An outreach activity to enhance biochemistry pedagogy.

Students are self-motivated to learn when provided opportunities that connect theory and real-world applications. Here, we describe for biochemistry majors a newborn screening-focused outreach activity that seeks to develop students' mastery of disciplinary content and soft skills (e.g.

Dietary carbohydrate restriction as the first approach in diabetes management: critical review and evidence base.

The inability of current recommendations to control the epidemic of diabetes, the specific failure of the prevailing low-fat diets to improve obesity, cardiovascular risk, or general health and the persistent reports of some serious side effects of commonly prescribed diabetic medications, in combination with the continued success of low-carbohydrate diets in the treatment of diabetes and metabolic syndrome without significant side effects, point to the need for a reappraisal of dietary guidelines. The benefits of carbohydrate restriction in diabetes are immediate and well documented. Concerns about the efficacy and safety are long term and conjectural rather than data driven.

Dietary carbohydrate restriction in type 2 diabetes mellitus and metabolic syndrome: time for a critical appraisal.

Current nutritional approaches to metabolic syndrome and type 2 diabetes generally rely on reductions in dietary fat. The success of such approaches has been limited and therapy more generally relies on pharmacology. The argument is made that a re-evaluation of the role of carbohydrate restriction, the historical and intuitive approach to the problem, may provide an alternative and possibly superior dietary strategy.

Cystinosis and cystinuria: differences in outcome.

Cystinosis and cystinuria, both recessive genetic disorders, are fundamentally different in their pathophysiologic mechanisms. Cystinosis is a disease of cystine storage in which the kidney is the initial, but not the sole target organ. Cystinuria is a disease of renal tubular cystine transport in which excessive loss of this insoluble amino acid causes precipitation at physiologic urine pH and concentration.

Pediatric obstructive uropathy: clinical trials.

As the powerful tools of molecular biology continue to delineate new concepts of pathogenesis of diseases, new molecular-level therapeutic modalities are certain to emerge. In order to design and execute clinical trials to evaluate outcomes of these new treatment modalities, we will soon need a new supply of investigators with training and experience in clinical research. The slowly-progressive nature of chronic pediatric kidney disease often results in diagnosis being made at a time remote from initial insult, and the inherently slow rate of progression makes changes difficult to measure.

Immunizations for patients with metabolic disorders.

Individuals with underlying metabolic disorders are a potential high-risk group for vaccine-preventable diseases. Newborn metabolic screening has provided a means of early identification and treatment for many of these disorders, whereas childhood immunization is one of the most effective means of decreasing the morbidity and mortality resulting from communicable diseases worldwide. There are very few contraindications to the routine administration of vaccines to the healthy, immunocompetent individual.

The role of nutrition in chronic renal insufficiency of childhood: how much do we know?

Dietary protein restriction in the treatment of symptomatic renal failure has been utilized for many years, especially as a means for reduction of 'fixed acid" load. Studies in animal models of renal failure suggest that low protein intake may retard the progression of renal disease as well. However, large, well-organized investigations into this question in humans have fallen prey to difficulties that are almost impossible to overcome.

Tumor-necrosis-factor-related apoptosis-inducing-ligand (TRAIL)-mediated death of neurons in living human brain tissue is inhibited by flupirtine-maleate.

Neuronal damage mediated by the TRAIL-system might be involved in the pathogenesis of neuroinflammatory diseases of the central nervous system. Here we used an investigator-independent approach to quantify TRAIL-mediated death of total CNS cells and neurons in a living human brain slice culture system, a model which is much closer to the in vivo situation than dissociated cell culture. We observed dose-dependent TRAIL-mediated death of both total human CNS cells and neurons, which was prevented by flupirtine-maleate, a centrally acting analgesic drug with proposed neuroprotective properties.

Hyperammonemia, bane of the brain.

Ammonia, normally produced from catabolism of amino acids, is a deadly neurotoxin. As such, the concentration of free ammonia in the blood is very tightly regulated and is exceeded by two orders of magnitude by its physiologic derivative, urea. The normal capacity for urea production far exceeds the rate of free ammonia production by protein catabolism under normal circumstances, such that any increase in free blood ammonia concentration is a reflection of either biochemical or pharmacologic impairment of urea cycle function or fairly extensive hepatic damage.

Evaluation of the hypertensive infant: a rational approach to diagnosis.

This article reviews the literature and describes a methodologic approach to the diagnosis of hypertension in the young infant. The numerous etiologies of hypertension have been discussed and normative blood pressure data for neonates and infants have been provided. Techniques for accurate blood pressure measurement in the intensive care setting and for routine outpatient settings, are discussed.

Comparison of neuronal density and subfield sizes in the hippocampus of CD95L-deficient (gld), CD95-deficient (lpr) and nondeficient mice.

During brain development, the majority of neurons undergo programmed cell death. It is now clear that caspases are involved in this process of selective induction of neuronal apoptosis, yet the signals for this caspase activation remain undefined. As an upstream activator of these enzymes, the death receptor CD95 (Fas, APO1) was recently shown on neurons in the cornu ammonis (CA)2 and CA3 hippocampal subfields of early postnatal mice and rats.

Obstructive uropathy: an important cause of chronic renal failure in children.

Care of individuals with renal failure continues to impose a tremendous burden on our national health care budget. Chronic renal failure is the final common denominator of a multiplicity of pathologic processes, some of which progress silently over years. Of these, obstructive uropathy is a prominent cause of kidney failure, accounting for 16.