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Biomedical subjects

E A Buck

Publications and source records attributed to E A Buck.

6 recordsLinked to original sources

Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of 418 cases of sudden death in the first year of life.

OBJECTIVE: Fatty acid oxidation (FAO) disorders are frequently reported as the cause of sudden and unexpected death, but their postmortem recognition remains difficult. We have devised a biochemical protocol in which informative findings in liver tissue are microvesicular steatosis, elevated concentrations of C8-C16 fatty acids, glucose depletion, and low carnitine concentration. STUDY DESIGN: We analyzed 27 cases representing five FAO disorders and compared the results with those obtained in a retrospective blinded analysis of 418 cases of sudden infant death (313 SIDS, 45 infections, and 34 accidents and abuse). RESULTS: All cases of accidents and abuse correctly tested negative. Among the others, 25 (6%) showed at least two abnormal findings. Of these, 14 closely matched the biochemical profiles seen in specific FAO disorders. These included 2 cases with medium-chain acyl-CoA dehydrogenase deficiency, 4 cases consistent with glutaric acidemia type 2, 4 cases with either very long-chain acylcoenzyme A dehydrogenase deficiency or long-chain 3-hydroxy-acyl-coenzyme A dehydrogenase deficiency, and 4 cases predicted to be affected with carnitine uptake defect. CONCLUSION: The results of this study support the view that approximately 5% of all cases of sudden infant death are likely caused by an FAO disorder.

3-Hydroxyacyl CoA Dehydrogenases

Cloning, expression, and chromosomal assignment of the human mitochondrial intermediate peptidase gene (MIPEP).

The mitochondrial intermediate peptidase of Saccharomyces cerevisiae (YMIP) is a component of the yeast mitochondrial protein import machinery critically involved in the biogenesis of the oxidative phosphorylation (OXPHOS) system. This leader peptidase removes specific octapeptides from the amino terminus of nuclear-encoded OXPHOS subunits and components of the mitochondrial genetic apparatus. To address the biologic role of the human peptidase [MIPEP gene, HMIP polypeptide], we have initiated its molecular and functional characterization. A full-length cDNA was isolated by screening a human liver library using a rat MIP (RMIP) cDNA as a probe. The encoded protein contained a typical mitochondrial leader peptide and showed 92 and 54% homology to RMIP and YMIP, respectively. A survey of human mitochondrial protein precursors revealed that, similar to YMIP, HMIP is primarily involved in the maturation of OXPHOS-related proteins. Northern analysis showed that the MIPEP gene is differentially expressed in human tissues, with the highest levels of expression in the heart, skeletal muscle, and pancreas, three organ systems that are frequently affected in OXPHOS disorders. Using fluorescence in situ hybridization, the MIPEP locus was assigned to 13q12. This information offers the possibility of testing the potential involvement of HMIP in the pathophysiology of nuclear-driven OXPHOS disorders.

Amino Acid Sequence

The nurse's role in facilitating compliance in clients with hypertension.

Afflicting approximately 50 million people, hypertension costs over $500 million per year and accounts for the largest number of physician office visits per year and the greatest use of prescription drugs. Nurses play a vital role in assessing and managing hypertensive clients' and promoting clients' compliance with treatment regimens.

Algorithms

Issues in liability insurance and the nursing consultant.

Since the number of lawsuits involving consultants has increased in recent years, it is important for clinical nurse specialists to be aware of the causes for these lawsuits and to protect themselves from the possibility of litigation. Maintaining open communication with clients, using an evaluation tool as a means for ensuring quality, drawing up a legal contract, and carrying adequate liability insurance are four important ways to protect oneself from the possibility of legal action.

Contract Services

Weaning from mechanical ventilatory support.

Weaning patients from mechanical ventilatory support continues to be a major challenge in critical care units. This article discusses recent research in the area of weaning and identifies specific parameters related to successful weaning in clinical practice.

Clinical Nursing Research