We have established the first prospective, collaborative study of spinal muscular atrophy, the second most common neuromuscular disease of childhood. One hundred and forty-one patients have been evaluated on at least four occasions over a 3-year period. The patients have been grouped by age of onset, as well as by function at the time of initial evaluation. The muscle strength of 96 patients aged 5 years or older was evaluated at 6-month intervals using a fixed myometry system. The new observations made are: (1) The present classification schema is not valid; for example, 49 patients with onset of weakness before 6 months of age (type I or Werdnig-Hoffmann disease), whose life span is said to be only 2 to 4 years, participated in the study and are 4 months to 31 years of age. (2) Thirty-seven patients were evaluated over an 18-month period. None lost strength during this time but four lost function. Although the period of observation was short, the results suggest that the loss of function in patients with spinal muscular atrophy might be explained by a process other than cell death that allows patient strength to be maintained and simultaneously prevents the motor unit from achieving its normal adult potential. ( J Child Neurol 1992;7:347-353).
Background A positive urine culture often drives initiation of antimicrobials even in the absence of symptoms. Our objectives were to evaluate the knowledge and practice patterns related to ordering urine cultures in patients with indwelling urinary catheters. Methods We performed chart reviews of catheter-associated urinary tract infections (CAUTIs) at our academic health care system between October 1, 2015, and September 30, 2017, to assess practice patterns related to the assessment of potential CAUTIs. Following this, we surveyed physicians and nurses about indications for ordering urine cultures in catheterized patients between January 11, 2018, and April 17, 2018. The accuracy of these indications was assessed based on Infectious Diseases Society of America CAUTI and asymptomatic bacteriuria guidelines. Results On chart review, we identified 184 CAUTIs in 2 years. In 159 episodes (86%), urine cultures were ordered inappropriately. In 114 episodes (62%), CAUTI criteria were met by “pan-culturing” rather than symptom-directed testing. Twenty cases (11%) experienced partial or delayed management of other infections, drug adverse events, and Clostridioides difficile infections (CDIs). On our survey, we received 405 responses, for a response rate of 45.3%. Mean scores varied by occupation and level of training. Nurses were more likely than physicians to consider change in appearance (61% vs 23%; P < .05) and odor (74% vs 42%; P < .05) of urine as indications to order urine cultures. Conclusions Our data reveal specific knowledge gaps among physicians and nurses related to ordering urine cultures in catheterized patients. The practice of pan-culturing and inappropriate urine culture orders may contribute to overdiagnosis of surveillance CAUTIs, delay in diagnosis of alternative infections, and excess CDIs.
Thirteen male patients (average age, 15.6 years) underwent intraarticular hip arthrodesis for unilateral disabling hip disease. Follow-up averaged 6.6 years. Seven patients had excellent or good Harris hip scores. Ten patients had low back pain, and seven patients had knee pain on the side of the fused hip. Limb-length discrepancy averaged 3.7 cm. A progressive hip adduction drift (average, 7 degrees) occurred during follow-up. Patients whose hips were fused in a position of 20-25 degrees flexion and whose limb-length discrepancies were < 2 cm had significantly lower incidences of back pain. Cybex muscle testing revealed significantly decreased quadriceps strength (29%) on the hip-fusion side. Gait laboratory analysis demonstrated decreased velocity, stride length, and cadence. There were two mildly symptomatic nonunions. Based on these results, we recommend that the hip fusion be positioned in 20 degrees flexion and 0 degree abduction. The joint must be completely debrided down to viable bone to maximize chances for union. Maintaining a limb-length discrepancy of < 2 cm is essential to minimize the incidence of low back pain, quadriceps deficiency, and abnormal gait parameters.
Background: Solid organ transplant recipients are at increased risk of COVID-19associated morbidity and mortality. Aims: We describe a nosocomial outbreak investigation on an immunocompromised inpatient unit. Methods: Patients positive for SARS-CoV-2 were identified. An epidemiologic investigation was assisted with whole genome sequencing of positive samples. Results: Two patients were identified as potential index cases; one presented with diarrhea and was initially not isolated, and the other developed hypoxemia on hospital day 18 before testing positive. Following identification of a SARS-CoV-2 cluster, the unit was closed and all patients and staff received surveillance testing revealing eight additional positive patients and staff members. Whole genome sequencing confirmed an outbreak. Enhanced infection prevention practices mitigated further spread. Asymptomatic patients with COVID-19 were successfully treated with bamlanivimab. Discussion: Preventing SARS-CoV-2 outbreaks in transplant units poses unique challenges as patients may have atypical presentations of COVID-19. Immunocompromised patients who test positive for SARS-CoV-2 while asymptomatic may benefit from monoclonal antibody therapy to prevent disease progression. All hospital staff members working with immunocompromised patients should be promptly encouraged to follow infection prevention behaviors and receive SARS-CoV-2 vaccination. Conclusion: SARS-CoV-2 outbreaks on immunocompromised units can be mitigated through prompt identification of cases and robust infection prevention practices.
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