The New England journal of medicine
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Our previous studies predicted a functional relationship between the plasma proteins alpha 1-antitrypsin and antithrombin III. To elucidate this relationship we investigated the plasma of a 14-year-old boy who had died from an episodic bleeding disorder. A variant alpha 1-antitrypsin was identified in which the methionine at position 358 had been replaced by an arginine. ⋯ This finding indicates that the reactive center of alpha 1-antitrypsin is methionine 358, which acts as a bait for elastase, just as the normal reactive center of antithrombin III is arginine 393, which acts as a bait for thrombin. The independence of the new thrombin inhibitor from heparin control explains the bleeding disorder; it also indicates that heparin normally acts directly on antithrombin III, revealing its inherent inhibitory activity. The episodic nature of the bleeding was a consequence of the mutant protein's being an acute-phase reactant, the level of which increased several-fold after trauma.
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Pediatric nursing homes were established in Massachusetts in 1971, in response to the crisis created by the deinstitutionalization of children with multiple severe handicaps for whom care in the community was unrealistic. Although these residential beds provided short-term relief for the system, failure both to plan for the improved long-term survival of the children and to develop alternative community services for those whose functional and medical status changed has left fundamental problems unresolved. A review of the 421 residents of pediatric nursing homes since 1972 shows that most have been bedridden and nonverbal, requiring provision of basic care: feeding, dressing, maintaining hygiene, and sensory and motor stimulation. ⋯ About 4 per cent died each year, and another 5 per cent were discharged; few beds were available for new admissions. Low standards of care and financial disincentives have resulted in less than optimal educational and rehabilitative services. Other states are facing similar problems, which can be resolved only through better funding and coordination of the relevant state agencies.