Use of intravenous immunoglobulins (IVIg) to treat a child with pancytopenia and hypoplastic marrow.
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Biomedical subjects
Publications and source records attributed to J M Levy.
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Sixteen patients seen over a 9-month period ending in August 1990 were offered transcatheter closure of their ASD with a custom-made "buttoned" double-disc device. The study was approved by the Institutional Review Board and informed consent was obtained in each case. The device consists of an occluder, a counteroccluder, and a loading wire and is delivered to the ASD site via an 8F sheath. Parents of two children elected surgical closure. In five children the stretched diameter of the ASD was too large (greater than 20 mm) and transcatheter closure was not attempted. These seven children underwent elective surgical closure without incident. In one child the defect measured 5 mm and the Qp:Qs was 1.4:1 and therefore ASD closure was not recommended. In the remaining eight children transcatheter closure was attempted. In two of the children the occluder pulled through the ASD and was successfully retrieved and the children later underwent uneventful elective surgical closure. The device was implanted across the ASD in six children. In one child the device dislodged from the ASD site within minutes after implantation and the child was sent to emergency surgery, where the device was removed and the ASD was closed. In the remaining five patients, aged 7 months to 45 years (weight 3.6 to 50 kg), with a Qp:Qs range of 1.3 to 2.3 and a stretched diameter of 10 to 19 mm, the ASD closure was successful with 25 to 40 mm size devices. Repeat echo-Doppler studies 2 weeks and 3 months after the procedure in all patients and 6 months later in two children did not reveal any residual shunt. It is concluded that (1) the custom-made "buttoned" double-disc device can be implanted across the ASD safely and effectively via an 8F sheath, thus making transcatheter ASD closure feasible even in very young infants; (2) measurement of stretched diameter of the ASD in the catheterization laboratory is a useful guide for selection of an appropriate-sized device; and (3) additional clinical trials are warranted to confirm the efficacy and safety of the device.
There is little information available about the provision of supported employment services for individuals from diverse cultural, ethnic and economic backgrounds. To fill this gap, in relation to the specific experiences of urban youth with mental retardation, we initiated an agency based longitudinal study of our employment training programmes. Data from the first year follow up of 45 young adults with mental retardation is presented. Information regarding all phases of the job training and job placement process was examined. The major findings are that 71% of the trainees were employed during the first nine months post-training, with approximately 30% of the trainees working for six months or more. Overall, 75% of the trainees' placements were in service industries and the amount of direct support the trainees required decreased over time. Additional findings and their implications for programme development are discussed in relation to the unique needs of urban young adults with developmental disabilities.
On January 1, 1992, the Medicare program unveiled a new method for paying physicians known as the Medicare Fee Schedule (MFS). The new fee schedule is a complex system of administrative pricing based on the resource inputs used in producing physician services. The MFS consists of three parts: 1) a Relative Value Scale, which assigns to each medical service a value relative to all other services; 2) a conversion factor, which converts the relative values into dollars; and 3) a geographic adjustment factor, which adjusts payments based on geographic differences in the cost of producing physician services. In this article, the following are addressed: how the relative values were determined; how the geographic adjustment factor was constructed; and how the conversion factor was calculated. In addition, balance billing limits and the Medicare Volume Performance Standards (MVPS) are described. Computer simulations of the impact of the MFS on payments to physicians are presented. The authors found that the MFS will 1) redistribute payments away from surgeons, radiologists, and other procedure-based specialties toward the primary care specialties; 2) redistribute payments away from urban areas toward rural areas; and 3) redistribute payments away from invasive procedures and diagnostic tests toward evaluation and management services. The authors conclude with a discussion of the future refinements of the MFS, its applicability to other payers, and whether it will accomplish its intended purposes.
Executives (N = 341) responsible for hiring decisions in Fortune 500 industrial and service corporations returned a mail questionnaire measuring their attitudes toward persons with severe disabilities and their employability. Responses indicated that attitudes were favorable to persons with disabilities and to their employability, both in terms of advantages for the individual and lack of disadvantages for others in the work setting. Significant differences in attitudes existed among subgroups of executives. Executives who had contact with persons with disabilities in the corporate work world had more positive attitudes than did executives who did not have such experiences.
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Clinical, echo-Doppler, and pathologic data of a rare case of tricuspid atresia in association with truncus arteriosus are presented. There are only six patients (including the patient reported here) with this anomaly in whom detailed pathologic and/or clinical descriptions are available in the literature. An additional six cases were mentioned in the literature but without any details. A prevalence rate of 1.4% among tricuspid atresia cases is estimated. All infants presented with symptoms of cyanosis and/or congestive heart failure within a few days to 2 months after birth and died shortly thereafter. Cardiomegaly and increased pulmonary vascular markings on the chest x-ray film and left axis deviation with left ventricular hypertrophy on the ECG are usually present. Echocardiographic, catheterization, and angiocardiographic studies are helpful in documenting anatomic and physiologic features of this anomaly. Pathologically, the muscular type of tricuspid atresia, type I or II truncus arteriosus, a large subtruncal VSD, and a hypoplastic right ventricle are present. It is concluded that coexistence of tricuspid atresia and truncus arteriosus is rare; clinical, laboratory, and pathologic features are distinctive; the clinical presentation early in life with rapid demise is common; and prompt palliative surgery immediately after recognition of this anomaly should be undertaken.
