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Biomedical subjects

C Shear

Publications and source records attributed to C Shear.

5 recordsLinked to original sources

Phenylketonuria in adulthood: a collaborative study.

During 1967-1983, the Maternal and Child Health Division of the Public Health Services funded a collaborative study of 211 newborn infants identified on newborn screening as having phenylketonuria (PKU). Subsequently, financial support was provided by the National Institute of Child Health and Human Development (NICHD). The infants were treated with a phenylalanine (Phe)-restricted diet to age 6 years and then randomized either to continue the diet or to discontinue dietary treatment altogether. One hundred and twenty-five of the 211 children were then followed until 10 years of age. In 1998, NICHD scheduled a Consensus Development Conference on Phenylketonuria and initiated a study to follow up the participants from the original Collaborative Study to evaluate their present medical, nutritional, psychological, and socioeconomic status. Fourteen of the original clinics (1967-1983) participated in the Follow-up Study effort. Each clinic director was provided with a list of PKU subjects who had completed the original study (1967-1983), and was asked to evaluate as many as possible using a uniform protocol and data collection forms. In a subset of cases, magnetic resonance imaging and spectroscopy (MRI/MRS) were performed to study brain Phe concentrations. The medical evaluations revealed that the subjects who maintained a phenylalanine-restricted diet reported fewer problems than the diet discontinuers, who had an increased rate of eczema, asthma, mental disorders, headache, hyperactivity and hypoactivity. Psychological data showed that lower intellectual and achievement test scores were associated with dietary discontinuation and with higher childhood and adult blood Phe concentrations. Abnormal MRI results were associated with higher brain Phe concentrations. Early dietary discontinuation for subjects with PKU is associated with poorer outcomes not only in intellectual ability, but also in achievement test scores and increased rates of medical and behavioural problems.

Adult↗

Correlation of non-high-density lipoprotein cholesterol with apolipoprotein B: effect of 5 hydroxymethylglutaryl coenzyme A reductase inhibitors on non-high-density lipoprotein cholesterol levels.

Apolipoprotein B has been shown to be a better predictor of coronary heart disease than low-density lipoprotein (LDL) cholesterol, and non-high-density lipoprotein (non-HDL) cholesterol may also be a better parameter for coronary heart disease risk assessment and as a target for therapy. Data from the Atorvastatin Comparative Cholesterol Efficacy and Safety Study (ACCESS) were used to assess the correlation between lipid and apolipoprotein B levels before and after lipid-lowering therapy and to examine the effects of 5 hydroxymethylglutaryl coenzyme A reductase inhibitors on lipids and apolipoprotein B. The 54-week study randomized 3,916 hypercholesterolemic patients to atorvastatin, fluvastatin, lovastatin, pravastatin, or simvastatin, initiated at recommended starting doses with titrations as needed at weeks 6, 12, and 18 to achieve National Cholesterol Education Program LDL targets. Compared with LDL cholesterol, non-HDL cholesterol correlated better with apolipoprotein B levels at baseline (r = 0.914, p <0.0001) and at week 54 (r = 0.938, p <0.0001), and the correlation was strong across all baseline triglyceride strata. At starting doses, atorvastatin (10 mg) lowered non-HDL cholesterol by 33.3% compared with 26.6% with simvastatin (10 mg), 24.1% with lovastatin (20 mg), 17.2% with fluvastatin (20 mg), and 17.0% with pravastatin (10 mg). Atorvastatin also provided greater reductions in non-HDL cholesterol after dose titration, and a greater percentage of patients taking atorvastatin achieved non-HDL cholesterol targets. Baseline triglyceride did not affect non-HDL cholesterol reductions with any of the 5 hydroxymethylglutaryl coenzyme A reductase inhibitors. Fewer patients achieved non-HDL cholesterol targets than LDL cholesterol targets, particularly among high-risk patients, implying that if non-HDL cholesterol was used as a target for treatment, more patients would need to be treated more aggressively than National Cholesterol Education Program guidelines require.

Anticholesteremic Agents↗

Field test reliability of a new lens opacity rating system utilizing slit-lamp examination.

A field test of a lens opacity rating system was run. Findings were graded in 7 categories: Cortex: (1) Waterclefts, Vacuoles, & Flakes (WVF, 4 grades), (2) Wedges & Spokes graded by number of Quadrants affected (WS-Q), and (3) Inward extent of Wedges & Spokes (WS-I, 3 grades); Nucleus: (4) Opacification (NUC-O) and (5) Coloration (NUC-C), each using 4 grades; and Subcapsular areas: (6) Anterior (SUB-A) and (7) Posterior (SUB-P) opacification, each using 5 grades. An instruction booklet with corresponding guidelines, sketches, and photographs was developed. Reproducibility of grading was tested in 3 clinics using 16 ophthalmologist-examiners. At each clinic, examiners were randomly paired. They independently performed slit-lamp exams of assigned patients (9 on average) on the same day (inter-examiner) and 1-2 weeks later (intra-examiner). To test intra-examiner reliability, examiners were divided into two groups (one from each pair), with gradings analyzed by group. For each group, 53 to 58 patients had evaluable examinations. Intra-examiner concordance for each lens category (defined as at most one grade difference in one lens) was as follows (for examiner groups 1 and 2, resp.): WVF: 98% & 97%; WS-Q: 96% & 100%; WS-I: 100% & 100%; NUC-O: 98% & 100%; NUC-C: 100% & 98%; SUB-A: 90% & 100%; and, SUB-P: 92% & 100%. Inter-examiner concordances were slightly lower than those found for intra-examiner. Grading lens findings in the manner described above appears to have potential utility in large scale studies.

Aged↗