BACKGROUND To evaluate the impact of sampling rate on the predictive capability of continuous fetal heart rate (FHR) variability (fHRV) monitoring for detecting fetal acidemia during labor, we tested the performance of the root mean square of successive differences (RMSSD) in R-R intervals from the ECG when acquired with the sampling rate of 4 Hz currently available in FHR monitors, in comparis...

We investigated whether hypoxemia without acidemia affects ductus venosus (DV) blood velocity waveform pattern in sheep fetuses with intact placenta and whether worsening acidemia and impending fetal death are related to changes in DV velocimetry in fetuses with increased placental vascular resistance. A total of 34 fetuses were instrumented at 115-136/145 days of gestation. Placental embolizat...

The profiles of urinary volatiles from patients with phenylketonuria, maple syrup urine disease, isovaleric acidemia, or trimethylaminuria (fish-odor syndrome) were in each case vastly different from the normal urinary volatiles profile. In the maple syrup urine disease case, metabolites that occur distal to the block were found and a mechanism for their formation is suggested. A new major meta...

Isovaleric acidemia (IVA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricte...

Compostos orgânicos provenientes de cinco amostras de sedimentos e um perfil com 75 cm foram coletados na Lagoa da Conceição, localizada na Ilha de Santa Catarina, com o objetivo de avaliar fontes de matéria orgânica. Os resultados que foram baseados na relação C:N:P, abundância relativa dos esteróis (com predomínio dos C 27 ), álcoois (0,2-1,2 μg g) e hidrocarbonetos lineares (0,4-5,0 μg g), e...

Пропионовая ацидемия (ПА) - редкое наследственное заболевание с аутосомно-рецессивным типом наследования, относится к «классическим», т.е. наиболее часто диагностируемым органическим ацидемиям. В данной работе представлена биохимическая и молекулярно-генетическая характеристика 16 пациентов ПА, выявленных в Российской Федерации. У большинства обследованных первые симптомы проявились месяцы жизн...

In fetal sheep, the electrocorticogram (ECOG) recorded directly from the cortex during repetitive heart rate (FHR) decelerations induced by umbilical cord occlusions (UCO) predictably correlates with worsening hypoxic-acidemia. In human fetal monitoring during labor, the equivalent electroencephalogram (EEG) can be recorded noninvasively from the scalp. We tested the hypothesis that combined fe...

To evaluate the effect of delivery mode on fetal stress hormones and acid-base status and also to investigate the relationship between fetal acidemia and these hormones. 64 women with term pregnancies were studied. All had singleton, healthy pregnancies. Twenty one women were delivered by spontaneous vaginal route, 23 by vaginal route following oxytocin infusion and 20 by elective caesarean sec...

In the search for the mechanism by which hyperammonemia complicates propionic and methylmalonic acidemia the effects of a series of acyl-coenzyme A (CoA) derivatives were studied on the activity of N-acetylglutamate synthetase in rat liver mitochondria using acetyl-CoA as substrate. Propionyl-CoA was found to be a competitive inhibitor. The inhibition constant of 0.71 mM is in the range of conc...