Report of a case of Incontinentia pigmenti (IP) of the Bloch-Sulzberger type, with neurological features, in a 3 month-old deceased infant. The neuropathological examination revealed signs of a destructive process which had occurred in the perinatal period: ulegyria, cavities of white matter and patchy scar softening of the cerebellar cortex. In addition, there was a diffuse inflammatory process involving the pia-arachnoid and brain tissue: perivascular cuffs of lymphocytes, histiocytes and eosinophilic polymorphs, and mononuclear nodules. No prenatal malformations or vascular lesions were found. These findings are discussed in the light of both the relevant literature on neurological abnormalities in IP and the only already reported neuropathological examination by O'Doherty and Norman (1968). Two main hypotheses might account for the frequency of neurological disturbances in IP, 1. a peculiar sensitivity of the brain to anoxia and/or ischemia: 2. its involvement by the specific inflammatory process which occurs both in the skin as other tissues in this genetically determined disease (Perié et al., 1976).
This paper highlights some of the problems associated with lipid therapy in the primary and secondary prevention of cardiovascular disorders and to make some potentially useful suggestions in the context of managed care. For managed care organizations, financial and logistical issues create obstacles to the provision of primary prevention of cardiovascular disease. These current obstacles necessitate the generation of external forces, perhaps regulatory or standards agencies, that may help increase accountability in managed care organizations for midterm and distant outcomes. In contrast, the provision of secondary prevention by managed care organizations has fewer limitations. One of the major challenges in secondary prevention, however, is the low rate of physician compliance with national treatment guidelines and standards. Among possible explanations for this observation are limitations in health data collection and integration. Improvements in data management are vital to the achievement of treatment goal optimization in secondary prevention.
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