The Seminole Indians of Oklahoma: morphology and serology.
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Three sisters, each with chondrocalcinosis/arthropathy, are described who have the clinical, laboratory and pathologic findings characteristic of the adult form of hypophosphatasia. Premature loss of adult teeth, arthralgias and pain from bilateral femoral pseudo-fractures were associated with subnormal circulating alkaline phosphatase levels, phosphoethanolaminuria and osteomalacia diagnosed by iliac crest biopsy. Assay of alkaline phosphatase activity in the blood of kindred members revealed hypophosphatasemia in one of two younger brothers. Several subjects in subsequent generations also had suspiciously low alkaline phosphatase activity, but did not have histories of significant dental, bone or joint disease. Review of the medical records of the sisters' parents, aunts and uncles revealed normal alkaline phosphatase levels in their father and five of his siblings, but consistently low levels in their mother and two of her siblings. Despite hypophosphatasemia, the sisters' mother and her siblings lived to old age without clinical or radiographic evidence of bone disease. Our findings suggest that although adult hypophosphatasia can be transmitted as a dominant trait in some kindreds, there is considerable variation in the clinical expression of the biochemical defect. One person, generation or family may manifest clinical bone disease and arthropathy whereas the biochemical defect may be present but remain asymptomatic in others. Furthermore, in some cases, the adult form of hypophosphatasia may represent a developmental disorder with hypophosphatasemia appearing during adulthood.
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BACKGROUND: In a previous cross-sectional study of age-associated peripheral neuropathy (AAPN), we found that a history of hypertension was protective. The purpose of this study, conducted in the same cohort, was to investigate further this association in the same subjects and in a comparison group of older subjects with diabetes mellitus. METHODS: Baseline data from 584 subjects involved in a longitudinal study of primary care patients 65 years of age and older, with no history of 10 medical conditions known to cause peripheral neuropathy, were analyzed for associations between peripheral neuropathy by examination and history of hypertension, number of antihypertensive medications, systolic blood pressure, diastolic blood pressure, pulse pressure, and orthostatic hypotension. In addition, we examined associations between neuropathy at baseline and use of specific classes of antihypertensive medications and non-steroidal anti-inflammatory agents (NSAIDs) in year 3. The analyses were repeated in 110 subjects with diabetes mellitus. RESULTS: History of hypertension, but not the other hypertension-related variables, was negatively associated (OR, 0.60; 95% CI: 0.40 to 0.90) with AAPN after controlling for age and body mass index (BMI). In 287 subjects evaluated in the 3rd year of the study, beta-blocking agents (OR, 3.56; 95% CI: 1.58 to 8.03) and NSAIDs (OR, 2.65; 95% CI: 1.37 to 5.10) were positively associated with AAPN. In subjects with diabetes mellitus, a history of hypertension was again protective, but current pulse pressure (OR, 1.03; 95% CI: 1.00 to 1.05) was a positive predictor of peripheral neuropathy. There were interesting interactions between pulse pressure and military service and pulse pressure and BMI in diabetic patients. CONCLUSIONS: The negative association between hypertension and AAPN remains unexplained. The positive association between pulse pressure and neuropathy in diabetic subjects supports findings from previous studies and suggests that AAPN and the neuropathy of diabetes may be distinct entities. The incidental finding of an association between NSAIDs and AAPN is concerning and should be investigated further.
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Hepatitis C is a growing public health concern in the United States. All physicians should be aware of who should be tested for HCV, based upon risk factors for infection, as well as how to manage persons found to have positive screening tests. The following recommendations focus primarily on the role of the primary care physician in the initial evaluation and management of persons who test positive for HCV and are excerpted from the Centers for Disease Control document "Recommendations for Prevention and Control of Hepatitis C Virus (HCV) Infection and HCV-Related Chronic Disease," Oct. 16, 1998/47(RR19); 1-39.
This notice describes requirements for the review activities of Utilization and Quality Control Peer Review Organizations (PROs) for the next contract cycle in the States listed above. Section 1153(h)(1) of the Social Security Act requires us to publish any new policy or procedure adopted by the Secretary that affects substantially the performance of PRO contract obligations at least 30 days before the date the policy or procedure is to be used. Specifically, this notice describes significant changes in the way in which cases will be selected for review and also describes continuing requirements. This notice also implements provisions of the Omnibus Budget Reconciliation Act of 1990 and announces the future direction of the PRO program.
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