Carnitine deficiency, organic acidemias, and Reye's syndrome

Relative carnitine deficiency is important in the pathophysiology of several disorders, including Reye's syndrome and organic acidemias. In acute clinical crises, carnitine serves as a "buffer," trapping toxic acyl compounds. Mitochondrial failure develops in carnitine deficiency when there is insufficient tissue carnitine available to buffer toxic acyl-CoA metabolites. Toxic levels of acyl-CoA impair the citrate cycle, gluconeogenesis, the urea cycle, and fatty-acid oxidation. Carnitine replacement therapy is safe and induces excretion of toxic acyl groups in the urine.     

An alternate practice sale/retirement arrangement. A way out

Eventually every orthodontist will leave his practice because of illness, death, or retirement. Two widely used methods to accomplish this separation are described. They are "sale of the practice" or "integrating an associate into the practice." This "alternate arrangement" provides that the outgoing orthodontist complete his own cases. The new man gradually, but rapidly, assumes control; the senior man relinquishes control and responsibility. Immediate income is provided for the incoming orthodontist, continuing income for the outgoing orthodontist. The psychologic needs of both individuals are thoroughly addressed.     

Between a hygiene rock and a hygienic hard place: Avoiding SARS-CoV-2 while needing environmental exposures for immunity

Suboptimal understanding of concepts related to hygiene by the general public, clinicians and researchers is a persistent problem in health and medicine. Although hygiene is necessary to slow or prevent deadly pandemics of infectious disease such as coronavirus disease 2019 (COVID-19), hygiene can have unwanted effects. In particular, some aspects of hygiene cause a loss of biodiversity from the human body, characterized by the almost complete removal of intestinal worms (helminths) and protists. Research spanning more than half a century documents that this loss of biodiversity results in an increased propensity for autoimmune disease, allergic disorders, probably neuropsychiatric problems and adverse reactions to infectious agents. The differences in immune function between communities with and communities without helminths have become so pronounced that the reduced lethality of severe acute respiratory syndrome coronavirus 2 in low-income countries compared to high-income countries was predicted early in the COVID-19 pandemic. This prediction, based on the maladaptive immune responses observed in many cases of COVID-19 in high-income countries, is now supported by emerging data from low-income countries. Herein, hygiene is subdivided into components involving personal choice versus components instituted by community wide systems such as sewage treatment facilities and water treatment plants. The different effects of personal hygiene and systems hygiene are described, and appropriate measures to alleviate the adverse effects of hygiene without losing the benefits of hygiene are discussed. Finally, text boxes are provided to function as stand-alone, public-domain handouts with the goal of informing the public about hygiene and suggesting solutions for biomedical researchers and policy makers.Lay Summary: Hygiene related to sewer systems and other technology can have adverse effects on immune function, and is distinct from personal hygiene practices such as hand washing and social distancing. Dealing with the drawbacks of hygiene must be undertaken without compromising the protection from infectious disease imposed by hygiene.     

Treatment of infants with ureteropelvic junction obstruction: findings from the PURSUIT network

International Urology and Nephrology - Studies based on administrative databases show that infant pyeloplasty is associated with minority race/ethnicity but lack clinical data that may influence...     

Persistent sex disparity in liver transplantation rates

BackgroundStudies have demonstrated that there are sex disparities in the rate of liver transplantation. However, little is known statistically about whether this disparity is caused by liver compartment size, Model for End-Stage Liver Disease adjustments, or regional differences.MethodsWe use retrospective data from the United Network for Organ Sharing Standard Treatment Analysis and Research data files for liver transplantation from 1995 through 2012. The final sample consists of 150,149 patients. These data contain information on all individuals who registered for the liver transplant waiting list as well as updated outcome data. Linear probability and logistic regression models were both used.ResultsWomen were 4.8 percentage points less likely to receive a transplant. Adjustment for race, weight, body mass index, region, education, and other characteristics attenuated the sex difference by roughly 19% (from 4.8 to 3.9 percentage points). The disparity was consistent across the 11 United Network for Organ Sharing allocation regions. Comparing the heaviest women to the lightest men, the disparity flipped. Pairwise comparisons between men and women of various sizes suggest that disparities in favor of men increase with the ratio of male-to-female size.ConclusionOur results document persistent sex disparity in liver transplantation, only 19% of which is explained by size differentials between men and women. Differences in rates of transplantation are increasing in the ratio of male-to-female height and weight, suggesting that some of the disparity is explained by differences in liver compartment size.     

Seroprevalence studies using a recombinant norwalk virus protein enzyme immunoassay

A recombinant Norwalk virus (NV) protein enzyme immunoassay was used to study the age of acquisition of NV IgG in various populations. In London, England, there was little evidence of infection during the first 2 years of life. However, the prevalence of NV IgG rose steadily throughout the period that children attend school, reaching a peak of 70% in the group aged 11–16 years. High levels of maternal antibody were detected in infants aged <3 months. Comparison of the acquisition of antibodies to three strains of human calicivirus in Japanese children in northern Japan indicated that although the majority had experienced infection with strains Japan and UK1 by the age of 12 years, only 22% possessed antibodies to NV. In Australian aborigines NV infection occurs early in life; by the age of 6 years over 90% of children were seropositive. © 1994 Wiley-Liss, Inc.     

Halothane alters the oxygen consumption-oxygen delivery relationship compared with conscious state

The authors' objectives were as follows: 1) to characterize for the first time the relationship between whole body O2 delivery (DO2) and O2 consumption (VO2) in adult conscious dogs; and 2) to asses the effects of the inhalational anesthetic, halothane, on that relationship. DO2 was varied over a wide range in chronically instrumented dogs by gradual inflation and deflation of a hydraulic occluder implanted around the thoracic inferior vena cava to alter venous return and cardiac output. VO2 was measured at different values of DO2 in dogs in the fully conscious state and again during halothane anesthesia. A "binning" technique indicated that halothane decreased VO2 (P less than 0.01) at any given value of DO2 over a broad range of VO2. A two-line piecewise linear regression analysis technique indicated that halothane decreased (P less than 0.01) the critical O2 delivery (COD) from 20 +/- 3 to 10 +/- 1 ml.kg-1.min-1 and increased (P less than 0.01) O2 extraction at COD from 31 +/- 3 to 40 +/- 2%. However, the DO2-VO2 plots measured in both conscious and halothane-anesthetized dogs did not exhibit a discrete discontinuity but rather were closely fit (correlation coefficient = 0.98) by an exponential equation of the following form: O2 extraction = B1.(1 - exp (-DO2/B2))/DO2, where B1 is the delivery-independent estimate of VO2 and B2 is the "delivery constant," i.e., the DO2 associated with a VO2 equal to 63% of B1. Halothane decreased B1 (P less than 0.01) from 5.3 +/- 0.1 to 3.9 +/- 0.1 ml.kg-1.min-1 and decreased B2 (P less than 0.01) from 5.6 +/- 0.3 to 3.6 +/- 0.3 ml.kg-1.min-1 compared with that measured in conscious dogs. Thus, compared with the conscious state, halothane anesthesia alters the fundamental relationship between DO2 and VO2 and may have a beneficial effect on tissue oxygenation at low values of DO2.