The authors discuss a patient with a large (15 x 10 x 12 cm), bony soft tissue intradural arteriovenous malformation (AVM) who presented with a 20-year history of back pain. Plain radiographs revealed a destructive lesion. Magnetic resonance imaging showed serpentine vascular structures within the thecal sac and lytic bone lesions with multiple level involvement. A Craig needle biopsy by the referring physician, as well as an open biopsy by the authors, caused severe bleeding. There was no evidence of neoplasm. Selective spinal angiography demonstrated a metameric juvenile type AVM whose intradural component was fed by the artery of Adamkiewicz. Because the patient had no neurological deficit on presentation, the options of embolization and then surgery were considered too risky. The patient is being followed conservatively.
A mail survey of the largest businesses in the US, the Fortune 500 industrial and service corporations, examined the attitudes of personnel and human resource executives towards the employability of persons with severe disabilities and actual corporate practices regarding the employment of persons with disabilities. The 341 executives who responded are found to be favourable to the employment of persons with disabilities, especially if the executives work in corporations that have hired persons with disabilities within the past three years, and/or have had personally good contact with persons with disabilities in the past. Almost two-thirds of the corporations have a policy regarding hiring persons with disabilities; over one-half have hired such persons within the past three years; and over one-third have supported work programmes.
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Beginning in 1992, the Medicare program will pay physicians by the Medicare Fee Schedule, a system of geographically adjusted standardized payment rates based in part on the Resource-Based Relative Value Scale developed by Hsaio et al and in part on current Medicare payments. In our simulations of the Medicare Fee Schedule, we find that (1) redistributions of Medicare-allowed charges across specialities will be substantial but approximately only half the size projected by Hsaio, (2) there will be large redistributions among geographic areas that tend to compound the specialty redistributions, and (3) there will be wide variation within specialties as to how individual providers are affected. The majority of the redistributive impact of the Medicare Fee Schedule is attributable to implementation of a geographically adjusted system of standardized payments rather than to the particular work values developed by Hsiao et al in the Resource-Based Relative Value Scale.
Six children with cyanotic congenital heart defects, aged 6 to 60 months, underwent percutaneous balloon angioplasty of a narrowed Blalock-Taussig (BT) shunt to improve arterial oxygen saturation. The indication for angioplasty was a cyanotic heart defect not amenable to total surgical correction, either because of the age and size at presentation or because of anatomic complexity, but at the same time requiring palliation of pulmonary oligemia. Following balloon angioplasty, there was an increase in arterial oxygen saturation from 71 +/- 8% to 81 +/- 6% (p less than 0.05). In one child with long segment narrowing, there was no significant improvement in oxygen saturation, and this child underwent an additional BT shunt on the contralateral side. On follow-up 3 to 12 months after balloon angioplasty, the oxygen saturations remained improved (78 +/- 10%) in the remaining five patients. In two children with either complete or almost complete blockage of the BT shunt, we were unable to advance any catheter across the shunt but we were able to advance a 2 or 3 mm balloon on a wire and dilate the shunt, followed by introduction of a catheter carrying a larger balloon for angioplasty. This has permitted us to obtain the pulmonary artery pressure directly (this information was of obvious value in patient management) and resulted in an improvement in the arterial oxygen saturation. It is concluded that (1) balloon angioplasty of narrowed BT shunts is feasible, effective, and safe and (2) even completely occluded shunts can be cannulated and the balloon dilated with the newly available balloon-on-wire devices.
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Sixteen infants and children with valvular aortic stenosis underwent percutaneous balloon aortic valvuloplasty over a 36-month period ending August 1988. The mean systolic pressure gradient across the aortic valve decreased from 72 +/- 21 (mean +/- standard deviation) to 28 +/- 13 mm Hg (p less than 0.001) immediately after valvuloplasty; the degree of aortic insufficiency did not significantly increase. Follow-up catheterization (in 10 patients) and Doppler data (in all 16 patients) were available 3 to 32 months (mean 12 months) after valvuloplasty and revealed a residual aortic valvular gradient of 37 +/- 23 mm Hg, which continues to be significantly lower (p less than 0.001) than that before valvuloplasty. There was no increase in aortic insufficiency. On the basis of follow-up data, the 16 children were divided into 2 groups: group I with good results (gradients less than or equal to 49 mm Hg), 12 patients; and group II with poor results (gradients greater than or equal to 50 mm Hg), 4 patients. All 4 patients in group II required repeat balloon valvuloplasty or surgical valvotomy; none from group I required these procedures. Seventeen general, anatomic, physiologic and technical variables were examined by a multivariate logistic regression analysis to identify factors associated with restenosis; these risk factors were: age less than or equal to 3 years; and immediate aortic valvular gradient after valvuloplasty greater than or equal to 30 mm Hg. The immediate and intermediate-term follow-up results of balloon aortic valvuloplasty are encouraging. Recognition of the risk factors may help identify potential candidates for recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)