Early-infantile galactosialidosis: prenatal presentation and postnatal follow-up.

Galactosialidosis (GS) is an autosomal recessive condition caused by combined deficiency of the lysosomal enzymes beta-galactosidase and alpha-neuraminidase. The combined deficiency has been found to result from a defect in protective protein/cathepsin A (PPCA), an intralysosomal protein which protects these enzymes from premature proteolytic processing. The most severe form of GS, the early-infantile form, results in early onset of edema, ascites, visceromegaly, and skeletal dysplasia. We report a case of early-infantile GS in a male infant who presented with nonimmune fetal hydrops (NIH), "coarse" facial appearance, massive fluid-filled inguinal hernias, multiple telangiectasia, and diffuse hypopigmentation; he subsequently developed visceromegaly. The diagnosis of GS was confirmed biochemically and the defect in PPCA characterized at the protein level. Examination of fetal peripheral blood smears sampled at 30 weeks gestation demonstrated vacuolation of lymphocytes, suggesting blood film examination may be a useful screening tool for cases of NIH where a metabolic disorder is suspected. Skeletal radiography at birth demonstrated punctate epiphyses of the femora, calcanei, and sacrum. We present a discussion of and differential diagnosis for this radiographic finding. To the best of our knowledge, this is the first case of early-infantile GS presenting with stippled epiphyses.     

Transitions From Skilled Nursing Facility to Home: The Relationship of Early Outpatient Care to Hospital Readmission

Background

Many adults are discharged to skilled nursing facilities (SNFs) prior to returning home from the hospital. Patient characteristics and factors that can help to prevent postdischarge adverse outcomes are poorly understood.

Physician recruitment: guidelines for a safer tomorrow

As hospitals are challenged to recruit and retain qualified medical staff, they must continually consider the legal ramifications of their actions. Developing a physician-hospital alliance may conceptually be an excellent idea within a medical-service area, but rulings by the Office of the Inspector General and the Internal Revenue Service can challenge the decisions of hospital administrators and boards. Writing for the National Institute of Physician Recruitment and Retention, Roger G. Bonds and Michael R. Callahan address legal ramifications of physician-hospital alliances.     

Two-dimensional echocardiographically guided pericardiocentesis: experience in 117 consecutive patients

Pericardiocentesis guided by 2-dimensional echocardiography has been used at the Mayo Clinic since April 1980. The 2-dimensional examination localizes the pericardial fluid. Particular note is made of the place on the body wall closest to the fluid. An entry track that permits puncture of the pericardial sac without damage to any vital structure is then selected for the pericardiocentesis needle. Between April 1980 and March 1984, 132 consecutive pericardiocenteses in 117 patients were done by this technique. The volume of fluid obtained ranged from 75 to 1,700 ml (mean 650). Seventy percent of the taps were done for therapy, 21% for diagnosis, and 9% for both therapy and diagnosis. A Teflon-sheathed "intracath" needle was used to complete 80% of the pericardiocenteses. In the other 20%, a large catheter was secondarily introduced and connected to a closed drainage system. There were no deaths related to the procedure. One symptomatic pneumothorax occurred. There were 3 minor complications. Two-dimensional echocardiographic imaging of the heart and pericardial fluid permits a safe and effective means of performing pericardiocentesis.     

Physician report cards and rankings yield long-lasting hand hygiene compliance exceeding 90?%

IntroductionHand hygiene is an effective, low-cost intervention that prevents the spread of multidrug-resistant bacteria. Despite mandatory education and reminders, compliance by physicians in our hospital remained stubbornly low. Our objective was to study whether surveillance by our unit coordinator (secretary) paired with regular feedback to chiefs of service would increase physician hand hygiene compliance in the ICU.MethodThe ICU unit coordinator was trained to observe and measure hand hygiene compliance. Data were collected on hand hygiene compliance at room entry and exit for 9 months. Percentage compliance for each medical and surgical subspecialty was reported to chiefs of service at the end of each month. Comparative rankings by service were widely distributed throughout the physician organization and the medical center.ResultsThe hand hygiene compliance rate among physicians increased from 65.1 % to 91.6 % during the study period (p <0.0001). More importantly in the succeeding 24 months after study completion, physician hand hygiene compliance remained >90 % in every month.ConclusionsPhysician hand hygiene compliance increased as a consequence of the surveillance conducted by a full-time ICU team member, leading to a highly significant increase in the number of observations. In turn, this allowed for specific comparative monthly feedback to individual chiefs of service. Over the next 2 years after the study ended, these gains were sustained, suggesting an enduring culture change in physician behavior.