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Lethal arthrogryposis multiplex congenital (fetal akinesia deformation sequence, FADS).

Arthrogryposis multiplex congenital (AMC) is the presence at birth of multiple congenital contractures in an intact skeleton. The severity of the condition is highly variable and the possible underlying causes are numerous. Fetal immobility and lesions of the brain, spinal cord, peripheral nerves and muscle, along with mechanical restriction of the fetus in utero are the pathogenic mechanisms that need to be considered. Etiological factors that have been implicated in the development of AMC include genetic conditions, infections, drugs, toxins, maternal hyperthermia, and maternal illness. This review will concentrate on the severe end of the spectrum of AMC that results in disease that is lethal pre- or postnatally, and will discuss the pathology, pathogenesis, etiology, and practical approach to this diversely expressed condition.

Arthrogryposis↗

Purification and general characterization of FAD synthetase from rat liver.

Flavin adenine dinucleotide synthetase (ATP:FMN adenylyltransferase, EC 2.7.7.2) has been enriched more extensively than previously from fresh rat liver. For this, 10% homogenates in sucrose-phosphate buffer were treated with 0.1% Tween-20 prior to high-speed centrifugation to obtain soluble proteins. Those precipitated by 40% saturation with ammonium sulfate were subjected to stepwise addition of calcium phosphate gel to remove pyrophosphatase, and the remaining synthetase was further enriched by passage through a tricalcium phosphate column. An apparent yield of greater than 70% and purification over 70-fold was achieved from the high-speed supernatant fraction. The synthetase activity in solution at 4 degrees was largely lost within a week unless protected by thiols which could partly restore inactivated enzyme. The pH optimum for synthetase activity is near 7.7 when assayed with suitable concentrations of FMN, ATP, and Mg2+. Purified enzyme could be separated into lower (140,000) and higher (325,000) molecular weight components when subjected to molecular sieving on a Sephadex G-200 column.

Animals↗

The costs and consequences of management fads and politically driven regulatory oversight.

CONTEXT: The period from the mid-1980s to the present has seen a virtual revolution in how blood collection, testing, and transfusion are carried out. Most of the changes that have occurred represent changes in organization, underlying paradigms of transfusion safety, and approaches to solutions to perceived problems, rather than technical or scientific advances. These changes were instituted with little or no data to support their safety and efficacy, much less their cost-effectiveness. DESIGN: Using data on changes in fees charged by regional blood centers during this period, an estimate was generated of the costs attending the purely administrative and organizational changes in the field of transfusion medicine. RESULTS: The additional expenditures, excluding inflation, are in the range of $226 million per year. This figure does not include changes in infectious disease testing on donated blood, the substantive scientific advance during this period. CONCLUSIONS: In addition to the questions of safety and efficacy, which are traditionally considered prior to clinical or procedural changes of this magnitude, the question remains whether these expenditures are a reasonable trade-off of cost versus benefit. Similar applications of federal regulatory standards intended for manufacturing (eg, good manufacturing practices) and industrial management theory (eg, total quality management) are now being implemented or proposed for hospitals, health care delivery systems, and individual physician's practices. Thus, the case of transfusion medicine may provide some cautionary lessons for future proposed changes in health care delivery and medical practices.

Blood Transfusion↗

Just-in-time and stockless programs for hospitals: fad or trend?

The JIT and stockless approach to provider-supplier relationships has proven to be a win-win proposition for the partners that have implemented it in many manufacturing industries and health care organizations as well. This strategy will fundamentally impact the entire cost structure within the hospital supply distribution chain. rewards have proven attainable and more comprehensive than had been hoped in the health care applications. The sweeping changes the health care industry experienced during the 1980s are leading creative materiel managers to seize the initiative to improve the current operating costs of their hospitals. They do not want to be left behind "holding the inventory."

Commerce↗