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Bibliografia publikacji pracowników
Państwowej Szkoły Wyższej w Białej Podlaskiej

Baza tworzona przez Bibliotekę Akademii Bialskiej im. Jana Pawła II.


Zapytanie: PHENYLKETONURIA
Liczba odnalezionych rekordów: 4



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Nr opisu: rations in relation to Phe intake from detailed meal records. This study aimed to determine Phe tolerance in twin pregnancies. Methods: The reviewed cases included three women with PKU who each had a singleton and twin pregnancy (i.e., they were pregnant twice). All patients were under regular supervision to maintain Phe concentrations in a steady state and determine safe Phe intake. Restriction of Phe in the patient's diet was determined depending on the amount of Phe intake, which allowed for stable blood Phe concentrations within the target range. Results: In all three patients with PKU, the ratio of Phe tolerance during the course of the twin and singleton pregnancies was <1 for most of the pregnancy. The ratio of the increase in Phe tolerance between 29 and 34 weeks of gestation and that between 15 and 28 weeks of gestation was 0.66 and 1.17, 0.51 and 0.14, and 0.76 and 1.42 in the twin and singleton pairs of pregnancies, respectively. Conclusions: Our study shows that Phe tolerance in a twin pregnancy is not greater than that in a singleton pregnancy.^aphenylalanine tolerance^atwin pregnancy^adietary record^aenergy intake^agestational age^asingleton
Autorzy: , phenylalanine tolerancetwin pregnancydietary recordenergy intakegestational agesingleton, phenylalanine tolerancetwin pregnancydietary recordenergy intakegestational agesingleton.
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Słowa kluczowe ang.: es, respectively. Conclusions: Our study shows that Phe tolerance in a twin pregnancy is not greater than that in a singleton pregnancy.^aphenylalanine tolerance^atwin pregnancy^adietary record^aenergy intake^agestational age^asingleton
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Nr opisu: rovides sufficient phe for maintenance and growth plus special phe-free formulas with amino acids to meet requirements for protein, energy and micronutrients. Rules for predicting phenylalanine tolerance changes during the course of pregnancy are not clear. The purpose of this report is to describe phe tolerance in pregnant PKU patients with the PAH genotype p.R408W/p.R408W. Low-phe diet was started before the pregnancies. the reviewed cases included three PKU women with two pregnancies and four PKU women with one pregnancy. Phe restriction in a patient's diet was determined upon the amount of this amino acid intake, which allows for stable blood phe concentrations within the target range of 120-360ľmol/L. Daily phe tolerance increased from 344ą85mg in the pre-conceptional period up to 1348ą446mg at the end of the third trimester. Before delivery, the lowest and highest phe tolerance expressed per kg of the pregnant women's body weight were 6.8 mg and 26.9 mg respectively. Phe tolerance varies greatly even between pregnancies of patients with the same PKU genotype. Further researc
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Język publikacji: , women's body weight were 6.8 mg and 26.9 mg respectively. Phe tolerance varies greatly even between pregnancies of patients with the same PKU genotype. Further research on pregnant PKU patients is essential to identify biological regulators of phe tolerance during pregnancy and provide evidence-based guidelines to optimise the dietetic care.^aphenylalanine tolerance^apregnancy
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Słowa kluczowe ang.: n's body weight were 6.8 mg and 26.9 mg respectively. Phe tolerance varies greatly even between pregnancies of patients with the same PKU genotype. Further research on pregnant PKU patients is essential to identify biological regulators of phe tolerance during pregnancy and provide evidence-based guidelines to optimise the dietetic care.^aphenylalanine tolerance^apregnancy
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Wskaźnik Impact Factor ISI: czynnikami wpływającymi na homeostazę Phe w ciąży u chorych na fenyloketonurię.^afenyloketonuria^aphenylketonuria^aThe maternal phenylketonuria (PKU) syndrome is caused by high phenylalanine (Phe) level during pregnancy. Dietary treatment to control Phe concentration (low-Phe diet) can prevent sequelae like intellectual disability, microcephaly and birth defects. In this study, 2 singleton pregnancies resulting in healthy offspring in one woman with phenylketonuria are reported. A state of metabolic control was sustained both during the preconception period and pregnancy in the course of both pregnancies. In clinical practice, the empirical determination of Phe tolerance relies on frequent assessment of blood Phe concentrations in patients with PKU in relation to Phe intake assessed using food records. In contrast to predictions based on literature data stating that Phe tolerance intensively increases in the third trimester and a low Phe tolerance may indicate fetal PKU, Phe tolerance in both pregnancies showed a low increase in the third trimester and a more noticeable increase during the whole pregnancy: 453 mg (30.2%)/1653 mg (551%) and 200 mg (12.5%)/1560 mg (650%), respectively. Interestingly, the patterns of Phe increase were relatively similar only in the second half of pregnancy. Further research on determinants of Phe tolerance in pregnant women with PKU is needed.^atolerancja fenyloalaniny^aphenylalanine tolerance^aoddziaływania matczyno-płodowe^amaternal-fetal interactions
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