 19 years; and for alcohol abuse or dependence, 21 years. Findings also suggest that for respondents 18-30 years old, having a major depressive episode or anxiety disorder doubles the risk for later drug abuse or dependence.     

Severe cerebral edema in a patient with anasarca and hypernatremia.

We describe a woman whose fatal post-liver transplantation cerebral edema was unexpected and of unusual pathogenesis. Her severe cerebral edema is of considerable pathophysiologic interest: 1) it developed in the setting of marked anasarca and persistent hypernatremia, and 2) although hepatic function was poor, it was not considered sufficiently deranged to induce cerebral edema. Furthermore, there was no histologic evidence of hepatic rejection or antemortem hepatic necrosis. We postulate that an impairment of the blood brain barrier in association with a degree of hepatic dysfunction insufficient by itself to cause cerebral edema permitted the brain interstitial fluid volume to increase pari passu with ECF expansion. Cytotoxic cerebral edema and vascular engorgement may also have contributed to a life-threatening increase in intracranial pressure.