COVID-19 disease among children and young adults enrolled in the North American Pediatric Renal Trials and Collaborative Studies registry.

Background: Coronavirus disease of 2019 (COVID-19) has disproportionately affected adults with kidney disease. Data regarding outcomes among children with kidney disease are limited. The North American Pediatric Renal Trials Collaborative Studies Registry (NAPRTCS) has followed children with chronic kidney disease (CKD) since 1987 at 87 participating centers.

Arterial spin labeling magnetic resonance imaging quantifies tissue perfusion around foot ulcers.

Objective: Tools that quantify tissue perfusion of the foot are deficient, contributing to the uncertainty in predicting ulcer healing potential. This pilot study aims to quantify peri-wound foot perfusion at various tissue depths using a novel application of pseudo-continuous arterial spin labeling magnetic resonance imaging.

Methods: Ten diabetic patients with neuropathic wounds and 20 healthy volunteers without wounds were recruited.

Health and Dental Insurance and Health Care Utilization Among Children, Adolescents, and Young Adults With CKD: Findings From the CKiD Cohort Study.

Rationale & Objective: To understand the association between health and dental insurance status and health and dental care utilization, and their relationship with disease severity in a population with childhood-onset chronic kidney disease (CKD).

Study Design: Observational cohort study.

Settings & Participants: Nine hundred fifty-three participants contributing 4,369 person-visits (unit of analysis) in the United States enrolled in the Chronic Kidney Disease in Children (CKiD) Study from 2005 to 2019.

The Relationship Between Neighborhood Disadvantage and Kidney Disease Progression in the Chronic Kidney Disease in Children (CKiD) Cohort.

Rationale & Objective: To examine the relationship between neighborhood poverty and deprivation, chronic kidney disease (CKD) comorbidities, and disease progression in children with CKD.

Study Design: Observational cohort study.

Setting & Participants: Children with mild to moderate CKD enrolled in the CKiD (Chronic Kidney Disease in Children) study with available US Census data.

LGE Provides Incremental Prognostic Information Over Serum Biomarkers in AL Cardiac Amyloidosis.

Objectives: This study sought to determine the prognostic value of cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) in amyloid light chain (AL) cardiac amyloidosis.

Background: Cardiac involvement is the major determinant of mortality in AL amyloidosis. CMR LGE is a marker of amyloid infiltration of the myocardium.

Recommendations for the development of rare disease drugs using the accelerated approval pathway and for qualifying biomarkers as primary endpoints.

For rare serious and life-threatening disorders, there is a tremendous challenge of transforming scientific discoveries into new drug treatments. This challenge has been recognized by all stakeholders who endorse the need for flexibility in the regulatory review process for novel therapeutics to treat rare diseases. In the United States, the best expression of this flexibility was the creation of the Accelerated Approval (AA) pathway.

Decreasing hypothermia during delivery room stabilization of preterm neonates.

Background And Objective: Hypothermia during delivery room stabilization of very low birth weight (VLBW) newborns is independently associated with mortality, yet it occurred frequently both in collaborative networks and at our institution. We aimed to attain admission temperatures in the target range of 36 °C to 38 °C in ≥ 90% of inborn VLBW neonates through implementation of a thermoregulation bundle.

Methods: This quality improvement project extended over 60 consecutive months, using sequential plan-do-check-act cycles.

Use of chemical warming packs during delivery room resuscitation is associated with decreased rates of hypothermia in very low-birth-weight neonates.

Background: : Hypothermia is an independent contributor to neonatal mortality. All very low-birth-weight (VLBW) newborns have the potential to undergo cold stress or frank hypothermia during delivery room stabilization. Thus, clinicians aiming to maintain normothermia in VLBW neonates are compelled to use multiple adjuncts of unknown efficacy or safety.

Ossification patterns of the atlas vertebra.

Objective: The objective of this study was to characterize ossification patterns of the C1 (atlas) vertebra in children, to better differentiate normal variants from traumatic injury.

Materials And Methods: A retrospective review of all sinus and temporal bone CT examinations was performed for the period of 2002-2009. Patients 96 months old or younger for whom C1 level was at least partially imaged were included.

Sex, race, and socioeconomic disparities in kidney disease in children.

Racial and gender differences in the prevalence and treatment of chronic kidney disease in US children have been reported. Girls have lower rates of kidney transplantation than boys. Incidence of end-stage renal disease is twice as high among black patients compared with whites.

latheo encodes a subunit of the origin recognition complex and disrupts neuronal proliferation and adult olfactory memory when mutant.

The Drosophila latheo (lat) gene was identified in a behavioral screen for olfactory memory mutants. The original hypomorphic latP1 mutant (Boynton and Tully, 1992) shows a structural defect in adult brain. Homozygous lethal lat mutants lack imaginal discs, show little cell proliferation in the CNS of third instar larvae, and die as early pupae.

Genetic dissection of consolidated memory in Drosophila.

Behavioral and pharmacological experiments in many animal species have suggested that memory is consolidated from an initial, disruptable form into a long-lasting, stable form within a few hours after training. We combined these traditional approaches with genetic analyses in Drosophila to show that consolidated memory of conditioned (learned) odor avoidance 1 day after extended training consisted of two genetically distinct, functionally independent memory components: anesthesia-resistant memory (ARM) and long-term memory (LTM). ARM decayed away within 4 days, was resistant to hypothermic disruption, was insensitive to the protein synthesis inhibitor cycloheximide (CXM), and was disrupted by the radish single-gene mutation.

Rapid and accurate random urinary porphyrin quantitation.

Urinary metabolite profiling, using randomly voided samples, has become accepted practice for such compounds as organic acids. To date, however, published methods for examining urinary porphyrin excretion have been based upon examination of 24-h urine collections. The inherent difficulties of obtaining an accurate collection, coupled with the intrinsic liabilities of porphyrins suggest that a method based upon random void analysis would be of great use.

Tissue distribution of succinylacetone in the rat in vivo: a possible basis for neurotoxicity in hereditary infantile tyrosinemia.

Neurologic dysfunction is a significant component of hereditary infantile tyrosinemia, an autosomal recessive disorder of man. The specific enzyme defect leads to endogenous production of the biochemical marker compound, succinylacetone (SA). Earlier study of the role which SA plays in generation of the renal Fanconi syndrome, also associated with this disorder, led to speculation that SA might also have neurotoxic effects.

Physiological basis for an animal model of the renal Fanconi syndrome: use of succinylacetone in the rat.

1. The biochemical basis for the human renal Fanconi syndrome, including glucosuria, phosphaturia and aminoaciduria, remains enigmatic. This is due, in part, to the lack of an appropriate animal model.

latheo, a new gene involved in associative learning and memory in Drosophila melanogaster, identified from P element mutagenesis.

Genetic dissection of learning and memory in Drosophila has been limited by the existence of ethyl methanesulfonate (EMS)-induced mutations in only a small number of X-linked genes. To remedy this shortcoming, we have begun a P element mutagenesis to screen for autosomal mutations that disrupt associative learning and/or memory. The generation of "P-tagged" mutant alleles will expedite molecular cloning of these new genes.

Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules.

Succinylacetone (SA), a metabolic end-product found in urine from individuals with hereditary tyrosinemia and associated renal Fanconi syndrome and a known inhibitor of hepatic 5-aminolevulinic acid dehydratase (ALAD), has been used to study heme metabolism in isolated rat renal tubules. Heme biosynthetic porphyrin precursors are increased selectively in the presence of 4 mmol/1 SA. Total porphyrin content of the tubules are increased approximately 2-fold, while both ferrochelatase and heme oxygenase activities remain unaffected by SA.

Agents that modify EDRF formation alter antiplatelet properties of brain arteriolar endothelium in vivo.

In the presence of circulating Evans blue a helium-neon laser injures the endothelium of brain surface arterioles in situ. The injury is known to selectively eliminate endothelium-dependent responses. The present study documents in mice the fact that such endothelial sites become selectively attractive to passing platelets that have been activated as a result of more severe injury upstream.